Updated on 2024/10/07

Information

 

写真a

 
YAMASAKI RYO
 
Organization
Faculty of Medical Sciences Neurological Institute Associate Professor
Faculty of Medical Sciences Neurological Institute（Concurrent）
Faculty of Medical Sciences Neurological Institute（Concurrent）
Faculty of Medical Sciences Neurological Institute（Concurrent）
School of Medicine Department of Medicine（Concurrent）
Graduate School of Medical Sciences Department of Medicine（Concurrent）
Graduate School of Medical Sciences Department of Medical Sciences（Concurrent）
Title
Associate Professor
Contact information
メールアドレス
Tel
0926425340
Profile
We investigate the model mice of amyotrophic lateral sclerosis, from the view point of the neuroprotective function of non-neuronal cells like microglia, astrocytes, etc. We found the impaired activation ability of microglia harvested from ALS model mice. It indicates the impaired neuroprotective function of ALS model mice microglia. And also we are now starting the investigation of the white blood cell from the ALS patients.
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Research Areas

  • Life Science / Neuroscience-general

  • Life Science / Neurology

Degree

  • M.D, Ph.D

Research History

  • Kyushu University Faculty of Medical Sciences Associate Professor

    2016.7 - Present

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  • 2000.5~: 九州大学病院研修医 2002.4~2004.3 : 松山赤十字病院神経内科医員 2010.1~2012.3：米国クリーブランドクリニック 2012.4~2012.9：九州大学大学院医学研究院神経内科学 助教 2012.10~：九州大学大学院医学研究院神経治療学寄付講座 助教 2015.7~: 九州大学病院　講師 2016.7~: 九州大学大学院医学研究院　神経内科学　准教授

    2000.5~: 九州大学病院研修医 2002.4~2004.3 : 松山赤十字病院神経内科医員 2010.1~2012.3：米国クリーブランドクリニック 2012.4~2012.9：九州大学大学院医学研究院神経内科学 助教 2012.10~：九州大学大学院医学研究院神経治療学寄付講座 助教 2015.7~: 九州大学病院　講師 2016.7~: 九州大学大学院医学研究院　神経内科学　准教授

  • なし

Research Interests・Research Keywords

  • Research theme：multiple sclerosis

    Keyword：multiple sclerosis

    Research period： 2024

  • Research theme：microglia

    Keyword：microglia

    Research period： 2024

  • Research theme：macrophage

    Keyword：macrophage

    Research period： 2024

  • Research theme：Parkinson's disease

    Keyword：Parkinson's disease

    Research period： 2024

  • Research theme：Alzhemer's disease

    Keyword：Alzhemer's disease

    Research period： 2024

  • Research theme：neuropathy

    Keyword：neuropathy

    Research period： 2024

  • Research theme：amyotrophic lateral sclerosis

    Keyword：amyotrophic lateral sclerosis

    Research period： 2024

  • Research theme：Development of a national clinical epidemiological survey on Facial onset sensory and motor neuronopathy (FOSMN) and diagnostic treatment guidelines based on it and development of a patient registry

    Keyword：FOSMN, national clinical epidemiological survey, diagnostic guideline

    Research period： 2019.6 - 2021.3

  • Research theme：Development of brand-new treatment for the atopy-related allodynia in atopic diathesis model mice.

    Keyword：Bronchial asthma, microglia, astroglia, glial inflammation, allodynia

    Research period： 2015.4 - 2016.3

  • Research theme：Functional analysis of Connexins in multiple sclerosis model mice (EAE)

    Keyword：Multiple sclerosis, experimental autoimmune encephalomyelitis, connexin

    Research period： 2014.4

  • Research theme：Early strong intrathecal inflammation in cerebellar type multiple system atrophy by cerebrospinal fluid cytokine/chemokine profiles: a case control study

    Keyword：Multiple system atrophy, Cerebrospinal fluid, Cytokine, Interleukin-6, Monocyte chemoattractant protein-1, Magnetic resonance imaging

    Research period： 2014.4

  • Research theme：Induction and analysis of the atopic myelitis model mice. Atopic myelitis reone of the eosinophilic myelitis which are seen in atopic disease patients, and the number is increasing. They feel leg weakness. dysesthesia and general fatigue. We have established new diagnostic criteria (2012), and the number of patients are increasing.We are going to make mice model for bronchial asthma, atopic dermatitis and so on, to check the glial inflammation in central nervous system.

    Keyword：Microglia, atopic deaematitis

    Research period： 2012.10 - 2014.12

  • Research theme：Functional analysis of auto-antibody against neurofascin 155 in CIDP patients.

    Keyword：CIDP

    Research period： 2012.7

  • Research theme：Elucidation of the mechanisms of neural damage in chronic stage multiple sclerosis model mice with glial inflammation

    Keyword：Multiple Sclerosis, microglia, astroglia, connexins

    Research period： 2012.4 - 2016.3

  • Research theme：Elucidation of the mechanisms of the accumulation of mutant SOD1 peptide in ALS model mice.

    Keyword：ALS, mutant SOD1, microglia

    Research period： 2012.4 - 2015.3

  • Research theme：Elucidating the roles of immune cells in the inflammatory lesions of central nervous system

    Keyword：microglia, multiple sclerosis, demyelinating disorders, glial inflammations, cytokine

    Research period： 2012.4 - 2013.3

  • Research theme：I conduct clinical neurology and a neurosciences study. The mechanism of amyotrophic lateral sclerosis (ALS) still remain unknown, and the therapy is limited, too. The elucidation of the mechanism of ALS is the mission of the university, and the immediate therapy development is urgent business. The spinal cord of the ALS reveals the colonization of monocytes-based cells, activation with a denaturing dropout of the motor nerve, but the role of these cells is not apparent. It is very likely that it contributes to clinical condition elucidation to analyze the function of these cells in detail. We are analyzing the effect that a mechanism of the motor nerve degeneration and a non-neuronal cell give to these using an ALS model mouse.

    Keyword：Amyotrophic lateral sclerosis, microglia, SOD1

    Research period： 2008.1 - 2009.12

Awards

  • 2023年度日本神経学会賞(学術研究部門)

    2023.6   日本神経学会   ミクログリア・マクロファージを基点とする神経炎症が神経疾患に果たす役割の解明

  • 2023年度日本神経学会賞（学術研究部門）

    2023   日本神経学会  

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  • 第31回日本末梢神経学会賞　内科部門

    2020.9   日本末梢神経学会   上記学会での研究発表内容

  • 2018年日本神経免疫学会研究創世賞

    2018.9   日本神経免疫学会   同学会での発表内容について

  • 第29回日本末梢神経学会賞

    2018.9   日本末梢神経学会   同学会における発表内容について

  • パーキンソン病学術奨励賞

    2018.7   九州大学神経内科同門会   MSAとサイトカインに関する論文内容について

  • 教育委員会委員長賞

    2017.8   第４回MSサマーカレッジ  

  • 該当なし

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Papers

  • Astroglial connexin 43 is a novel therapeutic target for chronic multiple sclerosis model. Reviewed International journal

    Ezgi Ozdemir Takase, Ryo Yamasaki, Satoshi Nagata, Mitsuru Watanabe, Katsuhisa Masaki, Hiroo Yamaguchi, Jun-Ichi Kira, Hideyuki Takeuchi, Noriko Isobe

    Scientific reports   14 ( 1 )   10877 - 10877   2024.5   （ ISSN:2045-2322 ）

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    Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

    In chronic stages of multiple sclerosis (MS) and its animal model, experimental autoimmune encephalitis (EAE), connexin (Cx)43 gap junction channel proteins are overexpressed because of astrogliosis. To elucidate the role of increased Cx43, the central nervous system (CNS)-permeable Cx blocker INI-0602 was therapeutically administered. C57BL6 mice with chronic EAE initiated by MOG35-55 received INI-0602 (40 mg/kg) or saline intraperitoneally every other day from days post-immunization (dpi) 17-50. Primary astroglia were employed to observe calcein efflux responses. In INI-0602-treated mice, EAE clinical signs improved significantly in the chronic phase, with reduced demyelination and decreased CD3+ T cells, Iba-1+ and F4/80+ microglia/macrophages, and C3+GFAP+ reactive astroglia infiltration in spinal cord lesions. Flow cytometry analysis of CD4+ T cells from CNS tissues revealed significantly reduced Th17 and Th17/Th1 cells (dpi 24) and Th1 cells (dpi 50). Multiplex array of cerebrospinal fluid showed significantly suppressed IL-6 and significantly increased IL-10 on dpi 24 in INI-0602-treated mice, and significantly suppressed IFN-γ and MCP-1 on dpi 50 in the same group. In vitro INI-0602 treatment inhibited ATP-induced calcium propagations of Cx43+/+ astroglial cells to similar levels of those of Cx43-/- cells. Astroglial Cx43 hemichannels represent a novel therapeutic target for chronic EAE and MS.

    DOI： 10.1038/s41598-024-61508-2

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  • A nationwide survey of facial onset sensory and motor neuronopathy in Japan Reviewed International journal

    Ko S, Yamasaki R, Okui T, Shiraishi W, Watanabe M, Hashimoto Y, Kobayakawa Y, Kusunoki S, Kira JI, Isobe N.

    J Neurol Sci   459   122957 - 122957   2024.4   （ ISSN:0022-510X eISSN:1878-5883 ）

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    Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

    DOI： 10.1016/j.jns.2024.122957

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  • Iguratimod Ameliorates the Severity of Secondary Progressive Multiple Sclerosis in Model Mice by Directly Inhibiting IL-6 Production and Th17 Cell Migration via Mitigation of Glial Inflammation Invited Reviewed International journal

    Nagata S, Yamasaki R, Takase EO, Iida K, Watanabe M, Masaki K, Wijering MHC, Yamaguchi H, Kira JI, Isobe N.

    Biology (Basel)   12 ( 9 )   2023.9   （ ISSN:2079-7737 eISSN:2079-7737 ）

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    Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

    We previously reported a novel secondary progressive multiple sclerosis (SPMS) model, progressive experimental autoimmune encephalomyelitis (pEAE), in oligodendroglia-specific Cx47-inducible conditional knockout (Cx47 icKO) mice. Based on our prior study showing the efficacy of iguratimod (IGU), an antirheumatic drug, for acute EAE treatment, we aimed to elucidate the effect of IGU on the SPMS animal model. We induced pEAE by immunizing Cx47 icKO mice with myelin oligodendrocyte glycoprotein peptide 35-55. IGU was orally administered from 17 to 50 days post-immunization. We also prepared a primary mixed glial cell culture and measured cytokine levels in the culture supernatant after stimulation with designated cytokines (IL-1α, C1q, TNF-α) and lipopolysaccharide. A migration assay was performed to evaluate the effect of IGU on the migration ability of T cells toward mixed glial cell cultures. IGU treatment ameliorated the clinical signs of pEAE, decreased the demyelinated area, and attenuated glial inflammation on immunohistochemical analysis. Additionally, IGU decreased the intrathecal IL-6 level and infiltrating Th17 cells. The migration assay revealed reduced Th17 cell migration and IL-6 levels in the culture supernatant after IGU treatment. Collectively, IGU successfully mitigated the clinical signs of pEAE by suppressing Th17 migration through inhibition of IL-6 production by proinflammatory-activated glial cells.

    DOI： 10.3390/biology12091217

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  • Clearance of peripheral nerve misfolded mutant protein by infiltrated macrophages correlates with motor neuron disease progression. International journal

    Wataru Shiraishi, Ryo Yamasaki, Yu Hashimoto, Senri Ko, Yuko Kobayakawa, Noriko Isobe, Takuya Matsushita, Jun-Ichi Kira

    Scientific reports   11 ( 1 )   16438 - 16438   2021.8

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    Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

    DOI： 10.1038/s41598-021-96064-6

  • Early strong intrathecal inflammation in cerebellar type multiple system atrophy by cerebrospinal fluid cytokine/chemokine profiles: a case control study. Reviewed International journal

    Ryo Yamasaki

    J Neuroinflammation   2017.4

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  • Allergic Inflammation Leads to Neuropathic Pain via Glial Cell Activation. Reviewed International journal

    Ryo Yamasaki

    J Neurosci   2016.11

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  • Differential roles of microglia and monocytes in the inflamed central nervous system. Reviewed International journal

    Ryo Yamasaki, Richard Ransohoff

    J Exp Med   2014.7

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  • Anti-neurofascin antibody in patients with combined central and peripheral demyelination. Reviewed International journal

    Ryo Yamasaki, Nobutoshi Kawamura, Takuya Matsushita, Matsuse Dai, Hiroyuki Murai, Jun-ichi Kira

    Neurology   2013.8

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  • Restoration of microglial function by granulocyte-colony stimulating factor in ALS model mice. Reviewed

    Ryo Yamasaki, Tanaka M, Fukunaga M, Tateishi T, Kikuchi H, Motomura K, Matsushita T, Ohyagi Y, Kira J

    J Neuroimmunol.   2010.12

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  • Involvement of lysosomal storage-induced p38 MAP kinase activation in the overproduction of nitric oxide by microglia in the cathepsin D-deficient mice. Reviewed International journal

    Ryo Yamasaki, Jian Zhang, Ichiro Koshiishi, Dewi F. Sastradipura Suniarti, Zhou Wu, Christoph Peters, Michael Schwake, Yasuo Uchiyama, Jun-ichi Kira, Paul Saftig, Hideo Utsumi, Hiroshi Nakanishi

    Molecular and Cellular Neuroscience   2007.8

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  • A rapidly progressive multiple system atrophy-cerebellar variant model presenting marked glial reactions with inflammation and spreading of α-synuclein oligomers and phosphorylated α-synuclein aggregates. Reviewed International journal

    Hiroo Yamaguchi, Yuji Nishimura, Dai Matsuse, Hiroaki Sekiya, Katsuhisa Masaki, Tatsunori Tanaka, Toru Saiga, Masaya Harada, Yuu-Ichi Kira, Dennis W Dickson, Kei Fujishima, Eriko Matsuo, Kenji F Tanaka, Ryo Yamasaki, Noriko Isobe, Jun-Ichi Kira

    Brain, behavior, and immunity   121   122 - 141   2024.10   （ ISSN:0889-1591 eISSN:1090-2139 ）

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    Multiple system atrophy (MSA) is a severe α-synucleinopathy facilitated by glial reactions; the cerebellar variant (MSA-C) preferentially involves olivopontocerebellar fibres with conspicuous demyelination. A lack of aggressive models that preferentially involve olivopontocerebellar tracts in adulthood has hindered our understanding of the mechanisms of demyelination and neuroaxonal loss, and thus the development of effective treatments for MSA. We therefore aimed to develop a rapidly progressive mouse model that recaptures MSA-C pathology. We crossed Plp1-tTA and tetO-SNCA*A53T mice to generate Plp1-tTA::tetO-SNCA*A53T bi-transgenic mice, in which human A53T α-synuclein-a mutant protein with enhanced aggregability-was specifically produced in the oligodendrocytes of adult mice using Tet-Off regulation. These bi-transgenic mice expressed mutant α-synuclein from 8 weeks of age, when doxycycline was removed from the diet. All bi-transgenic mice presented rapidly progressive motor deterioration, with wide-based ataxic gait around 22 weeks of age and death around 30 weeks of age. They also had prominent demyelination in the brainstem/cerebellum. Double immunostaining demonstrated that myelin basic protein was markedly decreased in areas in which SM132, an axonal marker, was relatively preserved. Demyelinating lesions exhibited marked ionised calcium-binding adaptor molecule 1-, arginase-1-, and toll-like receptor 2-positive microglial reactivity and glial fibrillary acidic protein-positive astrocytic reactivity. Microarray analysis revealed a strong inflammatory response and cytokine/chemokine production in bi-transgenic mice. Neuronal nuclei-positive neuronal loss and patchy microtubule-associated protein 2-positive dendritic loss became prominent at 30 weeks of age. However, a perceived decrease in tyrosine hydroxylase-positive neurons in the substantia nigra pars compacta in bi-transgenic mice compared with wild-type mice was not significant, even at 30 weeks of age. Wild-type, Plp1-tTA, and tetO-SNCA*A53T mice developed neither motor deficits nor demyelination. In bi-transgenic mice, double immunostaining revealed human α-synuclein accumulation in neurite outgrowth inhibitor A (Nogo-A)-positive oligodendrocytes beginning at 9 weeks of age; its expression was further increased at 10 to 12 weeks, and these increased levels were maintained at 12, 24, and 30 weeks. In an α-synuclein-proximity ligation assay, α-synuclein oligomers first appeared in brainstem oligodendrocytes as early as 9 weeks of age; they then spread to astrocytes, neuropil, and neurons at 12 and 16 weeks of age. α-Synuclein oligomers in the brainstem neuropil were most abundant at 16 weeks of age and decreased thereafter; however, those in Purkinje cells successively increased until 30 weeks of age. Double immunostaining revealed the presence of phosphorylated α-synuclein in Nogo-A-positive oligodendrocytes in the brainstem/cerebellum as early as 9 weeks of age. In quantitative assessments, phosphorylated α-synuclein gradually and successively accumulated at 12, 24, and 30 weeks in bi-transgenic mice. By contrast, no phosphorylated α-synuclein was detected in wild-type, tetO-SNCA*A53T, or Plp1-tTA mice at any age examined. Pronounced demyelination and tubulin polymerisation, promoting protein-positive oligodendrocytic loss, was closely associated with phosphorylated α-synuclein aggregates at 24 and 30 weeks of age. Early inhibition of mutant α-synuclein expression by doxycycline diet at 23 weeks led to fully recovered demyelination; inhibition at 27 weeks led to persistent demyelination with glial reactions, despite resolving phosphorylated α-synuclein aggregates. In conclusion, our bi-transgenic mice exhibited progressively increasing demyelination and neuroaxonal loss in the brainstem/cerebellum, with rapidly progressive motor deterioration in adulthood. These mice showed marked microglial and astrocytic reactions with inflammation that was closely associated with phosphorylated α-synuclein aggregates. These features closely mimic human MSA-C pathology. Notably, our model is the first to suggest that α-synuclein oligomers may spread from oligodendrocytes to neurons in transgenic mice with human α-synuclein expression in oligodendrocytes. This model of MSA is therefore particularly useful for elucidating the in vivo mechanisms of α-synuclein spreading from glia to neurons, and for developing therapies that target glial reactions and/or α-synuclein oligomer spreading and aggregate formation in MSA.

    DOI： 10.1016/j.bbi.2024.07.004

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  • Safety, tolerability, and efficacy of subcutaneous efgartigimod in patients with chronic inflammatory demyelinating polyradiculoneuropathy (ADHERE): a multicentre, randomised-withdrawal, double-blind, placebo-controlled, phase 2 trial. Reviewed International journal

    Jeffrey A Allen, Jie Lin, Ivana Basta, Tina Dysgaard, Christian Eggers, Jeffrey T Guptill, Kelly G Gwathmey, Channa Hewamadduma, Erik Hofman, Yessar M Hussain, Satoshi Kuwabara, Gwendal Le Masson, Frank Leypoldt, Ting Chang, Marta Lipowska, Murray Lowe, Giuseppe Lauria, Luis Querol, Mihaela-Adriana Simu, Niraja Suresh, Anissa Tse, Peter Ulrichts, Benjamin Van Hoorick, Ryo Yamasaki, Richard A Lewis, Pieter A van Doorn

    The Lancet. Neurology   23 ( 10 )   1013 - 1024   2024.10   （ ISSN:14744422 ）

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    BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system that can lead to severe disability from muscle weakness and sensory disturbances. Around a third of patients do not respond to currently available treatments, and many patients with a partial response have residual neurological impairment, highlighting the need for effective alternatives. Efgartigimod alfa, a human IgG1 antibody Fc fragment, has demonstrated efficacy and safety in patients with generalised myasthenia gravis. We evaluated the safety, tolerability, and efficacy of subcutaneous efgartigimod PH20 in adults with CIDP. METHODS: ADHERE, a multistage, double-blind, placebo-controlled trial, enrolled participants with CIDP from 146 clinical sites from Asia-Pacific, Europe, and North America. Participants with evidence of clinically meaningful deterioration entered an open-label phase of weekly 1000 mg subcutaneous efgartigimod PH20 for no longer than 12 weeks (stage A). Those with confirmed evidence of clinical improvement (ECI; treatment responders) entered a randomised-withdrawal phase of 1000 mg subcutaneous efgartigimod PH20 weekly treatment versus placebo for a maximum of 48 weeks (stage B). Participants were randomised (1:1) through interactive response technology and stratified by their adjusted Inflammatory Neuropathy Cause and Treatment (aINCAT) score change during stage A and their most recent CIDP medication within 6 months before screening. Investigators, the clinical research organisation, and participants were masked to the treatment. The primary endpoint in stage A, evaluated in the stage A safety population, was confirmed ECI (≥1 points aINCAT decrease, ≥4 points [centile metric] Inflammatory Rasch-built Overall Disability Scale increase, or ≥8 kPa grip strength increase after four injections and two consecutive visits). The primary endpoint in stage B, evaluated in the modified intention-to-treat population, was the risk of relapse (time to first aINCAT increase of ≥1 points). ADHERE is registered with ClinicalTrials.gov (NCT04281472) and EudraCT (2019-003076-39) and is completed. FINDINGS: Between April 15, 2020, and May 11, 2023, 629 participants were screened; 322 (114 female, 208 male) entered stage A, of whom 214 (66%, 95% CI 61·0-71·6) had confirmed ECI. In stage B, 221 participants were randomised (79 female, 142 male; 111 to subcutaneous efgartigimod PH20, 110 to placebo). Subcutaneous efgartigimod PH20 significantly reduced the risk of relapse versus placebo (hazard ratio 0·39 [95% CI 0·25-0·61]; p<0·0001). 31 (27·9% [19·6-36·3]) participants given subcutaneous efgartigimod PH20 had a relapse versus 59 (53·6% [44·3-63·0]) given placebo. In stage A, treatment-emergent adverse events (TEAEs) occurred in 204 (63%) participants and serious TEAEs in 21 (7%). In stage B, TEAEs occurred in 71 (64%) participants on subcutaneous efgartigimod PH20 and 62 (56%) participants on placebo, and serious TEAEs in six (5%) on subcutaneous efgartigimod PH20 and six (5%) on placebo. Three deaths occurred: two in stage A (one non-related and one unlikely related to treatment) and one in stage B (placebo group). INTERPRETATION: ADHERE showed the efficacy of subcutaneous efgartigimod PH20 in reducing the risk of relapse versus placebo in people with CIDP who responded to treatment. Further studies are needed to provide data on the longer-term effects of efgartigimod alfa and how it compares with currently available treatment options. FUNDING: argenx.

    DOI： 10.1016/S1474-4422(24)00309-0

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  • Heterotypic macrophages/microglia differentially contribute to retinal ischaemia and neovascularisation. Reviewed International journal

    Muneo Yamaguchi, Shintaro Nakao, Mitsuru Arima, Karis Little, Aditi Singh, Iori Wada, Yoshihiro Kaizu, Souska Zandi, Justus G Garweg, Tetsuya Matoba, Wataru Shiraishi, Ryo Yamasaki, Kensuke Shibata, Yasuhiro Go, Tatsuro Ishibashi, Akiyoshi Uemura, Alan W Stitt, Koh-Hei Sonoda

    Diabetologia   67 ( 10 )   2329 - 2345   2024.7   （ ISSN:0012-186X eISSN:1432-0428 ）

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    AIMS/HYPOTHESIS: Diabetic retinopathy is characterised by neuroinflammation that drives neuronal and vascular degenerative pathology, which in many individuals can lead to retinal ischaemia and neovascularisation. Infiltrating macrophages and activated retina-resident microglia have been implicated in the progression of diabetic retinopathy, although the distinct roles of these immune cells remain ill-defined. Our aim was to clarify the distinct roles of macrophages/microglia in the pathogenesis of proliferative ischaemic retinopathies. METHODS: Murine oxygen-induced retinopathy is commonly used as a model of ischaemia-induced proliferative diabetic retinopathy (PDR). We evaluated the phenotype macrophages/microglia by immunostaining, quantitative real-time RT-PCR (qRT-PCR), flow cytometry and scRNA-seq analysis. In clinical imaging studies of diabetic retinopathy, we used optical coherence tomography (OCT) and OCT angiography. RESULTS: Immunostaining, qRT-PCR and flow cytometry showed expression levels of M1-like macrophages/microglia markers (CD80, CD68 and nitric oxide synthase 2) and M2-like macrophages/microglia markers (CD206, CD163 and macrophage scavenger receptor 1) were upregulated in areas of retinal ischaemia and around neo-vessels, respectively. scRNA-seq analysis of the ischaemic retina revealed distinct ischaemia-related clusters of macrophages/microglia that express M1 markers as well as C-C chemokine receptor 2. Inhibition of Rho-kinase (ROCK) suppressed CCL2 expression and reduced CCR2-positive M1-like macrophages/microglia in areas of ischaemia. Furthermore, the area of retinal ischaemia was reduced by suppressing blood macrophage infiltration not only by ROCK inhibitor and monocyte chemoattractant protein-1 antibody but also by GdCl3. Clinical imaging studies of diabetic retinopathy using OCT indicated potential involvement of macrophages/microglia represented by hyperreflective foci in areas of reduced perfusion. CONCLUSIONS/INTERPRETATION: These results collectively indicated that heterotypic macrophages/microglia differentially contribute to retinal ischaemia and neovascularisation in retinal vascular diseases including diabetic retinopathy. This adds important new information that could provide a basis for a more targeted, cell-specific therapeutic approach to prevent progression to sight-threatening PDR.

    DOI： 10.1007/s00125-024-06215-3

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  • Elucidation of the role of microglia-macrophage-based neuroinflammation in neurological diseases Reviewed

    Yamasaki, R

    NEUROLOGY AND CLINICAL NEUROSCIENCE   2024.5   （ ISSN:2049-4173 ）

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    Publisher：Neurology and Clinical Neuroscience  

    Objective: To elucidate the roles of microglia and macrophages in neuroinflammation and their dual impact on the progression of neurological diseases. Background: Microglia and macrophages are integral components of the central and peripheral nervous systems, where they play important roles in maintaining homeostasis, influencing disease progression, and facilitating repair mechanisms. Methods: The review integrates recent advancements in single-cell RNA sequencing that highlight the extensive heterogeneity of microglia and macrophages, which helps in understanding their wide-ranging roles in different neurological conditions. Results: The analysis reveals that microglia and macrophages have a dual nature, capable of both exacerbating and mitigating disease processes across various conditions, including Alzheimer’s disease, multiple sclerosis, stroke, peripheral nerve disorders, and brain tumors. Conclusion: Modulating the activity of microglia and macrophages offers a promising avenue for therapeutic interventions in neurological disorders. There is a critical need for further research to fully leverage their therapeutic potential in targeting neuroinflammation to enhance patient outcomes.

    DOI： 10.1111/ncn3.12829

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  • Focal 18F-fluorodeoxyglucose uptake in spinal dural arteriovenous fistula: A report of two cases(タイトル和訳中) Reviewed

    Hayashida Hitoshi, Masaki Katsuhisa, Ogata Hidenori, Yamaguchi Takahiro, Tanaka Koji, Arimura Koichi, Maruoka Yasuhiro, Kikuchi Kazufumi, Togao Osamu, Yamasaki Ryo, Isobe Noriko

    Neurology and Clinical Neuroscience   12 ( 3 )   201 - 204   2024.5

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  • Association between hypothalamic Alzheimer's disease pathology and body mass index: The Hisayama study. Reviewed International journal

    Kaoru Yagita, Hiroyuki Honda, Tomoyuki Ohara, Sachiko Koyama, Hideko Noguchi, Yoshinao Oda, Ryo Yamasaki, Noriko Isobe, Toshiharu Ninomiya

    Neuropathology : official journal of the Japanese Society of Neuropathology   44 ( 5 )   388 - 400   2024.4   （ ISSN:0919-6544 eISSN:1440-1789 ）

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    The hypothalamus is the region of the brain that integrates the neuroendocrine system and whole-body metabolism. Patients with Alzheimer's disease (AD) have been reported to exhibit pathological changes in the hypothalamus, such as neurofibrillary tangles (NFTs) and amyloid plaques (APs). However, few studies have investigated whether hypothalamic AD pathology is associated with clinical factors. We investigated the association between AD-related pathological changes in the hypothalamus and clinical pictures using autopsied brain samples obtained from deceased residents of a Japanese community. A total of 85 autopsied brain samples were semi-quantitatively analyzed for AD pathology, including NFTs and APs. Our histopathological studies showed that several hypothalamic nuclei, such as the tuberomammillary nucleus (TBM) and lateral hypothalamic area (LHA), are vulnerable to AD pathologies. NFTs are observed in various neuropathological states, including normal cognitive cases, whereas APs are predominantly observed in AD. Regarding the association between hypothalamic AD pathologies and clinical factors, the degree of APs in the TBM and LHA was associated with a lower body mass index while alive, after adjusting for sex and age at death. However, we found no significant association between hypothalamic AD pathology and the prevalence of hypertension, diabetes, or dyslipidemia. Our study showed that a lower BMI, which is a poor prognostic factor of AD, might be associated with hypothalamic AP pathology and highlighted new insights regarding the disruption of the brain-whole body axis in AD.

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  • 特集 筋萎縮性側索硬化症(ALS)への多彩な連携とリハビリテーション医療 新規ALS治療薬とリハビリテーション医療

    山﨑 亮

    J. of Clinical Rehabilitation   33 ( 2 )   142 - 149   2024.2   （ ISSN:09185259 ）

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    DOI： 10.32118/cr033020142

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  • Multiple dural arteriovenous fistulas showing isolated subcortical white matter T<sub>2</sub> hyperintensity with gadolinium enhancement Reviewed

    M.D. Hayashida Hitoshi, M.D. Ph.D. Masaki Katsuhisa, M.D. Ph.D. Ogata Hidenori, M.D. Harada Ayumi, M.D. Ph.D. Arimura Koichi, M.D. Ph.D. Yamasaki Ryo, M.D. Ph.D. Isobe Noriko

    Rinsho Shinkeigaku   64 ( 8 )   572 - 578   2024   （ ISSN:0009918X eISSN:18820654 ）

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    <p>We describe a 44-year-old man with a complaint of atonic seizures of the left upper limb, followed by generalized seizures. Brain MRI showed isolated juxtacortical white matter T<sub>2</sub> hyperintensity with gadolinium (Gd) enhancement of the adjacent cortical gray matter and subcortical white matter in the right frontal convexity. Treatment with levetiracetam was effective for seizure suppression, and he had no other neurological abnormalities. Human leukocyte antigen typing revealed B54 and Cw1, which indicated the possibility of neuro-Sweet disease. However, a general examination, which included vital signs and eye and skin findings, was normal. A cerebrospinal fluid test showed a mild elevation in protein levels without pleocytosis and a normal range of interleukin-6. Electroencephalography showed intermittent slow waves without epileptic discharge in the bilateral temporal lobes. We detected subtle flow voids in the pia mater of the left frontal lobe, which suggested cerebrovascular disease, and specifically, the possibility of dural arteriovenous fistulas. Computed tomography angiography showed abnormally dilated perimedullary veins in the left frontal lobe. Cerebral angiography confirmed the existence of four dural arteriovenous fistulas, which included two retrograde leptomeningeal venous drainages in the right frontal cortical veins supplied by the anterior branch of the right middle meningeal artery. The other dural arteriovenous fistulas were retrograde leptomeningeal venous drainages in the left frontal cortical veins supplied by the anterior and posterior convexity branches of the left middle meningeal artery. The patient underwent successful endovascular embolization of all dural arteriovenous fistulas with Onyx injection. A follow-up MRI showed gradual improvement of the T<sub>2</sub> hyperintensity and Gd enhancement. He remained seizure-free for 2 years following endovascular embolization.</p>

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  • ALS脊髄の異常タンパクドレナージチューブとしての末梢神経機能と浸潤マクロファージの働き

    山﨑 亮

    ファルマシア   60 ( 5 )   409 - 413   2024   （ ISSN:00148601 eISSN:21897026 ）

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    <p>難病中の難病である筋萎縮性側索硬化症の病態解明と治療法開発を目指し、著者らは全く新たな観点=脊髄前角細胞から末梢へ伸びる末梢神経軸索を「導管」に見立て、運ばれてくる異常蛋白の除去機構を疾患モデルを用いて検討した。その結果、マクロファージは確かに異常蛋白のクリアランスに寄与しており、これらのマクロファージ除去により脊髄前角細胞の脱落が促進した。末梢神経軸索輸送を介した異常蛋白の運搬除去に、マクロファージが重要な役割を果たしていることが明らかとなった。</p>

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  • Connexins Control Glial Inflammation in Various Neurological Diseases Reviewed

    Yamasaki, R

    INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES   24 ( 23 )   2023.12   （ ISSN:1661-6596 eISSN:1422-0067 ）

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    Connexins (Cxs) form gap junctions through homotypic/heterotypic oligomerization. Cxs are initially synthesized in the endoplasmic reticulum, then assembled as hexamers in the Golgi apparatus before being integrated into the cell membrane as hemichannels. These hemichannels remain closed until they combine to create gap junctions, directly connecting neighboring cells. Changes in the intracellular or extracellular environment are believed to trigger the opening of hemichannels, creating a passage between the inside and outside of the cell. The size of the channel pore depends on the Cx isoform and cellular context-specific effects such as posttranslational modifications. Hemichannels allow various bioactive molecules, under ~1 kDa, to move in and out of the host cell in the direction of the electrochemical gradient. In this review, we explore the fundamental roles of Cxs and their clinical implications in various neurological dysfunctions, including hereditary diseases, ischemic brain disorders, degenerative conditions, demyelinating disorders, and psychiatric illnesses. The influence of Cxs on the pathomechanisms of different neurological disorders varies depending on the circumstances. Hemichannels are hypothesized to contribute to proinflammatory effects by releasing ATP, adenosine, glutamate, and other bioactive molecules, leading to neuroglial inflammation. Modulating Cxs’ hemichannels has emerged as a promising therapeutic approach.

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  • Caution to Poor Adherence With Immunosuppressant Medication That Causes Coma-Onset Autoimmune Encephalitis: A Case Report and Literature Review Reviewed International journal

    Toshida K, Toshima T, Itoh S, Yoshiya S, Mukaino T, Fujii T, Watanabe M, Yamasaki R, Isobe N, Yoshizumi T.

    Transplantation proceedings   55 ( 8 )   1968 - 1971   2023.10   （ ISSN:0041-1345 eISSN:1873-2623 ）

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    Autoimmune encephalitis after liver transplantation (LT) is a rare disorder. This is because patients are usually in an immunosuppressed state after LT. Here, we report a rare case of autoantibody-negative autoimmune-encephalitis-induced coma after living-donor (LD) LT. A 45-year-old woman who underwent LDLT for primary biliary cholangitis (PBC) was brought to our hospital with the chief complaint of cognitive deficiency and an episode of memory loss. Physical examination, laboratory tests, and cerebrospinal fluid analysis revealed no significant findings. However, diffusion-weighted magnetic resonance imaging showed hyperintensity in the bilateral hippocampus. No autoantibodies associated with autoimmune encephalitis were detected. The diagnosis of antibody-negative autoimmune encephalitis was made on the basis of low immunosuppressive drug levels in the blood (indicative of poor adherence) and the presence of PBC as the autoimmune disease. The patient regained consciousness after intravenous methylprednisolone pulse therapy and plasma exchange. This case highlights that when examining patients with impaired consciousness after LDLT, it is important to consider autoimmune encephalitis as a potential diagnosis.

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  • エクリズマブからラブリズマブへの切り替え後増悪傾向となった全身型重症筋無力症の一例 Reviewed

    坂上 晴紀, 吉村 廣紀, 渡邉 充, 入江 剛史, 田中 栄蔵, 山崎 亮, 磯部 紀子

    臨床神経学   63 ( 10 )   681 - 681   2023.10   （ ISSN:0009-918X eISSN:1882-0654 ）

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  • 重症筋無力症・心筋炎・肝炎を合併したirAE筋炎に対し免疫治療が有効であった1例 Reviewed

    芹野 南美, 眞崎 勝久, 向野 隆彦, 入江 剛史, 山崎 亮, 磯部 紀子

    臨床神経学   63 ( 10 )   680 - 680   2023.10   （ ISSN:0009-918X eISSN:1882-0654 ）

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  • 視床下部アルツハイマー病理はBMI低値と相関する 久山町研究

    八木田 薫, 本田 裕之, 小原 知之, 山崎 亮, 磯部 紀子, 二宮 利治

    Dementia Japan   37 ( 4 )   686 - 686   2023.10   （ ISSN:1342-646X ）

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  • ALSモデルマウスの脊髄におけるマクロファージ/ミクログリア動態の検討

    江 千里, 橋本 侑, 小早川 優子, 山崎 亮, 磯部 紀子

    神経免疫学   28 ( 1 )   221 - 221   2023.9   （ ISSN:0918-936X ）

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  • 脱髄性ニューロパチー患者における大径線維障害と小径線維障害の関連についての検討

    藤井 敬之, 緒方 英紀, 田代 匠, 井ノ上 有香, 山崎 亮, 磯部 紀子

    神経免疫学   28 ( 1 )   198 - 198   2023.9   （ ISSN:0918-936X ）

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  • 活性化ミクログリアが産生する蛋白に着目した運動神経細胞変性相関因子の同定と診断バイオマーカーの探求

    林 信太郎, 山崎 亮, 小早川 優子, 吉良 潤一, 磯部 紀子

    神経免疫学   28 ( 1 )   206 - 206   2023.9   （ ISSN:0918-936X ）

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  • 中枢神経作用コネキシン阻害薬による多系統萎縮症小脳型モデルマウスの治療

    原田 雅也, 眞崎 勝久, 松瀬 大, 山口 浩雄, 西村 由宇慈, 高瀬オズデミール エズギ, 田中 栄蔵, 田中 辰典, 山崎 亮, 竹内 英之, 谷脇 考恭, 磯部 紀子, 吉良 潤一

    神経免疫学   28 ( 1 )   191 - 191   2023.9   （ ISSN:0918-936X ）

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  • コネキシン30欠損はグリア炎症を抑制することでALSモデルマウスの発症早期の疾患進行を緩和する Reviewed

    橋本 侑, 山崎 亮, 江 千里, 小早川 優子, 眞崎 勝久, 松瀬 大, 松下 拓也, 磯部 紀子

    神経免疫学   28 ( 1 )   221 - 221   2023.9   （ ISSN:0918-936X ）

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  • Exosome Connexin43-Truncated Isoforms and Bound RNAs Distinctively Associated with Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder Reviewed

    Kira, JI; Maimaitijiang, G; Sakoda, A; Masaki, K; Watanabe, M; Nagata, S; Ozdemir, E; Yamasaki, R; Isobe, N; Zhang, X; Imamura, T; Nakamura, Y

    ANNALS OF NEUROLOGY   94   S54 - S55   2023.9   （ ISSN:0364-5134 eISSN:1531-8249 ）

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  • Astroglial Connexin 43 is a Novel Therapeutic Target for a Chronic Multiple Sclerosis Model

    Ozdemir, E; Yamasaki, R; Nagata, S; Watanabe, M; Yamaguchi, H; Masaki, K; Kira, J; Takeuchi, H; Isobe, N

    MULTIPLE SCLEROSIS JOURNAL   29 ( 7 )   NP2 - NP2   2023.6   （ ISSN:1352-4585 eISSN:1477-0970 ）

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  • Early and extensive alterations of glial connexins, distal oligodendrogliopathy type demyelination, and nodal/paranodal pathology are characteristic of multiple system atrophy. Reviewed International journal

    Yuji Nishimura, Katsuhisa Masaki, Dai Matsuse, Hiroo Yamaguchi, Tatsunori Tanaka, Eriko Matsuo, Shotaro Hayashida, Mitsuru Watanabe, Takuya Matsushita, Shoko Sadashima, Naokazu Sasagasako, Ryo Yamasaki, Noriko Isobe, Toru Iwaki, Jun-Ichi Kira

    Brain pathology (Zurich, Switzerland)   33 ( 3 )   e13131   2023.5   （ ISSN:1015-6305 eISSN:1750-3639 ）

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    The pathological hallmark of multiple system atrophy (MSA) is aberrant accumulation of phosphorylated α-synuclein in oligodendrocytes, forming glial cytoplasmic inclusions (GCIs). Extensive demyelination occurs particularly in the olivopontocerebellar and striatonigral pathways, but its precise mechanism remains elusive. Glial connexins (Cxs), which form gap junction channels between astrocytes and oligodendrocytes, play critical roles in myelin maintenance, and have not been studied in MSA. Therefore, we immunohistochemically investigated glial Cx changes in the cerebellar afferent fibers in 15 autopsied patients with MSA. We classified demyelinating lesions into three stages based on Klüver-Barrera staining: early (Stage I), intermediate (Stage II), and late (Stage III) stages showing subtle, moderate, and severe myelin reduction, respectively. Myelin-associated glycoprotein, but not myelin oligodendrocyte glycoprotein, was preferentially decreased in Stage I, suggesting distal oligodendrogliopathy type demyelination. Accumulation of phosphorylated α-synuclein in oligodendrocytes was frequently seen in Stage I but less frequently observed in Stages II and III. Tubulin polymerization-promoting protein (TPPP/p25α)-positive oligodendrocytes were preserved in Stage I but successively decreased in Stages II and III. Even at Stage I, Cx32 was nearly absent from myelin, despite the relative preservation of other nodal proteins, such as neurofascin, claudin-11/oligodendrocyte-specific protein, and contactin-associated protein 1, which successively decreased in the later stages. Cx32 was re-distributed in the oligodendrocyte cytoplasm and co-localized with GCIs. Cx47 gradually decreased at the oligodendrocyte surface in a stage-dependent manner but was not co-localized with GCIs. Astrocytic Cx43 was down-regulated in Stage I but up-regulated in Stages II and III, reflecting astrogliosis. Cx43/Cx47 gap junctions significantly decreased from Stage I to III. Activated microglia/macrophages and T cells infiltrated in Stage I rather than Stages II and III. Therefore, early and extensive alterations of glial Cxs, particularly Cx32 loss, occur in MSA and may accelerate distal oligodendrogliopathy type demyelination and nodal/paranodal dysfunction through disruption of inter-glial communication.

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  • 急性片麻痺で発症し,両側側脳室周囲病変を呈したCADASILの一例 Reviewed

    本河 悠太, 眞崎 勝久, 向野 隆彦, 山川 良太, 山崎 亮, 吾郷 哲朗, 高瀬 敬一郎, 植田 明彦, 植田 光晴, 磯部 紀子

    臨床神経学   63 ( 5 )   330 - 330   2023.5   （ ISSN:0009-918X eISSN:1882-0654 ）

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  • 小脳性運動失調で発症し,中枢性尿崩症と骨病変から診断し得たErdheim-Chester病の1例 Reviewed

    芹野 南美, 眞崎 勝久, 向野 隆彦, 松吉 彩乃, 山崎 亮, 磯部 紀子

    臨床神経学   63 ( 5 )   335 - 335   2023.5   （ ISSN:0009-918X eISSN:1882-0654 ）

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  • High-dose ubiquinol supplementation in multiple-system atrophy: a multicentre, randomised, double-blinded, placebo-controlled phase 2 trial Reviewed International journal

    Mitsui J, Matsukawa T, Uemura Y, Kawahara T, Chikada A, Porto KJL, Naruse H, Tanaka M, Ishiura H, Toda T, Kuzuyama H, Hirano M, Wada I, Ga T, Moritoyo T, Takahashi Y, Mizusawa H, Ishikawa K, Yokota T, Kuwabara S, Sawamoto N, Takahashi R, Abe K, Ishihara T, Onodera O, Matsuse D, Yamasaki R, Kira JI, Katsuno M, Hanajima R, Ogata K, Takashima H, Matsushima M, Yabe I, Sasaki H, Tsuji S.

    EClinicalMedicine   59   101920   2023.4   （ eISSN:2589-5370 ）

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  • Anti-LGI4 Antibody Is a Novel Juxtaparanodal Autoantibody for Chronic Inflammatory Demyelinating Polyneuropathy. Reviewed International journal

    Xu Zhang, Jun-Ichi Kira, Hidenori Ogata, Tomohiro Imamura, Mikio Mitsuishi, Takayuki Fujii, Masaki Kobayashi, Kazuo Kitagawa, Yukihiro Namihira, Yusuke Ohya, Guzailiayi Maimaitijiang, Ryo Yamasaki, Yuko Fukata, Masaki Fukata, Noriko Isobe, Yuri Nakamura

    Neurology(R) neuroimmunology & neuroinflammation   10 ( 2 )   2023.3   （ ISSN:2332-7812 ）

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    BACKGROUND AND OBJECTIVES: The objective of this study was to discover novel nodal autoantibodies in chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: We screened for autoantibodies that bind to mouse sciatic nerves and dorsal root ganglia (DRG) using indirect immunofluorescence (IFA) assays with sera from 113 patients with CIDP seronegative for anti-neurofascin 155 and anticontactin-1 antibodies and 127 controls. Western blotting, IFA assays using HEK293T cells transfected with relevant antigen expression plasmids, and cell-based RNA interference assays were used to identify target antigens. Krox20 and Periaxin expression, both of which independently control peripheral nerve myelination, was assessed by quantitative real-time PCR after application of patient and control sera to Schwann cells. RESULTS: Sera from 4 patients with CIDP, but not control sera, selectively bound to the nodal regions of sciatic nerves and DRG satellite glia (p = 0.048). The main immunoglobulin G (IgG) subtype was IgG4. IgG from these 4 patients stained a 60-kDa band on Western blots of mouse DRG and sciatic nerve lysates. These features indicated leucine-rich repeat LGI family member 4 (LGI4) as a candidate antigen. A commercial anti-LGI4 antibody and IgG from all 4 seropositive patients with CIDP showed the same immunostaining patterns of DRG and cultured rat Schwann cells and bound to the 60-kDa protein in Western blots of LGI4 overexpression lysates. IgG from 3 seropositive patients, but none from controls, bound to cells cotransfected with plasmids containing LGI4 and a disintegrin and metalloprotease domain-containing protein 22 (ADAM22), an LGI4 receptor. In cultured rat Schwann and human melanoma cells constitutively expressing LGI4, LGI4 siRNA effectively downregulated LGI4 and reduced patients' IgG binding compared with scrambled siRNA. Application of serum from a positive patient to Schwann cells expressing ADAM22 significantly reduced the expression of Krox20, but not Periaxin. Anti-LGI4 antibody-positive patients had a relatively old age at onset (mean age 58 years), motor weakness, deep and superficial sensory impairment with Romberg sign, and extremely high levels of CSF protein. Three patients showed subacute CIDP onset resembling Guillain-Barré syndrome. DISCUSSION: IgG4 anti-LGI4 antibodies are found in some elderly patients with CIDP who present subacute sensory impairment and motor weakness and are worth measuring, particularly in patients with symptoms resembling Guillain-Barré syndrome.

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  • Effect of smoking on disease activity in multiple sclerosis patients treated with dimethyl fumarate or fingolimod. Reviewed International journal

    Eizo Tanaka, Mitsuru Watanabe, Shoko Fukumoto, Katsuhisa Masaki, Ryo Yamasaki, Takuya Matsushita, Noriko Isobe

    Multiple sclerosis and related disorders   70   104513 - 104513   2023.2   （ ISSN:2211-0348 eISSN:2211-0356 ）

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    BACKGROUND: In relapsing-remitting multiple sclerosis (RRMS), smoking is a known risk factor for disease susceptibility and disability progression. However, its impact on the efficacy of oral disease-modifying drugs (DMDs) is unclear. Therefore, we initiated a single-center, retrospective, observational study to investigate the relationship between smoking and disease activity in RRMS patients under oral DMDs. METHODS: We retrospectively enrolled RRMS patients who initiated oral DMDs (fingolimod or dimethyl fumarate) at our hospital between January 2012 and December 2019. Clinical data and smoking status at oral DMD initiation were collected up to December 2020. We conducted survival analyses for relapse and any disease activity, defined as relapse or MRI disease activity, among patients with distinct smoking statuses. RESULTS: We enrolled 103 RRMS patients under oral DMDs including 19 (18.4%) current smokers at baseline. Proportions of relapses and any disease activity during follow-up were higher in current smokers (relapse: p = 0.040, any disease activity: p = 0.004) and time from initiating oral DMDs to relapse was shorter in current smokers (log-rank test: p = 0.011; Cox proportional hazard analysis: hazard ratio (HR) 2.72 [95% confidence interval (CI) 1.22-6.09], p = 0.015) than in non-smokers. Time from initiating oral DMDs to any disease activity was also shorter in current smokers (log-rank test: p = 0.016; Cox proportional hazard analysis: HR 2.18 [95% CI 1.14-4.19], p = 0.019) than in non-smokers. The survival curves for relapse and any disease activity were not different between the former smoker and never-smoker groups. Multivariate survival analysis showed current smoking was an independent risk factor for relapse or any disease activity after adjusting for covariates (relapse: HR 2.54 [95% CI 1.06-6.10], p = 0.037; any disease activity: HR 3.47 [95% CI 1.27-9.50], p = 0.015). CONCLUSION: Smoking was a risk factor for disease activity in RRMS patients under oral DMD treatment. RRMS patients should be advised to stop smoking even after the initiation of DMDs.

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  • 踵膝試験での右下肢の「さまよう動き」を契機に診断に至った大脳皮質基底核症候群(CBS)の1例 Reviewed

    内和田 英人, 藤井 敬之, 緒方 英紀, 山崎 亮, 磯部 紀子

    臨床神経学   63 ( 2 )   112 - 112   2023.2   （ ISSN:0009-918X eISSN:1882-0654 ）

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  • Hereditary myopathy with early respiratory failure(HMERF)の1例 Reviewed

    宮崎 哲, 緒方 英紀, 吉村 基, 向野 隆彦, 渡邉 充, 山崎 亮, 本田 裕之, 西野 一三, 磯部 紀子

    臨床神経学   63 ( 2 )   114 - 114   2023.2   （ ISSN:0009-918X eISSN:1882-0654 ）

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  • A case report of anti-N-methyl-D-aspartate receptor encephalitis with chromosomally integrated human herpesvirus 6

    Kazunori Iwao, Mitsuru Watanabe, Takahiko Mukaino, Takayuki Fujii, Ryo Yamasaki, Noriko Isobe

    NEUROLOGY AND CLINICAL NEUROSCIENCE   11 ( 1 )   52 - 54   2023.1   （ ISSN:2049-4173 ）

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    Chromosomally integrated human herpesvirus 6 (ciHHV6) is a condition where HHV6-DNA is integrated into the host germline genome. ciHHV6 can be misdiagnosed as active HHV6 infection. We report a 30-year-old woman presenting with psychological symptoms without a history of immunodeficiency. She had an ovarian teratoma and anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in the cerebrospinal fluid (CSF) with HHV6-DNA in the serum and CSF. The final diagnosis was anti-NMDAR encephalitis and ciHHV6 because laparoscopic oophorectomy and immunotherapy ameliorated her symptoms and HHV6-DNA was detected in her oral mucosa cells. This case suggests the need to assess whether HHV6-DNA is related to infection or ciHHV6 when HHV6-DNA is detected in the CSF of patients with encephalitis.

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  • 染色体に組み込まれたヒトヘルペスウイルス6を伴う抗NMDA受容体脳炎の症例報告(A case report of anti-N-methyl-D-aspartate receptor encephalitis with chromosomally integrated human herpesvirus 6) Reviewed

    Iwao Kazunori, Watanabe Mitsuru, Mukaino Takahiko, Fujii Takayuki, Yamasaki Ryo, Isobe Noriko

    Neurology and Clinical Neuroscience   11 ( 1 )   52 - 54   2023.1

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    脳脊髄液(CSF)で抗NMDA受容体(NMDAR)抗体とヒトヘルペスウイルス6(HHV6)-DNA陽性を示し、抗NMDAR脳炎および染色体に組み込まれたHHV6(ciHHV6)と最終診断された1例について報告した。症例は30歳女性で、Day0に亜急性の情動不安と錯乱状態を呈し頭痛を伴い、Day39に自宅の二階から飛び降りた。救急搬送されたが妄想が認められたため精神病院に移送され、移送先の医療施設で自己免疫性脳炎が疑われたことから、Day67に見当識障害、幻視、被害妄想、妄想気分、情緒不安定、激越を伴った状態で当院に移送された。CSFには軽度の髄液細胞増加、高タンパク血症、抗NMDAR抗体が検出され、血清とCSFからはHHV6-DNA陽性が示されたが、他のヘルペスウイルスDNAは陰性であった。脳波では突発活動を伴わないdiffuse background slowingが検出され、脳MRI検査では正常所見であったが、HHV6脳炎と抗NMDAR脳炎を疑い、抗ウイルス薬投与とメチルプレドニゾロン静注後に、静注免疫グロブリンが投与された。Day82に左卵巣腫瘍に対し腹腔鏡下卵巣摘出術を行い、血漿交換を行ったところ症状は改善され、CSF中細胞数とタンパク質も正常値に低下し、脳波も正常化した。しかし、6週間のガンシクロビル投与後も血清/CSFともにHHV6-DNA陽性、口腔粘膜細胞もHHV6-DNA陽性で、ガンシクロビル投与を中止したが、症状の増悪はみられなかった。以上の所見から、本例は抗NMDAR脳炎とciHHV6と最終診断された。

  • Dietary 7-ketocholesterol exacerbates myocardial ischemia-reperfusion injury in mice through monocyte/macrophage-mediated inflammation Invited Reviewed International journal

    Uchikawa T, Matoba T, Kawahara T, Baba I, Katsuki S, Koga JI, Hashimoto Y, Yamasaki R, Ichi I, Akita H, Tsutsui H

    Scientific reports   12 ( 1 )   14902   2022.12

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  • Connexin 30 deficiency ameliorates disease progression at the early phase in a mouse model of amyotrophic lateral sclerosis by suppressing glial inflammation Invited Reviewed International journal

    Hashimoto Y, Yamasaki R, Senri K, Matsuo E, Kobayakawa Y, Masaki K, Matsuse D, Isobe N

    IJMS   23 ( 24 )   16046   2022.12

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  • Identifying hyperreflective foci in diabetic retinopathy via VEGF-induced local self-renewal of CX3CR1+ vitreous resident macrophages Invited Reviewed International journal

    Yamaguchi M, Nakao S, Wada I, Matoba T, Arima M, Kaizu Y, Shirane M, Ishikawa K, Nakama T, Murakami Y, Mizuochi M, Shiraishi W, Yamasaki R, Hisatomi T, Ishibashi T, Shibuya M, Stitt AW, Sonoda KH

    Diabetes   71 ( 12 )   2685 - 2701   2022.12

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  • Non-IgG4抗体neurofascin155抗体陽性症例における臨床像の検討

    緒方 英紀, Abdelhadi Amina, 山崎 亮, 吉良 潤一, 磯部 紀子

    末梢神経   33 ( 2 )   303 - 303   2022.12   （ ISSN:0917-6772 ）

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  • 上腕二頭筋生検で診断に至らず,外眼筋組織で遺伝子診断に至った慢性進行性外眼筋麻痺の1例 Reviewed

    白石 渉, 立石 貴久, 橋本 侑, 山崎 亮, 吉良 潤一, 磯部 紀子

    臨床神経学   62 ( 12 )   946 - 951   2022.12   （ ISSN:0009-918X eISSN:1882-0654 ）

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    症例は初診時48歳の男性.緩徐進行性の複視を主訴に当科を受診した.家族歴はなく,エドロホニウムテストは陰性だった.血液検査で乳酸とピルビン酸値の上昇を認め,ミトコンドリア病を疑い,上腕二頭筋を生検したが筋病理,遺伝子検査とも異常は認められなかった.患者は後日,複視に対し眼筋縫縮術を施行された.その際に得られた組織で,ミトコンドリア遺伝子の多重欠失を認め,慢性進行性外眼筋麻痺の診断に至った.本例は上腕二頭筋組織に異常を認めず,外眼筋組織で遺伝子異常を認め,診断に至った.ミトコンドリア病では,ミトコンドリア遺伝子異常の組織特異性が指摘されており,生検を施行する際には罹患筋を選択することが望ましい.(著者抄録)

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    Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J01550&link_issn=&doc_id=20221220320006&doc_link_id=10.5692%2Fclinicalneurol.cn-001798&url=https%3A%2F%2Fdoi.org%2F10.5692%2Fclinicalneurol.cn-001798&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

  • A case report of anti-N-methyl-d-aspartate receptor encephalitis with chromosomally integrated human herpesvirus 6 Reviewed

    Iwao Kazunori, Watanabe Mitsuru, Mukaino Takahiko, Fujii Takayuki, Yamasaki Ryo, Isobe Noriko

    Neurology and Clinical Neuroscience   11 ( 1 )   52 - 54   2022.10   （ ISSN:20494173 ）

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    Chromosomally integrated human herpesvirus 6 (ciHHV6) is a condition where HHV6- DNA is integrated into the host germline genome. ciHHV6 can be misdiagnosed as active HHV6 infection. We report a 30-year-old woman presenting with psychological symptoms without a history of immunodeficiency. She had an ovarian teratoma and anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in the cerebrospinal fluid (CSF) with HHV6-DNA in the serum and CSF. The final diagnosis was anti-NMDAR encephalitis and ciHHV6 because laparoscopic oophorectomy and immunotherapy ameliorated her symptoms and HHV6-DNA was detected in her oral mucosa cells. This case suggests the need to assess whether HHV6-DNA is related to infection or ciHHV6 when HHV6-DNA is detected in the CSF of patients with encephalitis.

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  • Synucleionopathy-Associated Microglia Uncovered by a Novel Multiple System Atrophy-Cerebellar Type (MSA-c) Mouse Model Reviewed

    Kira, J; Yamaguchi, H; Nishimura, Y; Matsuse, D; Masaki, K; Yamasaki, R; Isobe, N

    ANNALS OF NEUROLOGY   92   S125 - S126   2022.10   （ ISSN:0364-5134 eISSN:1531-8249 ）

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  • 筋萎縮性側索硬化症モデルマウスにおけるコネキシン30の発現調節異常の役割(The role of dysregulated connexin 30 expression in amyotrophic lateral sclerosis model mice)

    Hashimoto Yu, Ko Senri, Kobayakawa Yuko, Yamasaki Ryo, Isobe Noriko

    臨床神経学   62 ( Suppl. )   S377 - S377   2022.10   （ ISSN:0009-918X eISSN:1882-0654 ）

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  • 末梢神経疾患1 定量的感覚検査を用いた自己免疫性ノドパチーにおける小径線維障害の検討

    藤井 敬之, 緒方 英紀, 山崎 亮, 磯部 紀子

    神経免疫学   27 ( 1 )   138 - 138   2022.10   （ ISSN:0918-936X ）

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  • 定量的IgG4抗neurofascin 155抗体、抗contactin-1抗体測定ELISAキットの有用性

    緒方 英紀, 小早川 優子, 川嵜 淳史, 竹内 智枝理, 山崎 亮, 吉良 潤一, 磯部 紀子

    神経免疫学   27 ( 1 )   202 - 202   2022.10   （ ISSN:0918-936X ）

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  • 一過性脳虚血発作様の発作を繰り返した神経核内封入体病の1例

    倉沢 亮, 渡邉 充, 向野 隆彦, 入江 剛史, 山崎 亮, 磯部 紀子

    臨床神経学   62 ( 10 )   815 - 815   2022.10   （ ISSN:0009-918X eISSN:1882-0654 ）

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  • その他 多系統萎縮症におけるconnexin蛋白群の早期かつ広範な変化と脱髄病態との関連

    西村 由宇慈, 眞崎 勝久, 松瀬 大, 山口 浩雄, 田中 辰典, 貞島 祥子, 笹ヶ迫 直一, 山崎 亮, 磯部 紀子, 岩城 徹, 吉良 潤一

    神経免疫学   27 ( 1 )   147 - 147   2022.10   （ ISSN:0918-936X ）

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  • その他 カルシウム結合蛋白に着目したミクログリアからの筋萎縮性側索硬化症重症度関連ケモカイン放出因子の解明

    林 信太郎, 山崎 亮, 吉良 潤一, 磯部 紀子

    神経免疫学   27 ( 1 )   146 - 146   2022.10   （ ISSN:0918-936X ）

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  • Teaching Video NeuroImage: Reversible Parkinsonism Caused by Lumboperitoneal Shunt Overdrainage. Reviewed International journal

    Hajime Takeuchi, Katsuhisa Masaki, Hidenori Ogata, Satoshi Nagata, Takafumi Shimogawa, Ryo Yamasaki, Noriko Isobe

    Neurology   99 ( 11 )   486 - 488   2022.9   （ ISSN:0028-3878 eISSN:1526-632X ）

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    DOI： 10.1212/WNL.0000000000200994

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  • Dietary 7-ketocholesterol exacerbates myocardial ischemia–reperfusion injury in mice through monocyte/macrophage-mediated inflammation Reviewed International journal

    Tomoki Uchikawa, Tetsuya Matoba, Takuro Kawahara, Isashi Baba, Shunsuke Katsuki, Jun-ichiro Koga, Yu Hashimoto, Ryo Yamasaki, Ikuyo Ichi, Hidetaka Akita, Hiroyuki Tsutsui

    Scientific Reports   12 ( 1 )   14902 - 14902   2022.9   （ ISSN:2045-2322 eISSN:2045-2322 ）

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    Abstract

    Emerging evidence suggests that 7-ketocholesterol (7-KC), one of the most abundant dietary oxysterols, causes inflammation and cardiovascular diseases. Here we show the deteriorating effects of dietary 7-KC on myocardial ischemia–reperfusion (IR) injury and detailed the molecular mechanisms. A high-fat high-cholesterol diet containing 7-KC (7KWD) for 3 weeks increased the plasma 7-KC level compared with high-fat high-cholesterol diet in mice. In wild-type mice but not in CCR2<sup>−/−</sup> mice, dietary 7-KC increased the myocardial infarct size after IR. Flow cytometry revealed that the ratio of Ly-6C<sup>high</sup> inflammatory monocytes to total monocytes was increased in the 7KWD group. Unbiased RNA sequencing using murine primary macrophages revealed that 7-KC regulated the expression of transcripts related to inflammation and cholesterol biosynthesis. We further validated that in vitro, 7-KC induced endoplasmic reticulum stress, mitochondrial reactive oxygen species production, and nuclear factor-kappa B activation, which are associated with increased mRNA levels of proinflammatory cytokines. Administration of N-acetyl-l-cysteine or siRNA-mediated knockdown of PKR-like endoplasmic reticulum kinase or endoplasmic reticulum oxidase 1α suppressed the levels of 7-KC-induced inflammation. Dietary 7-KC exacerbates myocardial IR injury through monocyte/macrophage-mediated inflammation. Endoplasmic reticulum stress and oxidative stress are involved in the 7-KC-induced proinflammatory response in macrophages.

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  • A novel quantitative indicator for disease progression rate in amyotrophic lateral sclerosis. Reviewed International journal

    Yuko Kobayakawa, Koji Todaka, Yu Hashimoto, Senri Ko, Wataru Shiraishi, Junji Kishimoto, Jun-Ichi Kira, Ryo Yamasaki, Noriko Isobe

    Journal of the neurological sciences   442   120389 - 120389   2022.8   （ ISSN:0022-510X eISSN:1878-5883 ）

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    OBJECTIVE: The current study sought to develop a new indicator for disease progression rate in amyotrophic lateral sclerosis (ALS). METHODS: We used a nonparametric method to score diverse patterns of decline in the percentage of predicted forced vital capacity (%FVC) in patients with ALS. This involved 6317 longitudinal %FVC data sets from 920 patients in the Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) database volunteered by PRO-ACT Consortium members. To assess the utility of the derived scores as a disease indicator, we examined changes over time, the association with prognosis, and correlation with the Risk Profile of the Treatment Research Initiative to Cure ALS (TRICALS). Our local cohort (n = 92) was used for external validation. RESULTS: We derived scores ranging from 35 to 106 points to construct the FVC Decline Pattern scale (FVC-DiP). Individuals' FVC-DiP scores were determined from a single measurement of %FVC and disease duration at assessment. Although the %FVC declined over the disease course (p < 0.0001), the FVC-DiP remained relatively stable. Low FVC-DiP scores were associated with rapid disease progression. Using our cohort, we demonstrated an association between FVC-DiP and the survival prognosis, the stability of the FVC-DiP per individual, and a correlation between FVC-DiP scores and the TRICALS Risk Profile (r2 = 0.904, p < 0.0001). CONCLUSIONS: FVC-DiP scores reflected patterns of declining %FVC over the natural course of ALS and indicated the disease progression rate. The FVC-DiP may enable easy assessment of disease progression patterns and could be used for assessing treatment efficacy.

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  • Activation of Microglia and Impairment of Neurogenesis through DNA Repair Enzyme, MUTYH, in the Pathogenesis of Alzheimer's Disease Reviewed

    Abolhassani, N; Mizuno, Y; Mazzei, G; Sakumi, K; Saito, T; Saido, TC; Ninomiya, T; Iwaki, T; Yamasaki, R; Kira, JI; Nakabeppu, Y

    ENVIRONMENTAL AND MOLECULAR MUTAGENESIS   63   79 - 79   2022.8   （ ISSN:0893-6692 eISSN:1098-2280 ）

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  • DEVELOPMENT OF STANDARDIZED ELISA KITS FOR IGG4 ANTI-NEUROFASCIN 155 AND ANTI-CONTACTIN-1 ANTIBODIES Reviewed

    Ogata, H; Kobayakawa, Y; Amina, A; Yamasaki, R; Kawasaki, A; Takeuchi, C; Kira, J; Isobe, N

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   27   S172 - S172   2022.7   （ ISSN:1085-9489 eISSN:1529-8027 ）

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  • RECIPE: A PHASE II RANDOMIZED CONTROLLED TRIAL OF RITUXIMAB FOR REFRACTORY CIDP WITH IGG4 AUTOANTIBODIES Reviewed

    Iijima, M; Shimizu, S; Fukami, Y; Koike, H; Kaida, K; Mori, M; Kuwabara, S; Koga, M; Kanda, T; Ogata, H; Yamasaki, R; Kira, J; Katsuno, M

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   27   S164 - S165   2022.7   （ ISSN:1085-9489 eISSN:1529-8027 ）

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  • The study of facial expression recognition from facial expression discrimination threshold in patients with temporal lobe epilepsy Reviewed

    Arakawa Tomomi, Mukaino Takahiko, Yamada Emi, Uehara Taira, Fukui Keiko, Yamasaki Ryo, Kira Junichi, Uchida Shinya

    Japanese Journal of Neuropsychology   38 ( 2 )   155 - 165   2022.6   （ ISSN:09111085 eISSN:21899401 ）

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    The study of facial expression recognition from facial expression discrimination threshold in patients with temporal lobe epilepsy
    The present study investigated the ability of facial emotion recognition in patients with temporal lobe epilepsy (TLE). Total of 12 TLE patients and 32 healthy controls were participated. Two tasks consisted of morphing movie task and static picture task were used. Emotions of happiness, anger, sadness and surprise were examined. In the morphing movie task, movies in which face was gradually morphed from neutral emotion expression to maximum emotion expression were presented to the participants, then they were asked to respond when they judged that facial emotion was changed from neutral expression. Reaction time from onset of movie presentation to response was used to calculate facial emotion discrimination threshold. In the static picture task, static image in which face with maximum facial emotion expression was presented, and participants were asked to judge type of emotion. The results showed that the facial emotion discrimination thresholds in emotions of surprise and anger were significantly higher in TLE patients compared with healthy control in the morphing task. No errors were found in the static picture task. TLE patients were able to recognize the facial expression of static image with maximum emotion expression as well as the healthy group, but showed the increase of facial emotion discrimination threshold. The morphing movie task could be detectable increase of facial emotion discrimination threshold in TLE patients.

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  • [Small Fiber Neuropathy with Inadequate Response to Medical Therapy: Diagnosis of The Etiology of Small Fiber Neuropathy and Treatment Option].

    Ryo Yamasaki

    Brain and nerve = Shinkei kenkyu no shinpo   74 ( 5 )   608 - 613   2022.5   （ ISSN:18816096 eISSN:13448129 ）

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    Small-fiber neuropathy (SFN) has few significant laboratory findings and is difficult to diagnose. In 70% of the cases, the cause of SFN is unknown. Among the cases with known etiology, 50% are associated with diabetes, and the causes are autoimmune, amyloidosis, or multifactorial. In recent years, a specific autoantibody-positive group has been identified and has attracted attention because immunotherapy was successful in the autoantibody-positive SFN groups. In the cases reporting to our department, abnormalities could not be detected by various tests, including nerve conduction studies, and the response to symptomatic treatment was poor. An abnormality was identified in the current perception threshold test result, and a positive blood anti-plexin D1 antibody was detected via enzyme-linked immunosorbent assay. Therefore, autoimmune SFN was diagnosed, and plasma exchange therapy was remarkably effective. Subsequently, we aim to introduce general treatments for SFN and COVID-19-related SFN.

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  • LGI4 IS A NOVEL AUTOANTIGEN FOR NODOPATHY TYPE CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY

    Zhang, X; Ogata, H; Imamura, T; Fujii, T; Yamasaki, R; Kira, J

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   27   S8 - S9   2022.5   （ ISSN:1085-9489 eISSN:1529-8027 ）

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  • Deficiency of MTH1 and/or OGG1 increases the accumulation of 8-oxoguanine in the brain of the AppNL-G-F/NL-G-F knock-in mouse model of Alzheimer's disease, accompanied by accelerated microgliosis and reduced anxiety-like behavior. Reviewed International journal

    Yuri Mizuno, Nona Abolhassani, Guianfranco Mazzei, Takashi Saito, Takaomi C Saido, Ryo Yamasaki, Jun-Ichi Kira, Yusaku Nakabeppu

    Neuroscience research   177   118 - 134   2022.4   （ ISSN:0168-0102 eISSN:1872-8111 ）

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    Oxidative stress is a major risk factor for Alzheimer's disease (AD). Among various oxidized molecules, the marked accumulation of an oxidized form of guanine, 8-oxo-7,8-dihydroguanine (8-oxoG), is observed in the AD brain. 8-oxo-2'-deoxyguanosine triphosphatase (MTH1) and 8-oxoG DNA glycosylase (OGG1) minimize the 8-oxoG accumulation in DNA, and their expression is decreased in the AD brain. MTH1 and/or OGG1 may suppress the pathogenesis of AD; however, their exact roles remain unclear. We evaluated the roles of MTH1 and OGG1 during the pathogenesis of AD using AppNL-G-F/NL-G-F knock-in mice (a preclinical AD model). Six-month-old female AppNL-G-F/NL-G-F mice with MTH1 and/or OGG1 deficiency exhibited reduced anxiety-related behavior, but their cognitive and locomotive functions were unchanged; the alteration was less evident in 12-month-old mice. MTH1 and/or OGG1 deficiency accelerated the 8-oxoG accumulation and microgliosis in the amygdala and cortex of six-month-old mice; the alteration was less evident in 12-month-old mice. Astrocytes and neurons were not influenced. We showed that MTH1 and OGG1 are essential for minimizing oxidative DNA damage in the AppNL-G-F/NL-G-F brain, and the effects are age-dependent. MTH1 and/or OGG1 deficiency reduced anxiety-related behavior in AppNL-G-F/NL-G-F mice with a significant acceleration of the 8-oxoG burden and microgliosis, especially in the cortex and amygdala.

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  • SEMA3E-Plexin D1経路に着目した筋萎縮側索硬化症の病態解明

    藤井 敬之, 本田 裕之, 山崎 亮, 吉村 基, 岩城 徹, 磯部 紀子

    日本内科学会雑誌   111 ( Suppl. )   208 - 208   2022.2

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  • Facial onset sensory and motor neuronopathy(FOSMN)の全国臨床疫学調査結果

    江 千里, 山崎 亮, 奥井 佑, 白石 渉, 渡邉 充, 橋本 侑, 小早川 優子, 楠 進, 吉良 潤一, 磯部 紀子

    日本内科学会雑誌   111 ( Suppl. )   206 - 206   2022.2

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  • Facial onset sensory and motor neuronopathy(FOSMN)の全国臨床疫学調査結果

    江 千里, 山崎 亮, 奥井 佑, 白石 渉, 渡邉 充, 橋本 侑, 小早川 優子, 楠 進, 吉良 潤一, 磯部 紀子

    日本内科学会雑誌   111 ( Suppl. )   206 - 206   2022.2   （ ISSN:0021-5384 eISSN:1883-2083 ）

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  • SEMA3E-Plexin D1経路に着目した筋萎縮側索硬化症の病態解明

    藤井 敬之, 本田 裕之, 山崎 亮, 吉村 基, 岩城 徹, 磯部 紀子

    日本内科学会雑誌   111 ( Suppl. )   208 - 208   2022.2   （ ISSN:0021-5384 eISSN:1883-2083 ）

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  • Chronic progressive external ophthalmoplegia that could not be diagnosed by biceps muscle biopsy, but was genetically diagnosed by extraocular muscle biopsy Reviewed

    M.D. Ph.D. Shiraishi Wataru, M.D. Ph.D. Tateishi Takahisa, M.D. Hashimoto Yu, M.D. Ph.D. Yamasaki Ryo, M.D. Ph.D. Kira Jun-ichi, M.D. Ph.D. Isobe Noriko

    Rinsho Shinkeigaku   62 ( 12 )   946 - 951   2022   （ ISSN:0009918X eISSN:18820654 ）

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    Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：Societas Neurologica Japonica  

    <p>A 48-year-old Japanese male experienced slowly progressive diplopia. He had no family history and was negative for the edrophonium chloride test. Blood analysis showed elevated lactic acid and pyruvic acid levels, suggesting mitochondrial disease. A muscle biopsy from the biceps brachii was performed, but no pathological or genetical mitochondrial abnormalities were detected. Subsequently, he underwent muscle plication for diplopia in which the right inferior rectus muscle was biopsied. Genetic examination of genomic DNA extracted from the extraocular muscle tissue revealed multiple mitochondrial gene deletions, with a heteroplasmy rate of approximately 35%, resulting in the diagnosis of chronic progressive external ophthalmoplegia. In mitochondrial diseases, the tissue distribution of mitochondria with disease-associated variants in mtDNA should be noted, and it is important to select the affected muscle when performing a biopsy for an accurate diagnosis.</p>

    DOI： 10.5692/clinicalneurol.cn-001798

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  • 脊髄炎や脱髄と鑑別を要した脊髄硬膜動静脈瘻(SDAVF)の1例 Reviewed

    林田 仁志, 眞崎 勝久, 緒方 英紀, 山崎 亮, 磯部 紀子

    臨床神経学   61 ( 12 )   887 - 887   2021.12

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  • 抗NF155抗体陽性CIDPの寛解 無治療または少量ステロイド薬で神経症候・検査所見が改善した3症例の検討

    緒方 英紀, 川嵜 淳史, 山嵜 亮, 吉良 潤一, 磯部 紀子

    末梢神経   32 ( 2 )   256 - 256   2021.12

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  • くも膜下出血後の水頭症に対するLPシャント術後にパーキンソン症候群をきたした1例 Reviewed

    竹内 創, 眞崎 勝久, 緒方 英紀, 山崎 亮, 磯部 紀子

    臨床神経学   61 ( 12 )   889 - 889   2021.12

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  • IgG4抗LGI4抗体はランビエ絞輪juxta-paranodeを標的とするCIDPの新たな自己抗体である

    張 旭, 緒方 英紀, 今村 友裕, 藤井 敬之, 山嵜 亮, 磯部 紀子, 吉良 潤一

    末梢神経   32 ( 2 )   239 - 239   2021.12

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  • 脊髄C7、8前角障害により右手第3、4指下垂指を来した一例 Reviewed

    倉沢 亮, 眞崎 勝久, 松下 拓也, 松瀬 大, 山崎 亮, 磯部 紀子

    臨床神経学   61 ( 11 )   780 - 780   2021.11

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  • 抗PM/Scl-75抗体陽性で全身性強皮症に壊死性筋症を合併した一例 Reviewed

    津村 周, 前田 泰宏, 眞崎 勝久, 松下 拓也, 渡邉 充, 山崎 亮, 吉良 潤一, 磯部 紀子

    臨床神経学   61 ( 11 )   779 - 779   2021.11

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  • 間接蛍光抗体法による多発性硬化症患者血清中の抗オリゴデンドロサイト抗体の検討

    藤井 敬之, 宮地 佑希野, 山崎 亮, 土本 大介, 迫田 礼子, 福元 尚子, 松下 拓也, 中別府 雄作, 吉良 潤一, 磯部 紀子

    神経免疫学   26 ( 1 )   122 - 122   2021.10

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  • 脊髄炎と鑑別を要した、腰椎レベルにシャントのある脊髄硬膜動静脈瘻(SDAVF)4例の臨床症状・画像所見の検討

    林田 仁志, 眞崎 勝久, 緒方 英紀, 松瀬 大, 山崎 亮, 磯部 紀子

    神経免疫学   26 ( 1 )   127 - 127   2021.10

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  • 胸腺腫を合併した抗contactin-1抗体陽性ニューロパチー3例の臨床的特徴

    緒方 英紀, 山崎 亮, 西嶌 春生, 桑原 基, 磯部 紀子

    神経免疫学   26 ( 1 )   108 - 108   2021.10

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  • 胸腺腫を合併した抗contactin-1抗体陽性ニューロパチー3例の臨床的特徴

    緒方 英紀, 山崎 亮, 西嶌 春生, 桑原 基, 磯部 紀子

    神経免疫学   26 ( 1 )   108 - 108   2021.10

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  • 小脳型多系統萎縮症/一次進行型多発性硬化症モデルマウスの樹立とこれら疾患の新規創薬ターゲットの探索

    山口 浩雄, 松瀬 大, 眞崎 勝久, 雑賀 徹, 西村 由宇慈, 田中 辰典, 田中 謙二, 山崎 亮, 吉良 潤一, 磯部 紀子

    神経治療学   38 ( 6 )   S274 - S274   2021.10

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  • 多系統萎縮症の早期脱髄病巣における活性化ミクログリア・マクロファージとコネキシン蛋白群脱落

    眞崎 勝久, 西村 由宇慈, 松瀬 大, 山口 浩雄, 田中 辰典, 山崎 亮, 岩城 徹, 吉良 潤一, 磯部 紀子

    神経免疫学   26 ( 1 )   139 - 139   2021.10

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  • 多発性硬化症の疾患修飾薬使用下での再発に関連する遺伝因子

    松下 拓也, 田中 栄蔵, 渡邉 充, 福元 尚子, 山本 健, 山崎 亮, 吉良 潤一, 磯部 紀子

    神経免疫学   26 ( 1 )   112 - 112   2021.10

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  • アストログリアコネキシン43は慢性多発性硬化症モデルに対する新規治療標的である(Astroglial connexin 43 is a novel therapeutic target for chronic multiple sclerosis model)

    Ozdemir Ezgi, 山崎 亮, 永田 諭, 山口 浩雄, 真崎 勝久, 吉良 潤一, 竹内 英之, 磯部 紀子

    神経免疫学   26 ( 1 )   129 - 129   2021.10

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  • LGI4-IgG4はLGI4とADAM22の蛋白間相互作用を阻害して脱髄を起こすCIDPの新たなノド抗体である

    張 旭, 緒方 英紀, 今村 友裕, 藤井 敬之, グザリアイ・ママティジャン, 山崎 亮, 磯部 紀子, 吉良 潤一

    神経免疫学   26 ( 1 )   109 - 109   2021.10

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  • 筋萎縮性側索硬化症に対する治療法開発を推進するための新規分類法の策定

    小早川 優子, 戸高 浩司, 橋本 侑, 山崎 亮, 吉良 潤一

    臨床神経学   61 ( Suppl. )   S267 - S267   2021.9

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  • Chromosomally integrated HHV-6を伴った抗NMDA受容体脳炎の1例

    岩尾 和紀, 渡邉 充, 向野 隆彦, 藤井 敬之, 山崎 亮, 磯部 紀子

    26 ( 2 )   45 - 45   2021.9

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  • Serum Anti-oligodendrocyte Autoantibodies in Patients With Multiple Sclerosis Detected by a Tissue-Based Immunofluorescence Assay International journal

    Yukino Miyachi, Takayuki Fujii, Ryo Yamasaki, Daisuke Tsuchimoto, Kyoko Iinuma, Ayako Sakoda, Shoko Fukumoto, Takuya Matsushita, Katsuhisa Masaki, Noriko Isobe, Yusaku Nakabeppu, Jun-ichi Kira

    Frontiers in Neurology   12   681980 - 681980   2021.8

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    DOI： 10.3389/fneur.2021.681980

  • Rapidly spreading seizures arise from large-scale functional brain networks in focal epilepsy International journal

    Taira Uehara, Hiroshi Shigeto, Takahiko Mukaino, Jun Yokoyama, Toshiki Okadome, Ryo Yamasaki, Katsuya Ogata, Nobutaka Mukae, Ayumi Sakata, Shozo Tobimatsu, Jun-ichi Kira

    NEUROIMAGE   237   118104 - 118104   2021.8

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    DOI： 10.1016/j.neuroimage.2021.118104

  • PON1 Q192R is associated with high platelet reactivity with clopidogrel in patients undergoing elective neurointervention: A prospective single-center cohort study International journal

    Koji Tanaka, Shoji Matsumoto, Gulibahaer Ainiding, Ichiro Nakahara, Hidehisa Nishi, Tetsuya Hashimoto, Tsuyoshi Ohta, Nobutake Sadamasa, Ryota Ishibashi, Masanori Gomi, Makoto Saka, Haruka Miyata, Sadayoshi Watanabe, Takuya Okata, Kazutaka Sonoda, Junpei Koge, Kyoko M. Iinuma, Konosuke Furuta, Izumi Nagata, Keitaro Matsuo, Takuya Matsushita, Noriko Isobe, Ryo Yamasaki, Jun-ichi Kira

    PLOS ONE   16 ( 8 )   e0254067 - e0254067   2021.8

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    DOI： 10.1371/journal.pone.0254067

  • 新規小脳型多系統萎縮症モデルのCSF-1R阻害剤治療

    松瀬 大, 山口 浩雄, 眞崎 勝久, 西村 由宇慈, 田中 辰典, 雑賀 徹, 田中 謙二, 山崎 亮, 吉良 潤一, 磯部 紀子

    パーキンソン病・運動障害疾患コングレスプログラム・抄録集   15回   92 - 92   2021.7

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  • Early postnatal allergic airway inflammation induces dystrophic microglia leading to excitatory postsynaptic surplus and autism-like behavior International journal

    Ban yu Saitoh, Eizo Tanaka, Norio Yamamoto, Daan van Kruining, Kyoko Iinuma, Yuko Nakamuta, Hiroo Yamaguchi, Ryo Yamasaki, Koichiro Matsumoto, Jun ichi Kira

    Brain, Behavior, and Immunity   95   362 - 380   2021.7

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    DOI： 10.1016/j.bbi.2021.04.008

  • Antiplexin D1 Antibodies Relate to Small Fiber Neuropathy and Induce Neuropathic Pain in Animals. International journal

    Takayuki Fujii, Eun-Jae Lee, Yukino Miyachi, Ryo Yamasaki, Young-Min Lim, Kyoko Iinuma, Ayako Sakoda, Kwang-Kuk Kim, Jun-Ichi Kira

    Neurology(R) neuroimmunology & neuroinflammation   8 ( 5 )   2021.7

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    DOI： 10.1212/NXI.0000000000001028

  • 抗neurofascin155抗体陽性の中枢末梢連合脱髄症の1例

    山本 燎, 岩中 行己男, 橋本 智代, 足立 弘明, 岡田 和将, 緒方 英紀, 山崎 亮

    臨床神経学   61 ( 6 )   430 - 430   2021.6

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  • Brain gray matter astroglia-specific connexin 43 ablation attenuates spinal cord inflammatory demyelination. International journal

    Journal of neuroinflammation   18 ( 1 )   126 - 126   2021.6

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    DOI： 10.1186/s12974-021-02176-1

  • [Granulomatosis with Polyangiitis: Diagnosis and Treatment Strategies].

    Ryo Yamasaki

    Brain and nerve = Shinkei kenkyu no shinpo   73 ( 5 )   503 - 510   2021.5

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    DOI： 10.11477/mf.1416201792

  • [A case of brain tuberculoma resembling a malignant tumor].

    Wataru Shiraishi, Takahisa Tateishi, Kazutaka Sonoda, Ryo Yamasaki, Jun-Ich Kira

    Rinsho shinkeigaku = Clinical neurology   61 ( 4 )   253 - 257   2021.4

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    DOI： 10.5692/clinicalneurol.cn-001557

  • Serum IgG anti-GD1a antibody and mEGOS predict outcome in Guillain-Barré syndrome. International journal

    91 ( 12 )   1339 - 1342   2020.12

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    OBJECTIVE: Approximately 15%-20% of patients with Guillain-Barré syndrome (GBS) are unable to walk independently at 6 months from the onset of neurological symptom. The modified Erasmus GBS outcome score (mEGOS) has been reported as a prognostic tool.Herein we investigated the association between a poor outcome, inability to walk independently at 6 months and presence of antiganglioside antibodies. METHODS: The clinical and serological data of 177 patients with GBS were retrospectively collected in Japan to assess the associations between a poor outcome and serum IgG antibodies against each ganglioside (GM1, GD1a, GalNAc-GD1a, GQ1b and GT1a). In addition, we investigated whether the combination of mEGOS and serum IgG antibodies against gangliosides is useful in predicting a poor outcome. RESULTS: The patients with IgG anti-GD1a antibodies more frequently showed poor outcomes than those without these antibodies (9 (36%) of 25 vs 8 (6%) of 127 patients, p<0.001). Particularly, 80% showed a poor outcome when they had both serum IgG anti-GD1a antibody and a high mEGOS of ≥10 on day 7 of admission. CONCLUSIONS: The combination of serum IgG anti-GD1a antibodies and a high mEGOS could help in making a more accurate prognosis of patients than mEGOS alone, especially for predicting poor outcomes.

    DOI： 10.1136/jnnp-2020-323960

  • Early decrease in intermediate monocytes in peripheral blood is characteristic of multiple system atrophy-cerebellar type. International journal

    Dai Matsuse, Ryo Yamasaki, Guzailiayi Maimaitijiang, Hiroo Yamaguchi, Katsuhisa Masaki, Noriko Isobe, Takuya Matsushita, Jun-Ichi Kira

    Journal of neuroimmunology   349   577395 - 577395   2020.12

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    DOI： 10.1016/j.jneuroim.2020.577395

  • 筋萎縮性側索硬化症の予後を反映した新規分類法の策定

    小早川 優子, 戸高 浩司, 橋本 侑, 山崎 亮, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   8 ( 1 )   94 - 94   2020.11

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  • Mendiondoモデルによるアルツハイマー病治療薬の効果判定 機能的MRI研究

    山下 謙一郎, 上原 平, 菊池 一史, 栂尾 理, 樋渡 昭雄, 谷脇 予志秀, 山崎 亮, 吉良 潤一, 飛松 省三

    臨床神経学   60 ( Suppl. )   S401 - S401   2020.11

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  • Optic, trigeminal, and facial neuropathy related to anti-neurofascin 155 antibody. International journal

    Hidenori Ogata, Xu Zhang, Saeko Inamizu, Ken-Ichiro Yamashita, Ryo Yamasaki, Takuya Matsushita, Noriko Isobe, Akio Hiwatashi, Shozo Tobimatsu, Jun-Ichi Kira

    Annals of clinical and translational neurology   7 ( 11 )   2297 - 2309   2020.11

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    DOI： 10.1002/acn3.51220

  • Distinct microglial and macrophage distribution patterns in the concentric and lamellar lesions in Baló's disease and neuromyelitis optica spectrum disorders International journal

    Shotaro Hayashida, Katsuhisa Masaki, Satoshi O. Suzuki, Ryo Yamasaki, Mitsuru Watanabe, Sachiko Koyama, Noriko Isobe, Takuya Matsushita, Kazuya Takahashi, Takeshi Tabira, Toru Iwaki, Jun‐ichi Kira

    30 ( 6 )   1144 - 1157   2020.11

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    TMEM119 and purinergic receptor P2Y12 (P2RY12), which are not expressed by recruited peripheral blood macrophages, are proposed to discriminate microglia from macrophages. Therefore, we investigated the distribution patterns of microglia and macrophages in 10 concentric lesions from four autopsied Baló's disease cases and one neuromyelitis optica spectrum disorder (NMOSD) case, using quantitative immunohistochemistry for the markers TMEM119, P2RY12, CD68, CD163 and GLUT5. Three cases with Baló's disease had distal oligodendrogliopathy (DO) showing preferential loss of myelin-associated glycoprotein and early active demyelination in the outermost demyelinating layer (termed DMY-MO). In DMY-MO with DO, TMEM119-positive activated microglia expressing upregulated GLUT5 but markedly downregulated P2RY12 were significantly increased. These activated microglia expressed inducible nitric oxide synthase. Oligodendrocytes and their precursors showed apoptotic-like nuclear condensation in DMY-MO. TMEM119-negative and CD68/CD163-positive macrophages were distributed throughout the lesion center of DMY-MO with DO and these cells demonstrated foamy morphology only in the inner portion but not in the outer portion. In concentric demyelinating lesions from another Baló's case and lamellar demyelinating lesions in an NMOSD case, which had late active demyelination without DO, the densities of TMEM119-, GLUT5- and P2RY12-positive microglia with ramified morphology were significantly increased in myelinated layers but not in demyelinating layers. In particular, in the NMOSD case, TMEM119-positive microglia were confined to the outer portion of the myelinated layers. CD68-positive macrophages with foamy morphology also expressing CD163 accumulated in myelinated as well as in demyelinated layers. These findings suggest that activated microglia expressing TMEM119 and GLUT5, but not P2RY12, are associated with apoptosis of oligodendrocytes in the leading edge of Baló's concentric lesions with DO, whereas TMEM119-, GLUT5- and P2RY12-positive microglia with ramified morphology are associated with myelin preservation in concentric lesions without DO in Baló's disease and NMOSD. These two types of microglia appear to play distinct roles in the formation of concentric lesions.

    DOI： 10.1111/bpa.12898

  • 筋萎縮性側索硬化症の予後を反映した新規分類法の策定

    小早川 優子, 戸高 浩司, 橋本 侑, 山崎 亮, 吉良 潤一

    日本難病看護学会誌   25 ( 1 )   74 - 74   2020.10

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  • Immunotherapy-refractory vacuolar myopathy with mucin deposition in scleromyxedema: A possible role of fibroblast growth factor 2. International journal

    Yuki Yanagihara, Shintaro Hayashi, Junpei Koge, Hiroyuki Honda, Ryo Yamasaki, Yuichi Yamada, Yoshinao Oda, Toru Iwaki, Jun-Ichi Kira

    Neuropathology : official journal of the Japanese Society of Neuropathology   40 ( 5 )   492 - 495   2020.10

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    DOI： 10.1111/neup.12659

  • 発作時頻脈・発作時徐脈の双方を記録しえた一例

    岡留 敏樹, 向野 隆彦, 横山 淳, 藤井 敬之, 渡邉 恵利子, 酒田 あゆみ, 重藤 寛史, 山崎 亮

    てんかん研究   38 ( 2 )   181 - 181   2020.9

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  • Painful trigeminal neuropathy associated with anti-Plexin D1 antibody. Reviewed International journal

    Takayuki Fujii, Ryo Yamasaki, Yukino Miyachi, Kyoko Iinuma, Yu Hashimoto, Noriko Isobe, Takuya Matsushita, Jun-Ichi Kira

    Neurology(R) neuroimmunology & neuroinflammation   7 ( 5 )   2020.9

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    DOI： 10.1212/NXI.0000000000000819

  • Two susceptible HLA-DRB1 alleles for multiple sclerosis differentially regulate anti-JC virus antibody serostatus along with fingolimod. International journal

    Mitsuru Watanabe, Yuri Nakamura, Noriko Isobe, Masami Tanaka, Ayako Sakoda, Fumie Hayashi, Yuji Kawano, Ryo Yamasaki, Takuya Matsushita, Jun-Ichi Kira

    Journal of neuroinflammation   17 ( 1 )   206 - 206   2020.7

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    DOI： 10.1186/s12974-020-01865-7

  • Disconnection of the right superior parietal lobule from the precuneus is associated with memory impairment in oldest-old Alzheimer's disease patients. International journal

    Pukovisa Prawiroharjo, Ken-Ichiro Yamashita, Koji Yamashita, Osamu Togao, Akio Hiwatashi, Ryo Yamasaki, Jun-Ichi Kira

    Heliyon   6 ( 7 )   e04516   2020.7

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    DOI： 10.1016/j.heliyon.2020.e04516

  • Risk HLA-DRB1 alleles differentially influence brain and lesion volumes in Japanese patients with multiple sclerosis. Reviewed International journal

    Shoko Fukumoto, Yuri Nakamura, Mitsuru Watanabe, Noriko Isobe, Takuya Matsushita, Ayako Sakoda, Akio Hiwatashi, Koji Shinoda, Ryo Yamasaki, Akira Tsujino, Jun-Ichi Kira

    Journal of the neurological sciences   413   116768 - 116768   2020.6

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    DOI： 10.1016/j.jns.2020.116768

  • Risk HLA-DRB1 alleles differentially influence brain and lesion volumes in Japanese patients with multiple sclerosis Reviewed

    Shoko Fukumoto, Yuri Nakamura, Mitsuru Watanabe, Noriko Isobe, Takuya Matsushita, Ayako Sakoda, Akio Hiwatashi, Koji Shinoda, Ryo Yamasaki, Akira Tsujino, Jun ichi Kira

    Journal of the Neurological Sciences   413   2020.6

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    DOI： 10.1016/j.jns.2020.116768

  • Differences between predictive factors for early neurological deterioration due to hemorrhagic and ischemic insults following intravenous recombinant tissue plasminogen activator Reviewed

    Koji Tanaka, Shoji Matsumoto, Konosuke Furuta, Takeshi Yamada, Sukehisa Nagano, Kei ichiro Takase, Taketo Hatano, Ryo Yamasaki, Jun ichi Kira

    Journal of Thrombosis and Thrombolysis   49 ( 4 )   545 - 550   2020.5

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    DOI： 10.1007/s11239-019-02015-4

  • メキシレチンが著効したCLCN1変異(P480T)を伴うThomsen病の一例 Reviewed

    吉良 雄一, 田中 弘二, 緒方 英紀, 松瀬 大, 山崎 亮, 西野 一三, 吉良 潤一

    臨床神経学   60 ( 4 )   307 - 307   2020.4

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  • Insulin deficiency promotes formation of toxic amyloid-β42 conformer co-aggregating with hyper-phosphorylated tau oligomer in an Alzheimer's disease model. Reviewed International journal

    137   104739 - 104739   2020.4

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    The toxic conformer of amyloid β-protein (Aβ) ending at 42 (Aβ42), which contains a unique turn conformation at amino acid residue positions 22 and 23 and tends to form oligomers that are neurotoxic, was reported to play a critical role in the pathomechanisms of Alzheimer's disease (AD), in which diabetes mellitus (DM)-like mechanisms are also suggested to be operative. It remains to be established whether the attenuation of insulin signaling is involved in an increase of toxic Aβ42 conformer levels. The present study investigated the association between impaired insulin metabolism and formation of toxic Aβ42 conformers in the brains of an AD mouse model. In particular, we studied whether insulin deficiency or resistance affected the formation of toxic Aβ42 conformers in vivo. We induced insulin deficiency and resistance in 3xTg-AD mice, a mouse AD model harboring two familial AD-mutant APP (KM670/671NL) and PS1 (M146 V) genes and a mutant TAU (P301L) gene, by streptozotocin (STZ) injection and a high fructose diet (HFuD), respectively. Cognitive impairment was significantly worsened by STZ injection but not by HFuD. Dot blot analysis revealed significant increases in total Aβ42 levels and the ratio of toxic Aβ42 conformer/total Aβ42 in STZ-treated mice compared with control and HFuD-fed mice. Immunostaining showed the accumulation of toxic Aβ42 conformers and hyper-phosphorylated tau protein (p-tau), which was more prominent in the cortical and hippocampal neurons of STZ-treated mice compared with HFuD-fed and control mice. HFuD-fed mice showed only a mild-to-moderate increase of these proteins compared with controls. Toxic Aβ42 conformers were co-localized with p-tau oligomers (Pearson's correlation coefficient = 0.62) in the hippocampus, indicating their co-aggregation. Toxic Aβ42 conformer levels were inversely correlated with pancreatic insulin secretion capacity as shown by fasting immunoreactive insulin levels in STZ-treated mice (correlation coefficient = -0.5879, p = .04441), but not HFuD-fed mice, suggesting a decrease in serum insulin levels correlates with toxic Aβ42 conformer formation. Levels of p-Akt and phosphorylated glycogen synthase kinase-3β measured by a homogeneous time-resolved fluorescence assay were significantly lower in STZ-treated mice than in HFuD-fed mice, suggesting a greater inhibition of brain insulin signaling by STZ than HFuD, although both levels were significantly decreased in these groups compared with controls. Iba1-positive and NOS2-positive areas in the cortex and hippocampus were significantly increased in STZ-treated mice and to a lesser extent in HFuD-fed mice compared with controls. These findings suggest that insulin deficiency rather than insulin resistance and the resultant impairment of brain insulin signaling facilitates the formation of toxic Aβ42 conformer and its co-aggregation with p-tau oligomers, and that insulin deficiency is an important pathogenic factor in the progression of AD.

    DOI： 10.1016/j.nbd.2020.104739

  • Insulin deficiency promotes formation of toxic amyloid-β42 conformer co-aggregating with hyper-phosphorylated tau oligomer in an Alzheimer's disease model Reviewed

    137   2020.4

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    The toxic conformer of amyloid β-protein (Aβ) ending at 42 (Aβ42), which contains a unique turn conformation at amino acid residue positions 22 and 23 and tends to form oligomers that are neurotoxic, was reported to play a critical role in the pathomechanisms of Alzheimer's disease (AD), in which diabetes mellitus (DM)-like mechanisms are also suggested to be operative. It remains to be established whether the attenuation of insulin signaling is involved in an increase of toxic Aβ42 conformer levels. The present study investigated the association between impaired insulin metabolism and formation of toxic Aβ42 conformers in the brains of an AD mouse model. In particular, we studied whether insulin deficiency or resistance affected the formation of toxic Aβ42 conformers in vivo. We induced insulin deficiency and resistance in 3xTg-AD mice, a mouse AD model harboring two familial AD-mutant APP (KM670/671NL) and PS1 (M146 V) genes and a mutant TAU (P301L) gene, by streptozotocin (STZ) injection and a high fructose diet (HFuD), respectively. Cognitive impairment was significantly worsened by STZ injection but not by HFuD. Dot blot analysis revealed significant increases in total Aβ42 levels and the ratio of toxic Aβ42 conformer/total Aβ42 in STZ-treated mice compared with control and HFuD-fed mice. Immunostaining showed the accumulation of toxic Aβ42 conformers and hyper-phosphorylated tau protein (p-tau), which was more prominent in the cortical and hippocampal neurons of STZ-treated mice compared with HFuD-fed and control mice. HFuD-fed mice showed only a mild-to-moderate increase of these proteins compared with controls. Toxic Aβ42 conformers were co-localized with p-tau oligomers (Pearson's correlation coefficient = 0.62) in the hippocampus, indicating their co-aggregation. Toxic Aβ42 conformer levels were inversely correlated with pancreatic insulin secretion capacity as shown by fasting immunoreactive insulin levels in STZ-treated mice (correlation coefficient = −0.5879, p =.04441), but not HFuD-fed mice, suggesting a decrease in serum insulin levels correlates with toxic Aβ42 conformer formation. Levels of p-Akt and phosphorylated glycogen synthase kinase-3β measured by a homogeneous time-resolved fluorescence assay were significantly lower in STZ-treated mice than in HFuD-fed mice, suggesting a greater inhibition of brain insulin signaling by STZ than HFuD, although both levels were significantly decreased in these groups compared with controls. Iba1-positive and NOS2-positive areas in the cortex and hippocampus were significantly increased in STZ-treated mice and to a lesser extent in HFuD-fed mice compared with controls. These findings suggest that insulin deficiency rather than insulin resistance and the resultant impairment of brain insulin signaling facilitates the formation of toxic Aβ42 conformer and its co-aggregation with p-tau oligomers, and that insulin deficiency is an important pathogenic factor in the progression of AD.

    DOI： 10.1016/j.nbd.2020.104739

  • Double positivity for anti-N-methyl-d-aspartate receptor and anti-aquaporin-4 antibodies in a patient presenting with hypersomnolence, personality change, and reduced spontaneity

    Motoi Yoshimura, Taira Uehara, Eizo Tanaka, Toshiki Okadome, Takahiro Yamaguchi, Yasuhiro Maeda, Noriko Isobe, Takuya Matsushita, Ryo Yamasaki, Jun ichi Kira

    Clinical and Experimental Neuroimmunology   11 ( S1 )   53 - 56   2020.3

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    DOI： 10.1111/cen3.12571

  • Novel animal model of multiple sclerosis The glial connexin gap junction as an environmental tuner for neuroinflammation Reviewed

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   11 ( S1 )   34 - 40   2020.3

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    DOI： 10.1111/cen3.12568

  • Double positivity for anti-N-methyl-d-aspartate receptor and anti-aquaporin-4 antibodies in a patient presenting with hypersomnolence, personality change, and reduced spontaneity Reviewed

    Motoi Yoshimura, Taira Uehara, Eizo Tanaka, Toshiki Okadome, Takahiro Yamaguchi, Yasuhiro Maeda, Noriko Isobe, Takuya Matsushita, Ryo Yamasaki, Jun ichi Kira

    Clinical and Experimental Neuroimmunology   11 ( S1 )   53 - 56   2020.3

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    DOI： 10.1111/cen3.12571

  • Anti-plexin D1 antibody–mediated neuropathic pain Reviewed

    11 ( S1 )   48 - 52   2020.3

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    Neuropathic pain (NeP) is an intractable pain caused by a lesion or disease of the somatosensory nervous system. NeP is often challenging to manage because most of the mechanisms remain to be elucidated. Recent investigations in the field of autoimmune neurology have demonstrated that specific autoantibodies against antigens in the somatosensory pathway can cause NeP. Detection of pathogenic autoantibodies in NeP adds to the understanding of the mechanism of pain, which might aid in the development of novel immunotherapies. Therefore, it is necessary to explore novel NeP-related autoantibodies to improve the management of intractable pain. Recently, we screened serum autoantibodies that bound to pain-conducting small dorsal root ganglion (DRG) neurons and their nerve terminals in the dorsal horns of NeP patients. We detected a novel autoantibody that bound to unmyelinated C-fiber–type small DRG neurons. The positive rate in patients with NeP was 10%. We identified plexin D1 as the target antigen. NeP patients with plexin D1-IgG developed burning pain and thermal hyperalgesia. The main comorbidities were allergy, collagen vascular disease, and cancer. Plasma exchange and intravenous methylprednisolone pulse therapy are effective for NeP in patients with plexin D1-IgG, indicating that these autoantibodies might be pathogenic in NeP. Indeed, our in vitro study demonstrated that plexin D1-IgG induced the membrane hyperpermeability of DRG neurons. In this review, we describe the discovery of plexin D1-IgG and discuss the association between plexin D1 and pain, allergy, and cancer.

    DOI： 10.1111/cen3.12570

  • Unique HLA haplotype associations in IgG4 anti-neurofascin 155 antibody-positive chronic inflammatory demyelinating polyneuropathy. Reviewed International journal

    Hidenori Ogata, Noriko Isobe, Xu Zhang, Ryo Yamasaki, Takayuki Fujii, Akira Machida, Nobutoshi Morimoto, Kenichi Kaida, Teruaki Masuda, Yukio Ando, Motoi Kuwahara, Susumu Kusunoki, Yuri Nakamura, Takuya Matsushita, Jun-Ichi Kira

    Journal of neuroimmunology   339   577139 - 577139   2020.2

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    DOI： 10.1016/j.jneuroim.2019.577139

  • The effects of chronic subthalamic stimulation on nonmotor symptoms in advanced Parkinson's disease, revealed by an online questionnaire program. Reviewed International journal

    Minako Kawaguchi, Kazuhiro Samura, Yasushi Miyagi, Tsuyoshi Okamoto, Ryo Yamasaki, Nobutaka Sakae, Fumiaki Yoshida, Koji Iihara

    Acta neurochirurgica   162 ( 2 )   247 - 255   2020.2

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    DOI： 10.1007/s00701-019-04182-y

  • Corrigendum to "Higher postictal parasympathetic activity following greater ictal heart rate increase in right- than left-sided seizures" [Epilepsy Behav 97 (2019) 161-168]. Reviewed International journal

    Mitsunori Shimmura, Taira Uehara, Katsuya Ogata, Hiroshi Shigeto, Tomoko Maeda, Ayumi Sakata, Ryo Yamasaki, Jun-Ichi Kira

    Epilepsy & behavior : E&B   103 ( Pt A )   106865 - 106865   2020.2

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    DOI： 10.1016/j.yebeh.2019.106865

  • Central nervous system-specific antinuclear antibodies in patients with multiple sclerosis. Reviewed International journal

    Takayuki Fujii, Ryo Yamasaki, Yukino Miyachi, Satoshi Nagata, Guzailiayi Maimaitijiang, Yuri Nakamura, Koji Shinoda, Takuya Matsushita, Noriko Isobe, Jun-Ichi Kira

    Journal of the neurological sciences   409   116619 - 116619   2020.2

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    DOI： 10.1016/j.jns.2019.116619

  • A case of overlapping adult-onset linear scleroderma and Parry-Romberg syndrome presenting with widespread ipsilateral neurogenic involvement. Reviewed International journal

    Ryo Yamasaki, Tomomi Yonekawa, Saeko Inamizu, Koji Shinoda, Hirofumi Ochi, Takuya Matsushita, Noriko Isobe, Gaku Tsuji, Shoko Sadashima, Yuki Kuma, Yoshinao Oda, Toru Iwaki, Masutaka Furue, Jun-Ichi Kira

    Neuropathology : official journal of the Japanese Society of Neuropathology   40 ( 1 )   109 - 115   2020.2

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    DOI： 10.1111/neup.12614

  • Unique HLA haplotype associations in IgG4 anti-neurofascin 155 antibody-positive chronic inflammatory demyelinating polyneuropathy Reviewed

    Hidenori Ogata, Noriko Isobe, Xu Zhang, Ryo Yamasaki, Takayuki Fujii, Akira Machida, Nobutoshi Morimoto, Kenichi Kaida, Teruaki Masuda, Yukio Ando, Motoi Kuwahara, Susumu Kusunoki, Yuri Nakamura, Takuya Matsushita, Jun ichi Kira

    Journal of Neuroimmunology   339   2020.2

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    DOI： 10.1016/j.jneuroim.2019.577139

  • The effects of chronic subthalamic stimulation on nonmotor symptoms in advanced Parkinson’s disease, revealed by an online questionnaire program Reviewed

    162 ( 2 )   247 - 255   2020.2

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    Background: This study was designed to detect and assess the frequency and severity of nonmotor symptoms (NMSs) in advanced Parkinson’s disease (PD) and to investigate the effects of subthalamic nucleus deep brain stimulation (STN-DBS) on NMSs. Methods: We developed an online PC–based questionnaire program to assess NMSs in PD. Twenty-six PD patients who underwent bilateral STN-DBS were assessed. The NMS questionnaire consisted of 54 NMSs in three categories, based on Witjas et al. (2002). For each NMS, the patients were asked whether or not it was present, whether or not the fluctuating manifestations correlated with the timing of levodopa-induced motor fluctuations, and how severe the NMS was. Patients were assessed by this system before surgery and at the follow-up visit, 3 to 6 months after surgery. At the postoperative assessment, patients were also assessed on preoperative NMSs using recall. Results: The most frequent preoperative NMSs were constipation and visual disorders, while the most frequent postoperative NMSs were difficulty in memorizing and pollakiuria. The ranking of most frequent NMSs changed from before to after surgery. NMSs of drenching sweats, dysphagia, and constipation were significantly ameliorated, while NMSs of dyspnea and slowness of thinking were significantly deteriorated after surgery. The preoperative assessment by postoperative recall gave very different results from that of the preoperative assessment. Conclusion: An online questionnaire system to assess NMSs in patients with advanced PD suggested that STN-DBS might influence the frequencies of some kinds of NMSs.

    DOI： 10.1007/s00701-019-04182-y

  • Environmental risk factors for multiple sclerosis in Japanese people Reviewed

    Ayako Sakoda, Takuya Matsushita, Yuri Nakamura, Mitsuru Watanabe, Koji Shinoda, Katsuhisa Masaki, Noriko Isobe, Ryo Yamasaki, Jun ichi Kira

    Multiple Sclerosis and Related Disorders   38   2020.2

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    DOI： 10.1016/j.msard.2019.101872

  • Corrigendum to “Higher postictal parasympathetic activity following greater ictal heart rate increase in right- than left-sided seizures” (Epilepsy & Behavior (2019) 97 (161–168), (S1525505019300174), (10.1016/j.yebeh.2019.05.026)) Reviewed

    103   2020.2

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    The authors regret that the legend of Fig. 4 in the above article contained errors. The sample sizes for right-sided seizures (n = 27) and left-sided seizures (n = 27) are wrong. The corrected sentence is as follows: Red lines indicate right-sided seizures (n = 24), and blue lines indicate left-sided seizures (n = 24). This is a simple typographical error and does not change the conclusions of the paper. The authors would like to apologize for any inconvenience caused.

    DOI： 10.1016/j.yebeh.2019.106865

  • Central nervous system-specific antinuclear antibodies in patients with multiple sclerosis Reviewed

    Takayuki Fujii, Ryo Yamasaki, Yukino Miyachi, Satoshi Nagata, Guzailiayi Maimaitijiang, Yuri Nakamura, Koji Shinoda, Takuya Matsushita, Noriko Isobe, Jun ichi Kira

    Journal of the Neurological Sciences   409   2020.2

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    DOI： 10.1016/j.jns.2019.116619

  • A case of overlapping adult-onset linear scleroderma and Parry-Romberg syndrome presenting with widespread ipsilateral neurogenic involvement Reviewed

    Ryo Yamasaki, Tomomi Yonekawa, Saeko Inamizu, Koji Shinoda, Hirofumi Ochi, Takuya Matsushita, Noriko Isobe, Gaku Tsuji, Shoko Sadashima, Yuki Kuma, Yoshinao Oda, Toru Iwaki, Masutaka Furue, Jun ichi Kira

    Neuropathology   40 ( 1 )   109 - 115   2020.2

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    DOI： 10.1111/neup.12614

  • Branchial myorhythmia in a case of systemic lupus erythematosus Reviewed

    Hayato Une, Dai Matsuse, Taira Uehara, Yoshikazu Kikuchi, Saeko Inamizu, Ryo Yamasaki, Shozo Tobimatsu, Hiroshi Shibasaki, Jun ichi Kira

    Journal of the Neurological Sciences   408   2020.1

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    DOI： 10.1016/j.jns.2019.116501

  • Oligodendroglial connexin 47 regulates neuroinflammation upon autoimmune demyelination in a novel mouse model of multiple sclerosis. Reviewed International journal

    Yinan Zhao, Ryo Yamasaki, Hiroo Yamaguchi, Satoshi Nagata, Hayato Une, Yiwen Cui, Katsuhisa Masaki, Yuko Nakamuta, Kyoko Iinuma, Mitsuru Watanabe, Takuya Matsushita, Noriko Isobe, Jun-Ichi Kira

    Proceedings of the National Academy of Sciences of the United States of America   117 ( 4 )   2160 - 2169   2020.1

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    DOI： 10.1073/pnas.1901294117

  • Branchial myorhythmia in a case of systemic lupus erythematosus. Reviewed International journal

    Hayato Une, Dai Matsuse, Taira Uehara, Yoshikazu Kikuchi, Saeko Inamizu, Ryo Yamasaki, Shozo Tobimatsu, Hiroshi Shibasaki, Jun-Ichi Kira

    Journal of the neurological sciences   408   116501 - 116501   2020.1

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    DOI： 10.1016/j.jns.2019.116501

  • Spinal cord involvement by atrophy and associations with disability are different between multiple sclerosis and neuromyelitis optica spectrum disorder Reviewed

    Y. Nakamura, Z. Liu, S. Fukumoto, K. Shinoda, A. Sakoda, T. Matsushita, S. Hayashida, N. Isobe, M. Watanabe, A. Hiwatashi, R. Yamasaki, J. i. Kira

    European Journal of Neurology   27 ( 1 )   92 - 99   2020.1

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    DOI： 10.1111/ene.14038

  • Oligodendroglial connexin 47 regulates neuroinflammation upon autoimmune demyelination in a novel mouse model of multiple sclerosis Reviewed

    Yinan Zhao, Ryo Yamasaki, Hiroo Yamaguchi, Satoshi Nagata, Hayato Une, Yiwen Cui, Katsuhisa Masaki, Yuko Nakamuta, Kyoko Iinuma, Mitsuru Watanabe, Takuya Matsushita, Noriko Isobe, Jun Ichi Kira

    Proceedings of the National Academy of Sciences of the United States of America   117 ( 4 )   2160 - 2169   2020.1

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    DOI： 10.1073/pnas.1901294117

  • Immunotherapy-refractory vacuolar myopathy with mucin deposition in scleromyxedema A possible role of fibroblast growth factor 2 Reviewed

    Yuki Yanagihara, Shintaro Hayashi, Junpei Koge, Hiroyuki Honda, Ryo Yamasaki, Yuichi Yamada, Yoshinao Oda, Toru Iwaki, Jun ichi Kira

    Neuropathology   2020.1

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    DOI： 10.1111/neup.12659

  • Novel Neuropathic Pain Mechanisms Associated With Allergic Inflammation Reviewed

    Takayuki Fujii, Ryo Yamasaki, Jun Ichi Kira

    Frontiers in Neurology   10   2019.12

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    DOI： 10.3389/fneur.2019.01337

  • 筋萎縮性側索硬化症モデルマウス末梢神経に蓄積するミスフォールド蛋白の末梢血マクロファージによる除去を介した神経保護作用の解明

    白石 渉, 山崎 亮, 小早川 優子, 吉良 潤一

    末梢神経   30 ( 2 )   291 - 291   2019.12

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  • 延髄病変に伴い急性心不全をきたした多発性硬化症の一例

    原 佑碩, 稲水 佐江子, 緒方 英紀, 松下 拓也, 山崎 亮, 吉良 潤一

    臨床神経学   59 ( 12 )   855 - 855   2019.12

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  • 大動脈弁置換術後にchoreaと精神症状を呈し、シデナム舞踏病が疑われた一例

    小俵 響子, 岡留 敏樹, 向野 隆彦, 横山 淳, 横山 拓, 田中 弘二, 上原 平, 松瀬 大, 山崎 亮, 飛松 省三, 吉良 潤一

    臨床神経学   59 ( 12 )   852 - 852   2019.12

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  • Differences between predictive factors for early neurological deterioration due to hemorrhagic and ischemic insults following intravenous recombinant tissue plasminogen activator. Reviewed International journal

    Koji Tanaka, Shoji Matsumoto, Konosuke Furuta, Takeshi Yamada, Sukehisa Nagano, Kei-Ichiro Takase, Taketo Hatano, Ryo Yamasaki, Jun-Ichi Kira

    Journal of thrombosis and thrombolysis   49 ( 4 )   545 - 550   2019.12

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    DOI： 10.1007/s11239-019-02015-4

  • ALSに対する臨床試験の対象患者群の設定に関する現状と問題点

    小早川 優子, 戸高 浩司, 白石 渉, 山崎 亮, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   7 ( 1 )   138 - 138   2019.11

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  • 抗plexin D1抗体測定系の確立と髄液サイトカインの網羅的解析

    藤井 敬之, 山崎 亮, 宮地 佑希野, 飯沼 今日子, Zhang Xu, 吉良 潤一

    日本自律神経学会総会プログラム・抄録集   72回   100 - 100   2019.11

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  • 急性期虚血性脳卒中におけるCTとDWIの撮影時間差はCT、DWI各ASPECTS間の差と関連する

    田中 弘二, 古田 興之介, 松本 省二, 山田 猛, 長野 祐久, 高瀬 敬一郎, 波多野 武人, 山崎 亮, 吉良 潤一

    臨床神経学   59 ( Suppl. )   S292 - S292   2019.11

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  • バロー病と視神経脊髄炎の層状脱髄巣における特異的なTMEM119陽性ミクログリアの分布

    眞崎 勝久, 林田 翔太郎, 鈴木 諭, 山崎 亮, 松下 拓也, 高橋 和也, 田平 武, 岩城 徹, 吉良 潤一

    臨床神経学   59 ( Suppl. )   S308 - S308   2019.11

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  • Temporal Trends in Clinical Characteristics and Door-to-Needle Time in Patients Receiving Intravenous Tissue Plasminogen Activator: A Retrospective Study of 4 Hospitals in Japan. Reviewed International journal

    Koji Tanaka, Shoji Matsumoto, Takeshi Yamada, Sukehisa Nagano, Kei-Ichiro Takase, Taketo Hatano, Ryo Yamasaki, Jun-Ichi Kira

    Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association   28 ( 11 )   104305 - 104305   2019.11

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    DOI： 10.1016/j.jstrokecerebrovasdis.2019.104305

  • Modified diffusion-weighted imaging-Alberta Stroke Program Early Computed Tomography Score including deep white matter lesions predicts symptomatic intracerebral hemorrhage following intravenous thrombolysis. Reviewed International journal

    Koji Tanaka, Shoji Matsumoto, Konosuke Furuta, Takeshi Yamada, Sukehisa Nagano, Kei-Ichiro Takase, Taketo Hatano, Ryo Yamasaki, Jun-Ichi Kira

    Journal of thrombosis and thrombolysis   50 ( 1 )   174 - 180   2019.11

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    DOI： 10.1007/s11239-019-01979-7

  • Intrathecal cytokine profile in neuropathy with anti-neurofascin 155 antibody. Reviewed International journal

    Hidenori Ogata, Xu Zhang, Ryo Yamasaki, Takayuki Fujii, Akira Machida, Nobutoshi Morimoto, Kenichi Kaida, Teruaki Masuda, Yukio Ando, Motoi Kuwahara, Susumu Kusunoki, Yuri Nakamura, Takuya Matsushita, Noriko Isobe, Jun-Ichi Kira

    Annals of clinical and translational neurology   6 ( 11 )   2304 - 2316   2019.11

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    DOI： 10.1002/acn3.50931

  • Environmental risk factors for multiple sclerosis in Japanese people. Reviewed International journal

    Ayako Sakoda, Takuya Matsushita, Yuri Nakamura, Mitsuru Watanabe, Koji Shinoda, Katsuhisa Masaki, Noriko Isobe, Ryo Yamasaki, Jun-Ichi Kira

    Multiple sclerosis and related disorders   38   101872 - 101872   2019.11

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    DOI： 10.1016/j.msard.2019.101872

  • Temporal Trends in Clinical Characteristics and Door-to-Needle Time in Patients Receiving Intravenous Tissue Plasminogen Activator A Retrospective Study of 4 Hospitals in Japan Reviewed

    Koji Tanaka, Shoji Matsumoto, Takeshi Yamada, Sukehisa Nagano, Kei ichiro Takase, Taketo Hatano, Ryo Yamasaki, Jun ichi Kira

    Journal of Stroke and Cerebrovascular Diseases   28 ( 11 )   2019.11

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    DOI： 10.1016/j.jstrokecerebrovasdis.2019.104305

  • Intrathecal cytokine profile in neuropathy with anti-neurofascin 155 antibody Reviewed

    Hidenori Ogata, Xu Zhang, Ryo Yamasaki, Takayuki Fujii, Akira Machida, Nobutoshi Morimoto, Kenichi Kaida, Teruaki Masuda, Yukio Ando, Motoi Kuwahara, Susumu Kusunoki, Yuri Nakamura, Takuya Matsushita, Noriko Isobe, Jun ichi Kira

    Annals of Clinical and Translational Neurology   6 ( 11 )   2304 - 2316   2019.11

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    DOI： 10.1002/acn3.50931

  • Current understanding of autoimmune encephalitis and encephalopathy Reviewed

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   10 ( 4 )   209 - 210   2019.11

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    DOI： 10.1111/cen3.12544

  • Brainstem posterior reversible encephalopathy syndrome in a case with Guillain–Barré syndrome Reviewed

    10 ( 4 )   267 - 271   2019.11

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    Background: Posterior reversible encephalopathy syndrome (PRES) is characterized by reversible vasogenic brain edema on magnetic resonance imaging. PRES is frequently associated with blood pressure (BP) fluctuation. Although Guillain–Barré syndrome (GBS) is often complicated by BP fluctuation, PRES is rarely reported. Here, we describe the first reported case of GBS in a patient who developed PRES mainly affecting the brainstem. Case presentation: A 43-year-old man presented with impaired consciousness and was hospitalized after a diagnosis of infectious meningoencephalitis. His consciousness was improved by treatment; however, he presented with polyneuropathy and BP fluctuation. We diagnosed him with GBS and started intravenous immunoglobulin therapy (IVIg); however, his consciousness became impaired again after IVIg. Brain magnetic resonance imaging showed hyperintense areas of the pons, cerebellar peduncle, midbrain and basal ganglia in the apparent diffusion coefficient image and the fluid-attenuated inversion recovery image. Diffusion-weighted imaging showed that hyperintense and hypointense lesions were present within the same regions. We diagnosed brainstem PRES complicated with GBS. Achievement of BP control improved his consciousness and hyperintense lesions on the diffusion-weighted imaging and apparent diffusion coefficient image map. Conclusions: BP fluctuation and IVIg might have caused PRES in the present case. Neurologists should consider PRES as a differential diagnosis when consciousness is impaired in GBS, especially at the time of IVIg therapy or BP fluctuation.

    DOI： 10.1111/cen3.12530

  • Anti-neurofascin autoantibody and demyelination Reviewed

    Jun ichi Kira, Ryo Yamasaki, Hidenori Ogata

    Neurochemistry International   130   2019.11

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    DOI： 10.1016/j.neuint.2018.12.011

  • Intractable axonal neuropathy with multifocal peripheral nerve swelling in neuromyelitis optica spectrum disorders A case report Reviewed

    Yuri Mizuno, Koji Shinoda, Mitsuru Watanabe, Hidenori Ogata, Noriko Isobe, Takuya Matsushita, Ryo Yamasaki, Kimihiro Tanaka, Haruki Koike, Masahisa Katsuno, Jun ichi Kira

    Multiple Sclerosis and Related Disorders   35   16 - 18   2019.10

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    DOI： 10.1016/j.msard.2019.06.033

  • 眼窩上神経の高度腫大に起因する複視が示唆された抗NF155抗体陽性CIDPの一例

    吉良 雄一, 劉 景晨, 松本 英展, 高柳 宏樹, 緒方 英紀, 松田 やよい, 坂本 竜一, 田中 弘二, 松瀬 大, 山崎 亮, 小川 佳宏, 吉良 潤一

    臨床神経学   59 ( 10 )   685 - 685   2019.10

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  • 抗Plexin D1抗体が陽性であった長期経過のFOSMN症候群の一例

    橋本 侑, 田代 匠, 藤井 敬之, 宮地 佑希野, 田中 弘二, 松瀬 大, 山崎 亮, 吉良 潤一

    臨床神経学   59 ( 10 )   685 - 685   2019.10

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  • Possible regulatory roles of miRNAs in APP-KI AD model mice(和訳中)

    今村 友裕, 浅井 宏英, 山崎 亮, 吉良 潤一

    33 ( 4 )   551 - 551   2019.10

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  • Intractable axonal neuropathy with multifocal peripheral nerve swelling in neuromyelitis optica spectrum disorders: A case report. Reviewed International journal

    Yuri Mizuno, Koji Shinoda, Mitsuru Watanabe, Hidenori Ogata, Noriko Isobe, Takuya Matsushita, Ryo Yamasaki, Kimihiro Tanaka, Haruki Koike, Masahisa Katsuno, Jun-Ichi Kira

    Multiple sclerosis and related disorders   35   16 - 18   2019.10

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    DOI： 10.1016/j.msard.2019.06.033

  • 基礎研究 筋萎縮性側索硬化症の脊髄白質に出現するgalectin-3陽性ミクログリアはTDP-43病理と正相関する

    林 信太郎, 山崎 亮, 岡本 幸市, 吉良 潤一

    神経免疫学   24 ( 1 )   122 - 122   2019.9

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  • 基礎研究 オリゴデンドロサイト特異的αシヌクレイン蓄積による小脳型多系統萎縮症・一次進行型多発性硬化症モデル

    眞崎 勝久, 松瀬 大, 山口 浩雄, 雑賀 徹, 西村 由宇慈, 田中 辰典, 渡邉 充, 田中 謙二, 山崎 亮, 吉良 潤一

    神経免疫学   24 ( 1 )   124 - 124   2019.9

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  • 基礎研究 Cx47は神経炎を調節する 二次性進行型多発性硬化症モデルの作製(Basic research Cx47 regulates neuro-inflammation: creation of a 1 secondary-progressive multiple sclerosis model)

    趙 奕楠, 山崎 亮, 山口 浩雄, 吉良 潤一

    神経免疫学   24 ( 1 )   123 - 123   2019.9

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  • Serum GFAP and neurofilament light as biomarkers of disease activity and disability in NMOSD. Reviewed International journal

    Mitsuru Watanabe, Yuri Nakamura, Zuzanna Michalak, Noriko Isobe, Christian Barro, David Leppert, Takuya Matsushita, Fumie Hayashi, Ryo Yamasaki, Jens Kuhle, Jun-Ichi Kira

    Neurology   93 ( 13 )   e1299-e1311   2019.9

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    DOI： 10.1212/WNL.0000000000008160

  • Cathepsin E in neutrophils contributes to the generation of neuropathic pain in experimental autoimmune encephalomyelitis. Reviewed International journal

    Yuka Harada, Jing Zhang, Kazuhisa Imari, Ryo Yamasaki, Junjun Ni, Zhou Wu, Kenji Yamamoto, Jun-Ichi Kira, Hiroshi Nakanishi, Yoshinori Hayashi

    Pain   160 ( 9 )   2050 - 2062   2019.9

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    DOI： 10.1097/j.pain.0000000000001596

  • Serum GFAP and neurofilament light as biomarkers of disease activity and disability in NMOSD Reviewed

    Mitsuru Watanabe, Yuri Nakamura, Zuzanna Michalak, Noriko Isobe, Christian Barro, David Leppert, Takuya Matsushita, Fumie Hayashi, Ryo Yamasaki, Jens Kuhle, Jun Ichi Kira

    Neurology   93 ( 13 )   E1299 - E1311   2019.9

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    DOI： 10.1212/WNL.0000000000008160

  • Cathepsin E in neutrophils contributes to the generation of neuropathic pain in experimental autoimmune encephalomyelitis Reviewed

    Yuka Harada, Jing Zhang, Kazuhisa Imari, Ryo Yamasaki, Junjun Ni, Zhou Wu, Kenji Yamamoto, Jun Ichi Kira, Hiroshi Nakanishi, Yoshinori Hayashi

    Pain   160 ( 9 )   2050 - 2062   2019.9

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    DOI： 10.1097/j.pain.0000000000001596

  • 基礎研究 脳皮質アストログリアはコネキシン43を介して遠隔性に脊髄炎症を制御している

    山崎 亮, 宇根 隼人, インディアサリ・ウルファカメリア, 永田 諭, 山口 浩雄, 吉良 潤一

    神経免疫学   24 ( 1 )   125 - 125   2019.9

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  • 末梢神経・筋 抗plexin D1抗体測定系の確立と疾患スペクトラムの実態解明

    藤井 敬之, 山崎 亮, 宮地 佑希野, 飯沼 今日子, 吉良 潤一

    神経免疫学   24 ( 1 )   111 - 111   2019.9

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  • 抗neurofascin 155抗体関連ニューロパチー全国調査結果

    緒方 英紀, 山崎 亮, 磯部 紀子, 角谷 真人, 海田 賢一, 松井 真, 桑原 聡, 楠 進, 吉良 潤一

    神経免疫学   24 ( 1 )   92 - 92   2019.9

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  • Higher postictal parasympathetic activity following greater ictal heart rate increase in right- than left-sided seizures Reviewed

    Mitsunori Shimmura, Taira Uehara, Katsuya Ogata, Hiroshi Shigeto, Tomoko Maeda, Ayumi Sakata, Ryo Yamasaki, Jun ichi Kira

    Epilepsy and Behavior   97   161 - 168   2019.8

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    DOI： 10.1016/j.yebeh.2019.05.026

  • 視神経脊髄炎の再発と鑑別を要したEBV脊髄神経根炎の一例

    松本 航, 中村 優理, 雑賀 徹, 松下 拓也, 山崎 亮, 吉良 潤一

    臨床神経学   59 ( 8 )   553 - 553   2019.8

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  • シェーグレン症候群による三叉神経障害と鑑別を要したFOSMN症候群の1例

    居積 晃希, 松下 拓也, 雑賀 徹, 渡邉 充, 山崎 亮, 吉良 潤一

    臨床神経学   59 ( 8 )   550 - 550   2019.8

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  • Multiple mtDNA deletions due to mitochondrion toxicity of anti-hepadnaviral drugs: Comments to the letter from J. Finsterer. Reviewed International journal

    Takayuki Fujii, Hiroyuki Honda, Ryo Yamasaki, Toru Iwaki, Jun-Ichi Kira

    Neuropathology : official journal of the Japanese Society of Neuropathology   39 ( 4 )   326 - 327   2019.8

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    DOI： 10.1111/neup.12563

  • Higher postictal parasympathetic activity following greater ictal heart rate increase in right- than left-sided seizures. Reviewed International journal

    Mitsunori Shimmura, Taira Uehara, Katsuya Ogata, Hiroshi Shigeto, Tomoko Maeda, Ayumi Sakata, Ryo Yamasaki, Jun-Ichi Kira

    Epilepsy & behavior : E&B   97   161 - 168   2019.8

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    DOI： 10.1016/j.yebeh.2019.05.026

  • Discriminative clinical and neuroimaging features of motor-predominant hereditary diffuse leukoencephalopathy with axonal spheroids and primary progressive multiple sclerosis A preliminary cross-sectional study Reviewed

    Ban yu Saitoh, Ryo Yamasaki, Akio Hiwatashi, Takuya Matsushita, Shintaro Hayashi, Yoshihiro Mitsunaga, Yasuhiro Maeda, Noriko Isobe, Kunihiro Yoshida, Shu ichi Ikeda, Jun ichi Kira

    Multiple Sclerosis and Related Disorders   31   22 - 31   2019.6

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    DOI： 10.1016/j.msard.2019.03.008

  • Novel pathogenic XK mutations in McLeod syndrome and interaction between XK protein and chorein. Reviewed International journal

    Yuka Urata, Masayuki Nakamura, Natsuki Sasaki, Nari Shiokawa, Yoshiaki Nishida, Kaoru Arai, Hanae Hiwatashi, Izumi Yokoyama, Shinsuke Narumi, Yasuo Terayama, Takenobu Murakami, Yoshikazu Ugawa, Hiroki Sakamoto, Satoshi Kaneko, Yusuke Nakazawa, Ryo Yamasaki, Shoko Sadashima, Toshiaki Sakai, Hiroaki Arai, Akira Sano

    Neurology. Genetics   5 ( 3 )   e328   2019.6

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    DOI： 10.1212/NXG.0000000000000328

  • Discriminative clinical and neuroimaging features of motor-predominant hereditary diffuse leukoencephalopathy with axonal spheroids and primary progressive multiple sclerosis: A preliminary cross-sectional study. Reviewed International journal

    Ban-Yu Saitoh, Ryo Yamasaki, Akio Hiwatashi, Takuya Matsushita, Shintaro Hayashi, Yoshihiro Mitsunaga, Yasuhiro Maeda, Noriko Isobe, Kunihiro Yoshida, Shu-Ichi Ikeda, Jun-Ichi Kira

    Multiple sclerosis and related disorders   31   22 - 31   2019.6

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    DOI： 10.1016/j.msard.2019.03.008

  • Novel pathogenic XK mutations in McLeod syndrome and interaction between XK protein and chorein Reviewed

    Yuka Urata, Masayuki Nakamura, Natsuki Sasaki, Nari Shiokawa, Yoshiaki Nishida, Kaoru Arai, Hanae Hiwatashi, Izumi Yokoyama, Shinsuke Narumi, Yasuo Terayama, Takenobu Murakami, Yoshikazu Ugawa, Hiroki Sakamoto, Satoshi Kaneko, Yusuke Nakazawa, Ryo Yamasaki, Shoko Sadashima, Toshiaki Sakai, Hiroaki Arai, Akira Sano

    Neurology: Genetics   5 ( 3 )   2019.6

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    DOI： 10.1212/NXG.0000000000000328

  • Cerebrospinal fluid cytokine/chemokine/growth factor profiles in idiopathic hypertrophic pachymeningitis Reviewed

    Xu Zhang, Takayuki Fujii, Hidenori Ogata, Ryo Yamasaki, Katsuhisa Masaki, Yiwen Cui, Takuya Matsushita, Noriko Isobe, Jun ichi Kira

    Journal of Neuroimmunology   330   38 - 43   2019.5

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    DOI： 10.1016/j.jneuroim.2019.01.010

  • 長期の経過で緩徐進行し両上肢遠位部に限局した症状を呈したCIDP(MADSAM)の一例

    松本 航, 稲水 佐江子, 山下 謙一郎, 山崎 亮, 吉良 潤一

    臨床神経学   59 ( 5 )   312 - 312   2019.5

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  • アルツハイマー病におけるミクログリアを介した病的タンパクの伝播について

    浅井 宏英, 今村 友裕, 山崎 亮, 吉良 潤一

    日本老年医学会雑誌   56 ( Suppl. )   82 - 82   2019.5

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  • Painful legs and moving toes(PLMT)による足趾不随意運動を呈した一例

    田代 匠, 稲水 佐江子, 山下 謙一郎, 山崎 亮, 吉良 潤一

    臨床神経学   59 ( 5 )   310 - 310   2019.5

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  • MOG antibody disease manifesting as progressive cognitive deterioration and behavioral changes with primary central nervous system vasculitis. Reviewed International journal

    Toshikazu Baba, Koji Shinoda, Mitsuru Watanabe, Shoko Sadashima, Dai Matsuse, Noriko Isobe, Ryo Yamasaki, Kimihiko Kaneko, Toshiyuki Takahashi, Toru Iwaki, Jun-Ichi Kira

    Multiple sclerosis and related disorders   30   48 - 50   2019.5

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    DOI： 10.1016/j.msard.2019.01.053

  • Cerebrospinal fluid cytokine/chemokine/growth factor profiles in idiopathic hypertrophic pachymeningitis. Reviewed International journal

    Xu Zhang, Takayuki Fujii, Hidenori Ogata, Ryo Yamasaki, Katsuhisa Masaki, Yiwen Cui, Takuya Matsushita, Noriko Isobe, Jun-Ichi Kira

    Journal of neuroimmunology   330   38 - 43   2019.5

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    DOI： 10.1016/j.jneuroim.2019.01.010

  • MOG antibody disease manifesting as progressive cognitive deterioration and behavioral changes with primary central nervous system vasculitis Reviewed

    Toshikazu Baba, Koji Shinoda, Mitsuru Watanabe, Shoko Sadashima, Dai Matsuse, Noriko Isobe, Ryo Yamasaki, Kimihiko Kaneko, Toshiyuki Takahashi, Toru Iwaki, Jun ichi Kira

    Multiple Sclerosis and Related Disorders   30   48 - 50   2019.5

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    DOI： 10.1016/j.msard.2019.01.053

  • Toxic myopathy with multiple deletions in mitochondrial DNA associated with long-term use of oral anti-viral drugs for hepatitis B A case study Reviewed

    Takayuki Fujii, Kei ichiro Takase, Hiroyuki Honda, Nobutoshi Kawamura, Ryo Yamasaki, Michiyo Urata, Takeshi Uchiumi, Toru Iwaki, Jun ichi Kira

    Neuropathology   39 ( 2 )   162 - 167   2019.4

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    DOI： 10.1111/neup.12548

  • Toxic myopathy with multiple deletions in mitochondrial DNA associated with long-term use of oral anti-viral drugs for hepatitis B: A case study. Reviewed International journal

    Takayuki Fujii, Kei-Ichiro Takase, Hiroyuki Honda, Nobutoshi Kawamura, Ryo Yamasaki, Michiyo Urata, Takeshi Uchiumi, Toru Iwaki, Jun-Ichi Kira

    Neuropathology : official journal of the Japanese Society of Neuropathology   39 ( 2 )   162 - 167   2019.4

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    DOI： 10.1111/neup.12548

  • A novel model for treatment of hypertrophic pachymeningitis Reviewed

    Yiwen Cui, Katsuhisa Masaki, Xu Zhang, Ryo Yamasaki, Takayuki Fujii, Hidenori Ogata, Shotaro Hayashida, Hiroo Yamaguchi, Fuminori Hyodo, Hinako Eto, Sachiko Koyama, Kyoko Iinuma, Tomomi Yonekawa, Takuya Matsushita, Mari Yoshida, Kazunori Yamada, Mitsuhiro Kawano, Marie Malissen, Bernard Malissen, Jun-Ichi Kira

    Annals of Clinical and Translational Neurology   6 ( 3 )   431 - 444   2019.3

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    DOI： 10.1002/acn3.715

  • 糖尿病網膜症におけるCD206+CX3CR1+硝子体界面マクロファージ局所増殖

    山口 宗男, 中尾 新太郎, 和田 伊織, 海津 嘉弘, 有馬 充, 石川 桂二郎, 中間 崇仁, 白石 渉, 山崎 亮, 吉良 潤一, 石橋 達朗, 園田 康平

    日本眼科学会雑誌   123 ( 臨増 )   185 - 185   2019.3

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  • 糖尿病網膜症におけるCD206+CX3CR1+硝子体界面マクロファージ局所増殖

    山口 宗男, 中尾 新太郎, 和田 伊織, 海津 嘉弘, 有馬 充, 石川 桂二郎, 中間 崇仁, 白石 渉, 山崎 亮, 吉良 潤一, 石橋 達朗, 園田 康平

    日本眼科学会雑誌   123 ( 臨増 )   185 - 185   2019.3

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  • A novel model for treatment of hypertrophic pachymeningitis. Reviewed International journal

    Yiwen Cui, Katsuhisa Masaki, Xu Zhang, Ryo Yamasaki, Takayuki Fujii, Hidenori Ogata, Shotaro Hayashida, Hiroo Yamaguchi, Fuminori Hyodo, Hinako Eto, Sachiko Koyama, Kyoko Iinuma, Tomomi Yonekawa, Takuya Matsushita, Mari Yoshida, Kazunori Yamada, Mitsuhiro Kawano, Marie Malissen, Bernard Malissen, Junichi Kira

    Annals of clinical and translational neurology   6 ( 3 )   431 - 444   2019.3

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    DOI： 10.1002/acn3.715

  • Anti-plexin D1 antibodies are a novel biomarker for immune-mediated neuropathic pain Reviewed

    Takayuki Fujii, Ryo Yamasaki, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   10 ( 1 )   7 - 8   2019.2

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    DOI： 10.1111/cen3.12488

  • Functional connectivity change between posterior cingulate cortex and ventral attention network relates to the impairment of orientation for time in Alzheimer's disease patients. Reviewed International journal

    Ken-Ichiro Yamashita, Taira Uehara, Pukovisa Prawiroharjo, Koji Yamashita, Osamu Togao, Akio Hiwatashi, Yoshihide Taniwaki, Hidetsuna Utsunomiya, Takuya Matsushita, Ryo Yamasaki, Jun-Ichi Kira

    Brain imaging and behavior   13 ( 1 )   154 - 161   2019.2

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    DOI： 10.1007/s11682-018-9860-x

  • Functional connectivity change between posterior cingulate cortex and ventral attention network relates to the impairment of orientation for time in Alzheimer’s disease patients Reviewed

    13 ( 1 )   154 - 161   2019.2

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    Alzheimer’s disease (AD) patients exhibit various cognitive dysfunctions, including impairment of orientation for time (OT). The brain regions underlying OT impairment remain to be elucidated. A previous single-photon emission computed tomography study has indicated hypoperfusion of the posterior cingulate cortex (PCC) in relation to deterioration of OT. In this study, we investigated whole brain functional connectivity changes of PCC using resting-state functional magnetic resonance imaging. Voxel-based functional connectivity with PCC was analyzed in OT-poor or OT-good AD patients, classified according to the mean OT scores of the Mini-Mental State Examination subscale. The connectivities of dorsal frontal lobe, and lateral parietal and lateral temporal lobes with PCC in the right hemisphere were reduced in the OT-poor AD group compared with the OT-good AD group. A subtraction connectivity map of OT score differences (OT-good minus OT-poor) revealed the right middle temporal gyrus near the temporo-parietal junction as a significantly connected region with PCC. These results suggest that the right posterior part of the middle temporal gyrus may play an important role in OT in conjunction with PCC, and that disconnection between PCC and the right ventral attention network may cause OT disturbance in AD patients.

    DOI： 10.1007/s11682-018-9860-x

  • Simultaneous MR neurography and apparent T2 mapping in brachial plexus Evaluation of patients with chronic inflammatory demyelinating polyradiculoneuropathy Reviewed

    Hiwatashi Akio, Osamu Togao, Koji Yamashita, kazufumi kikuchi, Daichi Momosaka, Hiroshi Nakatake, Ryo Yamasaki, Hidenori Ogata, Masami Yoneyama, Jun-Ichi Kira, Hiroshi Honda

    Magnetic Resonance Imaging   55   112 - 117   2019.1

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    DOI： 10.1016/j.mri.2018.09.025

  • 【Glymphatic system-脳のゴミ処理とその異常】Glymphatic systemと疾患 多発性硬化症におけるglymphatic systemの役割

    山崎 亮, 吉良 潤一

    37 ( 1 )   100 - 103   2019.1

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  • Simultaneous MR neurography and apparent T2 mapping in brachial plexus: Evaluation of patients with chronic inflammatory demyelinating polyradiculoneuropathy. Reviewed International journal

    Akio Hiwatashi, Osamu Togao, Koji Yamashita, Kazufumi Kikuchi, Daichi Momosaka, Hiroshi Nakatake, Ryo Yamasaki, Hidenori Ogata, Masami Yoneyama, Jun-Ichi Kira, Hiroshi Honda

    Magnetic resonance imaging   55   112 - 117   2019.1

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    DOI： 10.1016/j.mri.2018.09.025

  • Multiple mtDNA deletions due to mitochondrion toxicity of anti-hepadnaviral drugs Comments to the letter from J. Finsterer Reviewed

    Takayuki Fujii, Hiroyuki Honda, Ryo Yamasaki, Toru Iwaki, Jun Ichi Kira

    Neuropathology   39 ( 4 )   326 - 327   2019.1

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    DOI： 10.1111/neup.12563

  • Multiple Sclerosis

    Ryo Yamasaki, Jun ichi Kira

    Advances in Experimental Medicine and Biology   217 - 247   2019.1

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    DOI： 10.1007/978-981-32-9636-7_14

  • Modified diffusion-weighted imaging-Alberta Stroke Program Early Computed Tomography Score including deep white matter lesions predicts symptomatic intracerebral hemorrhage following intravenous thrombolysis Reviewed

    Koji Tanaka, Shoji Matsumoto, Konosuke Furuta, Takeshi Yamada, Sukehisa Nagano, Kei ichiro Takase, Taketo Hatano, Ryo Yamasaki, Jun ichi Kira

    Journal of Thrombosis and Thrombolysis   2019.1

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    DOI： 10.1007/s11239-019-01979-7

  • Creutzfeldt–Jakob disease-like diffusion-weighted imaging hyperintensity paralleled with neuropsychiatric symptoms in a patient with limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies Reviewed

    10 ( 3 )   204 - 206   2019.1

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    DOI： 10.1111/cen3.12525

  • A visual task management application for acute ischemic stroke care Reviewed

    Shoji Matsumoto, Hiroshi Koyama, Ichiro Nakahara, Akira Ishii, Taketo Hatano, Tsuyoshi Ohta, Koji Tanaka, Mitsushige Ando, Hideo Chihara, Wataru Takita, Keisuke Tokunaga, Takuro Hashikawa, Yusuke Funakoshi, Takahiko Kamata, Eiji Higashi, Sadayoshi Watanabe, Daisuke Kondo, Atsushi Tsujimoto, Konosuke Furuta, Takuma Ishihara, Tetsuya Hashimoto, Junpei Koge, Kazutaka Sonoda, Takako Torii, Hideaki Nakagaki, Ryo Yamasaki, Izumi Nagata, Jun Ichi Kira

    Frontiers in Neurology   10 ( OCT )   2019.1

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    DOI： 10.3389/fneur.2019.01118

  • Novel disease-modifying anti-rheumatic drug iguratimod suppresses chronic experimental autoimmune encephalomyelitis by down-regulating activation of macrophages/microglia through an NF-κB pathway Reviewed

    8 ( 1 )   2018.12

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    We aimed to elucidate the effects of iguratimod, a widely used anti-rheumatic drug with no severe side effects, on chronic experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis (MS). Iguratimod was orally administered to mice immunised with myelin oligodendrocyte glycoprotein peptide 35-55. Preventive administration of iguratimod from the time of immunisation was found to markedly reduce the clinical severity of acute and chronic EAE. Pathologically, iguratimod treatment significantly reduced demyelination and infiltration of CD3 + T, F4/80 + , and CD169 + cells into the spinal cord, and suppressed macrophage/microglia activation in the parenchyma at the acute and chronic stages compared with vehicle treatment. Therapeutic administration of iguratimod after the onset of clinical symptoms significantly ameliorated the clinical severity of chronic EAE and reduced demyelination, T helper (Th)1/Th17 cell infiltration, macrophage/microglia activation, and nuclear factor (NF)-κB p65 and cyclooxygenase-2 expression in the spinal cord. In vitro, iguratimod treatment inhibited nuclear translocation of NF-κB p65 and down-regulated pro-inflammatory responses in macrophages and microglia. Our results suggest that iguratimod ameliorates acute and chronic EAE by suppressing inflammatory cell infiltration and immune cell activation, partly through inhibition of NF-κB p65, supporting the therapeutic potential of this drug for not only acute, but also chronic MS.

    DOI： 10.1038/s41598-018-20390-5

  • 膜性腎症を伴うCIDPにおける抗paranode、抗podocyte抗体、並びに抗contactin1抗体陽性CIDPとの病像の比較

    橋本 侑, 緒方 英紀, 山崎 亮, 張 旭, 笹栗 毅和, 立石 貴久, 秋山 真一, 丸山 彰一, 吉良 潤一

    末梢神経   29 ( 2 )   301 - 301   2018.12

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  • 膜性腎症を伴うCIDPにおける抗paranode、抗podocyte抗体、並びに抗contactin1抗体陽性CIDPとの病像の比較

    橋本 侑, 緒方 英紀, 山崎 亮, 張 旭, 笹栗 毅和, 立石 貴久, 秋山 真一, 丸山 彰一, 吉良 潤一

    末梢神経   29 ( 2 )   301 - 301   2018.12

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  • 末梢神経疾患の新しい疾患概念 自己抗体陽性CIDPの臨床像および病態機序

    緒方 英紀, 山崎 亮, 吉良 潤一

    末梢神経   29 ( 2 )   142 - 146   2018.12

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    近年、慢性炎症性多発神経炎症例の一部でランビエ絞輪部およびその周辺の膜蛋白に対する自己抗体が複数同定された。特にランビエ傍絞輪部に局在するneurofascin 155(NF155)および、contactin 1に対する自己抗体は診断的価値が証明され、病的意義についても明らかにされつつある。本稿では抗NF155抗体を中心に自己抗体陽性CIDPの臨床像および病態機序について概説する。(著者抄録)

  • 抗neurofascin155抗体陽性CIDPにおけるT細胞性免疫の寄与と免疫遺伝学的背景因子

    山崎 亮, 緒方 英紀, 張 旭, 町田 明, 森本 展年, 海田 賢一, 増田 曜章, 安東 由喜雄, 桑原 基, 楠 進, 吉良 潤一

    末梢神経   29 ( 2 )   256 - 256   2018.12

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  • 抗Neurofascin 155抗体陽性CIDPの分子生物的基盤と臨床像

    緒方 英紀, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( Suppl. )   S119 - S119   2018.12

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  • 抗Neurofascin 155抗体陽性CIDPの分子生物的基盤と臨床像

    緒方 英紀, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( Suppl. )   S119 - S119   2018.12

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  • 抗NF155抗体陽性CIDPを効率よく検出する指標の同定 暫定診断基準案に基づいた全国調査による解析結果

    緒方 英紀, 山崎 亮, 松下 拓也, 海田 賢一, 松井 真, 桑原 聡, 楠 進, 吉良 潤一

    末梢神経   29 ( 2 )   255 - 255   2018.12

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  • 急性期虚血性脳卒中に対する経静脈的血栓溶解療法後の早期神経症候増悪例の頻度と特徴

    田中 弘二, 松本 省二, 山田 猛, 長野 祐久, 高瀬 敬一郎, 波多野 武人, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( Suppl. )   S228 - S228   2018.12

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  • 急性期虚血性脳卒中に対する経静脈的血栓溶解療法後の早期神経症候増悪例の頻度と特徴

    田中 弘二, 松本 省二, 山田 猛, 長野 祐久, 高瀬 敬一郎, 波多野 武人, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( Suppl. )   S228 - S228   2018.12

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  • 安静時機能的MRIを用いたアルツハイマー病治療薬による脳機能的結合変化の研究

    山下 謙一郎, 上原 平, Pukovisa Prawiroharjo, 山下 孝二, 栂尾 理, 樋渡 昭雄, 谷脇 予志秀, 宇都宮 英綱, 山崎 亮, 本田 浩, 吉良 潤一

    臨床神経学   58 ( Suppl. )   S340 - S340   2018.12

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  • 安静時機能的MRIを用いたアルツハイマー病治療薬による脳機能的結合変化の研究

    山下 謙一郎, 上原 平, Pukovisa Prawiroharjo, 山下 孝二, 栂尾 理, 樋渡 昭雄, 谷脇 予志秀, 宇都宮 英綱, 山崎 亮, 本田 浩, 吉良 潤一

    臨床神経学   58 ( Suppl. )   S340 - S340   2018.12

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  • 多系統萎縮症の病態形成における自然免疫の関与とバイオマーカーの探索

    松瀬 大, 山口 浩雄, Maimaitijiang Guzailiayi, 樋渡 昭雄, 松下 拓也, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( Suppl. )   S321 - S321   2018.12

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  • 多系統萎縮症の病態形成における自然免疫の関与とバイオマーカーの探索

    松瀬 大, 山口 浩雄, Maimaitijiang Guzailiayi, 樋渡 昭雄, 松下 拓也, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( Suppl. )   S321 - S321   2018.12

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  • Lumbar plexus in patients with chronic inflammatory demyelinating polyradiculoneuropathy: evaluation with simultaneous T2 mapping and neurography method with SHINKEI. Reviewed International journal

    Akio Hiwatashi, Osamu Togao, Koji Yamashita, Kazufumi Kikuchi, Daichi Momosaka, Hiroshi Nakatake, Ryo Yamasaki, Hidenori Ogata, Masami Yoneyama, Jun-Ichi Kira, Hiroshi Honda

    The British journal of radiology   91 ( 1092 )   20180501 - 20180501   2018.12

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    DOI： 10.1259/bjr.20180501

  • Duplication and deletion upstream of LMNB1 in autosomal dominant adult-onset leukodystrophy. Reviewed International journal

    Naomi Mezaki, Takeshi Miura, Kotaro Ogaki, Makoto Eriguchi, Yuri Mizuno, Kenichi Komatsu, Hiroki Yamazaki, Natsuki Suetsugi, Sumihiro Kawajiri, Ryo Yamasaki, Takanobu Ishiguro, Takuya Konno, Hiroaki Nozaki, Kensaku Kasuga, Yasuyuki Okuma, Jun-Ichi Kira, Hideo Hara, Osamu Onodera, Takeshi Ikeuchi

    Neurology. Genetics   4 ( 6 )   e292   2018.12

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    DOI： 10.1212/NXG.0000000000000292

  • Duplication and deletion upstream of LMNB1 in autosomal dominant adult-onset leukodystrophy Reviewed

    Naomi Mezaki, Takeshi Miura, Kotaro Ogaki, Makoto Eriguchi, Yuri Mizuno, Kenichi Komatsu, Hiroki Yamazaki, Natsuki Suetsugi, Sumihiro Kawajiri, Ryo Yamasaki, Takanobu Ishiguro, Takuya Konno, Hiroaki Nozaki, Kensaku Kasuga, Yasuyuki Okuma, Jun-Ichi Kira, Hideo Hara, Osamu Onodera, Takeshi Ikeuchi

    Neurology: Genetics   4 ( 6 )   2018.12

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    DOI： 10.1212/NXG.0000000000000292

  • Chronic inflammatory demyelinating polyneuropathy with concurrent membranous nephropathy An anti-paranode and podocyte protein antibody study and literature survey Reviewed

    Yu Hashimoto, Hidenori Ogata, Ryo Yamasaki, Takakazu Sasaguri, Senri Ko, kenichiro yamashita, Zhang Xu, Takuya Matsushita, Takahisa Tateishi, Shin'Ichi Akiyama, Shoichi Maruyama, Akifumi Yamamoto, Jun-Ichi Kira

    Frontiers in Neurology   9 ( NOV )   2018.11

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    DOI： 10.3389/fneur.2018.00997

  • 難病法施行後の難病医療ネットワーク事業の実態 都道府県アンケートより

    岩木 三保, 小早川 優子, 原田 幸子, 白石 渉, 山崎 亮, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   5 ( 2 )   46 - 49   2018.11

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    難病法施行後の難病医療提供体制整備事業の実態を明らかにした。47都道府県庁の難病担当部局課係を対象とする質問紙調査を行った(回収率100&#37;)。難病医療ネットワーク事業は98&#37;実施されていた。難病医療コーディネーターは、42都道府県に60名配置されていた。難病法施行後も難病医療ネットワーク事業の県による差異は大きく、共通して専門的な知識を有する人材の確保の困難さが課題であることがわかった。難病相談・支援センター等との有機的な連携により難病患者の支援体制がより充実すると期待される。(著者抄録)

  • 海綿静脈洞部硬膜動静脈瘻コイル塞栓術後に進行性に外眼筋麻痺をきたした一例

    居積 晃希, 稲水 佐江子, 山下 謙一郎, 松瀬 大, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( 11 )   713 - 713   2018.11

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  • 抗neurofascin155抗体陽性CIDPにおける免疫遺伝学的背景因子とT細胞性免疫の関与

    緒方 英紀, 山崎 亮, 張 旭, 町田 明, 森本 展年, 海田 賢一, 増田 曜章, 安東 由喜雄, 桑原 基, 楠 進, 吉良 潤一

    神経治療学   35 ( 6 )   S226 - S226   2018.11

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  • 抗neurofascin155抗体陽性CIDPにおける免疫遺伝学的背景因子とT細胞性免疫の関与

    緒方 英紀, 山崎 亮, 張 旭, 町田 明, 森本 展年, 海田 賢一, 増田 曜章, 安東 由喜雄, 桑原 基, 楠 進, 吉良 潤一

    神経治療学   35 ( 6 )   S226 - S226   2018.11

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  • 急性純粋脳神経運動麻痺を呈し、ギラン・バレー症候群(GBS)の亜型と考えられた症例

    馬場 俊和, 篠田 紘司, 渡邉 充, 松瀬 大, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( 11 )   715 - 715   2018.11

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  • GBS/CIDP:病態と治療の新たな展開-1 GBS/CIDPの自己抗体 蛋白抗原

    緒方 英紀, 山崎 亮, 吉良 潤一

    神経治療学   35 ( 6 )   S144 - S144   2018.11

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  • GBS/CIDP:病態と治療の新たな展開-1 GBS/CIDPの自己抗体 蛋白抗原

    緒方 英紀, 山崎 亮, 吉良 潤一

    神経治療学   35 ( 6 )   S144 - S144   2018.11

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  • Facial onset sensory and motor neuronopathy症候群は早期からoral phase dysphagiaを呈する

    渡邉 充, 白石 渉, 山崎 亮, 磯部 紀子, 吉良 潤一

    神経治療学   35 ( 6 )   S212 - S212   2018.11

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  • Facial onset sensory and motor neuronopathy症候群は早期からoral phase dysphagiaを呈する

    渡邉 充, 白石 渉, 山崎 亮, 磯部 紀子, 吉良 潤一

    神経治療学   35 ( 6 )   S212 - S212   2018.11

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  • Downregulation of neuronal and dendritic Connexin36-made electrical synapses without glutamatergic axon terminals in spinal anterior horn cells from the early stage of amyotrophic lateral sclerosis Reviewed

    Yuko Kobayakawa, Katsuhisa Masaki, Ryo Yamasaki, Wataru Shiraishi, Shotaro Hayashida, Shintaro Hayashi, Koichi Okamoto, Takuya Matsushita, Jun-Ichi Kira

    Frontiers in Neuroscience   12 ( NOV )   2018.11

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    DOI： 10.3389/fnins.2018.00894

  • Connexin 30 deficiency attenuates chronic but not acute phases of experimental autoimmune encephalomyelitis through induction of neuroprotective microglia Reviewed

    Mei Fang, Ryo Yamasaki, Guangrui Li, Katsuhisa Masaki, Hiroo Yamaguchi, Atsushi Fujita, Noriko Isobe, Jun-Ichi Kira

    Frontiers in Immunology   9 ( NOV )   2018.11

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    DOI： 10.3389/fimmu.2018.02588

  • 問題症例の神経筋診断 左肩に限局する不随意運動を呈した一例

    稲水 佐江子, 藤下 幸穂, 山下 謙一郎, 磯部 紀子, 山崎 亮, 吉良 潤一

    臨床神経生理学   46 ( 5 )   405 - 405   2018.10

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  • A comparison of brain magnetic resonance imaging lesions in multiple sclerosis by race with reference to disability progression Reviewed

    yuri nakamura, Laura Gaetano, Takuya Matsushita, Altermatt Anna, Till Sprenger, Ernst Wilhelm Radue, Jens Wuerfel, Lorena Bauer, Michael Amann, Koji Shinoda, Noriko Isobe, Ryo Yamasaki, Takahiko Saida, Ludwig Kappos, Jun-Ichi Kira

    Journal of Neuroinflammation   15 ( 1 )   2018.9

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    DOI： 10.1186/s12974-018-1295-1

  • 末梢神経 抗neurofascin155抗体陽性CIDPにおける免疫遺伝学的背景因子と髄液サイトカインプロフィール

    緒方 英紀, 山崎 亮, 町田 明, 森本 展年, 海田 賢一, 増田 曜章, 安東 由喜雄, 桑原 基, 楠 進, 吉良 潤一

    神経免疫学   23 ( 1 )   104 - 104   2018.9

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  • 孤発性筋萎縮性側索硬化症の無症候性脊髄と症候性脊髄におけるグリア炎症の免疫病理学的解析

    林 信太郎, 山崎 亮, 岡本 幸市, 吉良 潤一

    神経免疫学   23 ( 1 )   125 - 125   2018.9

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  • 多発性硬化症ではインターフェロンβ治療によるVδ1-Vδ2-Vγ9-γδ型T細胞の増加が良好な予後と関連する

    ママティジャン・グザリアイ, 渡邉 充, 篠田 紘司, 中村 優理, 真崎 勝久, 松下 拓也, 山崎 亮, 吉開 泰信, 吉良 潤一

    神経免疫学   23 ( 1 )   139 - 139   2018.9

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  • 基礎研究・動物モデル 神経障害性疼痛における抗Plexin D1抗体の後根神経節細胞に対する病原性の検討

    藤井 敬之, 山崎 亮, 飯沼 今日子, 土本 大介, 林 良憲, 中西 博, 中別府 雄作, 吉良 潤一

    神経免疫学   23 ( 1 )   111 - 111   2018.9

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  • 基礎研究・動物モデル 希突起神経膠細胞特異的Cx47欠失により産生した新規続発性進行性MSモデル(Oligodendroglia-specific Cx47 deletion produces a novel secondary-progressive MS model)

    趙 奕楠, 山崎 亮, バイエリン・マリオン, 山口 浩雄, 吉良 潤一

    神経免疫学   23 ( 1 )   110 - 110   2018.9

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  • 基礎研究・動物モデル 筋萎縮性側索硬化症モデルマウスにおける末梢神経炎症を介した中枢神経保護機構の解明

    山崎 亮, 白石 渉, 吉良 潤一

    神経免疫学   23 ( 1 )   109 - 109   2018.9

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  • バロー病と視神経脊髄炎の層状脱髄巣における髄鞘保持層への特異的なTMEM119陽性ミクログリアの分布

    林田 翔太郎, 山崎 亮, 眞崎 勝久, 鈴木 諭, 松下 拓也, 高橋 和也, 田平 武, 岩城 徹, 吉良 潤一

    神経免疫学   23 ( 1 )   138 - 138   2018.9

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  • 【ニューロパチー-Basics & Updates】ニューロパチートピックス 抗neurofascin155抗体陽性中枢末梢連合脱髄症

    山崎 亮, 吉良 潤一

    36 ( 9 )   1084 - 1087   2018.9

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  • MS/NMO 血清GFAPとニューロフィラメント軽鎖は視神経脊髄炎関連疾患や多発性硬化症の障害度や再発率と相関する

    渡邉 充, 中村 優理, Michalak Zuzanna, 林 史恵, Barro Christian, 磯部 紀子, 松下 拓也, 山崎 亮, Kuhle Jens, 吉良 潤一

    神経免疫学   23 ( 1 )   92 - 92   2018.9

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  • A comparison of brain magnetic resonance imaging lesions in multiple sclerosis by race with reference to disability progression. Reviewed International journal

    Yuri Nakamura, Laura Gaetano, Takuya Matsushita, Altermatt Anna, Till Sprenger, Ernst-Wilhelm Radue, Jens Wuerfel, Lorena Bauer, Michael Amann, Koji Shinoda, Noriko Isobe, Ryo Yamasaki, Takahiko Saida, Ludwig Kappos, Jun-Ichi Kira

    Journal of neuroinflammation   15 ( 1 )   255 - 255   2018.9

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    DOI： 10.1186/s12974-018-1295-1

  • A Novel Autoantibody against Plexin D1 in Patients with Neuropathic Pain Reviewed

    Takayuki Fujii, Ryo Yamasaki, Kyoko Iinuma, Daisuke Tsuchimoto, Yoshinori Hayashi, Ban yu Saitoh, Takuya Matsushita, Mizuho A. Kido, Shinichi Aishima, Hiroshi Nakanishi, Yusaku Nakabeppu, Jun ichi Kira

    Annals of Neurology   84 ( 2 )   208 - 224   2018.8

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    DOI： 10.1002/ana.25279

  • Branchial myorhythmiaを呈したneuropsychiatric SLEの1例

    宇根 隼人, 江 千里, 菊池 良和, 篠田 紘司, 渡邉 充, 上原 平, 松瀬 大, 山崎 亮, 飛松 省三, 柴崎 浩, 吉良 潤一

    臨床神経学   58 ( 8 )   544 - 544   2018.8

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  • Elevated end-diastolic ratio of the common carotid artery due to cerebral arteriovenous malformation: Two case reports. Reviewed International journal

    Koji Tanaka, Shoji Matsumoto, Takeshi Yamada, Daisuke Kondo, Hideo Chihara, Motohisa Koga, Taketo Hatano, Tomoya Miyagi, Ryo Yamasaki, Jun-Ichi Kira

    Radiology case reports   13 ( 4 )   917 - 920   2018.8

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    DOI： 10.1016/j.radcr.2018.06.007

  • Connexin 30 deficiency attenuates A2 astrocyte responses and induces severe neurodegeneration in a 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine hydrochloride Parkinson's disease animal model. Reviewed International journal

    Atsushi Fujita, Hiroo Yamaguchi, Ryo Yamasaki, Yiwen Cui, Yuta Matsuoka, Ken-Ichi Yamada, Jun-Ichi Kira

    Journal of neuroinflammation   15 ( 1 )   227 - 227   2018.8

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    DOI： 10.1186/s12974-018-1251-0

  • A Novel Autoantibody against Plexin D1 in Patients with Neuropathic Pain. Reviewed International journal

    Takayuki Fujii, Ryo Yamasaki, Kyoko Iinuma, Daisuke Tsuchimoto, Yoshinori Hayashi, Ban-Yu Saitoh, Takuya Matsushita, Mizuho A Kido, Shinichi Aishima, Hiroshi Nakanishi, Yusaku Nakabeppu, Jun-Ichi Kira

    Annals of neurology   84 ( 2 )   208 - 224   2018.8

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    DOI： 10.1002/ana.25279

  • Elevated end-diastolic ratio of the common carotid artery due to cerebral arteriovenous malformation Two case reports Reviewed

    Koji Tanaka, Shoji Matsumoto, Takeshi Yamada, Daisuke Kondo, Hideo Chihara, Motohisa Koga, Taketo Hatano, Tomoya Miyagi, Ryo Yamasaki, Jun-Ichi Kira

    Radiology Case Reports   13 ( 4 )   917 - 920   2018.8

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    DOI： 10.1016/j.radcr.2018.06.007

  • Connexin 30 deficiency attenuates A2 astrocyte responses and induces severe neurodegeneration in a 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine hydrochloride Parkinson's disease animal model Reviewed

    Atsushi Fujita, hiroo yamaguchi, Ryo Yamasaki, Yiwen Cui, Yuta Matsuoka, Ken-Ichi Yamada, Jun-Ichi Kira

    Journal of Neuroinflammation   15 ( 1 )   2018.8

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    DOI： 10.1186/s12974-018-1251-0

  • Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years. Reviewed

    Haruki Koike, Ryoji Nishi, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Naoki Atsuta, Tomohiko Nakamura, Masaaki Hirayama, Hidenori Ogata, Ryo Yamasaki, Jun-Ichi Kira, Masahisa Katsuno, Gen Sobue

    Internal medicine (Tokyo, Japan)   57 ( 14 )   2061 - 2066   2018.7

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    DOI： 10.2169/internalmedicine.0455-17

  • Oral phase dysphagia in facial onset sensory and motor neuronopathy Reviewed

    mitsuru watanabe, Wataru Shiraishi, Ryo Yamasaki, Noriko Isobe, Motohiro Sawatsubashi, Ryuji Yasumatsu, Takashi Nakagawa, Jun-Ichi Kira

    Brain and Behavior   8 ( 6 )   2018.6

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    DOI： 10.1002/brb3.999

  • 総頸動脈拡張末期血流速度比の左右差が術後に正常化した脳動静脈奇形の2例

    田中 弘二, 松本 省二, 山田 猛, 近藤 大祐, 古賀 誉久, 千原 英夫, 波多野 武人, 宮城 知也, 山崎 亮, 吉良 潤一

    31 ( 増刊 )   82 - 82   2018.6

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  • アレルギー性炎症に関連した神経障害性疼痛

    藤井 敬之, 山崎 亮, 吉良 潤一

    末梢神経   29 ( 1 )   35 - 41   2018.6

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    神経障害性疼痛(neuropathic pain:NeP)は、様々な病態を含む難治性疼痛の一つである。その病態機序はほとんど明らかとなっておらず、根治的な治療法は存在しない。私たちは、近年アレルギー性炎症を背景としたNePの新規発症機序を明らかにした。アトピー性疾患モデルマウスでは脊髄後角のグリア炎症がNePを惹起することを見出し、またNePを有するアレルギー性疾患患者の血清において、痛みに関連する後根神経節の小型ニューロンと脊髄後角に結合する自己抗体を見出した。アレルギー性炎症はNeP発症に関わる病態の一つと考えられる。(著者抄録)

  • Facial onset sensory and motor neuronopathyの病態と臨床

    渡邉 充, 白石 渉, 山崎 亮, 吉良 潤一

    末梢神経   29 ( 1 )   50 - 55   2018.6

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    Facial onset sensory and motor neuronopathy(FOSMN症候群)は、顔面の感覚障害で発症し、構音・嚥下障害を伴い、感覚障害、運動障害が次第に尾側に広がる特徴を有する孤発性の神経疾患である。緩徐進行性であり、剖検症例の特徴から、本疾患は神経変性疾患と考えられる。一方、免疫療法が部分的に有効な例があるため、免疫学的機序の関与も想定される。またFOSMN症候群では嚥下障害が予後規定因子と考えられ、早期から口腔相が障害される特徴を有する。そのため顔面の感覚障害で発症した患者を診た際には、FOSMN症候群を鑑別に挙げ、球症状の出現に注意を払うべきである。(著者抄録)

  • Safety and efficacy of eculizumab in Guillain-Barré syndrome: a multicentre, double-blind, randomised phase 2 trial. Reviewed International journal

    17 ( 6 )   519 - 529   2018.6

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    DOI： 10.1016/S1474-4422(18)30114-5

  • Oral phase dysphagia in facial onset sensory and motor neuronopathy. Reviewed International journal

    Mitsuru Watanabe, Wataru Shiraishi, Ryo Yamasaki, Noriko Isobe, Motohiro Sawatsubashi, Ryuji Yasumatsu, Takashi Nakagawa, Jun-Ichi Kira

    Brain and behavior   8 ( 6 )   e00999   2018.6

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    Language：English   Publishing type：Research paper (scientific journal)  

    DOI： 10.1002/brb3.999

  • Safety and efficacy of eculizumab in Guillain-Barré syndrome a multicentre, double-blind, randomised phase 2 trial Reviewed

    17 ( 6 )   519 - 529   2018.6

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    Background: Despite the introduction of plasmapheresis and immunoglobulin therapy, many patients with Guillain-Barré syndrome still have an incomplete recovery. Evidence from pathogenesis studies suggests the involvement of complement-mediated peripheral nerve damage. We aimed to investigate the safety and efficacy of eculizumab, a humanised monoclonal antibody against the complement protein C5, in patients with severe Guillain-Barré syndrome. Methods: This study was a 24 week, multicentre, double-blind, placebo-controlled, randomised phase 2 trial done at 13 hospitals in Japan. Eligible patients with Guillain-Barré syndrome were aged 18 years or older and could not walk independently (Guillain-Barré syndrome functional grade 3–5). Patients were randomly assigned (2:1) to receive 4 weeks of intravenous immunoglobulin plus either eculizumab (900 mg) or placebo; randomisation was done via a computer-generated process and web response system with minimisation for functional grade and age. The study had a parallel non-comparative single-arm outcome measure. The primary outcomes were efficacy (the proportion of patients with restored ability to walk independently [functional grade ≤2] at week 4) in the eculizumab group and safety in the full analysis set. For the efficacy endpoint, we predefined a response rate threshold of the lower 90% CI boundary exceeding 50%. This trial is registered with ClinicalTrials.gov, number, NCT02493725. Findings: Between Aug 10, 2015, and April 21, 2016, 34 patients were assigned to receive either eculizumab (n=23) or placebo (n=11). At week 4, the proportion of the patients able to walk independently (functional grade ≤2) was 61% (90% CI 42–78; n=14) in the eculizumab group, and 45% (20–73; n=5) in the placebo group. Adverse events occurred in all 34 patients. Three patients had serious adverse events: two in the eculizumab group (anaphylaxis in one patient and intracranial haemorrhage and abscess in another patient) and one in the placebo group (depression). The possibility that anaphylaxis and intracranial abscess were related to eculizumab could not be excluded. No deaths or meningococcal infections occurred. Interpretation: The primary outcome measure did not reach the predefined response rate. However, because this is a small study without statistical comparison with the placebo group, the efficacy and safety of eculizumab could be investigated in larger, randomised controlled trials. Funding: The Japan Agency for Medical Research and Development, Ministry of Health, Labor and Welfare, and Alexion Pharmaceuticals.

    DOI： 10.1016/S1474-4422(18)30114-5

  • HLA genotype and cortical lesions Response to the letter from Spencer et al. Reviewed

    Koji Shinoda, Takuya Matsushita, yuri nakamura, Katsuhisa Masaki, Ryo Yamasaki, Jun-Ichi Kira

    Multiple Sclerosis   24 ( 6 )   819 - 820   2018.5

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    DOI： 10.1177/1352458517734072

  • 高度な自律神経障害を呈した抗VGKC複合体抗体関連辺縁系脳炎の1例

    馬場 俊和, 藤田 篤史, 山下 謙一郎, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( 5 )   353 - 353   2018.5

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  • ALS医療ニーズと地域医療資源調査 在宅での医療処置や意思伝達装置に焦点をあてて

    小早川 優子, 岩木 三保, 山崎 亮, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   4 ( 2 )   32 - 37   2018.5

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    ALS患者と家族が必要としている医療資源を把握するため、全国的なアンケート調査を行った。集計の結果、訪問介護の頻度、痰の吸引やTPPV施行時の介護者の確保、TPPV導入時期、意思伝達装置導入時期に対する満足度が低いことがわかった。また障害者総合支援法による重度訪問介護、重度障害者入院時コミュニケーション支援事業に対する認知度と利用率の低さが明らかとなった。ALS患者の療養において介護者の負担軽減は依然として大きな課題であり、個々の患者の病状や介護者の状況にあわせ、適切な制度の利用や医療処置・福祉器具の導入を進めていけるよう、医療従事者および福祉関係者による積極的で継続的な関わりが必要と考えられる。(著者抄録)

  • Upregulation of Vesicular Glutamate Transporter 2 and STAT3 Activation in the Spinal Cord of Mice Receiving 3,3'-Iminodipropionitrile. Reviewed International journal

    Tomohiro Ohgomori, Ryo Yamasaki, Jun-Ichi Kira, Shozo Jinno

    Neurotoxicity research   33 ( 4 )   768 - 780   2018.5

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    DOI： 10.1007/s12640-017-9822-x

  • Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms in NMOSD. Reviewed International journal

    Yuri Mizuno, Koji Shinoda, Mitsuru Watanabe, Takuya Matsushita, Ryo Yamasaki, Jun-Ichi Kira

    Neurology(R) neuroimmunology & neuroinflammation   5 ( 3 )   e447   2018.5

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    DOI： 10.1212/NXI.0000000000000447

  • Renewal of the Editorial Board members: The dawn of a new era Reviewed

    Hiroyuki Murai, Ken-Ichi Kaida, Yuji Nakatsuji, Hideyuki Takeuchi, Koji Yamanaka, Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   9 ( 2 )   79 - 80   2018.5

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    DOI： 10.1111/cen3.12456

  • Hopkins syndrome following the first episode of bronchial asthma associated with enterovirus D68: a case report. Reviewed International journal

    Fumie Hayashi, Shintaro Hayashi, Dai Matsuse, Ryo Yamasaki, Keiji Yonekura, Jun-Ichi Kira

    BMC neurology   18 ( 1 )   71 - 71   2018.5

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    Language：English   Publishing type：Research paper (scientific journal)  

    DOI： 10.1186/s12883-018-1075-7

  • HLA-DRB1*04:05 allele is associated with intracortical lesions on three-dimensional double inversion recovery images in Japanese patients with multiple sclerosis. Reviewed International journal

    Koji Shinoda, Takuya Matsushita, Yuri Nakamura, Katsuhisa Masaki, Ryo Yamasaki, Hiroo Yamaguchi, Osamu Togao, Akio Hiwatashi, Jun-Ichi Kira

    Multiple sclerosis (Houndmills, Basingstoke, England)   24 ( 6 )   710 - 720   2018.5

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    DOI： 10.1177/1352458517707067

  • HLA genotype and cortical lesions: Response to the letter from Spencer et al. Reviewed International journal

    Koji Shinoda, Takuya Matsushita, Yuri Nakamura, Katsuhisa Masaki, Ryo Yamasaki, Jun-Ichi Kira

    Multiple sclerosis (Houndmills, Basingstoke, England)   24 ( 6 )   819 - 820   2018.5

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    DOI： 10.1177/1352458517734072

  • Renewal of the Editorial Board members The dawn of a new era Reviewed

    Hiroyuki Murai, Ken Ichi Kaida, Yuji Nakatsuji, Hideyuki Takeuchi, Koji Yamanaka, Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   9 ( 2 )   79 - 80   2018.5

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    DOI： 10.1111/cen3.12456

  • Upregulation of Vesicular Glutamate Transporter 2 and STAT3 Activation in the Spinal Cord of Mice Receiving 3,3′-Iminodipropionitrile Reviewed

    33 ( 4 )   768 - 780   2018.5

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    Chronic administration of 3,3′-iminodipropionitrile (IDPN) causes axonal impairment. Although controversy still remains, it has been suggested that IDPN intoxication mimics the axonopathy of amyotrophic lateral sclerosis (ALS). Interestingly, recent studies including our own showed that signal transducer and activator of transcription 3 (STAT3) in spinal α-motoneurons was activated in both IDPN-treated mice and SOD1G93A mice, a genetic model of familial ALS. Because activation of STAT3 occurs in response to various stimuli, such as axonal injury, ischemia, and excessive glutamate, here we focused on a potential link between phosphorylated STAT3 (pSTAT3, an active form) and vesicular glutamate transporter 2 (VGluT2, a regulator of glutamate storage and release) in IDPN-treated mice and SOD1G93A mice. Impairment of axonal transport was confirmed by western blot analysis: the expression levels of phosphorylated neurofilament H were elevated in both models. As shown in SOD1G93A mice, the expression frequencies of VGluT2 in synaptophysin-positive (SYP)+ presynaptic terminals around spinal α-motoneurons were significantly higher in IDPN-treated mice than in vehicle controls. The coverages of spinal α-motoneurons by VGluT2+ presynaptic terminals were more elevated around pSTAT3+ cells than around pSTAT3− cells in IDPN-treated mice and SOD1G93A mice. Considering that excessive glutamate is shown to be involved in axonal impairment and STAT3 activation, the present results suggest that IDPN-induced upregulation of VGluT2 may result in an increase in glutamate, which might cause axonopathy and induction of pSTAT3. The link between upregulation of VGluT2 and activation of STAT3 via glutamate may represent a common pathological feature of IDPN-treated mice and SOD1G93A mice.

    DOI： 10.1007/s12640-017-9822-x

  • Hopkins syndrome following the first episode of bronchial asthma associated with enterovirus D68 A case report Reviewed

    Fumie Hayashi, Shintaro Hayashi, Dai Matsuse, Ryo Yamasaki, Keiji Yonekura, Jun-Ichi Kira

    BMC Neurology   18 ( 1 )   2018.5

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    DOI： 10.1186/s12883-018-1075-7

  • Association of decreased percentage of Vδ2+Vγ9+ γδ T cells with disease severity in multiple sclerosis Reviewed

    9 ( APR )   2018.4

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    We recently reported that deletion-type copy number variations of the T cell receptor (TCR) γ, α, and δ genes greatly enhanced susceptibility to multiple sclerosis (MS). However, the effect of abnormal TCR γδ gene rearrangement on MS pathogenesis remains unknown. In the present study, we aimed to clarify γδ TCR repertoire alterations and their relationship to clinical and immunological parameters in MS patients by comprehensive flow cytometric immunophenotyping. Peripheral blood mononuclear cells obtained from 30 untreated MS patients in remission and 23 age- and sex-matched healthy controls (HCs) were stained for surface markers and intracellular cytokines after stimulation with phorbol 12-myristate 13-acetate and ionomycin, and analyzed by flow cytometry. MS patients showed significantly decreased percentages of Vδ2+ and Vδ2+Vγ9+ cells in γδ T cells (pcorr = 0.0297 and pcorr = 0.0288, respectively) and elevated Vδ1/Vδ2 ratios compared with HCs (p = 0.0033). The percentages of interferon (IFN)-γ+Vδ2+ and interleukin (IL)-17A+IFN-γ+Vδ2+ cells in γδ T cells, as well as IFN-γ+ cells in Vδ2+ γδ T cells, were significantly lower in MS patients than in HCs (pcorr < 0.0009, pcorr = 0.0135, and pcorr = 0.0054, respectively). The percentages of Vδ2+ and Vδ2+Vγ9+ cells in γδ T cells were negatively correlated with both the Expanded Disability Status Scale score (r = -0.5006, p = 0.0048; and r = -0.5040, p = 0.0045, respectively) and Multiple Sclerosis Severity Score (r = -0.4682, p = 0.0091; and r = -0.4706, p = 0.0087, respectively), but not with age at disease onset, disease duration, or annualized relapse rate. In HCs, the percentages of Vδ2+ and Vδ2+Vγ9+ cells of total CD3+ T cells had strong positive correlations with the percentage of CD25+CD127low/- cells in CD4+ T cells (r = 0.7826, p < 0.0001; and r = 0.7848, p < 0.0001, respectively), whereas such correlations were totally absent in MS patients. These findings suggest that decreased Vδ2+Vγ9+ γδ T cells are associated with disability in MS. Therefore, the Vδ1/Vδ2 ratio might be a candidate biomarker for predicting disease severity in MS.

    DOI： 10.3389/fimmu.2018.00748

  • Safety and efficacy of eculizumab in Guillain-Barr? syndrome: a multicentre, double-blind, randomised phase 2 trial. Reviewed

    Misawa Sonoko, Kuwabara Satoshi, Sato Yasunori, Yamaguchi Nobuko, Nagashima Kengo, Katayama Kanako, Sekiguchi Yukari, Iwai Yuta, Amino Hiroshi, Suichi Tomoki, Yokota Takanori, Nishida Yoichiro, Kanouchi Tadashi, Kohara Nobuo, Kawamoto Michi, Ishii Junko, Kuwabara Motoi, Suzuki Hidekazu, Hirata Koichi, Kokubun Norito, Masuda Ray, Kaneko Juntaro, Yabe Ichiro, Sasaki Hidenao, Kaida Ken-ichi, Takazaki Hiroshi, Suzuki Norihiro, Suzuki Shigeaki, Hiroyuki Nodera, Naoko Matsui, Tsuji Shoji, Koike Haruki, Yamasaki Ryo, Kusunki Susumu

    The Lancet Neurology   Vol.17 ( No.6 )   519 - 529   2018.4

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    Safety and efficacy of eculizumab in Guillain-Barr? syndrome: a multicentre, double-blind, randomised phase 2 trial.
    Despite the introduction of plasmapheresis and immunoglobulin therapy, many patients with Guillain-Barr? syndrome still have an incomplete recovery. Evidence from pathogenesis studies suggests the involvement of complement-mediated peripheral nerve damage. We aimed to investigate the safety and efficacy of eculizumab, a humanised monoclonal antibody against the complement protein C5, in patients with severe Guillain-Barr? syndrome. This study was a 24 week, multicentre, double-blind, placebo-controlled, randomised phase 2 trial done at 13 hospitals in Japan. Eligible patients with Guillain-Barr? syndrome were aged 18 years or older and could not walk independently (Guillain-Barr? syndrome functional grade 3-5). Patients were randomly assigned (2:1) to receive 4 weeks of intravenous immunoglobulin plus either eculizumab (900 mg) or placebo; randomisation was done via a computer-generated process and web response system with minimisation for functional grade and age. The study had a parallel non-comparative single-arm outcome measure. The primary outcomes were efficacy (the proportion of patients with restored ability to walk independently [functional grade ?2] at week 4) in the eculizumab group and safety in the full analysis set. For the efficacy endpoint, we predefined a response rate threshold of the lower 90% CI boundary exceeding 50%. This trial is registered with ClinicalTrials.gov, number, NCT02493725. Between Aug 10, 2015, and April 21, 2016, 34 patients were assigned to receive either eculizumab (n=23) or placebo (n=11). At week 4, the proportion of the patients able to walk independently (functional grade ?2) was 61% (90% CI 42-78; n=14) in the eculizumab group, and 45% (20-73; n=5) in the placebo group. Adverse events occurred in all 34 patients. Three patients had serious adverse events: two in the eculizumab group (anaphylaxis in one patient and intracranial haemorrhage and abscess in another patient) and one in the placebo group (depression). The possibility that anaphylaxis and intracranial abscess were related to eculizumab could not be excluded. No deaths or meningococcal infections occurred. The primary outcome measure did not reach the predefined response rate. However, because this is a small study without statistical comparison with the placebo group, the efficacy and safety of eculizumab could be investigated in larger, randomised controlled trials. The Japan Agency for Medical Research and Development, Ministry of Health, Labor and Welfare, and Alexion Pharmaceuticals.

    DOI： 10.1016/S1474-4422(18)30114-5

  • Expansions of intronic TTTCA and TTTTA repeats in benign adult familial myoclonic epilepsy. Reviewed International journal

    50 ( 4 )   581 - 590   2018.4

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    DOI： 10.1038/s41588-018-0067-2

  • Expansions of intronic TTTCA and TTTTA repeats in benign adult familial myoclonic epilepsy Reviewed

    Hiroyuki Ishiura, Koichiro Doi, Jun Mitsui, Jun Yoshimura, Miho Kawabe Matsukawa, Asao Fujiyama, Yasuko Toyoshima, Akiyoshi Kakita, Hitoshi Takahashi, Yutaka Suzuki, Sumio Sugano, Wei Qu, Kazuki Ichikawa, Hideaki Yurino, Koichiro Higasa, Shota Shibata, Aki Mitsue, Masaki Tanaka, Yaeko Ichikawa, Yuji Takahashi, Hidetoshi Date, Takashi Matsukawa, Junko Kanda, Fumiko Kusunoki Nakamoto, Mana Higashihara, Koji Abe, Ryoko Koike, Mutsuo Sasagawa, Yasuko Kuroha, Naoya Hasegawa, Norio Kanesawa, Takayuki Kondo, Takefumi Hitomi, Masayoshi Tada, Hiroki Takano, Yutaka Saito, Kazuhiro Sanpei, Osamu Onodera, Masatoyo Nishizawa, Masayuki Nakamura, Takeshi Yasuda, Yoshio Sakiyama, Mieko Otsuka, Akira Ueki, Ken Ichi Kaida, Jun Shimizu, Ritsuko Hanajima, Toshihiro Hayashi, Yasuo Terao, Ryo Yamasaki

    Nature Genetics   50 ( 4 )   581 - 590   2018.4

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    DOI： 10.1038/s41588-018-0067-2

  • Connexins in health and disease Reviewed

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   9   30 - 36   2018.3

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    DOI： 10.1111/cen3.12433

  • 【多発性硬化症 最前線】多発性硬化症の病態とグリア炎症

    山崎 亮, 吉良 潤一

    神経眼科   35 ( 1 )   4 - 10   2018.3

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    多発性硬化症は代表的な脱髄性疾患で、再発寛解を繰り返す。再発を繰り返すと神経障害が蓄積され、数年から十数年で二次進行型に移行する。この慢性進行期は、末梢の免疫反応にかかわらず中枢神経系で徐々に神経障害が悪化していく。そのメカニズムに深く関わるのが、中枢のグリア細胞という非神経細胞の活性化(=グリア炎症)である。グリア炎症は様々な要因に左右されるが、その重要な一つがギャップ結合蛋白コネキシンの機能異常である。多発性硬化症の急性期にはアストログリアのコネキシンが低下し、慢性期では逆に発現上昇する一方、オリゴデンドログリアのコネキシンは低下し続ける。この現象が神経保護的に働くのか障害性に働くのか、現時点では不明である。このメカニズム解明が、現在まで治療法のない慢性進行期の多発性硬化症治療薬開発につながる。(著者抄録)

  • Connexins in health and disease

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   9   30 - 36   2018.3

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    DOI： 10.1111/cen3.12433

  • Anti-neurofascin 155 antibody-related neuropathy Reviewed

    Hidenori Ogata, Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   9 ( 1 )   54 - 64   2018.2

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    DOI： 10.1111/cen3.12444

  • Anti-neurofascin 155 antibody-related neuropathy

    Hidenori Ogata, Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   9 ( 1 )   54 - 64   2018.2

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    DOI： 10.1111/cen3.12444

  • Parallel fluctuation of anti-neurofascin 155 antibody levels with clinico-electrophysiological findings in patients with chronic inflammatory demyelinating polyradiculoneuropathy Reviewed

    Atsushi Fujita, Hidenori Ogata, Ryo Yamasaki, Takuya Matsushita, Jun-Ichi Kira

    Journal of the Neurological Sciences   384   107 - 112   2018.1

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    DOI： 10.1016/j.jns.2017.11.035

  • 傍脊柱筋に強い炎症がある抗PM/Scl-75抗体陽性多発筋炎の1例

    江 千里, 雑賀 徹, 山下 謙一郎, 前田 教寿, 渡邉 充, 松下 拓也, 山崎 亮, 岩城 徹, 吉良 潤一

    臨床神経学   58 ( 1 )   66 - 66   2018.1

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  • ナタリズマブ投与中に急性の構音・嚥下障害、顔面神経麻痺を認め、ギラン・バレー症候群を発症した多発性硬化症の一例

    野上 健一郎, 雑賀 徹, 山下 謙一郎, 松下 拓也, 山崎 亮, 吉良 潤一

    臨床神経学   58 ( 1 )   65 - 65   2018.1

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  • Parallel fluctuation of anti-neurofascin 155 antibody levels with clinico-electrophysiological findings in patients with chronic inflammatory demyelinating polyradiculoneuropathy. Reviewed International journal

    Atsushi Fujita, Hidenori Ogata, Ryo Yamasaki, Takuya Matsushita, Jun-Ichi Kira

    Journal of the neurological sciences   384   107 - 112   2018.1

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    DOI： 10.1016/j.jns.2017.11.035

  • Novel disease-modifying anti-rheumatic drug iguratimod suppresses chronic experimental autoimmune encephalomyelitis by down-regulating activation of macrophages/microglia through an NF-κB pathway. Reviewed International journal

    8 ( 1 )   1933 - 1933   2018.1

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    DOI： 10.1038/s41598-018-20390-5

  • Restoration of a conduction block after the long-term treatment of CIDP with anti-neurofascin 155 antibodies Follow-up of a case over 23 years Reviewed

    Haruki Koike, Ryoji Nishi, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Naoki Atsuta, Tomohiko Nakamura, Masaaki Hirayama, Hidenori Ogata, Ryo Yamasaki, Jun Ichi Kira, Masahisa Katsuno, Gen Sobue

    Internal Medicine   57 ( 14 )   2061 - 2066   2018.1

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    DOI： 10.2169/internalmedicine.0455-17

  • Lumbar plexus in patients with chronic inflammatory demyelinating polyradiculoneuropathy Evaluation with simultaneous T2 mapping and neurography method with SHINKEI Reviewed

    Akio Hiwatashi, Osamu Togao, Koji Yamashita, Kazufumi Kikuchi, Daichi Momosaka, Hiroshi Nakatake, Ryo Yamasaki, Hidenori Ogata, Masami Yoneyama, Jun Ichi Kira, Hiroshi Honda

    British Journal of Radiology   91 ( 1092 )   2018.1

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    DOI： 10.1259/bjr.20180501

  • The contribution of glial inflammation in the pathogenesis of multiple sclerosis Reviewed

    Ryo Yamasaki, Jun-Ichi Kira

    Neuro-Ophthalmology Japan   35 ( 1 )   4 - 10   2018.1

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    DOI： 10.11476/shinkeiganka.35.4

  • Markers for Guillain-Barré syndrome with poor prognosis a multi-center study Reviewed

    22 ( 4 )   433 - 439   2017.12

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    Guillain-Barré syndrome (GBS) is an acute monophasic neuropathy. Prognostic tools include the modified Erasmus GBS outcome score (mEGOS), Erasmus GBS respiratory insufficiency score (EGRIS), and the increase in serum IgG levels (ΔIgG) 2 weeks after intravenous immunoglobulin (IVIg) treatment. Given that proportions of GBS subtypes differ between Western countries and Japan, the usefulness of these tools in Japan or other countries remains unknown. We enrolled 177 Japanese patients with GBS from 15 university hospitals and retrospectively obtained mEGOS and EGRIS for all and ΔIgG status for 79 of them. High mEGOS scores on admission or on day 7 were significantly associated with poorer outcomes (unable to walk independently at 6 months). High EGRIS scores (≥5 points) were associated with an increased risk for mechanical ventilation. Patients with ΔIgG <1,108 mg/dl had significantly poorer outcomes. We suggest that mEGOS, EGRIS, and ΔIgG in GBS are clinically relevant in Japan.

    DOI： 10.1111/jns.12234

  • 特徴的な臨床症候と画像所見を認めたneurofascin155抗体陽性ニューロパチーの2例

    渡利 茉里, 村上 慶高, 植田 光晴, 安東 由喜雄, 緒方 英紀, 山崎 亮, 吉良 潤一

    末梢神経   28 ( 2 )   348 - 348   2017.12

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  • 全身neurographyで多発する紡錘状末梢神経肥厚を認め、病理学的に脱髄・炎症細胞浸潤を確認し得たMADSAMの一例

    緒方 英紀, 前田 泰宏, 岡 伸幸, 西田 圭一郎, 松下 拓也, 山崎 亮, 吉良 潤一

    末梢神経   28 ( 2 )   346 - 346   2017.12

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  • 「CRPSにおける最近の進歩」 自己抗体陽性CIDPの臨床像 抗neurofascin 155抗体陽性CIDPを中心に

    緒方 英紀, 山崎 亮, 吉良 潤一

    末梢神経   28 ( 2 )   193 - 196   2017.12

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    近年ランビエ傍絞輪部の細胞膜蛋白であるneurofascin 155およびcontactin-1に対する自己抗体と慢性炎症性脱髄性多発根ニューロパチー(CIDP)との関係が明らかにされつつある。特に抗neurofascin 155抗体陽性CIDPについてはこれまでに本邦から60例以上の報告がなされ、疾患概念が確立されつつある。私たちはこれまでに得られた知見を踏まえ、「抗neurofascin 155抗体関連ニューロパチー」として暫定診断基準を策定し、全国臨床調査を実施中である。(著者抄録)

  • Plexin D1は小径無髄後根神経節神経細胞を標的とした免疫介在性神経障害性疼痛の責任自己抗原である

    藤井 敬之, 山崎 亮, 飯沼 今日子, 土本 大介, 城戸 瑞穂, 相島 慎一, 中別府 雄作, 吉良 潤一

    末梢神経   28 ( 2 )   279 - 279   2017.12

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  • Facial onset sensory and motor neuronopathy(FOSMN症候群)の自験例5例の検討 免疫療法の効果と長期経過

    渡邉 充, 白石 渉, 山崎 亮, 吉良 潤一

    末梢神経   28 ( 2 )   299 - 299   2017.12

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  • Markers for Guillain-Barré syndrome with poor prognosis: a multi-center study. Reviewed International journal

    22 ( 4 )   433 - 439   2017.12

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    DOI： 10.1111/jns.12234

  • Polymorphic regulation of mitochondrial fission and fusion modifies phenotypes of microglia in neuroinflammation Reviewed

    Mitsuhiko Katoh, Bao Wu, Huy Bang Nguyen, Truc Quynh Thai, Ryo Yamasaki, Haiyan Lu, Anna M. Rietsch, Musab M. Zorlu, Youichi Shinozaki, Yurika Saitoh, Sei Saitoh, Takashi Sakoh, Kazuhiro Ikenaka, Schuichi Koizumi, Richard M. Ransohoff, Nobuhiko Ohno

    Scientific Reports   7 ( 1 )   2017.12

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    DOI： 10.1038/s41598-017-05232-0

  • Astroglial phagocytosis in central nervous system health and disease Reviewed

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   8 ( 4 )   285 - 286   2017.11

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    DOI： 10.1111/cen3.12413

  • 特徴的な臨床症候と治療経過を呈した抗neurofascin155抗体陽性ニューロパチー 当院での治療経験

    池田 知聡, 山元 雅典, 渡利 茉里, 増田 曜章, 植田 光晴, 安東 由喜雄, 緒方 英紀, 山崎 亮, 吉良 潤一

    神経治療学   34 ( 6 )   S202 - S202   2017.11

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  • Astroglial phagocytosis in central nervous system health and disease

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   8 ( 4 )   285 - 286   2017.11

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    DOI： 10.1111/cen3.12413

  • Neuron-specific methylome analysis reveals epigenetic regulation and tau-related dysfunction of BRCA1 in Alzheimer's disease. Reviewed International journal

    Tatsuo Mano, Kenichi Nagata, Takashi Nonaka, Airi Tarutani, Tomohiro Imamura, Tadafumi Hashimoto, Taro Bannai, Kagari Koshi-Mano, Takeyuki Tsuchida, Ryo Ohtomo, Junko Takahashi-Fujigasaki, Satoshi Yamashita, Yasumasa Ohyagi, Ryo Yamasaki, Shoji Tsuji, Akira Tamaoka, Takeshi Ikeuchi, Takaomi C Saido, Takeshi Iwatsubo, Toshikazu Ushijima, Shigeo Murayama, Masato Hasegawa, Atsushi Iwata

    Proceedings of the National Academy of Sciences of the United States of America   114 ( 45 )   E9645-E9654 - E9654   2017.11

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    DOI： 10.1073/pnas.1707151114

  • Neuron-specific methylome analysis reveals epigenetic regulation and tau-related dysfunction of BRCA1 in Alzheimer’s disease Reviewed

    114 ( 45 )   E9645 - E9654   2017.11

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    Alzheimer’s disease (AD) is a chronic neurodegenerative disease characterized by pathology of accumulated amyloid β (Aβ) and phosphorylated tau proteins in the brain. Postmortem degradation and cellular complexity within the brain have limited approaches to molecularly define the causal relationship between pathological features and neuronal dysfunction in AD. To overcome these limitations, we analyzed the neuron-specific DNA methylome of postmortem brain samples from AD patients, which allowed differentially hypomethylated region of the BRCA1 promoter to be identified. Expression of BRCA1 was significantly up-regulated in AD brains, consistent with its hypomethylation. BRCA1 protein levels were also elevated in response to DNA damage induced by Aβ. BRCA1 became mislocalized to the cytoplasm and highly insoluble in a tau-dependent manner, resulting in DNA fragmentation in both in vitro cellular and in vivo mouse models. BRCA1 dysfunction under Aβ burden is consistent with concomitant deterioration of genomic integrity and synaptic plasticity. The Brca1 promoter region of AD model mice brain was similarly hypomethylated, indicating an epigenetic mechanism underlying BRCA1 regulation in AD. Our results suggest deterioration of DNA integrity as a central contributing factor in AD pathogenesis. Moreover, these data demonstrate the technical feasibility of using neuron-specific DNA methylome analysis to facilitate discovery of etiological candidates in sporadic neurodegenerative diseases.

    DOI： 10.1073/pnas.1707151114

  • A novel mutation in FGD4 causes Charcot–Marie–Tooth disease type 4H with cranial nerve involvement Reviewed

    27 ( 10 )   959 - 961   2017.10

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    Charcot–Marie–Tooth disease type 4H (CMT4H) is a rare variant of autosomal recessive hereditary neuropathy. It is caused by FGD4 mutations and characterized by early infantile onset, slowly progressive distal muscle weakness, scoliosis, and myelin outfoldings visible in nerve biopsy samples. Here, we report a 65-year-old male born to consanguineous parents, who carries a novel homozygous FGD4 c.724C>T nonsense mutation. He developed lower limb weakness in his teens, which progressed slowly and was accompanied by diplopia, bilateral hearing loss, and erectile dysfunction from his twenties. At the age of 65, he was wheelchair-bound and had mild scoliosis, bilateral ophthalmoplegia, facial muscle weakness, inner ear hearing loss, distal-dominant weakness, and sensory disturbance, but no cognitive deterioration. Magnetic resonance imaging revealed enlarged bilateral trigeminal and facial nerves. Accordingly, we believe that this mutation causes slowly progressive sensorimotor neuropathy with apparent cranial nerve involvement, thereby further expanding the clinical spectrum of CMT4H.

    DOI： 10.1016/j.nmd.2017.07.011

  • 限局型全身性強皮症に多発単神経炎を合併し、MR neurographyで末梢神経腫大を認めた一例

    山元 伸昭, 山口 高弘, 篠田 紘司, 渡邉 充, 山崎 亮, 吉良 潤一

    臨床神経学   57 ( 10 )   643 - 643   2017.10

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  • 視神経障害を伴った抗neurofascin155抗体陽性CIDPの一例

    渡利 茉里, 植田 光晴, 安東 由喜雄, 緒方 英紀, 山崎 亮, 吉良 潤一

    臨床神経学   57 ( 10 )   643 - 643   2017.10

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  • 末梢神経 抗neurofascin 155抗体関連ニューロパチー暫定診断基準案の策定

    緒方 英紀, 山崎 亮, 松下 拓也, 角谷 真人, 海田 賢一, 松井 真, 桑原 聡, 楠 進, 吉良 潤一

    神経免疫学   22 ( 1 )   109 - 109   2017.10

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  • 基礎研究・実験モデル オリゴデンドログリアのコネキシン47欠失は慢性進行型実験的自己免疫性脳脊髄炎を悪化させる

    趙 奕楠, 山崎 亮, 山口 浩雄, 宇根 隼人, 飯沼 今日子, 真崎 勝久, 篠田 紘司, 吉良 潤一

    神経免疫学   22 ( 1 )   120 - 120   2017.10

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  • 基礎研究・実験モデル Cx30 KO miceにおける慢性進行期EAEの軽症化

    山崎 亮, 方 梅, 藤田 篤史, 山口 浩雄, 吉良 潤一

    神経免疫学   22 ( 1 )   119 - 119   2017.10

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  • 基礎研究・実験モデル 新生児期のアレルゲン暴露はsynaptopathyにより自閉症様行動を惹起する

    齋藤 万有, クルイニング・ダンバン, 山崎 亮, 吉良 潤一

    神経免疫学   22 ( 1 )   117 - 117   2017.10

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  • 基礎研究・実験モデル 筋萎縮性側索硬化症モデルマウスにおいてCCR2陽性末梢免疫細胞は病態保護的に働く

    白石 渉, 山崎 亮, 吉良 潤一

    神経免疫学   22 ( 1 )   116 - 116   2017.10

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  • 基礎研究・実験モデル 筋萎縮性側索硬化症の脊髄白質では樹状細胞とマクロファージは異なる局在を示す

    林 信太郎, 山崎 亮, 村井 弘之, 岡本 幸市, 吉良 潤一

    神経免疫学   22 ( 1 )   116 - 116   2017.10

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  • 世界初の肥厚性硬膜炎動物モデル作成とTGF-βシグナルを標的とした新規治療法開発

    崔 訳文, 真崎 勝久, 山崎 亮, 山口 浩雄, 林田 翔太郎, 兵藤 文紀, 江藤 比奈子, 米川 智, 松下 拓也, 吉良 潤一

    神経免疫学   22 ( 1 )   86 - 86   2017.10

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  • Paranodal axo-glial detachment in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies

    Koike H, Kadoya M, Kaida K. I, Nishi R, Ikeda S, Kawagashira Y, Lijima M, Kato D, Ogata H, Yamasaki R, Matsukawa N, Kira J. I, Katsuno M, Sobue G

    JOURNAL OF THE NEUROLOGICAL SCIENCES   381   59-60   2017.10

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    Paranodal axo-glial detachment in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies

    DOI： 10.1016/j.jns.2017.08.226

  • MS・NMO2 視神経脊髄炎脳病巣の神経病理学的検討 大脳皮質の炎症細胞浸潤と脱髄を伴う限局性アストロサイトパチー

    林田 翔太郎, 眞崎 勝久, 鈴木 諭, 松下 拓也, 山崎 亮, 末長 敏彦, 高橋 和也, 岩城 徹, 吉良 潤一

    神経免疫学   22 ( 1 )   112 - 112   2017.10

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  • MS・NMO2 γδ型T細胞受容体のレパトワ偏倚は多発性硬化症の臨床的障害度と有意に関連する

    篠田 紘司, グザリアイ・ママティジャン, 中村 優理, 眞崎 勝久, 松下 拓也, 山崎 亮, 吉開 泰信, 吉良 潤一

    神経免疫学   22 ( 1 )   114 - 114   2017.10

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  • MR neurographyで多発性の末梢神経腫大・神経根腫大を認めた抗AQP4抗体陽性視神経脊髄炎関連疾患の一例

    水野 裕理, 篠田 紘司, 渡邉 充, 馬場 俊和, 田中 公裕, 山崎 亮, 吉良 潤一

    臨床神経学   57 ( 10 )   640 - 640   2017.10

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  • Lamin B1関連常染色体優性遺伝性白質脳症 コピー数多型と臨床的特徴

    目崎 直実, 三浦 健, 大垣 光太郎, 江里口 誠, 水野 裕理, 小松 研一, 山崎 博輝, 末次 南月, 河尻 澄宏, 山崎 亮, 野崎 洋明, 春日 健作, 大熊 泰之, 吉良 潤一, 原 英夫, 小野寺 理, 池内 健

    31 ( 4 )   596 - 596   2017.10

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  • Lamin B1関連常染色体優性遺伝性白質脳症 コピー数多型と臨床的特徴

    目崎 直実, 三浦 健, 大垣 光太郎, 江里口 誠, 水野 裕理, 小松 研一, 山崎 博輝, 末次 南月, 河尻 澄宏, 山崎 亮, 野崎 洋明, 春日 健作, 大熊 泰之, 吉良 潤一, 原 英夫, 小野寺 理, 池内 健

    31 ( 4 )   596 - 596   2017.10

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  • IgG2抗Plexin D1抗体は免疫介在性神経障害性疼痛の新規バイオマーカーである

    藤井 敬之, 山崎 亮, 飯沼 今日子, 土本 大介, 城戸 瑞穂, 相島 慎一, 中別府 雄作, 吉良 潤一

    神経免疫学   22 ( 1 )   85 - 85   2017.10

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  • Focal cortical astrocytopathy lesions with demyelination and inflammatory cell infiltrates in neuromyelitis optica spectrum disorder: a neuropathological study of eleven autopsied cases

    S. Hayashida, K. Masaki, S. O. Suzuki, T. Matsushita, R. Yamasaki, T. Suenaga, K. Takahashi, T. Iwaki, J. -I. Kira

    MULTIPLE SCLEROSIS JOURNAL   23   173 - 173   2017.10

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  • Establishing a Hypertrophic Pachymeningitis Model Mice and Successful Treatment with Tumor Growth Factor-beta Blockade

    Ryo Yamasaki, Yiwen Cui, Katsuhisa Masaki, Shotaro Hayashida, Jun-ichi Kira

    ANNALS OF NEUROLOGY   82   S26 - S26   2017.10

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  • Astroglial and Oligodendroglial Connexins Differentially Modulate Acute and Chronic Experimental Autoimmune Encephalomyelitis

    Jun-Ichi Kira, Ryo Yamasaki, Hayato Une, Mei Fang, Yinan Zhao, Guanrui Li, Kyoko Iinuma, Katsuhisa Masaki, Kouji Shinoda, Hiroo Yamaguchi

    ANNALS OF NEUROLOGY   82   S124 - S124   2017.10

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    Language：English   Publishing type：Research paper (other academic)  

  • An Anti-Plexin D1 Autoantibody Is Associated with Immunotherapy-Responsive Neuropathic Pain

    Takayuki Fujii, Ryo Yamasaki, Kyoko Iinuma, Daisuke Tsuchimoto, Mizuho A. Kido, Shinichi Aishima, Yusaku Nakabeppu, Jun-Ichi Kira

    ANNALS OF NEUROLOGY   82   S119 - S119   2017.10

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  • A distinct repertoire of the gamma delta T cell population is associated with disease severity of multiple sclerosis

    K. Shinoda, G. Maimaitijiang, Y. Nakamura, K. Masaki, T. Matsushita, R. Yamasaki, Y. Yoshikai, J. -I. Kira

    MULTIPLE SCLEROSIS JOURNAL   23   201 - 201   2017.10

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  • Slowed abduction during smooth pursuit eye movement in episodic ataxia type 2 with a novel CACNA1A mutation. Reviewed International journal

    Mitsunori Shimmura, Taira Uehara, Kenichiro Yamashita, Hiroshi Shigeto, Ryo Yamasaki, Kinya Ishikawa, Jun-Ichi Kira

    Journal of the neurological sciences   381   4 - 6   2017.10

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    DOI： 10.1016/j.jns.2017.07.040

  • Measurement Conditions of End-Diastolic Ratio of Common Carotid Arteries Alter Diagnostic Ability for Large Artery Intracranial Occlusive Disease. Reviewed International journal

    Koji Tanaka, Shoji Matsumoto, Hiroyuki Murai, Ryo Yamasaki, Jun-Ichi Kira

    Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association   26 ( 10 )   2421 - 2426   2017.10

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    DOI： 10.1016/j.jstrokecerebrovasdis.2017.05.038

  • A novel mutation in FGD4 causes Charcot-Marie-Tooth disease type 4H with cranial nerve involvement. Reviewed International journal

    Daisuke Kondo, Koji Shinoda, Ken-Ichiro Yamashita, Ryo Yamasaki, Akihiro Hashiguchi, Hiroshi Takashima, Jun-Ichi Kira

    Neuromuscular disorders : NMD   27 ( 10 )   959 - 961   2017.10

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    DOI： 10.1016/j.nmd.2017.07.011

  • Slowed abduction during smooth pursuit eye movement in episodic ataxia type 2 with a novel CACNA1A mutation Reviewed

    Mitsunori Shimmura, Taira Uehara, kenichiro yamashita, Hiroshi Shigeto, Ryo Yamasaki, Kinya Ishikawa, Jun-Ichi Kira

    Journal of the Neurological Sciences   381   4 - 6   2017.10

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    Language：English   Publishing type：Research paper (scientific journal)  

    DOI： 10.1016/j.jns.2017.07.040

  • Measurement Conditions of End-Diastolic Ratio of Common Carotid Arteries Alter Diagnostic Ability for Large Artery Intracranial Occlusive Disease Reviewed

    Koji Tanaka, Shoji Matsumoto, Hiroyuki Murai, Ryo Yamasaki, Jun-Ichi Kira

    Journal of Stroke and Cerebrovascular Diseases   26 ( 10 )   2421 - 2426   2017.10

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    DOI： 10.1016/j.jstrokecerebrovasdis.2017.05.038

  • CLINICO-ELECTROPHYSIOLOGICAL CORRELATION WITH ANTI-NEUROFASCIN155 ANTIBODY LEVELS IN THE ANTIBODY-POSITIVE CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY PATIENTS

    H. Ogata, A. Fujita, R. Yamasaki, T. Matsushita, J. Kira

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   22 ( 3 )   348 - 349   2017.9

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  • 難病医療コーディネーターに対する多職種のニーズ調査結果

    岩木 三保, 福重 麻耶, 原田 幸子, 山崎 亮, 白石 渉, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   5 ( 1 )   110 - 110   2017.9

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  • 福岡県在宅重症難病患者レスパイト入院事業のレスパイト受入病院アンケート調査報告

    原田 幸子, 岩木 三保, 福重 麻耶, 白石 渉, 山崎 亮, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   5 ( 1 )   66 - 66   2017.9

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  • 福岡県在宅重症難病患者レスパイト入院事業の利用者アンケート調査報告

    岩木 三保, 福重 麻耶, 原田 幸子, 白石 渉, 山崎 亮, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   5 ( 1 )   65 - 65   2017.9

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  • アレルギー学からみた内科疾患 病態解明から新規治療の開発へ アレルギー学からみた脳神経疾患の新展開

    吉良 潤一, 藤井 敬之, 山崎 亮

    日本内科学会雑誌   106 ( 9 )   1812 - 1820   2017.9

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  • PARANODAL DISSECTION IN CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY WITH ANTI-NEUROFASCIN 155 AND ANTI-CONTACTIN 1 ANTIBODIES

    H. Koike, M. Kadoya, K. Kaida, S. Ikeda, Y. Kawagashira, M. Iijima, D. Kato, H. Ogata, R. Yamasaki, N. Matsukawa, J. Kira, M. Katsuno, G. Sobue

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   22 ( 3 )   318 - 318   2017.9

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  • Differential activation of neuronal and glial STAT3 in the spinal cord of the SOD1G93A mouse model of amyotrophic lateral sclerosis Reviewed

    Tomohiro Ohgomori, Ryo Yamasaki, Hideyuki Takeuchi, Kenji Kadomatsu, Jun-Ichi Kira, Shozo Jinno

    European Journal of Neuroscience   46 ( 4 )   2001 - 2014   2017.8

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    DOI： 10.1111/ejn.13650

  • 脳幹病巣により頭部自律神経症状を伴う短時間持続性片側神経痛様頭痛発作(SUNA)を呈した抗AQP4抗体陽性視神経脊髄炎の一例

    水野 裕理, 篠田 紘司, 渡邉 充, 山崎 亮, 吉良 潤一

    臨床神経学   57 ( 8 )   461 - 461   2017.8

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  • Re-emergence of a tumefactive demyelinating lesion after initiation of fingolimod therapy. Reviewed International journal

    Yu Hashimoto, Koji Shinoda, Eizo Tanaka, Taira Uehara, Takuya Matsushita, Ryo Yamasaki, Jun-Ichi Kira

    Journal of the neurological sciences   379   167 - 168   2017.8

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    DOI： 10.1016/j.jns.2017.06.002

  • Lumbar plexus in patients with chronic inflammatory demyelinating polyneuropathy: Evaluation with 3D nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (3D SHINKEI). Reviewed International journal

    Akio Hiwatashi, Osamu Togao, Koji Yamashita, Kazufumi Kikuchi, Ryotato Kamei, Daichi Momosaka, Hidenori Ogata, Ryo Yamasaki, Masami Yoneyama, Jun-Ichi Kira, Hiroshi Honda

    European journal of radiology   93   95 - 99   2017.8

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    DOI： 10.1016/j.ejrad.2017.05.031

  • Differential activation of neuronal and glial STAT3 in the spinal cord of the SOD1G93A mouse model of amyotrophic lateral sclerosis. Reviewed International journal

    Tomohiro Ohgomori, Ryo Yamasaki, Hideyuki Takeuchi, Kenji Kadomatsu, Jun-Ichi Kira, Shozo Jinno

    The European journal of neuroscience   46 ( 4 )   2001 - 2014   2017.8

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    DOI： 10.1111/ejn.13650

  • Re-emergence of a tumefactive demyelinating lesion after initiation of fingolimod therapy Reviewed

    Yu Hashimoto, Koji Shinoda, Eizo Tanaka, Taira Uehara, Takuya Matsushita, Ryo Yamasaki, Jun-Ichi Kira

    Journal of the Neurological Sciences   379   167 - 168   2017.8

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    DOI： 10.1016/j.jns.2017.06.002

  • Lumbar plexus in patients with chronic inflammatory demyelinating polyneuropathy Evaluation with 3D nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (3D SHINKEI) Reviewed

    Hiwatashi Akio, Osamu Togao, Koji Yamashita, kazufumi kikuchi, Ryotato Kamei, Daichi Momosaka, Hidenori Ogata, Ryo Yamasaki, Masami Yoneyama, Jun-Ichi Kira, Hiroshi Honda

    European Journal of Radiology   93   95 - 99   2017.8

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    DOI： 10.1016/j.ejrad.2017.05.031

  • Differential activation of neuronal and glial STAT3 in the spinal cord of the SOD1(G93A) mouse model of amyotrophic lateral sclerosis Reviewed

    Tomohiro Ohgomori, Ryo Yamasaki, Hideyuki Takeuchi, Kenji Kadomatsu, Jun-Ichi Kira, Shozo Jinno

    European Journal of Neuroscience   46 ( 4 )   2001 - 2014   2017.8

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    DOI： 10.1111/ejn.13650

  • Differential involvement of vesicular and glial glutamate transporters around spinal α-motoneurons in the pathogenesis of SOD1G93A mouse model of amyotrophic lateral sclerosis Reviewed

    356   114 - 124   2017.7

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    From a view point of the glutamate excitotoxicity theory, several studies have suggested that abnormal glutamate homeostasis via dysfunction of glial glutamate transporter-1 (GLT-1) may underlie neurodegeneration in amyotrophic lateral sclerosis (ALS). However, the detailed role of GLT-1 in the pathogenies of ALS remains controversial. To assess this issue, here we elucidated structural alterations associated with dysregulation of glutamate homeostasis using SOD1G93A mice, a genetic model of familial ALS. We first examined the viability of α-motoneurons in the lumbar spinal cord of SOD1G93A mice. Measurement of the soma size and density indicated that α-motoneurons might be intact at 9 weeks of age (presymptomatic stage), then soma shrinkage began at 15 weeks of age (progressive stage), and finally neuronal density declined at 21 weeks of age (end stage). Next, we carried out the line profile analysis, and found that the coverage of α-motoneurons by GLT-1-positive (GLT-1+) astrocytic processes was decreased only at 21 weeks of age, while the reduction of coverage of α-motoneurons by synaptophysin-positive (SYP+) presynaptic terminals began at 15 weeks of age. Interestingly, the coverage of α-motoneurons by VGluT2+ presynaptic terminals was transiently increased at 9 weeks of age, and then gradually decreased towards 21 weeks of age. On the other hand, there were no time-dependent alterations in the coverage of α-motoneurons by GABAergic presynaptic terminals. These findings suggest that VGluT2 and GLT-1 may be differentially involved in the pathogenesis of ALS via abnormal glutamate homeostasis at the presymptomatic stage and end stage of disease, respectively.

    DOI： 10.1016/j.neuroscience.2017.05.014

  • Plexin D1は小径無髄後根神経節神経細胞を標的とした免疫介在性神経障害性疼痛の責任自己抗原である

    藤井敬之, 山崎亮, 飯沼今日子, 土本大介, 城戸瑞穂, 相島慎一, 中別府雄作, 吉良潤一

    日本末梢神経学会学術集会プログラム・抄録   28th   83   2017.7

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  • Differential activation of neuronal and glial STAT3 in the spinal cord of the SOD1G93A mouse model of amyotrophic lateral sclerosis.

    Ohgomori T, Yamasaki R, Takeuchi H, Kadomatsu K, Kira JI, Jinno S

    Neuroscience   356   114-124   2017.7

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    Differential activation of neuronal and glial STAT3 in the spinal cord of the SOD1G93A mouse model of amyotrophic lateral sclerosis.

    DOI： 10.1016/j.neuroscience.2017.05.014

  • Polymorphic regulation of mitochondrial fission and fusion modifies phenotypes of microglia in neuroinflammation. Reviewed International journal

    Mitsuhiko Katoh, Bao Wu, Huy Bang Nguyen, Truc Quynh Thai, Ryo Yamasaki, Haiyan Lu, Anna M Rietsch, Musab M Zorlu, Youichi Shinozaki, Yurika Saitoh, Sei Saitoh, Takashi Sakoh, Kazuhiro Ikenaka, Schuichi Koizumi, Richard M Ransohoff, Nobuhiko Ohno

    Scientific reports   7 ( 1 )   4942 - 4942   2017.7

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    DOI： 10.1038/s41598-017-05232-0

  • Differential involvement of vesicular and glial glutamate transporters around spinal α-motoneurons in the pathogenesis of SOD1G93A mouse model of amyotrophic lateral sclerosis. Reviewed International journal

    356   114 - 124   2017.7

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    DOI： 10.1016/j.neuroscience.2017.05.014

  • Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies. Reviewed International journal

    Ryo Yamasaki

    J Neurol Neurosurg Psychiatry   2017.6

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  • Stiff-person症候群やIsaacs症候群との鑑別を要した汎下垂体機能低下症の1例

    水野 裕理, 山口 浩雄, 上原 平, 山下 謙一郎, 山崎 亮, 吉良 潤一

    臨床神経学   57 ( 6 )   298 - 302   2017.6

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    症例は56歳女性。出産時大量出血した既往がある。53歳頃より両下肢の筋硬直と腰曲がりが徐々に出現した。初診時は股関節と膝関節の可動域制限があり、触診で下肢筋に硬直と疼痛を認めた。抗VGKC複合体抗体が弱陽性だったことから、stiff-person症候群やIsaacs症候群を疑ったがジアセパム内服に反応しなかった。一方、血液検査で内分泌学的異常を認めたため精査し、出産時出血後の汎下垂体機能低下症と診断した。ホルモン補充療法の開始後下肢筋の硬直が著明に改善した。汎下垂体機能低下症でstiff-person症候群様の症状を来す例を稀に認め、鑑別に挙げる必要がある。(著者抄録)

  • Astrocytic connexin 30 deletion leads to hypersensitivity in a MPTP mouse model of Parkinson's disease

    A. Fujita, H. Yamaguchi, Y. Matsuoka, K. -I. Yamada, R. Yamasaki, J. -I. Kira

    GLIA   65   E182 - E182   2017.6

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  • Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies. Reviewed International journal

    Haruki Koike, Masato Kadoya, Ken-Ichi Kaida, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Daisuke Kato, Hidenori Ogata, Ryo Yamasaki, Noriyuki Matsukawa, Jun-Ichi Kira, Masahisa Katsuno, Gen Sobue

    Journal of neurology, neurosurgery, and psychiatry   88 ( 6 )   465 - 473   2017.6

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    DOI： 10.1136/jnnp-2016-314895

  • Cerebral blood flow laterality derived from arterial spin labeling as a biomarker for assessing the disease severity of parkinson's disease. Reviewed International journal

    Koji Yamashita, Akio Hiwatashi, Osamu Togao, Kazufumi Kikuchi, Hiroo Yamaguchi, Yuriko Suzuki, Ryotaro Kamei, Ryo Yamasaki, Jun-Ichi Kira, Hiroshi Honda

    Journal of magnetic resonance imaging : JMRI   45 ( 6 )   1821 - 1826   2017.6

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    DOI： 10.1002/jmri.25489

  • A case of stiff-person syndrome due to secondary adrenal insufficiency. Reviewed

    Yuri Mizuno, Hiroo Yamaguchi, Taira Uehara, Kenichiro Yamashita, Ryo Yamasaki, Jun-Ichi Kira

    Rinsho shinkeigaku = Clinical neurology   57 ( 6 )   298 - 302   2017.6

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    Language：Japanese   Publishing type：Research paper (scientific journal)  

    DOI： 10.5692/clinicalneurol.cn-001008

  • Paranodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies Reviewed

    Haruki Koike, Masato Kadoya, Ken Ichi Kaida, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Daisuke Kato, Hidenori Ogata, Ryo Yamasaki, Noriyuki Matsukawa, Jun-Ichi Kira, Masahisa Katsuno, Gen Sobue

    Journal of Neurology, Neurosurgery and Psychiatry   88 ( 6 )   465 - 473   2017.6

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    DOI： 10.1136/jnnp-2016-314895

  • Cerebral blood flow laterality derived from arterial spin labeling as a biomarker for assessing the disease severity of parkinson's disease Reviewed

    Koji Yamashita, Hiwatashi Akio, Osamu Togao, kazufumi kikuchi, hiroo yamaguchi, Yuriko Suzuki, Ryotaro Kamei, Ryo Yamasaki, Jun-Ichi Kira, Hiroshi Honda

    Journal of Magnetic Resonance Imaging   45 ( 6 )   1821 - 1826   2017.6

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    DOI： 10.1002/jmri.25489

  • Cerebral blood flow laterality derived from arterial spin labeling as a biomarker for assessing the disease severity of parkinson's disease. Reviewed International journal

    Ryo Yamasaki

    J Magn Reson Imaging.   2017.6

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  • HLA-DRB1*04:05 allele is associated with intracortical lesions on three-dimensional double inversion recovery images in Japanese patients with multiple sclerosis. Reviewed International journal

    Ryo Yamasaki

    Mult Scler   2017.5

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  • Relationship between Th1 cells and astrocytic connexin 43 gap junctions in multiple sclerosis

    Mitsuru Watanabe, Ryo Yamasaki, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   8 ( 2 )   101 - 102   2017.5

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    DOI： 10.1111/cen3.12384

  • Early and extensive spinal white matter involvement in neuromyelitis optica. Reviewed International journal

    Shotaro Hayashida, Katsuhisa Masaki, Tomomi Yonekawa, Satoshi O Suzuki, Akio Hiwatashi, Takuya Matsushita, Mitsuru Watanabe, Ryo Yamasaki, Toshihiko Suenaga, Toru Iwaki, Hiroyuki Murai, Jun-Ichi Kira

    Brain pathology (Zurich, Switzerland)   27 ( 3 )   249 - 265   2017.5

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    DOI： 10.1111/bpa.12386

  • Relationship between Th1 cells and astrocytic connexin 43 gap junctions in multiple sclerosis Reviewed

    Mitsuru Watanabe, Ryo Yamasaki, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   8 ( 2 )   101 - 102   2017.5

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    DOI： 10.1111/cen3.12384

  • HLA-DRB1*04:05 allele is associated with intracortical lesions on three-dimensional double inversion recovery images in Japanese patients with multiple sclerosis Reviewed

    Koji Shinoda, Takuya Matsushita, Yuri Nakamura, Katsuhisa Masaki, Ryo Yamasaki, hiroo yamaguchi, Osamu Togao, Akio Hiwatashi, Jun-Ichi Kira

    Multiple Sclerosis   1352458517707067   2017.5

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    DOI： 10.1177/1352458517707067

  • Apomorphine Therapy for Neuronal Insulin Resistance in a Mouse Model of Alzheimer's Disease. Reviewed International journal

    Ryo Yamasaki

    J Alzheimer's disease   2017.5

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  • Differential involvement of vesicular and glial glutamate transporters around spinal α-motoneurons in the pathogenesis of SOD1(G93A) mouse model of amyotrophic lateral sclerosis. Reviewed International journal

    2017.5

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  • Early and extensive spinal white matter involvement in neuromyelitis optica. Invited Reviewed International journal

    Ryo Yamasaki

    Brain Pathol   2017.5

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  • Early strong intrathecal inflammation in cerebellar type multiple system atrophy by cerebrospinal fluid cytokine/chemokine profiles A case control study Reviewed

    Ryo Yamasaki, Hiroo Yamaguchi, Takuya Matsushita, Takayuki Fujii, Akio Hiwatashi, Jun ichi Kira

    Journal of neuroinflammation   14 ( 1 )   2017.4

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    DOI： 10.1186/s12974-017-0863-0

  • [Longitudinal Ultrasonography Detected a Guidewire Outside the Internal Jugular Vein Left during Adult Central Venous Catheter Placement]. Reviewed

    Ryo Yamasaki, Kenji Kayashima

    Masui. The Japanese journal of anesthesiology   66 ( 4 )   420 - 423   2017.4

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  • MRI CISS(constructive interference in steady state)法により出血源の硬膜欠損部が同定でき、硬膜閉鎖術が有効であった脳表ヘモジデリン沈着症の1例 Reviewed

    迫田 礼子, 山下 謙一郎, 林田 光正, 岩本 幸英, 山崎 亮, 吉良 潤一

    臨床神経学   57 ( 4 )   180 - 183   2017.4

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    症例は64歳男性である。1998年に頭部を打撲し慢性硬膜下血腫を来した既往がある。2000年から頭痛、ふらつき、聴力低下が出現し緩徐に増悪した。2011年、他院精査で血性髄液を認め、MRIでT2/T2*強調画像にて脳・脊髄表面に沿った低信号病変を認めた。脳表ヘモジデリン沈着症と診断されるも出血源は不明で、止血剤も無効であった。2015年当科受診時、水平性注視方向性眼振、両側高度感音性難聴、四肢・体幹失調を認め、MRIではCISS法にてTh2～3レベル硬膜前面に欠損を認めた。硬膜欠損に対して硬膜欠損閉鎖術を行ったところ、血性髄液の改善を認め、術後頭痛、失調の改善を認めた。(著者抄録)

    DOI： 10.5692/clinicalneurol.cn-000960

  • Early strong intrathecal inflammation in cerebellar type multiple system atrophy by cerebrospinal fluid cytokine/chemokine profiles: a case control study. Reviewed International journal

    Ryo Yamasaki, Hiroo Yamaguchi, Takuya Matsushita, Takayuki Fujii, Akio Hiwatashi, Jun-Ichi Kira

    Journal of neuroinflammation   14 ( 1 )   89 - 89   2017.4

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    DOI： 10.1186/s12974-017-0863-0

  • Evaluation of chronic inflammatory demyelinating polyneuropathy: 3D nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (3D SHINKEI). Reviewed International journal

    Ryo Yamasaki

    Eur Radiol   2017.2

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  • The Effects of the HLA-DRB1*04:05 Allele on Intracortical Lesions Detected by 3-Dimensional Double Inversion Recovery Imaging in Japanese Patients with Multiple Sclerosis

    K. Shinoda, T. Matsushita, Y. Nakamura, K. Masaki, R. Yamasaki, O. Togao, A. Hiwatashi, J-I Kira

    MULTIPLE SCLEROSIS JOURNAL   23 ( 2 )   313 - 313   2017.2

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  • Experimental Autoimmune Encephalomyelitis (EAE) is Ameliorated in Mice with Gray Matter (GM) Astrocyte-Specific Inducible Conditional Connexin 43 Knock-Out (Cx43cKO)

    Hayato Une, Hiroo Yamaguchi, Yinan Zhao, Koji Shinoda, Katsuhisa Masaki, Magdalena Goetz, Ryo Yamasaki, Jun-ichi Kira

    MULTIPLE SCLEROSIS JOURNAL   23 ( 2 )   319 - 319   2017.2

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  • Distinct Repertoires of CD4 and CD8 T Cells in Multiple Sclerosis Patients with and without Deletion-type Copy Number Variations

    Guzailiayi Maimaitijiang, Koji Shinoda, Yuri Nakamura, Katsuhisa Masaki, Takuya Matsushita, Ryo Yamasaki, Yasunobu Yoshikai, Jun-ichi Kira

    MULTIPLE SCLEROSIS JOURNAL   23 ( 2 )   315 - 315   2017.2

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  • Allergic inflammation leads to neuropathic pain through glial cell activation

    Ryo Yamasaki, Takayuki Fujii

    Clinical and Experimental Neuroimmunology   8 ( 1 )   7 - 8   2017.2

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    DOI： 10.1111/cen3.12372

  • Evaluation of chronic inflammatory demyelinating polyneuropathy: 3D nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (3D SHINKEI). Reviewed International journal

    Akio Hiwatashi, Osamu Togao, Koji Yamashita, Kazufumi Kikuchi, Hidenori Ogata, Ryo Yamasaki, Masami Yoneyama, Jun-Ichi Kira, Hiroshi Honda

    European radiology   27 ( 2 )   447 - 453   2017.2

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    DOI： 10.1007/s00330-016-4406-3

  • Evaluation of chronic inflammatory demyelinating polyneuropathy 3D nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (3D SHINKEI) Reviewed

    Akio Hiwatashi, Osamu Togao, Koji Yamashita, Kazufumi Kikuchi, Hidenori Ogata, Ryo Yamasaki, Masami Yoneyama, Jun ichi Kira, Hiroshi Honda

    European Radiology   27 ( 2 )   447 - 453   2017.2

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    DOI： 10.1007/s00330-016-4406-3

  • Allergic inflammation leads to neuropathic pain through glial cell activation Reviewed

    Ryo Yamasaki, Takayuki Fujii

    Clinical and Experimental Neuroimmunology   8 ( 1 )   7 - 8   2017.2

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    DOI： 10.1111/cen3.12372

  • A case of stiff-person syndrome due to secondary adrenal insufficiency Reviewed

    Yuri Mizuno, Hiroo Yamaguchi, Taira Uehara, Kenichiro Yamashita, Ryo Yamasaki, Jun Ichi Kira

    Clinical Neurology   57 ( 6 )   298 - 302   2017.1

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    DOI： 10.5692/clinicalneurol.cn-001008

  • Apomorphine Therapy for Neuronal Insulin Resistance in a Mouse Model of Alzheimer's Disease Reviewed

    Norimichi Nakamura, Yasumasa Ohyagi, Tomohiro Imamura, Yuki T. Yanagihara, Kyoko M. Iinuma, Naoko Soejima, Hiroyuki Murai, Ryo Yamasaki, Jun Ichi Kira

    Journal of Alzheimer's Disease   58 ( 4 )   1151 - 1161   2017.1

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    DOI： 10.3233/JAD-160344

  • A case of superficial siderosis ameliorated after closure of dural deficit detected by MRI-CISS (constructive interference in steady state) imaging Reviewed

    Ayako Sakoda, Ken Ichiro Yamashita, Mitsumasa Hayashida, Yukihide Iwamoto, Ryo Yamasaki, Jun Ichi Kira

    Clinical Neurology   57 ( 4 )   180 - 183   2017.1

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    DOI： 10.5692/clinicalneurol.cn-000960

  • Th1 cells downregulate connexin 43 gap junctions in astrocytes via microglial activation. Reviewed International journal

    Ryo Yamasaki

    Sci Rep   2016.12

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  • IgG4 anti-neurofascin155 antibodies in chronic inflammatory demyelinating polyradiculoneuropathy: Clinical significance and diagnostic utility of a conventional assay. Reviewed International journal

    Masato Kadoya, Kenichi Kaida, Haruki Koike, Hiroshi Takazaki, Hidenori Ogata, Kota Moriguchi, Jun Shimizu, Eiichiro Nagata, Shunya Takizawa, Atsuro Chiba, Ryo Yamasaki, Jun-Ichi Kira, Gen Sobue, Katsunori Ikewaki

    Journal of neuroimmunology   301   16 - 22   2016.12

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    DOI： 10.1016/j.jneuroim.2016.10.013

  • Th1 cells downregulate connexin 43 gap junctions in astrocytes via microglial activation Reviewed

    Mitsuru Watanabe, Katsuhisa Masaki, Ryo Yamasaki, Jun Kawanokuchi, Hideyuki Takeuchi, Takuya Matsushita, Akio Suzumura, Jun Ichi Kira

    Scientific reports   6   2016.12

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    DOI： 10.1038/srep38387

  • Minocycline-induced human herpesvirus 6 encephalomyelitis with drastically disseminated contrast-enhanced lesions Reviewed

    K. Shinoda, T. Iwata, Y. Nakamura, K. Masaki, T. Matsushita, R. Yamasaki, J. I. Kira

    European Journal of Neurology   23 ( 12 )   e76 - e77   2016.12

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    DOI： 10.1111/ene.13190

  • IgG4 anti-neurofascin155 antibodies in chronic inflammatory demyelinating polyradiculoneuropathy Clinical significance and diagnostic utility of a conventional assay Reviewed

    Masato Kadoya, Kenichi Kaida, Haruki Koike, Hiroshi Takazaki, Hidenori Ogata, Kota Moriguchi, Jun Shimizu, Eiichiro Nagata, Shunya Takizawa, Atsuro Chiba, Ryo Yamasaki, Jun ichi Kira, Gen Sobue, Katsunori Ikewaki

    Journal of Neuroimmunology   301   16 - 22   2016.12

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    DOI： 10.1016/j.jneuroim.2016.10.013

  • Autoantibodies in chronic inflammatory neuropathies Reviewed

    Ryo Yamasaki

    Brain and Nerve   68 ( 12 )   1415 - 1421   2016.12

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  • Th1 cells downregulate connexin 43 gap junctions in astrocytes via microglial activation. Reviewed International journal

    Mitsuru Watanabe, Katsuhisa Masaki, Ryo Yamasaki, Jun Kawanokuchi, Hideyuki Takeuchi, Takuya Matsushita, Akio Suzumura, Jun-Ichi Kira

    Scientific reports   6   38387 - 38387   2016.12

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    DOI： 10.1038/srep38387

  • 抗neurofascin155抗体陽性CIDP症例における臨床・神経伝導検査・抗体検査所見の経時的変化からみた早期再発マーカーの同定

    緒方 英紀, 山崎 亮, 吉良 潤一

    末梢神経   27 ( 2 )   265 - 265   2016.12

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  • 慢性免疫性ニューロパチーのメカニズム CIDPとランビエ絞輪部を標的とした自己抗体

    緒方 英紀, 山崎 亮, 吉良 潤一

    末梢神経   27 ( 2 )   164 - 169   2016.12

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    近年、ランビエ絞輪部およびその周辺の分子機構の解明が進み、自己抗体の標的部位としての側面にも注目が集まるようになった。特にランビエ傍絞輪部の細胞膜蛋白であるcontactin-1およびneurofascin155に対する自己抗体が慢性炎症性脱髄性多発根ニューロパチー症例の一部で陽性になることが立て続けに報告され、臨床的特徴が明らかにされつつある。治療反応性に関する報告もなされ、今後これらの自己抗体が診断および治療選択マーカーとして用いられることが期待される。(著者抄録)

  • アレルギー疾患におけるET1/EDNRB経路を介するアロディニアの発生

    山崎 亮, 藤井 敬之, 方 梅, 王 冰, 城戸 瑞穂, 吉良 潤一

    末梢神経   27 ( 2 )   280 - 280   2016.12

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  • Minocycline-induced human herpesvirus 6 encephalomyelitis with drastically disseminated contrast-enhanced lesions

    K. Shinoda, T. Iwata, Y. Nakamura, K. Masaki, T. Matsushita, R. Yamasaki, J. -I. Kira

    EUROPEAN JOURNAL OF NEUROLOGY   23 ( 12 )   E76 - E77   2016.12

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    DOI： 10.1111/ene.13190

  • Recurrent Hemorrhagic Venous Infarctions Caused by Thrombosis of a Pontine Developmental Venous Anomaly and Protein S Mutation. Reviewed International journal

    Ryo Yamasaki

    Cerebrovasc Dis   2016.11

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  • Recurrent Hemorrhagic Venous Infarctions Caused by Thrombosis of a Pontine Developmental Venous Anomaly and Protein S Mutation. Reviewed International journal

    Yuri Nakamura, Kei-Ichiro Takase, Takuya Matsushita, Satoshi Yoshimura, Ryo Yamasaki, Hiroyuki Murai, Kazufumi Kikuchi, Jun-Ichi Kira

    Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association   25 ( 11 )   e216-e217 - e217   2016.11

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    DOI： 10.1016/j.jstrokecerebrovasdis.2016.08.040

  • Peripheral blood T cell subset characteristics of multiple sclerosis in remission phase correlate with annualized relapse rates Reviewed

    Zi-Ye Song, Yuri Nakamura, Ryo Yamasaki, Yuji Kawano, Koji Shinoda, Maimaitijiang Guzailiayi, Katsuhisa Masaki, Hiroo Yamaguchi, Takuya Matsushita, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   7 ( 4 )   346 - 352   2016.11

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    DOI： 10.1111/cen3.12321

  • Clear detection of lower medullary lesions by three-dimensional double inversion recovery imaging in a patient with neuromyelitis optica spectrum disorder Reviewed

    Satoshi Nagata, Koji Shinoda, Taira Uehara, Takuya Matsushita, Ryo Yamasaki, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   7 ( 4 )   355 - 356   2016.11

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    DOI： 10.1111/cen3.12330

  • Allergic Inflammation Leads to Neuropathic Pain via Glial Cell Activation. Reviewed International journal

    Ryo Yamasaki, Takayuki Fujii, Bing Wang, Katsuhisa Masaki, Mizuho A Kido, Mari Yoshida, Takuya Matsushita, Jun-Ichi Kira

    The Journal of neuroscience : the official journal of the Society for Neuroscience   36 ( 47 )   11929 - 11945   2016.11

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    DOI： 10.1523/JNEUROSCI.1981-16.2016

  • Recurrent Hemorrhagic Venous Infarctions Caused by Thrombosis of a Pontine Developmental Venous Anomaly and Protein S Mutation Reviewed

    Yuri Nakamura, Kei ichiro Takase, Takuya Matsushita, Satoshi Yoshimura, Ryo Yamasaki, Hiroyuki Murai, Kazufumi Kikuchi, Jun ichi Kira

    Journal of Stroke and Cerebrovascular Diseases   25 ( 11 )   e216 - e217   2016.11

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    DOI： 10.1016/j.jstrokecerebrovasdis.2016.08.040

  • Peripheral blood T cell subset characteristics of multiple sclerosis in remission phase correlate with annualized relapse rates Reviewed

    Zi Ye Song, Yuri Nakamura, Ryo Yamasaki, Yuji Kawano, Koji Shinoda, Maimaitijiang Guzailiayi, Katsuhisa Masaki, Hiroo Yamaguchi, Takuya Matsushita, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   7 ( 4 )   346 - 352   2016.11

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    DOI： 10.1111/cen3.12321

  • Clear detection of lower medullary lesions by three-dimensional double inversion recovery imaging in a patient with neuromyelitis optica spectrum disorder Reviewed

    Satoshi Nagata, Koji Shinoda, Taira Uehara, Takuya Matsushita, Ryo Yamasaki, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   7 ( 4 )   355 - 356   2016.11

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    DOI： 10.1111/cen3.12330

  • Allergic inflammation leads to neuropathic pain via glial cell activation Reviewed

    Ryo Yamasaki, Takayuki Fujii, Bing Wang, Katsuhisa Masaki, Mizuho A. Kido, Mari Yoshida, Takuya Matsushita, Jun Ichi Kira

    Journal of Neuroscience   36 ( 47 )   11929 - 11945   2016.11

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    DOI： 10.1523/JNEUROSCI.1981-16.2016

  • IgG4 anti-neurofascin155 antibodies in chronic inflammatory demyelinating polyradiculoneuropathy: Clinical significance and diagnostic utility of a conventional assay. Reviewed International journal

    Ryo Yamasaki

    J Neuroimmunol   2016.11

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  • Ultrasonographic Identification of the Thyrocervical Trunk and Vertebral Artery in Adults to Decrease Complications. Reviewed International journal

    Kenji Kayashima, Ryo Yamasaki

    Turkish journal of anaesthesiology and reanimation   44 ( 5 )   275 - 275   2016.10

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  • フィンゴリモド投与によりtumefactive demyelinating lesion(TDL)を伴って再発した多発性硬化症の一例

    田中 栄蔵, 橋本 侑, 茶谷 裕, 山崎 亮, 吉良 潤一

    臨床神経学   56 ( 10 )   724 - 724   2016.10

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  • Ultrasonographic identification of the thyrocervical trunk and vertebral artery in adults to decrease complications

    Kenji Kayashima, Ryo Yamasaki

    Turk Anesteziyoloji ve Reanimasyon Dernegi Dergisi   44 ( 5 )   275   2016.10

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    DOI： 10.5152/TJAR.2016.02679

  • Immunopathological Study of Glucose and Lactate Transporters in White and Gray Matter Lesions of Multiple Sclerosis and Neuromyelitis Optica

    Katsuhisa Masaki, Satoshi O. Suzuki, Shotaro Hayashida, Mitsuru Watanabe, Takuya Matsushita, Ryo Yamasaki, Makiko Suzuki, Toshihiko Suenaga, Toru Iwaki, Jun-ichi Kira

    ANNALS OF NEUROLOGY   80   S80 - S80   2016.10

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  • Functional Analysis of Cx30 in Experimental Autoimmune Encephalomyelitis

    Ryo Yamasaki, Mei Fang, Hiroo Yamaguchi, Jun-Ichi Kira

    ANNALS OF NEUROLOGY   80   S175 - S176   2016.10

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  • Efficacy of intravenous methylprednisolone pulse therapy in patients with multiple sclerosis and neuromyelitis optica. Reviewed International journal

    Ryo Yamasaki

    Mult Scler   2016.9

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  • 誘導性conditional connexin 43ノックアウトマウスにおけるExperimental autoimmune encephalomyelitis(Experimental autoimmune encephalomyelitis in mice with induced conditional connexin 43 knock-out)

    宇根 隼人, 山口 浩雄, Yinan Zhao, 篠田 紘司, 真崎 勝久, Magdalena Gotz, 山崎 亮, 吉良 潤一

    神経免疫学   21 ( 1 )   114 - 114   2016.9

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  • 筋萎縮性側索硬化症の脊髄では活性早期ミクログリアは灰白質に、活性後期ミクログリアは白質に分布する

    林 信太郎, 山崎 亮, 村井 弘之, 岡本 幸市, 吉良 潤一

    神経免疫学   21 ( 1 )   138 - 138   2016.9

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  • 実験的自己免疫性脳脊髄炎における大グリア細胞コネキシン30の新規の役割(Novel roles of astroglial connexin30 in experimental autoimmune encephalomyelitis)

    方 梅, 山崎 亮, 山口 浩雄, 真崎 勝久, 李 広瑞, 篠田 紘司, 吉良 潤一

    神経免疫学   21 ( 1 )   151 - 151   2016.9

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  • 多発性硬化症、視神経脊髄炎におけるグルコース・乳酸担体の神経病理学的検討

    眞崎 勝久, 鈴木 諭, 林田 翔太郎, 渡邉 充, 松下 拓也, 山崎 亮, 岩城 徹, 吉良 潤一

    神経免疫学   21 ( 1 )   121 - 121   2016.9

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  • VALUABLE OBJECTIVE MARKERS FOR PREDICTING ANTI-NEUROFASCIN 155 ANTIBODY STATUS AMONG CIDP PATIENTS

    H. Ogata, R. Yamasaki, N. Oka, M. Kuwahara, H. Suzuki, S. Kusunoki, Y. Yagi, T. Matsushita, T. Yokota, J. Kira

    JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM   21 ( 3 )   196 - 196   2016.9

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  • IFNγはミクログリアにIL-1β/TNFαを産生させてアストロサイトコネキシン43発現を低下させる

    渡邉 充, 眞崎 勝久, 山崎 亮, 川ノ口 潤, 竹内 英之, 錫村 明生, 吉良 潤一

    神経免疫学   21 ( 1 )   118 - 118   2016.9

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  • IFN gamma reduces expression of connexin43 in astrocytes via activation of microglia

    M. Watanabe, K. Masaki, R. Yamasaki, J. Kawanokuchi, H. Takeuchi, A. Suzumura, J. -I. Kira

    MULTIPLE SCLEROSIS JOURNAL   22   159 - 159   2016.9

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  • Latitude and HLA-DRB1*04:05 independently influence disease severity in Japanese multiple sclerosis: a cross-sectional study. Reviewed International journal

    Yuri Nakamura, Takuya Matsushita, Shinya Sato, Masaaki Niino, Toshiyuki Fukazawa, Satoshi Yoshimura, Shin Hisahara, Noriko Isobe, Shun Shimohama, Mitsuru Watanabe, Kazuto Yoshida, Hideki Houzen, Yusei Miyazaki, Ryo Yamasaki, Seiji Kikuchi, Jun-Ichi Kira

    Journal of neuroinflammation   13 ( 1 )   239 - 239   2016.9

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    DOI： 10.1186/s12974-016-0695-3

  • Efficacy of intravenous methylprednisolone pulse therapy in patients with multiple sclerosis and neuromyelitis optica. Reviewed International journal

    Ryo Yamasaki, Takuya Matsushita, Toshiyuki Fukazawa, Kazumasa Yokoyama, Kazuo Fujihara, Mieko Ogino, Takanori Yokota, Katsuichi Miyamoto, Masaaki Niino, Kyoichi Nomura, Ryo Tomioka, Masami Tanaka, Izumi Kawachi, Takashi Ohashi, Ken-Ichi Kaida, Makoto Matsui, Yuji Nakatsuji, Hirofumi Ochi, Hikoaki Fukaura, Takashi Kanda, Akiko Nagaishi, Kanae Togo, Hidehiro Mizusawa, Hiroyuki Murai, Jun-Ichi Kira

    Multiple sclerosis (Houndmills, Basingstoke, England)   22 ( 10 )   1337 - 48   2016.9

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    DOI： 10.1177/1352458515617248

  • Latitude and HLA-DRB1*04:05 independently influence disease severity in Japanese multiple sclerosis A cross-sectional study Reviewed

    Yuri Nakamura, Takuya Matsushita, Shinya Sato, Masaaki Niino, Toshiyuki Fukazawa, Satoshi Yoshimura, Shin Hisahara, Noriko Isobe, Shun Shimohama, Mitsuru Watanabe, Kazuto Yoshida, Hideki Houzen, Yusei Miyazaki, Ryo Yamasaki, Seiji Kikuchi, Jun ichi Kira

    Journal of neuroinflammation   13 ( 1 )   2016.9

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    DOI： 10.1186/s12974-016-0695-3

  • Efficacy of intravenous methylprednisolone pulse therapy in patients with multiple sclerosis and neuromyelitis optica Reviewed

    Ryo Yamasaki, Takuya Matsushita, Toshiyuki Fukazawa, Kazumasa Yokoyama, Kazuo Fujihara, Mieko Ogino, Takanori Yokota, Katsuichi Miyamoto, Masaaki Niino, Kyoichi Nomura, Ryo Tomioka, Masami Tanaka, Izumi Kawachi, Takashi Ohashi, Ken Ichi Kaida, Makoto Matsui, Yuji Nakatsuji, Hirofumi Ochi, Hikoaki Fukaura, Takashi Kanda, Akiko Nagaishi, Kanae Togo, Hidehiro Mizusawa, Hiroyuki Murai, Jun Ichi Kira

    Multiple Sclerosis   22 ( 10 )   1337 - 1348   2016.9

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    DOI： 10.1177/1352458515617248

  • T細胞受容体領域に欠失型copy number variationを有する多発性硬化症の免疫学的解析

    篠田 紘司, グザリアイ・ママティジャン, 中村 優理, 佐藤 眞也, 眞崎 勝久, 松下 拓也, 山崎 亮, 吉開 泰信, 吉良 潤一

    日本臨床免疫学会会誌   39 ( 4 )   389 - 389   2016.8

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  • 【ミクログリアと疼痛】末梢のアトピー素因に起因する疼痛とグリア細胞

    山崎 亮, 吉良 潤一

    細胞   48 ( 8 )   373 - 376   2016.8

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    気管支喘息やアトピー性皮膚炎、アレルギー性鼻炎などのアトピー性疾患は増加傾向にあるが、これらの末梢性アレルギー炎症が中枢神経系に及ぼす影響は明らかでない。我々は最近、気管支喘息モデルマウスが神経因性疼痛(異痛症、アロディニア)を呈することを発見した。これらのマウス脊髄ではミクログリアとアストログリアの活性化(=グリア炎症)が見られた。また、これらのグリア細胞表面ではエンドセリン受容体EDNRBの発現亢進を認め、EDNRBの選択的拮抗薬BQ788は同マウスのアロディニアをほぼ完全に抑制した。これらの結果から、アトピー素因を持つ患者では中枢グリア炎症が起こっている可能性が示唆された。グリア炎症は多発性硬化症や筋萎縮性側索硬化症などの神経疾患でもしばしば見られる所見であり、疾患の重症度との関連性が指摘されていることから、末梢のアレルギー炎症が中枢神経疾患を悪化させている可能性が考えられた。(著者抄録)

  • 【免疫性・炎症性神経疾患-病態解明から疾患修飾薬開発まで-】《中枢神経疾患》 アトピー性脊髄炎

    藤井 敬之, 山崎 亮, 吉良 潤一

    36 ( 7 )   678 - 681   2016.7

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    ＜ポイント＞アトピー性脊髄炎(AM)はアトピー性皮膚炎、気管支喘息等のアトピー患者でみられる原因不明の脊髄炎である。AM患者の80%でアロディニア・神経障害性疼痛が持続する。AMの病変は頸髄後索寄りに起こりやすく、病理組織学的特徴としてさまざまな程度の好酸球浸潤ならびにミクログリアとアストログリアの活性化を認める。AMの治療では、ステロイド療法ならびに血漿交換療法が有効である。(著者抄録)

  • 【免疫性・炎症性神経疾患-病態解明から疾患修飾薬開発まで-】《免疫性・炎症性神経疾患のトピックス》 ランビエ傍絞輪部を標的としたIgG4自己抗体によるCIDP

    緒方 英紀, 山崎 亮, 吉良 潤一

    36 ( 7 )   773 - 776   2016.7

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    Language：Japanese  

    ＜ポイント＞ランビエ傍絞輪部の細胞膜蛋白であるcontactin-1およびneurofascin 155(NF155)に対する自己抗体が一部のCIDP症例で陽性になることが相次いで報告されている。両蛋白に対する自己抗体のIgGサブクラスは大半の症例でIgG4が優位である。抗contactin-1抗体、抗NF155抗体陽性症例はそれぞれ特徴的な臨床・検査所見を呈する。抗contactin-1抗体、抗NF155抗体陽性症例に対してはIVIgのみの治療では不十分な可能性がある。今後両蛋白に対する自己抗体は診断および治療選択マーカーとして用いられることが期待される。(著者抄録)

  • Immune-mediated spastic paraparesis accompanied with high titres of voltage-gated potassium channel complex antibodies and myokymia/fasciculation Reviewed

    Ban Yu Saitoh, Shintaro Hayashi, Katsuya Ogata, Taira Uehara, Hikaru Doi, Osamu Watanabe, Ryo Yamasaki, Hiroyuki Murai, Jun Ichi Kira

    Journal of the Neurological Sciences   364   133 - 135   2016.5

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    DOI： 10.1016/j.jns.2016.03.028

  • Immune-mediated spastic paraparesis accompanied with high titres of voltage-gated potassium channel complex antibodies and myokymia/fasciculation. Reviewed International journal

    Ban-Yu Saitoh, Shintaro Hayashi, Katsuya Ogata, Taira Uehara, Hikaru Doi, Osamu Watanabe, Ryo Yamasaki, Hiroyuki Murai, Jun-Ichi Kira

    Journal of the neurological sciences   364   133 - 5   2016.5

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    DOI： 10.1016/j.jns.2016.03.028

  • Mutations in MME cause an autosomal-recessive Charcot-Marie-Tooth disease type 2. Reviewed International journal

    Ryo Yamasaki

    Ann Neurol.   2016.4

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  • The Low Frequency of Antibodies to Myelin Oligodendrocyte Glycoprotein in Japanese Opticospinal Multiple Sclerosis

    Sudarshini Ramanathan, Shinya Sato, Takuya Matsushita, Katsuhisa Masaki, Ryo Yamasaki, Russell Dale, Jun-ichi Kira, Fabienne Brilot

    NEUROLOGY   86   2016.4

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  • The Low Frequency of Antibodies to Myelin Oligodendrocyte Glycoprotein in Japanese Opticospinal Multiple Sclerosis

    Sudarshini Ramanathan, Shinya Sato, Takuya Matsushita, Katsuhisa Masaki, Ryo Yamasaki, Russell Dale, Jun-ichi Kira, Fabienne Brilot

    NEUROLOGY   86   2016.4

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  • Mutations in MME cause an autosomal-recessive Charcot-Marie-Tooth disease type 2. Reviewed International journal

    Yujiro Higuchi, Akihiro Hashiguchi, Junhui Yuan, Akiko Yoshimura, Jun Mitsui, Hiroyuki Ishiura, Masaki Tanaka, Satoshi Ishihara, Hajime Tanabe, Satoshi Nozuma, Yuji Okamoto, Eiji Matsuura, Ryuichi Ohkubo, Saeko Inamizu, Wataru Shiraishi, Ryo Yamasaki, Yasumasa Ohyagi, Jun-ichi Kira, Yasushi Oya, Hayato Yabe, Noriko Nishikawa, Shinsuke Tobisawa, Nozomu Matsuda, Masayuki Masuda, Chiharu Kugimoto, Kazuhiro Fukushima, Satoshi Yano, Jun Yoshimura, Koichiro Doi, Masanori Nakagawa, Shinichi Morishita, Shoji Tsuji, Hiroshi Takashima

    Annals of neurology   79 ( 4 )   659 - 72   2016.4

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    DOI： 10.1002/ana.24612

  • Mutations in MME cause an autosomal-recessive Charcot-Marie-Tooth disease type 2 Reviewed

    Yujiro Higuchi, Akihiro Hashiguchi, Junhui Yuan, Akiko Yoshimura, Jun Mitsui, Hiroyuki Ishiura, Masaki Tanaka, Satoshi Ishihara, Hajime Tanabe, Satoshi Nozuma, Yuji Okamoto, Eiji Matsuura, Ryuichi Ohkubo, Saeko Inamizu, Wataru Shiraishi, Ryo Yamasaki, Yasumasa Ohyagi, Jun Ichi Kira, Yasushi Oya, Hayato Yabe, Noriko Nishikawa, Shinsuke Tobisawa, Nozomu Matsuda, Masayuki Masuda, Chiharu Kugimoto, Kazuhiro Fukushima, Satoshi Yano, Jun Yoshimura, Koichiro Doi, Masanori Nakagawa, Shinichi Morishita, Shoji Tsuji, Hiroshi Takashima

    Annals of Neurology   79 ( 4 )   659 - 672   2016.4

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    DOI： 10.1002/ana.24612

  • Evaluation of chronic inflammatory demyelinating polyneuropathy: 3D nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (3D SHINKEI). Reviewed International journal

    Ryo Yamasaki

    Eur Radiol.   2016.4

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  • Early and Extensive Spinal White Matter Involvement in Neuromyelitis Optica. Reviewed International journal

    Ryo Yamasaki

    Brain Pathol   2016.4

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    DOI： 10.1111/bpa.12386

  • Mutations in MME cause an autosomal-recessive Charcot-Marie-Tooth disease type 2. Invited Reviewed International journal

    Ryo Yamasaki

    Ann Neurol   2016.3

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  • 指定難病最前線(volume8) アトピー性脊髄炎の疫学と診断基準

    藤井 敬之, 山崎 亮, 吉良 潤一

    新薬と臨牀   65 ( 3 )   378 - 382   2016.3

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  • IgG4抗neurofascin 155抗体陽性CIDPの臨床的特徴

    緒方 英紀, 山崎 亮, 樋渡 昭雄, 岡 伸幸, 河村 信利, 松瀬 大, 楠 進, 吉良 潤一

    日本内科学会雑誌   105 ( Suppl. )   267 - 267   2016.2

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  • 【一般内科疾患に伴う脊髄障害】Atopic myelitis

    藤井 敬之, 山崎 亮, 吉良 潤一

    脊椎脊髄ジャーナル   29 ( 2 )   119 - 123   2016.2

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  • A nationwide survey of combined central and peripheral demyelination in Japan Reviewed

    Hidenori Ogata, Dai Matsuse, Ryo Yamasaki, Nobutoshi Kawamura, Takuya Matsushita, Tomomi Yonekawa, Makoto Hirotani, Hiroyuki Murai, Jun Ichi Kira

    Journal of Neurology, Neurosurgery and Psychiatry   87 ( 1 )   29 - 36   2016.1

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    DOI： 10.1136/jnnp-2014-309831

  • 各種疾患 脱髄・免疫性代謝疾患 慢性炎症性脱髄性多発根ニューロパチーにおける自己抗体の重要性

    緒方 英紀, 山崎 亮, 吉良 潤一

    Annual Review神経   2016   206 - 212   2016.1

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    CIDPでは細胞性免疫のみならず液性免疫の関与が想定されているが,類縁疾患であるIgM-MGUS関連脱髄性ニューロパチーにおける抗MAG抗体ほど診断的価値が確立した自己抗体はこれまで同定されていない.近年,ランビエ絞輪部およびその周辺の分子機構の解明が進み,自己抗体の標的部位としての側面にも注目が集まるようになった.特にランビエ傍絞輪部の細胞膜蛋白であるcontactin-1およびneurofascin155に対する自己抗体とCIDPの関連が立て続けに報告され,陽性症例の臨床的特徴が明らかにされつつある.また治療反応性についての報告もなされ,今後これらの自己抗体が診断および治療選択マーカーとして用いられることが期待される.(著者抄録)

  • Antibodies to myelin oligodendrocyte glycoprotein are uncommon in Japanese opticospinal multiple sclerosis. Reviewed International journal

    Sudarshini Ramanathan, Shinya Sato, Takuya Matsushita, Katsuhisa Masaki, Ryo Yamasaki, Russell C Dale, Jun-Ichi Kira, Fabienne Brilot

    Multiple sclerosis (Houndmills, Basingstoke, England)   22 ( 1 )   127 - 8   2016.1

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    DOI： 10.1177/1352458515586089

  • A nationwide survey of combined central and peripheral demyelination in Japan. Reviewed International journal

    Hidenori Ogata, Dai Matsuse, Ryo Yamasaki, Nobutoshi Kawamura, Takuya Matsushita, Tomomi Yonekawa, Makoto Hirotani, Hiroyuki Murai, Jun-ichi Kira

    Journal of neurology, neurosurgery, and psychiatry   87 ( 1 )   29 - 36   2016.1

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    DOI： 10.1136/jnnp-2014-309831

  • Antibodies to myelin oligodendrocyte glycoprotein are uncommon in Japanese opticospinal multiple sclerosis Reviewed

    Sudarshini Ramanathan, Shinya Sato, Takuya Matsushita, Katsuhisa Masaki, Ryo Yamasaki, Russell C. Dale, Jun Ichi Kira, Fabienne Brilot

    Multiple Sclerosis   22 ( 1 )   127 - 128   2016.1

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    DOI： 10.1177/1352458515586089

  • Antibodies to myelin oligodendrocyte glycoprotein are uncommon in Japanese opticospinal multiple sclerosis Reviewed

    Sudarshini Ramanathan, Shinya Sato, Takuya Matsushita, Katsuhisa Masaki, Ryo Yamasaki, Russell C. Dale, Jun Ichi Kira, Fabienne Brilot

    Multiple Sclerosis   22 ( 1 )   127 - 128   2016.1

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    DOI： 10.1177/1352458515586089

  • Holocord involvement with sparing of the peripheral white matter rim in longitudinally extensive spinal cord lesions of neuromyelitis optica Reviewed

    Shotaro Hayashida, Katsuhisa Masaki, Takuya Matsushita, Mitsuru Watanabe, Ryo Yamasaki, Hiroyuki Murai, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   6   78 - 79   2015.12

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    DOI： 10.1111/cen3.12274

  • 視神経脊髄炎における長大な脊髄病巣の進展様式に関する神経画像的・神経病理学的研究

    林田 翔太郎, 眞崎 勝久, 米川 智, 松下 拓也, 山崎 亮, 鈴木 諭, 岩城 徹, 吉良 潤一

    臨床神経学   55 ( Suppl. )   S380 - S380   2015.12

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  • 抗neurofascin 155抗体陽性CIDPの臨床的特徴

    緒方 英紀, 山崎 亮, 樋渡 昭雄, 岡 伸幸, 河村 信利, 松瀬 大, 桑原 基, 鈴木 秀和, 楠 進, 池添 浩二, 岸田 日帯, 田中 章景, 松下 拓也, 村井 弘之, 吉良 潤一

    末梢神経   26 ( 2 )   306 - 306   2015.12

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  • 抗Neurofascin抗体関連自己免疫性脱髄疾患としてのcombined central and peripheral demyelination(CCPD)

    山崎 亮, 緒方 英紀, 河村 信利, 吉良 潤一

    末梢神経   26 ( 2 )   232 - 236   2015.12

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  • 多発性硬化症、視神経脊髄炎におけるグルコース・乳酸担体の神経病理学的検討

    眞崎 勝久, 鈴木 諭, 林田 翔太郎, 宇根 隼人, 渡邉 充, 松下 拓也, 山崎 亮, 岩城 徹, 吉良 潤一

    臨床神経学   55 ( Suppl. )   S263 - S263   2015.12

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  • 【ミクログリア】病態機能 ミクログリア活性化の機構 神経障害性(M1型)と神経保護性(M2型)へのミクログリア活性型転換誘導因子

    山崎 亮, 吉良 潤一

    33 ( 12 )   1354 - 1358   2015.12

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  • 【グリア研究の最先端】ヒト疾患グリア病 アトピー性脊髄炎とグリア

    藤井 敬之, 山崎 亮, 吉良 潤一

    生体の科学   66 ( 6 )   575 - 578   2015.12

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  • Holocord involvement with sparing of the peripheral white matter rim in longitudinally extensive spinal cord lesions of neuromyelitis optica

    Shotaro Hayashida, Katsuhisa Masaki, Takuya Matsushita, Mitsuru Watanabe, Ryo Yamasaki, Hiroyuki Murai, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   6   78 - 79   2015.12

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    DOI： 10.1111/cen3.12274

  • Copy number variations in multiple sclerosis and neuromyelitis optica Reviewed International journal

    Ryo Yamasaki

    ANNALS OF NEUROLOGY   78 ( 5 )   762 - 774   2015.11

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    DOI： 10.1002/ana.24511

  • ALS医療ニーズと地域医療資源調査 難病医療専門員へのニーズに焦点をあてて

    岩木 三保, 小早川 優子, 山崎 亮, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   3 ( 1 )   91 - 91   2015.11

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  • ALS医療ニーズと地域医療資源調査 医療行為・福祉機器に対するニーズに焦点をあてて

    小早川 優子, 岩木 三保, 山崎 亮, 吉良 潤一

    日本難病医療ネットワーク学会機関誌   3 ( 1 )   54 - 54   2015.11

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  • Copy number variations in multiple sclerosis and neuromyelitis optica. Reviewed International journal

    Shinya Sato, Ken Yamamoto, Takuya Matsushita, Noriko Isobe, Yuji Kawano, Kyoko Iinuma, Masaaki Niino, Toshiyuki Fukazawa, Yuri Nakamura, Mitsuru Watanabe, Tomomi Yonekawa, Katsuhisa Masaki, Satoshi Yoshimura, Hiroyuki Murai, Ryo Yamasaki, Jun-Ichi Kira

    Annals of neurology   78 ( 5 )   762 - 74   2015.11

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    DOI： 10.1002/ana.24511

  • Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy. Reviewed International journal

    Ryo Yamasaki

    Ann Clin Transl Neurol.   2015.10

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  • Peripheral Allergy/Atopy Induces Glial Activation Leading to Allodynia and Exacerbation of Autoimmune Central Nervous System Inflammation Via Endothelin-1

    Takayuki Fujii, Ryo Yamasaki, Bing Wang, Mei Fang, Mizuho Kido, Katsuhisa Masaki, Jun-ichi Kira

    ANNALS OF NEUROLOGY   78   S75 - S75   2015.10

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  • IgG4 Neurofascin 155 Antibody-Positive Hypertrophic Demyelinating Polyneuropathy

    Hidenori Ogata, Ryo Yamasaki, Akio Hiwatashi, Nobuyuki Oka, Nobutoshi Kawamura, Dai Matsuse, Motoi Kuwahara, Hidekazu Suzuki, Susumu Kusunoki, Yuichi Fujimoto, Koji Ikezoe, Hitaru Kishida, Fumiaki Tanaka, Takuya Matsushita, Hiroyuki Murai, Jun-ichi Kira

    ANNALS OF NEUROLOGY   78   S79 - S79   2015.10

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  • Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy. Reviewed International journal

    Hidenori Ogata, Ryo Yamasaki, Akio Hiwatashi, Nobuyuki Oka, Nobutoshi Kawamura, Dai Matsuse, Motoi Kuwahara, Hidekazu Suzuki, Susumu Kusunoki, Yuichi Fujimoto, Koji Ikezoe, Hitaru Kishida, Fumiaki Tanaka, Takuya Matsushita, Hiroyuki Murai, Jun-Ichi Kira

    Annals of clinical and translational neurology   2 ( 10 )   960 - 71   2015.10

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    DOI： 10.1002/acn3.248

  • Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy Reviewed

    Hidenori Ogata, Ryo Yamasaki, Akio Hiwatashi, Nobuyuki Oka, Nobutoshi Kawamura, Dai Matsuse, Motoi Kuwahara, Hidekazu Suzuki, Susumu Kusunoki, Yuichi Fujimoto, Koji Ikezoe, Hitaru Kishida, Fumiaki Tanaka, Takuya Matsushita, Hiroyuki Murai, Jun Ichi Kira

    Annals of Clinical and Translational Neurology   2 ( 10 )   960 - 971   2015.10

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    DOI： 10.1002/acn3.248

  • Extensive loss of astrocytic monocarboxylate transporter 4 in active lesions of multiple sclerosis and neuromyelitis optica

    K. Masaki, S. O. Suzuki, S. Hayashida, M. Watanabe, T. Matsushita, R. Yamasaki, M. Suzuki, T. Iwaki, J. -I. Kira

    MULTIPLE SCLEROSIS JOURNAL   21   69 - 70   2015.9

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  • 視神経脊髄炎における長大な脊髄病巣の進展様式に関する神経画像的・神経病理学的研究

    林田 翔太郎, 眞崎 勝久, 米川 智, 松下 拓也, 山崎 亮, 樋渡 昭雄, 鈴木 諭, 末長 敏彦, 岩城 徹, 吉良 潤一

    神経免疫学   20 ( 1 )   115 - 115   2015.9

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  • 脳炎・その他 活性化ミクログリアの形態差と脊髄内局在差がTDP-43病理形成に与える影響の解析

    林 信太郎, 眞崎 勝久, 山崎 亮, 村井 弘之, 岡本 幸市, 吉良 潤一

    神経免疫学   20 ( 1 )   107 - 107   2015.9

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  • 脳炎・その他 アレルギー素因マウスにおける異痛症(アロディニア)と中枢グリア炎症

    山崎 亮, 藤井 敬之, 方 梅, 王 氷, 城戸 瑞穂, 吉良 潤一

    神経免疫学   20 ( 1 )   107 - 107   2015.9

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  • 神経免疫の新展開 中枢末梢連合脱髄症における新規自己抗体について

    山崎 亮, 緒方 英紀, 河村 信利, 吉良 潤一

    日本臨床免疫学会会誌   38 ( 4 )   287 - 287   2015.9

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  • Th1 cells inhibit the expression of connexins in astrocytes

    M. Watanabe, K. Masaki, R. Yamasaki, J. Kawanokuchi, H. Takeuchi, A. Suzumura, J. -I. Kira

    MULTIPLE SCLEROSIS JOURNAL   21   135 - 135   2015.9

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  • MS基礎 多発性硬化症、視神経脊髄炎におけるグルコース・乳酸担体の神経病理学的検討

    眞崎 勝久, 鈴木 諭, 林田 翔太郎, 渡邉 充, 松下 拓也, 山崎 亮, 岩城 徹, 吉良 潤一

    神経免疫学   20 ( 1 )   96 - 96   2015.9

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  • MS基礎 Th1細胞によるアストロサイトのコネキシン発現抑制作用

    渡邉 充, 眞崎 勝久, 山崎 亮, 川ノ口 潤, 竹内 英之, 錫村 明生, 吉良 潤一

    神経免疫学   20 ( 1 )   95 - 95   2015.9

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  • Nationwide Japanese survey shows the characteristic features of combined central and peripheral demyelination Reviewed

    Hidenori Ogata, Ryo Yamasaki, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   6 ( 3 )   214 - 215   2015.8

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    DOI： 10.1111/cen3.12224

  • A mouse model of atopic diathesis displaying tactile allodynia with glial inflammation in the spinal cord

    R. Yamasaki, J-I. Kira

    GLIA   63   E356 - E356   2015.8

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  • Nationwide Japanese survey shows the characteristic features of combined central and peripheral demyelination Reviewed

    Hidenori Ogata, Ryo Yamasaki, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   6 ( 3 )   214 - 215   2015.8

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    DOI： 10.1111/cen3.12224

  • Distinct features of immunoglobulin G2 aquaporin-4 antibody carriers with neuromyelitis optica Reviewed

    Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Yuji Kawano, Katsuhisa Masaki, Satoshi Yoshimura, Hiroyuki Murai, Ryo Yamasaki, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   6 ( 2 )   154 - 158   2015.5

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    DOI： 10.1111/cen3.12179

  • Peripheral Blood T Cell Alterations in Myelitis with Various Causes

    Z. Y. Song, Y. Kawano, S. Sato, M. Watanabe, S. Imamura, T. Yonekawa, K. Masaki, T. Matsushita, R. Yamasaki, J. Kira

    MULTIPLE SCLEROSIS JOURNAL   21 ( 6 )   829 - 829   2015.5

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  • Peripheral Atopic Diathesis Induces Neuropathic Pain and Activation of Astrocytes in The Posterior Horns of The Spinal Cord

    T. Fujii, R. Yamasaki, B. Wang, M. Kido, K. Masaki, J. Kira

    MULTIPLE SCLEROSIS JOURNAL   21 ( 6 )   830 - 831   2015.5

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  • Distinct features of immunoglobulin G2 aquaporin-4 antibody carriers with neuromyelitis optica Reviewed

    Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Yuji Kawano, Katsuhisa Masaki, Satoshi Yoshimura, Hiroyuki Murai, Ryo Yamasaki, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   6 ( 2 )   154 - 158   2015.5

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    DOI： 10.1111/cen3.12179

  • Peripheral blood T cell dynamics predict relapse in multiple sclerosis patients on fingolimod Reviewed

    Zi Ye Song, Ryo Yamasaki, Yuji Kawano, Shinya Sato, Katsuhisa Masaki, Satoshi Yoshimura, Dai Matsuse, Hiroyuki Murai, Takuya Matsushita, Jun Ichi Kira

    PloS one   10 ( 4 )   2015.4

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    DOI： 10.1371/journal.pone.0124923

  • A nationwide survey of combined central and peripheral demyelination in Japan. Reviewed International journal

    Ryo Yamasaki

    J Neurol Neurosurg Psychiatry (in press).   2015.3

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  • Decreased serum vitamin D levels in Japanese patients with multiple sclerosis Invited Reviewed International journal

    Ryo Yamasaki

    JOURNAL OF NEUROIMMUNOLOGY   279   40 - 45   2015.2

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    DOI： 10.1016/j.jneuroim.2015.01.007

  • Decreased serum vitamin D levels in Japanese patients with multiple sclerosis. Reviewed International journal

    Masaaki Niino, Shinya Sato, Toshiyuki Fukazawa, Katsuhisa Masaki, Yusei Miyazaki, Dai Matsuse, Ryo Yamasaki, Eri Takahashi, Seiji Kikuchi, Jun-ichi Kira

    Journal of neuroimmunology   279   40 - 5   2015.2

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    DOI： 10.1016/j.jneuroim.2015.01.007

  • 各種疾患 脱髄・免疫性代謝疾患 中枢神経障害を合併したニューロパチーにおける抗neurofascin抗体の意義

    緒方 英紀, 山崎 亮, 吉良 潤一

    Annual Review神経   2015   206 - 211   2015.1

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    Neurofascinは中枢神経系・末梢神経系いずれにも存在し,ランビエ絞輪部の形成と維持に必要不可欠な細胞結合膜タンパクである.近年ランビエ絞輪部周辺の様々な分子やその機能が明らかにされ,炎症性脱髄性疾患の自己抗原としての一面にも注目が集まる中,私たちは中枢神経障害を合併したニューロパチー症例の血清中にneurofascinの主要アイソフォームであるneurofascin 155に対する自己抗体が存在することを報告した.本稿ではneurofascinの生理的役割および自己抗原としての側面,炎症性脱髄性疾患との関連について既報告をふまえ概説する.(著者抄録)

  • Distinct roles of microglia and monocytes in central nervous system inflammation and degeneration Reviewed

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   5 ( s1 )   41 - 48   2014.12

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    DOI： 10.1111/cen3.12162

  • 軸索スフェロイドを伴う遺伝性びまん性白質脳症と一次性進行型多発性硬化症の画像比較

    斎藤 万有, 林 信太郎, 吉田 邦弘, 山崎 亮, 村井 弘之, 池田 修一, 吉良 潤一

    臨床神経学   54 ( Suppl. )   S102 - S102   2014.12

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  • 脱髄性疾患におけるグルコース・乳酸トランスポーター発現の神経理学的検討

    真崎 勝久, 鈴木 諭, 山崎 亮, 宇根 隼人, 渡邉 充, 佐藤 眞也, 岩城 徹, 吉良 潤一

    臨床神経学   54 ( Suppl. )   S42 - S42   2014.12

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  • 筋萎縮性側索硬化症モデルマウスの病態における末梢性炎症細胞の機能解明

    山崎 亮, 吉良 潤一

    臨床神経学   54 ( Suppl. )   S37 - S37   2014.12

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  • 気管支喘息モデルマウスはミクログリア活性化とアロディニアを生じる

    山崎 亮, 王 氷, 津田 誠, 城戸 瑞穂, 井上 和秀, 吉良 潤一

    末梢神経   25 ( 2 )   347 - 347   2014.12

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  • 多発性硬化症一卵性双生児不一致例でのゲノムの相違

    河野 祐治, 佐藤 眞也, 渡邊 充, 今村 志穂子, 眞崎 勝久, 山崎 亮, 吉良 潤一

    臨床神経学   54 ( Suppl. )   S249 - S249   2014.12

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  • 多発性硬化症および視神経脊髄炎におけるT細胞受容体遺伝子領域のコピー数多型の同定

    佐藤 眞也, 山本 健, 松下 拓也, 磯部 紀子, 河野 祐治, 吉村 怜, 飯沼 今日子, 渡邉 充, 米川 智, 眞崎 勝久, 山崎 亮, 吉良 潤一, the Japan Multiple Sclerosis Genetics Consortium

    臨床神経学   54 ( Suppl. )   S65 - S65   2014.12

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  • 免疫細胞群からみた孤発性筋萎縮性側索硬化症の脊髄白質変性機序の再検討

    林 信太郎, 山崎 亮, 眞崎 勝久, 村井 弘之, 大八木 保政, 岡本 幸市, 吉良 潤一

    臨床神経学   54 ( Suppl. )   S98 - S98   2014.12

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  • 中枢・末梢連合脱髄症における特異性の高い抗neurofascin抗体測定法の開発

    緒方 英紀, 松瀬 大, 河村 信利, 松下 拓也, 山崎 亮, 吉良 潤一

    臨床神経学   54 ( Suppl. )   S38 - S38   2014.12

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  • 世界初の日本人肥厚性硬膜炎全国臨床疫学調査二次調査159例の解析結果報告

    米川 智, 村井 弘之, 宇津木 聡, 松下 拓也, 眞崎 勝久, 磯部 紀子, 山崎 亮, 吉田 眞理, 楠 進, 坂田 清美, 藤井 清孝, 吉良 潤一

    臨床神経学   54 ( Suppl. )   S1 - S1   2014.12

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  • Th1細胞によるアストロサイトのコネキシン発現抑制作用

    渡邉 充, 眞崎 勝久, 山崎 亮, 川ノ口 潤, 竹内 英之, 錫村 明生, 吉良 潤一

    臨床神経学   54 ( Suppl. )   S42 - S42   2014.12

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  • Distinct roles of microglia and monocytes in central nervous system inflammation and degeneration

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   5 ( 1 )   41 - 48   2014.12

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    DOI： 10.1111/cen3.12162

  • Distinct cytokine and T helper cell profiles between patients with multiple sclerosis who had or had not received interferon-beta Reviewed

    Hikaru Doi, Zi Ye Song, Satoshi Yoshimura, Takahisa Tateishi, Tomomi Yonekawa, Ryo Yamasaki, Hiroyuki Murai, Takuya Matsushita, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   5 ( 3 )   321 - 327   2014.10

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    DOI： 10.1111/cen3.12138

  • Distinct Value of Cerebrospinal Fluid Cytokines in Patients with Multiple System Atrophy and Spinocerebellar Degenerations

    Ryo Yamasaki, Takuya Matsushita, Yasumasa Ohyagi, Akio Hiwatashi, Takashi Yoshiura, Jun-ichi Kira

    ANNALS OF NEUROLOGY   76   S50 - S51   2014.10

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  • Deletion-Type Copy Number Variations at T Cell Receptor Genes Confer Susceptibility to Multiple Sclerosis and Neuromyelitis Optica

    Shinya Sato, Ken Yamamoto, Takuya Matsushita, Noriko Isobe, Yuji Kawano, Kyoko Iinuma, Tomomi Yonekawa, Katsuhisa Masaki, Satoshi Yoshimura, Ryo Yamasaki, Junichi Kira

    ANNALS OF NEUROLOGY   76   S110 - S111   2014.10

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  • Copy number variations in T cell receptor loci are associated with susceptibility to multiple sclerosis and neuromyelitis optica

    Shinya Sato, Ken Yamamoto, Takuya Matsushita, Noriko Isobe, Yuji Kawano, Kyoko Iinuma, Tomomi Yonekawa, Katsuhisa Masaki, Satoshi Yoshimura, Ryo Yamasaki, Jun-ichi Kira

    JOURNAL OF NEUROIMMUNOLOGY   275 ( 1-2 )   53 - 53   2014.10

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    DOI： 10.1016/j.jneuroim.2014.08.140

  • Early inhibition of tumor necrosis factor-α and interleukin-6 in muscle tissue as a therapy for dystrophinopathy in mdx mice Reviewed

    5 ( 3 )   371 - 377   2014.10

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    Objective Pro-inflammatory cytokines can exacerbate muscle fiber damage in dystrophinopathy. The aim of the present study was to identify cytokine/chemokine alterations in the muscle tissues of mdx mice, a model of dystrophinopathy, and the beneficial effects of anti-proinflammatory cytokine therapy. Methods A total of 23 cytokines and chemokines were quantitatively measured in muscle tissues from mdx mice by fluorescent bead-based immunoassay. The mdx mice were treated with anti-tumor necrosis factor-α (TNF-α) and anti-interleukin-6 (IL-6) drugs, and their physical condition was evaluated by Rotarod and muscle damage by histopathological analysis. Results Levels of TNF-α and IL-6 were elevated at 14 days (P14), before a transient increase of macrophage and neutrophil-activating pro-inflammatory cytokines/chemokines, such as C-C motif ligand 2 (CCL2), CCL4 and KC (mouse C-X-C motif ligand 8 homolog), at P20. Administration of an anti-TNF-α drug (etanercept) and an anti-IL-6 receptor antibody (MR16-1) from P7 improved physical performance, and reduced both the area of basophilic fibers that indicated degenerating/regenerating fibers and CD68-positive macrophage infiltration. Initiating therapy at P7 inhibited the elevation of CCL2, CCL4 and KC more effectively than at P13. Conclusions The early administration of anti-TNF-α and anti-IL-6 drugs attenuated muscle fiber degeneration in a mouse model of dystrophinopathy.

    DOI： 10.1111/cen3.12111

  • Distinct cytokine and T helper cell profiles between patients with multiple sclerosis who had or had not received interferon-beta Reviewed

    Hikaru Doi, Zi-Ye Song, Satoshi Yoshimura, Takahisa Tateishi, Tomomi Yonekawa, Ryo Yamasaki, Hiroyuki Murai, Takuya Matsushita, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   5 ( 3 )   321 - 327   2014.10

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    DOI： 10.1111/cen3.12138

  • Early inhibition of tumor necrosis factor-α and interleukin-6 in muscle tissue as a therapy for dystrophinopathy in mdx mice Reviewed

    5 ( 3 )   371 - 377   2014.10

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    Objective Pro-inflammatory cytokines can exacerbate muscle fiber damage in dystrophinopathy. The aim of the present study was to identify cytokine/chemokine alterations in the muscle tissues of mdx mice, a model of dystrophinopathy, and the beneficial effects of anti-proinflammatory cytokine therapy. Methods A total of 23 cytokines and chemokines were quantitatively measured in muscle tissues from mdx mice by fluorescent bead-based immunoassay. The mdx mice were treated with anti-tumor necrosis factor-α (TNF-α) and anti-interleukin-6 (IL-6) drugs, and their physical condition was evaluated by Rotarod and muscle damage by histopathological analysis. Results Levels of TNF-α and IL-6 were elevated at 14 days (P14), before a transient increase of macrophage and neutrophil-activating pro-inflammatory cytokines/chemokines, such as C-C motif ligand 2 (CCL2), CCL4 and KC (mouse C-X-C motif ligand 8 homolog), at P20. Administration of an anti-TNF-α drug (etanercept) and an anti-IL-6 receptor antibody (MR16-1) from P7 improved physical performance, and reduced both the area of basophilic fibers that indicated degenerating/regenerating fibers and CD68-positive macrophage infiltration. Initiating therapy at P7 inhibited the elevation of CCL2, CCL4 and KC more effectively than at P13. Conclusions The early administration of anti-TNF-α and anti-IL-6 drugs attenuated muscle fiber degeneration in a mouse model of dystrophinopathy.

    DOI： 10.1111/cen3.12111

  • A genome-wide copy number variation study identified T-cell receptor as a susceptibility gene for multiple sclerosis and neuromyelitis optica

    S. Sato, K. Yamamoto, T. Matsushita, N. Isobe, Y. Kawano, K. Iinuma, T. Yonekawa, K. Masaki, S. Yoshimura, R. Yamasaki, J-I Kira

    MULTIPLE SCLEROSIS JOURNAL   20   251 - 252   2014.9

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  • 筋萎縮性側索硬化症におけるグリア性炎症初期像の同定 発症早期組織と末期組織を用いた免疫病理学的検討

    林 信太郎, 山崎 亮, 真崎 勝久, 村井 弘之, 岡本 幸市, 吉良 潤一

    神経免疫学   19 ( 1 )   128 - 128   2014.9

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  • 末梢のアトピー炎症は脊髄ミクログリア活性化とアロディニアを惹起する

    王 冰, 山崎 亮, 城戸 瑞穂, 真崎 勝久, 吉良 潤一

    神経免疫学   19 ( 1 )   151 - 151   2014.9

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  • 多系統萎縮症と脊髄小脳変性症の鑑別における髄液サイトカインの意義

    山崎 亮, 松下 拓也, 大八木 保政, 樋渡 昭雄, 吉浦 敬, 吉良 潤一

    神経免疫学   19 ( 1 )   130 - 130   2014.9

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  • マウスの実験的自己免疫性脳脊髄炎(EAE)におけるNotch4の機能解析(Functional analysis of Notch4 in mouse experimental autoimmune encephalomyelitis (EAE))

    李 広瑞, 山崎 亮, 吉良 潤一

    神経免疫学   19 ( 1 )   121 - 121   2014.9

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  • アトピー体質の動物モデルにおけるEAEの調節(Modulation of EAE in an animal model of atopic diathesis)

    方 梅, 山崎 亮, 王 冰, 城戸 瑞穗, 吉良 潤一

    神経免疫学   19 ( 1 )   152 - 152   2014.9

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  • 【神経症候群(第2版)-その他の神経疾患を含めて-】先天異常/先天奇形 破壊性獲得性二次性障害 遺伝性脱髄性疾患

    山崎 亮, 吉良 潤一

    日本臨床   別冊 ( 神経症候群IV )   331 - 335   2014.9

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  • Fingolimod投与下のMS再発はTh17%・Th1%の一過性増加ではなくCD4+Tcm%が関係

    宋 子夜, 河野 佑治, 吉村 怜, 渡邊 充, 今村 志穂子, 米川 智, 真崎 勝久, 山崎 亮, 吉良 潤一

    神経免疫学   19 ( 1 )   161 - 161   2014.9

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  • Fingolimod投与下のMS再発はTh17%・Th1%の一過性増加ではなくCD4+Tcm%が関係

    宋 子夜, 河野 佑治, 吉村 怜, 渡邊 充, 今村 志穂子, 米川 智, 真崎 勝久, 山崎 亮, 吉良 潤一

    19 ( 2 )   225 - 225   2014.8

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  • 末梢のアトピー炎症は脊髄ミクログリア活性化とアロディニアを惹起する

    王 冰, 山崎 亮, 城戸 瑞穂, 真崎 勝久, 吉良 潤一

    19 ( 2 )   215 - 215   2014.8

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  • アトピー素因を持つモデル動物におけるEAEの調節(Modulation of EAE in an animal model of atopic diathesis)

    方 梅, 山崎 亮, 王 冰, 城戸 瑞穂, 吉良 潤一

    19 ( 2 )   216 - 216   2014.8

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  • A nationwide survey of hypertrophic pachymeningitis in Japan Reviewed

    Tomomi Yonekawa, Hiroyuki Murai, Satoshi Utsuki, Takuya Matsushita, Katsuhisa Masaki, Noriko Isobe, Ryo Yamasaki, Mari Yoshida, Susumu Kusunoki, Kiyomi Sakata, Kiyotaka Fujii, Jun Ichi Kira

    Journal of Neurology, Neurosurgery and Psychiatry   85 ( 7 )   732 - 739   2014.7

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    DOI： 10.1136/jnnp-2013-306410

  • Differential roles of microglia and monocytes in the inflamed central nervous system. Reviewed International journal

    Ryo Yamasaki, Haiyan Lu, Oleg Butovsky, Nobuhiko Ohno, Anna M Rietsch, Ron Cialic, Pauline M Wu, Camille E Doykan, Jessica Lin, Anne C Cotleur, Grahame Kidd, Musab M Zorlu, Nathan Sun, Weiwei Hu, LiPing Liu, Jar-Chi Lee, Sarah E Taylor, Lindsey Uehlein, Debra Dixon, Jinyu Gu, Crina M Floruta, Min Zhu, Israel F Charo, Howard L Weiner, Richard M Ransohoff

    The Journal of experimental medicine   211 ( 8 )   1533 - 49   2014.7

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    DOI： 10.1084/jem.20132477

  • A nationwide survey of hypertrophic pachymeningitis in Japan. Reviewed International journal

    Tomomi Yonekawa, Hiroyuki Murai, Satoshi Utsuki, Takuya Matsushita, Katsuhisa Masaki, Noriko Isobe, Ryo Yamasaki, Mari Yoshida, Susumu Kusunoki, Kiyomi Sakata, Kiyotaka Fujii, Jun-ichi Kira

    Journal of neurology, neurosurgery, and psychiatry   85 ( 7 )   732 - 9   2014.7

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    DOI： 10.1136/jnnp-2013-306410

  • Anti-KIR4.1 Antibody in Japanese Patients with Multiple Sclerosis and Other Neurological Diseases

    Y. Kawano, M. Watanabe, T. Yonekawa, K. Masaki, S. Sato, R. Yamasaki, S. Imamura, J. Kira

    MULTIPLE SCLEROSIS JOURNAL   20 ( 7 )   921 - 922   2014.6

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  • The Peripheral Blood T-cell Subset Dynamics Of Oral Fingolimod (FTY720)-Treated Multiple Sclerosis Patients

    Z. Y. Song, Y. Kawano, S. Yoshimura, M. Watanabe, S. Imamura, T. Yonekawa, K. Masaki, T. Matsushita, R. Yamasaki, J. Kira

    MULTIPLE SCLEROSIS JOURNAL   20 ( 7 )   930 - 930   2014.6

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  • Primary microgliopathy mimics primary progressive MS

    Kunihiro Yoshida, Ryo Yamasaki

    MULTIPLE SCLEROSIS JOURNAL   20 ( 7 )   904 - 904   2014.6

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  • Interleukin 2 Receptor Alpha Chain Gene Polymorphisms and Risk of Multiple Sclerosis and Neuromyelitis Optica

    G. Ainiding, Y. Kawano, S. Sato, N. Isobe, T. Matsushita, S. Yoshimura, T. Yonekawa, R. Yamasaki, J. Kira

    MULTIPLE SCLEROSIS JOURNAL   20 ( 7 )   923 - 923   2014.6

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  • Cytokine Receptor Expression in Monocyte Subsets in Multiple Sclerosis, Neuromyelitis Optica, and Amyotrophic Lateral Sclerosis

    Yi Wen Cui, Yuji Kawano, Nan Shi, Katsuhisa Masaki, Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Takahisa Tateishi, Ryo Yamasaki, Jun-ichi Kira

    MULTIPLE SCLEROSIS JOURNAL   20 ( 7 )   944 - 944   2014.6

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  • Combined Central And Peripheral Demyelination: Diagnostic Value Of Anti-Neurofascin Antibody And First Nationwide Survey In Japan

    H. Ogata, R. Yamasaki, D. Matsuse, N. Kawamura, T. Yonekawa, T. Matsushita, S. Imamura, S. Kusunoki, S. Nagayama, Y. Fukuda, H. Murai, J. Kira

    MULTIPLE SCLEROSIS JOURNAL   20 ( 7 )   909 - 909   2014.6

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  • Extensive dysregulations of oligodendrocytic and astrocytic connexins are associated with disease progression in an amyotrophic lateral sclerosis mouse model. Reviewed International journal

    Ryo Yamasaki, Masaki Katsuhisa, HAYASHI SHINTARO, Satoshi O Suzuki, Jun-ichi Kira

    J Neuroinflammation   2014.3

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  • 【神経症候群(第2版)-その他の神経疾患を含めて-】脱髄性疾患、遺伝性ニューロパチー 中枢性脱髄疾患 アトピー性脊髄炎

    山崎 亮, 吉良 潤一

    日本臨床   別冊 ( 神経症候群II )   822 - 826   2014.3

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  • Extensive dysregulations of oligodendrocytic and astrocytic connexins are associated with disease progression in an amyotrophic lateral sclerosis mouse model. Reviewed International journal

    Yiwen Cui, Katsuhisa Masaki, Ryo Yamasaki, Shihoko Imamura, Satoshi O Suzuki, Shintaro Hayashi, Shinya Sato, Yuko Nagara, Mami F Kawamura, Jun-ichi Kira

    Journal of neuroinflammation   11   42 - 42   2014.3

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    DOI： 10.1186/1742-2094-11-42

  • Extensive dysregulations of oligodendrocytic and astrocytic connexins are associated with disease progression in an amyotrophic lateral sclerosis mouse model Reviewed

    Yiwen Cui, Katsuhisa Masaki, Ryo Yamasaki, Shihoko Imamura, Satoshi O. Suzuki, Shintaro Hayashi, Shinya Sato, Yuko Nagara, Mami F. Kawamura, Jun ichi Kira

    Journal of neuroinflammation   11   2014.3

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    DOI： 10.1186/1742-2094-11-42

  • Interleukin 2 receptor α chain gene polymorphisms and risks of multiple sclerosis and neuromyelitis optica in southern Japanese. Reviewed International journal

    2014.2

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    Interleukin 2 receptor α subunit (IL2RA) is a genetic risk for multiple sclerosis (MS) in Caucasians. However, the association between MS and IL2RA in Japanese idiopathic demyelinating diseases of the central nervous system has not been examined.
    OBJECTIVE:
    To determine whether IL2RA gene polymorphisms confer risks of developing MS or neuromyelitis optica (NMO) in a Japanese population.
    METHODS:
    DNA samples were obtained from 115 MS patients, 75 NMO/NMO spectrum disorder (NMOSD) patients, and 238 healthy controls. The single nucleotide polymorphisms (SNPs) rs2104286, rs12722489, and rs7090512 were genotyped by real-time PCR using TaqMan SNP genotyping assays.
    RESULTS:
    No significant associations of the three IL2RA SNPs with the development of the diseases were observed. In MS patients only, the annualized relapse rates were significantly higher for the rs2104286-TT genotype than for the non-TT (CT+CC) genotype and for the rs12722489-CC genotype than for the non-CC genotype in females (p = 0.0138 for both), but not in males.
    CONCLUSIONS:
    Although the possibility that IL2RA is a risk factor for MS development was not confirmed in this Japanese population, IL2RA gene polymorphisms were able to modify the disease activity in female MS patients, but had no influence on either susceptibility or disease phenotype in NMO/NMOSD patients.

  • 中枢・末梢連合脱髄症(CCPD)における全国臨床疫学調査成績と抗neurofascin(NF)抗体陽性率の検討

    緒方 英紀, 松瀬 大, 松下 拓也, 河村 信利, 山崎 亮, 楠 進, 吉良 潤一

    日本内科学会雑誌   103 ( Suppl. )   221 - 221   2014.2

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  • Interleukin 2 receptor α chain gene polymorphisms and risks of multiple sclerosis and neuromyelitis optica in southern Japanese. Reviewed International journal

    337 ( 1-2 )   147 - 50   2014.2

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    DOI： 10.1016/j.jns.2013.11.037

  • A case of neuromyelitis optica harboring both anti-aquaporin-4 antibodies and a pathogenic mitochondrial DNA mutation for Leber's hereditary optic neuropathy. Reviewed International journal

    Wataru Shiraishi, Shintaro Hayashi, Takashi Kamada, Noriko Isobe, Ryo Yamasaki, Hiroyuki Murai, Yasumasa Ohyagi, Jun-ichi Kira

    Multiple sclerosis (Houndmills, Basingstoke, England)   20 ( 2 )   258 - 60   2014.2

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    DOI： 10.1177/1352458513513057

  • Interleukin 2 receptor α chain gene polymorphisms and risks of multiple sclerosis and neuromyelitis optica in southern Japanese Reviewed

    337 ( 1-2 )   147 - 150   2014.2

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    Background Interleukin 2 receptor α subunit (IL2RA) is a genetic risk for multiple sclerosis (MS) in Caucasians. However, the association between MS and IL2RA in Japanese idiopathic demyelinating diseases of the central nervous system has not been examined. Objective To determine whether IL2RA gene polymorphisms confer risks of developing MS or neuromyelitis optica (NMO) in a Japanese population. Methods DNA samples were obtained from 115 MS patients, 75 NMO/NMO spectrum disorder (NMOSD) patients, and 238 healthy controls. The single nucleotide polymorphisms (SNPs) rs2104286, rs12722489, and rs7090512 were genotyped by real-time PCR using TaqMan SNP genotyping assays. Results No significant associations of the three IL2RA SNPs with the development of the diseases were observed. In MS patients only, the annualized relapse rates were significantly higher for the rs2104286-TT genotype than for the non-TT (CT+CC) genotype and for the rs12722489-CC genotype than for the non-CC genotype in females (p = 0.0138 for both), but not in males. Conclusions Although the possibility that IL2RA is a risk factor for MS development was not confirmed in this Japanese population, IL2RA gene polymorphisms were able to modify the disease activity in female MS patients, but had no influence on either susceptibility or disease phenotype in NMO/NMOSD patients.

    DOI： 10.1016/j.jns.2013.11.037

  • A case of neuromyelitis optica harboring both anti-aquaporin-4 antibodies and a pathogenic mitochondrial DNA mutation for Leber's hereditary optic neuropathy Reviewed

    Wataru Shiraishi, Shintaro Hayashi, Takashi Kamada, Noriko Isobe, Ryo Yamasaki, Hiroyuki Murai, Yasumasa Ohyagi, Jun Ichi Kira

    Multiple Sclerosis   20 ( 2 )   258 - 260   2014.2

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    DOI： 10.1177/1352458513513057

  • Decreased CCR2 and CD62L expressions on peripheral blood classical monocytes in amyotrophic lateral sclerosis Reviewed

    Yi Wen Cui, Yuji Kawano, Ryo Yamasaki, Nan Shi, Katsuhisa Masaki, Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Takahisa Tateishi, Shintaro Hayashi, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   5 ( 1 )   92 - 96   2014.1

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    DOI： 10.1111/cen3.12088

  • The role of microglia in inflammatory demyelination lesion in the central nervous system Reviewed

    Ryo Yamasaki

    Clinical Neurology   54 ( 12 )   981 - 983   2014.1

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    DOI： 10.5692/clinicalneurol.54.981

  • Microglia in vivo and in vitro Reviewed

    Ryo Yamasaki

    Clinical and Experimental Neuroimmunology   5 ( 2 )   114 - 116   2014.1

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    DOI： 10.1111/cen3.12120

  • Differential roles of microglia and monocytes in the inflamed central nervous system Reviewed

    Ryo Yamasaki, Haiyan Lu, Oleg Butovsky, Nobuhiko Ohno, Anna M. Rietsch, Ron Cialic, Pauline M. Wu, Camille E. Doykan, Jessica Lin, Anne C. Cotleur, Grahame Kidd, Musab M. Zorlu, Nathan Sun, Weiwei Hu, Li Ping Liu, Jar Chi Lee, Sarah E. Taylor, Lindsey Uehlein, Debra Dixon, Jinyu Gu, Crina M. Floruta, Min Zhu, Israel F. Charo, Howard L. Weiner, Richard M. Ransohoff

    Journal of Experimental Medicine   211 ( 8 )   1533 - 1549   2014

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    DOI： 10.1084/jem.20132477

  • Anti-neurofascin antibodies in patients with combined central and peripheral demyelination Reviewed

    Ryo Yamasaki, Nobutoshi Kawamura

    Clinical and Experimental Neuroimmunology   4 ( 3 )   257 - 258   2013.12

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    DOI： 10.1111/cen3.12073

  • 日本人多発性硬化症の緩徐進行群と非緩徐進行群の臨床・免疫遺伝学的背景の比較

    渡邉 充, 吉村 怜, 磯部 紀子, 米川 智, 松下 拓也, 佐藤 眞也, 真崎 勝久, 山崎 亮, 河野 祐治, 吉良 潤一

    臨床神経学   53 ( 12 )   1594 - 1594   2013.12

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  • 日本人多発性硬化症における抗Kir4.1抗体の意義

    河野 祐治, 渡邉 充, 今村 志穂子, 米川 智, 真崎 勝久, 山崎 亮, 吉良 潤一

    臨床神経学   53 ( 12 )   1503 - 1503   2013.12

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  • 中枢・末梢連合脱髄症における抗neurofascin抗体陽性率の検討および全国臨床疫学調査

    緒方 英紀, 松瀬 大, 河村 信利, 山崎 亮, 米川 智, 今村 志穂子, 松下 拓也, 楠 進, 錫村 明生, 吉良 潤一

    臨床神経学   53 ( 12 )   1504 - 1504   2013.12

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  • 中枢・末梢連合脱髄症における全国臨床調査報告および抗neurofascin抗体陽性率の検討

    緒方 英紀, 松瀬 大, 松下 拓也, 河村 信利, 山崎 亮, 飛松 省三, 楠 進, 錫村 明生, 吉良 潤一

    末梢神経   24 ( 2 )   304 - 305   2013.12

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  • fingolimodによる末梢血T細胞サブセット動態

    宋 子夜, 河野 祐治, 吉村 怜, 渡邉 充, 今村 志穂子, 米川 智, 真崎 勝久, 山崎 亮, 吉良 潤一

    臨床神経学   53 ( 12 )   1570 - 1570   2013.12

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  • TDP-43(A315T)Tgマウスの脊髄運動ニューロンにおける核細胞質間輸送障害の解析

    長柄 祐子, 林 信太郎, 山崎 亮, 大八木 保政, 吉良 潤一

    臨床神経学   53 ( 12 )   1497 - 1497   2013.12

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  • IL-2受容体α鎖の遺伝子多型の日本人多発性硬化症における意義

    Gulibahaer Ainiding, 河野 祐治, 吉村 怜, 渡邉 充, 今村 志穂子, 米川 智, 真崎 勝久, 磯部 紀子, 松下 拓也, 山崎 亮, 吉良 潤一

    臨床神経学   53 ( 12 )   1570 - 1570   2013.12

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  • Efficacy of methylprednisolone pulse therapy for acute relapse in Japanese patients with multiple sclerosis and neuromyelitis optica: A multicenter retrospective analysis - 1. Whole group analysis

    Jun-Ichi Kira, Ryo Yamasaki, Satoshi Yoshimura, Toshiyuki Fukazawa, Kazumasa Yokoyama, Kazuo Fujihara, Mieko Ogino, Takanori Yokota, Katsuichi Miyamoto, Masaaki Niino, Kyoichi Nomura, Ryo Tomioka, Masami Tanaka, Izumi Kawachi, Takashi Ohashi, Kenichi Kaida, Makoto Matsui, Yuji Nakatsuji, Hirofumi Ochi, Hikoaki Fukaura, Takashi Kanda, Akiko Nagaishi, Kanae Togo, Hidehiro Mizusawa, Yuji Kawano

    Clinical and Experimental Neuroimmunology   4 ( 3 )   305 - 317   2013.12

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    DOI： 10.1111/cen3.12071

  • Anti-neurofascin antibodies in patients with combined central and peripheral demyelination

    Ryo Yamasaki, Nobutoshi Kawamura

    Clinical and Experimental Neuroimmunology   4 ( 3 )   257 - 258   2013.12

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    DOI： 10.1111/cen3.12073

  • ALSマウスモデルのグリア炎症におけるコネキシン蛋白と病態への関与

    崔 訳文, 真崎 勝久, 山崎 亮, 林 信太郎, 長柄 祐子, 河村 真実, 佐藤 眞也, 大八木 保政, 吉良 潤一

    臨床神経学   53 ( 12 )   1501 - 1501   2013.12

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  • Protein kinase C-η polymorphism rs2230500 does not confer disease susceptibility to multiple sclerosis or neuromyelitis optica Reviewed

    4 ( 3 )   283 - 287   2013.12

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    Objectives To determine whether the non-synonymous 1425G/A polymorphism (rs2230500), an Asian-specific single nucleotide polymorphism that increases the kinase activity and affects the function of immune cells, of the protein kinase C-η gene (PRKCH) confers the risk of developing idiopathic demyelinating diseases of the central nervous system in a Japanese population. Methods Blood samples were collected from 96 multiple sclerosis (MS) patients, 52 neuromyelitis optica (NMO)/NMO spectrum disorder (NMOSD) patients and 151 healthy controls. The polymorphism rs2230500 was genotyped by sequencing. Results No significant association was observed between the PRKCH rs2230500 polymorphism and the risk of either MS or NMO/NMOSD. Clinical characteristics were also unaffected by the rs2230500 status. Conclusions Although the possibility that PRKCH has some effect on MS and NMO/NMOSD risk cannot be completely excluded because of the small study sample size, the polymorphism rs2230500 did not appear to confer disease susceptibility to MS or NMO/NMOSD in this Japanese population. © 2013 Japanese Society for Neuroimmunology.

    DOI： 10.1111/cen3.12056

  • Protein kinase C-η polymorphism rs2230500 does not confer disease susceptibility to multiple sclerosis or neuromyelitis optica Reviewed

    4 ( 3 )   283 - 287   2013.12

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    Objectives To determine whether the non-synonymous 1425G/A polymorphism (rs2230500), an Asian-specific single nucleotide polymorphism that increases the kinase activity and affects the function of immune cells, of the protein kinase C-η gene (PRKCH) confers the risk of developing idiopathic demyelinating diseases of the central nervous system in a Japanese population. Methods Blood samples were collected from 96 multiple sclerosis (MS) patients, 52 neuromyelitis optica (NMO)/NMO spectrum disorder (NMOSD) patients and 151 healthy controls. The polymorphism rs2230500 was genotyped by sequencing. Results No significant association was observed between the PRKCH rs2230500 polymorphism and the risk of either MS or NMO/NMOSD. Clinical characteristics were also unaffected by the rs2230500 status. Conclusions Although the possibility that PRKCH has some effect on MS and NMO/NMOSD risk cannot be completely excluded because of the small study sample size, the polymorphism rs2230500 did not appear to confer disease susceptibility to MS or NMO/NMOSD in this Japanese population.

    DOI： 10.1111/cen3.12056

  • Efficacy of methylprednisolone pulse therapy for acute relapse in Japanese patients with multiple sclerosis and neuromyelitis optica A multicenter retrospective analysis - 1. Whole group analysis Reviewed

    Jun Ichi Kira, Ryo Yamasaki, Satoshi Yoshimura, Toshiyuki Fukazawa, Kazumasa Yokoyama, Kazuo Fujihara, Mieko Ogino, Takanori Yokota, Katsuichi Miyamoto, Masaaki Niino, Kyoichi Nomura, Ryo Tomioka, Masami Tanaka, Izumi Kawachi, Takashi Ohashi, Kenichi Kaida, Makoto Matsui, Yuji Nakatsuji, Hirofumi Ochi, Hikoaki Fukaura, Takashi Kanda, Akiko Nagaishi, Kanae Togo, Hidehiro Mizusawa, Yuji Kawano

    Clinical and Experimental Neuroimmunology   4 ( 3 )   305 - 317   2013.12

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    DOI： 10.1111/cen3.12071

  • A nationwide survey of hypertrophic pachymeningitis in Japan. Reviewed International journal

    Ryo Yamasaki, Tomomi Yonekawa, Hiroyuki Murai, Takuya Matsushita, Masaki Katsuhisa, Noriko Isobe, Jun-ichi Kira

    J Neurol Neurosurg Psychiatry   2013.11

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  • 視神経脊髄炎におけるT細胞受容体遺伝子のコピー数多型と表現型との関連

    佐藤 眞也, 松下 拓也, 磯部 紀子, 河野 祐治, 渡邉 充, 米川 智, 真崎 勝久, 山崎 亮, 山本 健, 吉良 潤一

    神経免疫学   18 ( 1 )   92 - 92   2013.11

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  • 末梢神経 中枢末梢連合脱髄症(CCPD)における新規自己抗体の同定とその意義

    山崎 亮, 河村 信利, 緒方 英紀, 松瀬 大, 米川 智, 河野 祐治, 今村 志穂子, 吉良 潤一

    神経免疫学   18 ( 1 )   94 - 94   2013.11

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  • 孤発性筋萎縮性側索硬化症の脊髄白質に浸潤する免疫細胞群の特徴 免疫組織化学的解析

    林 信太郎, 真崎 勝久, 山崎 亮, 村井 弘之, 大八木 保政, 岡本 幸市, 吉良 潤一

    神経免疫学   18 ( 1 )   120 - 120   2013.11

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  • Fingolimod治療時の末梢血Tcm%は多発性硬化症の再発予測因子である

    宋 子夜, 河野 祐治, 吉村 怜, 渡邊 充, 今村 志穂子, 米川 智, 真崎 勝久, 山崎 亮, 吉良 潤一

    神経免疫学   18 ( 1 )   152 - 152   2013.11

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  • ALSマウスモデルにおける病態の進行に伴うコネキシン蛋白群の変動

    崔 訳文, 真崎 勝久, 山崎 亮, 林 信太郎, 長柄 祐子, 河村 真実, 今村 志穂子, 佐藤 眞也, 吉良 潤一

    神経免疫学   18 ( 1 )   120 - 120   2013.11

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  • A NOTCH4 missense mutation confers resistance to multiple sclerosis in Japanese. Reviewed International journal

    Jian Huang, Satoshi Yoshimura, Noriko Isobe, Takuya Matsushita, Tomomi Yonekawa, Shinya Sato, Ryo Yamasaki, Jun-ichi Kira

    Multiple sclerosis (Houndmills, Basingstoke, England)   19 ( 13 )   1696 - 703   2013.11

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    DOI： 10.1177/1352458513482512

  • Intracellular accumulation of toxic turn amyloid-β is associated with endoplasmic reticulum stress in Alzheimer's disease Reviewed

    10 ( 1 )   11 - 20   2013.11

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    Amyloid-β3 protein (Aβ3) accumulates in the neurons of Alzheimer's disease (AD) patients at an early stage of the disease. Recently, we found that Aβ3 with a toxic turn at positions 22 and 23 accumulates in neurons in AD brain. Here, we studied the accumulation of Aβ3, toxic turn Aβ3 and high-molecular-weight Aβ3 oligomers in presenilin 1 (PS1) gene-transfected SH-SY5Y cells as well as in the brains of 3xTg-AD mice and AD patients. Immunostaining revealed that accumulation of toxic turn Aβ3 was promoted in G384A- and I143T-mutant PS1-transfected cells and further enhanced by co-transfection of cells with the Aβ3-precursor protein (Aβ3PP) gene. In contrast, accumulation of high-molecular-weight Aβ3 oligomers was promoted in mutant PS1 cells but attenuated by co-transfection of cells with the Aβ3PP gene. Toxic turn Aβ3 was detected in the neurons of 3xTg-AD mice aged 2 months, when the mice were cognitively unimpaired. In contrast, high-molecular-weight Aβ3 oligomers were detected in the neurons of 7-month-old mice, when memory dysfunction is apparent. Furthermore, immunostaining and western blotting for Rab4, Rab6 and GRP78 revealed increased levels of these proteins in mutant PS1 cells and their accumulation in the neurons of 3xTg-AD mice. Remarkably, GRP78 immunoreactivity was increased at 2 months of age. Double-label immunostaining of AD brain revealed an apparent association between toxic turn Aβ3 and GRP78, an endoplasmic reticulum (ER) stress marker. Intraneuronal accumulation of toxic turn Aβ3 may be associated with ER stress in the brains of AD model mice and AD patients at an early stage.

  • A NOTCH4 missense mutation confers resistance to multiple sclerosis in Japanese Reviewed

    Jian Huang, Satoshi Yoshimura, Noriko Isobe, Takuya Matsushita, Tomomi Yonekawa, Shinya Sato, Ryo Yamasaki, Jun Ichi Kira

    Multiple Sclerosis Journal   19 ( 13 )   1696 - 1703   2013.11

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    DOI： 10.1177/1352458513482512

  • A case of hereditary diffuse leukoencephalopathy with axonal spheroids caused by a de novo mutation in CSF1R masquerading as primary progressive multiple sclerosis Reviewed

    Ban Yu Saitoh, Ryo Yamasaki, Shintaro Hayashi, Satoshi Yoshimura, Takahisa Tateishi, Yasumasa Ohyagi, Hiroyuki Murai, Toru Iwaki, Kunihiro Yoshida, Jun Ichi Kira

    Multiple Sclerosis Journal   19 ( 10 )   1367 - 1370   2013.9

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    DOI： 10.1177/1352458513489854

  • TAR DNA-binding protein 43 pathology in a case clinically diagnosed with facial-onset sensory and motor neuronopathy syndrome: an autopsied case report and a review of the literature. Reviewed International journal

    Keita Sonoda, Kensuke Sasaki, Takahisa Tateishi, Ryo Yamasaki, Shintaro Hayashi, Nobutaka Sakae, Yasumasa Ohyagi, Toru Iwaki, Jun-ichi Kira

    Journal of the neurological sciences   332 ( 1-2 )   148 - 53   2013.9

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    DOI： 10.1016/j.jns.2013.06.027

  • Impaired cytoplasmic-nuclear transport of hypoxia-inducible factor-1α in amyotrophic lateral sclerosis. Reviewed International journal

    23 ( 5 )   534 - 46   2013.9

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    DOI： 10.1111/bpa.12040

  • A case of hereditary diffuse leukoencephalopathy with axonal spheroids caused by a de novo mutation in CSF1R masquerading as primary progressive multiple sclerosis. Reviewed International journal

    Ban-yu Saitoh, Ryo Yamasaki, Shintaro Hayashi, Satoshi Yoshimura, Takahisa Tateishi, Yasumasa Ohyagi, Hiroyuki Murai, Toru Iwaki, Kunihiro Yoshida, Jun-ichi Kira

    Multiple sclerosis (Houndmills, Basingstoke, England)   19 ( 10 )   1367 - 70   2013.9

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    DOI： 10.1177/1352458513489854

  • TAR DNA-binding protein 43 pathology in a case clinically diagnosed with facial-onset sensory and motor neuronopathy syndrome An autopsied case report and a review of the literature Reviewed

    Keita Sonoda, Kensuke Sasaki, Takahisa Tateishi, Ryo Yamasaki, Shintaro Hayashi, Nobutaka Sakae, Yasumasa Ohyagi, Toru Iwaki, Jun Ichi Kira

    Journal of the Neurological Sciences   332 ( 1-2 )   148 - 153   2013.9

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    DOI： 10.1016/j.jns.2013.06.027

  • Impaired cytoplasmic-nuclear transport of hypoxia-inducible factor-1α in amyotrophic lateral sclerosis Reviewed

    23 ( 5 )   534 - 546   2013.9

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    We investigated the mechanisms underlying abnormal vascular endothelial growth factor (VEGF) production in amyotrophic lateral sclerosis (ALS). We immunohistochemically studied VEGF, its receptors VEGFR1 and 2, and hypoxia-inducible factor-1α (HIF-1α) in autopsied ALS spinal cords. We also chronologically assessed the expression of HIF-1α, karyopherin β1, karyopherin β-cargo protein complex inhibitors and nuclear pore complex proteins in G93A mutant superoxide dismutase 1 (mSOD1) transgenic mice at presymptomatic, symptomatic and end stages. In ALS patients, compared with controls, HIF-1α immunoreactivity in the cytoplasm of anterior horn cells (AHCs) was significantly increased, while immunoreactivities for VEGF and VEGFRs were significantly decreased. Similar changes in HIF-1α and VEGF levels were observed in mSOD1 transgenic mice. HIF-1α co-localized with karyopherin β1 in the cytoplasm of AHCs and karyopherin β1 co-localized with nucleoporin 62 (Nup62) on the nuclear envelope. From the presymptomatic stage of mSOD1 transgenic mice, karyopherin β1 immunoreactivity in AHC nuclei significantly decreased and morphological irregularities of the Nup62-immunostained nuclear envelope became more pronounced with disease progression. Thus, in AHCs from mSOD1 transgenic mice, transport of cytoplasmic HIF-1α to the nuclear envelope and into the nucleus is impaired from the presymptomatic stage, suggesting that impaired cytoplasmic-nuclear transport of HIF-1α through the nuclear pore might precede motor neuron degeneration.

    DOI： 10.1111/bpa.12040

  • Connexin 43 astrocytopathy linked to rapidly progressive multiple sclerosis and neuromyelitis optica Reviewed International journal

    PLoS One   8 ( 8 )   2013.8

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    DOI： 10.1371/journal.pone.0072919.

  • Anti-KIR4.1 antibodies in Japanese patients with idiopathic central nervous system demyelinating diseases

    Mitsuru Watanabe, Ryo Yamasaki, Yuji Kawano, Shihoko Imamura, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 2 )   241 - 242   2013.8

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    DOI： 10.1111/cen3.12029

  • Distinct genetic profiles between Japanese multiple sclerosis patients with and without Barkhof brain lesions Reviewed

    Jian Huang, Noriko Isobe, Takuya Matsushita, Satoshi Yoshimura, Shinya Sato, Tomomi Yonekawa, Ryo Yamasaki, Hiroyuki Murai, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 2 )   173 - 180   2013.8

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    DOI： 10.1111/cen3.12017

  • Anti-neurofascin antibody in patients with combined central and peripheral demyelination. Reviewed International journal

    Nobutoshi Kawamura, Ryo Yamasaki, Tomomi Yonekawa, Takuya Matsushita, Susumu Kusunoki, Shigemi Nagayama, Yasuo Fukuda, Hidenori Ogata, Dai Matsuse, Hiroyuki Murai, Jun-Ichi Kira

    Neurology   81 ( 8 )   714 - 22   2013.8

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    DOI： 10.1212/WNL.0b013e3182a1aa9c

  • Alterations in chemokine receptor expressions on peripheral blood monocytes in multiple sclerosis and neuromyelitis optica Reviewed

    Yi Wen Cui, Yuji Kawano, Nan Shi, Katsuhisa Masaki, Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Takahisa Tateishi, Ryo Yamasaki, Hiroyuki Murai, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 2 )   201 - 205   2013.8

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    DOI： 10.1111/cen3.12039

  • Neuro2013 Reviewed

    Jun Ichi Kira, Ryo Yamasaki, Katsuhisa Masaki

    Clinical and Experimental Neuroimmunology   4 ( 2 )   243 - 245   2013.8

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    DOI： 10.1111/cen3.12042

  • Distinct genetic profiles between Japanese multiple sclerosis patients with and without Barkhof brain lesions Reviewed

    Jian Huang, Noriko Isobe, Takuya Matsushita, Satoshi Yoshimura, Shinya Sato, Tomomi Yonekawa, Ryo Yamasaki, Hiroyuki Murai, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 2 )   173 - 180   2013.8

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    DOI： 10.1111/cen3.12017

  • Connexin 43 Astrocytopathy Linked to Rapidly Progressive Multiple Sclerosis and Neuromyelitis Optica Reviewed

    Katsuhisa Masaki, Satoshi O. Suzuki, Takuya Matsushita, Takeshi Matsuoka, Shihoko Imamura, Ryo Yamasaki, Makiko Suzuki, Toshihiko Suenaga, Toru Iwaki, Jun Ichi Kira

    PloS one   8 ( 8 )   2013.8

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    DOI： 10.1371/journal.pone.0072919

  • Anti-neurofascin antibody in patients with combined central and peripheral demyelination Reviewed

    Nobutoshi Kawamura, Ryo Yamasaki, Tomomi Yonekawa, Takuya Matsushita, Susumu Kusunoki, Shigemi Nagayama, Yasuo Fukuda, Hidenori Ogata, Dai Matsuse, Hiroyuki Murai, Jun Ichi Kira

    Neurology   81 ( 8 )   714 - 722   2013.8

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    DOI： 10.1212/WNL.0b013e3182a1aa9c

  • Anti-KIR4.1 antibodies in Japanese patients with idiopathic central nervous system demyelinating diseases Reviewed

    Mitsuru Watanabe, Ryo Yamasaki, Yuji Kawano, Shihoko Imamura, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 2 )   241 - 242   2013.8

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    DOI： 10.1111/cen3.12029

  • Alterations in chemokine receptor expressions on peripheral blood monocytes in multiple sclerosis and neuromyelitis optica Reviewed

    Yi Wen Cui, Yuji Kawano, Nan Shi, Katsuhisa Masaki, Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Takahisa Tateishi, Ryo Yamasaki, Hiroyuki Murai, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 2 )   201 - 205   2013.8

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    DOI： 10.1111/cen3.12039

  • Case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids showing features common to multiple sclerosis Reviewed

    Kazutaka Sonoda, Ryo Yamasaki, Takuya Matsushita, Takeo Yoshimura, Hiroyuki Murai, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 1 )   104 - 106   2013.6

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    DOI： 10.1111/cen3.12028

  • Multimodality evoked potentials for discrimination of atopic myelitis and multiple sclerosis

    Yuji Kanamori, Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Hiroshi Shigeto, Nobutoshi Kawamura, Ryo Yamasaki, Hiroyuki Murai, Shozo Tobimatsu, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 1 )   29 - 35   2013.6

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    DOI： 10.1111/cen3.12018

  • Case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids showing features common to multiple sclerosis

    Kazutaka Sonoda, Ryo Yamasaki, Takuya Matsushita, Takeo Yoshimura, Hiroyuki Murai, Jun-Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 1 )   104 - 106   2013.6

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    DOI： 10.1111/cen3.12028

  • Multimodality evoked potentials for discrimination of atopic myelitis and multiple sclerosis Reviewed

    Yuji Kanamori, Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Hiroshi Shigeto, Nobutoshi Kawamura, Ryo Yamasaki, Hiroyuki Murai, Shozo Tobimatsu, Jun Ichi Kira

    Clinical and Experimental Neuroimmunology   4 ( 1 )   29 - 35   2013.6

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    DOI： 10.1111/cen3.12018

  • A case of hereditary diffuse leukoencephalopathy with axonal spheroids caused by a de novo mutation in CSF1R masquerading as primary progressive multiple sclerosis. Reviewed

    Saitoh BY, Ryo Yamasaki, Hayashi S, Yoshimura S, Tateishi T, Ohyagi Y, Murai H, Iwaki T, Yoshida K, Kira JI

    Mult Scler.   2013.5

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  • A NOTCH4 missense mutation confers resistance to multiple sclerosis in Japanese. Reviewed

    Huang J, Yoshimura S, Isobe N, Matsushita T, Yonekawa T, Sato S, Ryo Yamasaki, Kira JI

    Mult Scler.   2013.4

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  • Dysregulation of Surface Marker Expressions on Peripheral Blood Monocytes in Multiple Sclerosis

    Y. W. Cui, K. Masaki, R. Yamasaki, N. Shi, T. Tateishi, T. Matsushita, T. Yonekawa, J. Kira

    MULTIPLE SCLEROSIS JOURNAL   19 ( 5 )   675 - 676   2013.4

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  • Characteristic Cerebrospinal Fluid Cytokine/Chemokine Profiles in Neuromyelitis Optica, Relapsing Remitting or Primary Progressive Multiple Sclerosis Reviewed

    Takuya Matsushita, Takahisa Tateishi, Noriko Isobe, Tomomi Yonekawa, Ryo Yamasaki, Dai Matsuse, Hiroyuki Murai, Jun Ichi Kira

    PloS one   8 ( 4 )   2013.4

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    DOI： 10.1371/journal.pone.0061835

  • Characteristic cerebrospinal fluid cytokine/chemokine profiles in neuromyelitis optica, relapsing remitting or primary progressive multiple sclerosis. Reviewed

    Matsushita T, Tateishi T, Isobe N, Yonekawa T, Ryo Yamasaki, Matsuse D, Murai H, Kira J

    PLoS One   2013.4

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  • Intracellular accumulation of toxic turn amyloid-β is associated with endoplasmic reticulum stress in Alzheimer's disease. Reviewed

    2013.1

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  • Intracellular accumulation of toxic turn amyloid-β is associated with endoplasmic reticulum stress in Alzheimer's disease. Reviewed International journal

    10 ( 1 )   11 - 20   2013.1

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  • Impaired Cytoplasmic-Nuclear Transport of Hypoxia-Inducible Factor-1α in Amyotrophic Lateral Sclerosis. Reviewed

    2013.1

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  • ALSモデルマウスにおける脊髄前角細胞での核膜蛋白孔の形態変化の検討

    長柄 祐子, 立石 貴久, 河村 真実, 山崎 亮, 菊池 仁志, 大八木 保政, 吉良 潤一

    臨床神経学   52 ( 12 )   1601 - 1601   2012.12

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  • mSOD1-Tgマウスの急性神経傷害時の成熟に伴うミクログリアとT細胞の反応性低下

    河村 真実, 河村 信利, 立石 貴久, 山崎 亮, 長柄 祐子, 大八木 保政, 吉良 潤一

    臨床神経学   52 ( 12 )   1409 - 1409   2012.12

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  • Impaired cytoplasmic-nuclear transport (intranuclear changes and nuclear envelope alterations) occurs at the early pre-symptomatic stage of amyotrophic lateral sclerosis

    Y. Nagara, T. Tateishi, S. Hayashi, R. Yamasaki, M. Kawamura, H. Kikuchi, K. M. Iinuma, Y. Ohoyagi, J. -I. Kira

    EUROPEAN JOURNAL OF NEUROLOGY   19   276 - 276   2012.9

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  • Microglia sculpt postnatal neural circuits in an activity and complement-dependent manner.

    Schafer DP, Lehman EK, Kautzman AG, Koyama R, Mardinly AR, Yamasaki R, Ransohoff RM, Greenberg ME, Barres BA, Stevens B

    Neuron   2012.5

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  • Microglia sculpt postnatal neural circuits in an activity and complement-dependent manner. Reviewed International journal

    Dorothy P Schafer, Emily K Lehrman, Amanda G Kautzman, Ryuta Koyama, Alan R Mardinly, Ryo Yamasaki, Richard M Ransohoff, Michael E Greenberg, Ben A Barres, Beth Stevens

    Neuron   74 ( 4 )   691 - 705   2012.5

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    DOI： 10.1016/j.neuron.2012.03.026

  • Microglia Sculpt Postnatal Neural Circuits in an Activity and Complement-Dependent Manner Reviewed

    Dorothy P. Schafer, Emily K. Lehrman, Amanda G. Kautzman, Ryuta Koyama, Alan R. Mardinly, Ryo Yamasaki, Richard M. Ransohoff, Michael E. Greenberg, Ben A. Barres, Beth Stevens

    Neuron   74 ( 4 )   691 - 705   2012.5

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    DOI： 10.1016/j.neuron.2012.03.026

  • Impaired recruitment of neuroprotective microglia and T cells during acute neuronal injury coincides with increased neuronal vulnerability in an amyotrophic lateral sclerosis model. Reviewed International journal

    Kawamura MF, Yamasaki R, Kawamura N, Tateishi T, Nagara Y, Matsushita T, Ohyagi Y, Kira J

    Journal of experimental neurology   2012.4

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  • Impaired recruitment of neuroprotective microglia and T cells during acute neuronal injury coincides with increased neuronal vulnerability in an amyotrophic lateral sclerosis model. Reviewed International journal

    Mami Fukunaga Kawamura, Ryo Yamasaki, Nobutoshi Kawamura, Takahisa Tateishi, Yuko Nagara, Takuya Matsushita, Yasumasa Ohyagi, Jun-ichi Kira

    Experimental neurology   234 ( 2 )   437 - 45   2012.4

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    DOI： 10.1016/j.expneurol.2012.01.015

  • Impaired recruitment of neuroprotective microglia and T cells during acute neuronal injury coincides with increased neuronal vulnerability in an amyotrophic lateral sclerosis model Reviewed

    Mami Fukunaga Kawamura, Ryo Yamasaki, Nobutoshi Kawamura, Takahisa Tateishi, Yuko Nagara, Takuya Matsushita, Yasumasa Ohyagi, Jun ichi Kira

    Experimental Neurology   234 ( 2 )   437 - 445   2012.4

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    DOI： 10.1016/j.expneurol.2012.01.015

  • Role of CCR2 in immunobiology and neurobiology Reviewed

    Ryo Yamasaki, Liping Liu, Jessica Lin, Richard M. Ransohoff

    Clinical and Experimental Neuroimmunology   3 ( 1 )   16 - 29   2012.1

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    DOI： 10.1111/j.1759-1961.2011.00024.x

  • Thymoma-associated progressive encephalomyelitis with rigidity and myoclonus successfully treated with thymectomy and intravenous immunoglobulin Reviewed

    Taira Uehara, Hiroyuki Murai, Ryo Yamasaki, Hitoshi Kikuchi, Hiroshi Shigeto, Yasumasa Ohyagi, Jun Ichi Kira

    European Neurology   66 ( 6 )   328 - 330   2011.12

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    DOI： 10.1159/000332033

  • 進行期mSOD1-Tgマウスにおける急性神経細胞障害に対するミクログリアの反応性低下

    福永 真実, 河村 信利, 立石 貴久, 山崎 亮, 大八木 保政, 吉良 潤一

    臨床神経学   51 ( 12 )   1362 - 1362   2011.12

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  • mSOD G93Aマウスにおける脊髄前角細胞での核細胞質間輸送系 核膜孔蛋白の検討

    長柄 祐子, 立石 貴久, 福永 真実, 山崎 亮, 菊池 仁志, 吉良 潤一

    臨床神経学   51 ( 12 )   1362 - 1362   2011.12

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  • Thymoma-associated progressive encephalomyelitis with rigidity and myoclonus successfully treated with thymectomy and intravenous immunoglobulin. Reviewed

    Uehara T, Murai H, Ryo Yamasaki, Kikuchi H, Shigeto H, Ohyagi Y, Kira J

    Eur Neurol.   2011.11

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  • Preventive and therapeutic effects of the selective Rho-kinase inhibitor fasudil on experimental autoimmune neuritis. Reviewed

    Pineda AA, Minohara M, Kawamura N, Matsushita T, Ryo Yamasaki, Sun X, Piao H, Shimokara H, Kira J

    J Neurol Sci.   2011.7

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  • Preventive and therapeutic effects of the selective Rho-kinase inhibitor fasudil on experimental autoimmune neuritis. Reviewed International journal

    Arnold Angelo M Pineda, Motozumi Minohara, Nobutoshi Kawamura, Takuya Matsushita, Ryo Yamasaki, Xiaojia Sun, Hua Piao, Hiroaki Shimokawa, Jun-ichi Kira

    Journal of the neurological sciences   306 ( 1-2 )   115 - 20   2011.7

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    DOI： 10.1016/j.jns.2011.03.031

  • Preventive and therapeutic effects of the selective Rho-kinase inhibitor fasudil on experimental autoimmune neuritis Reviewed

    Arnold Angelo M. Pineda, Motozumi Minohara, Nobutoshi Kawamura, Takuya Matsushita, Ryo Yamasaki, Xiaojia Sun, Hua Piao, Hiroaki Shimokawa, Jun Ichi Kira

    Journal of the Neurological Sciences   306 ( 1-2 )   115 - 120   2011.7

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    DOI： 10.1016/j.jns.2011.03.031

  • Activation of glutathione peroxidase and inhibition of p53-related apoptosis by apomorphine. Reviewed

    Ma L, Ohyagi Y, Nakamura N, Iinuma KM, Miyoshi K, Himeno E, Soejima N, Yanagihara YT, Sakae N, Ryo Yamasaki, Kira J

    J Alzheimers Dis   2011.6

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  • Peripheral neuropathy, myelopathy, cerebellar ataxia, and subclinical optic neuropathy associated with copper deficiency occurring 23 years after total gastrectomy Reviewed

    Meiko Inaba, Takako Torii, Koji Shinoda, Ryo Yamasaki, Yasumasa Ohyagi, Jun-Ichi Kira

    Clinical Neurology   51 ( 6 )   412 - 416   2011.6

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    DOI： 10.5692/clinicalneurol.51.412

  • [Peripheral neuropathy, myelopathy, cerebellar ataxia, and subclinical optic neuropathy associated with copper deficiency occurring 23 years after total gastrectomy]. Reviewed

    Meiko Inaba, Takako Torii, Koji Shinoda, Ryo Yamasaki, Yasumasa Ohyagi, Jun-ichi Kira

    Rinsho shinkeigaku = Clinical neurology   51 ( 6 )   412 - 6   2011.6

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    DOI： 10.5692/clinicalneurol.51.412

  • 胃全摘術後23年後に末梢神経障害、脊髄症、小脳失調、潜在的視神経症を呈した銅欠乏の1例

    稲葉 明子, 鳥居 孝子, 篠田 紘司, 山崎 亮, 大八木 保政, 吉良 潤一

    臨床神経学   51 ( 6 )   412 - 416   2011.6

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    症例は61歳男性である。37歳で胃癌のため胃全摘を施行。60歳より下肢異常感覚が出現、61歳より歩行時のふらつきを自覚。神経学的に左上下肢の腱反射亢進、両下肢の失調、両下肢の異常感覚、Th12以下の触覚低下、両下肢の全感覚低下をみとめた。ロンベルグ徴候は陰性。電気生理学的検査では両側錐体路・後索障害、両下肢末梢神経障害、潜在的視神経障害をみとめた。血清銅10μg/dlと著明な低下をみとめ、銅欠乏による末梢神経障害、脊髄症、視神経症と診断し、銅補充療法を開始後、症状は改善した。本例は胃全摘術後23年の経過でこれらの神経障害を発症しており、胃切除後長期経過における神経障害では銅欠乏も考慮すべきである。(著者抄録)

  • Post-streptococcal chorea in an adult with bilateral striatal encephalitis. Reviewed

    Hagiwara K, Tominaga K, Okada Y, Kido M, Ryo Yamasaki, Shida N, Yamashita Y

    J Clin Neurosci.   2011.5

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  • Post-streptococcal chorea in an adult with bilateral striatal encephalitis. Reviewed International journal

    Koichi Hagiwara, Kayo Tominaga, Yoko Okada, Miwako Kido, Ryo Yamasaki, Norihiko Shida, Yoriaki Yamashita

    Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia   18 ( 5 )   708 - 9   2011.5

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    DOI： 10.1016/j.jocn.2010.08.024

  • Post-streptococcal chorea in an adult with bilateral striatal encephalitis Reviewed

    Koichi Hagiwara, Kayo Tominaga, Yoko Okada, Miwako Kido, Ryo Yamasaki, Norihiko Shida, Yoriaki Yamashita

    Journal of Clinical Neuroscience   18 ( 5 )   708 - 709   2011.5

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    DOI： 10.1016/j.jocn.2010.08.024

  • Apomorphine treatment in Alzheimer mice promoting amyloid-β degradation. Reviewed

    2011.2

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  • Apomorphine treatment in Alzheimer mice promoting amyloid-β degradation. Reviewed International journal

    69 ( 2 )   248 - 56   2011.2

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    DOI： 10.1002/ana.22319

  • Apomorphine treatment in Alzheimer mice promoting amyloid-β degradation Reviewed

    69 ( 2 )   248 - 256   2011.2

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    Objective: Intracellular amyloid β-protein (Aβ) contributes to neurodegeneration in Alzheimer disease (AD). Apomorphine (APO) is a dopamine receptor agonist for Parkinson disease and also protects against oxidative stress. Efficacy of APO for an AD mouse model and effects of APO on cell cultures are studied. Methods: The triple transgenic AD mouse model (3xTg-AD) has 2 familial AD-related gene mutations (APPKM670/671NL/PS1 M146V) and a tau gene mutation (TauP301L). Six-month-old 3xTg-AD mice were treated with subcutaneous injections of APO once a week for 1 month. Memory function was evaluated by Morris water maze before and after the treatment. Brain tissues were examined by immunohistochemical staining and Western blotting. Effects of APO on intracellular Aβ degradation, activity of Aβ-degrading enzymes, and protection against oxidative stress were studied in cultured SH-SY5Y cells. Results: After APO treatment, short-term memory function was dramatically improved. Significant decreases in the levels of intraneuronal Aβ, hyper-phosphorylated tau (p-tau), p53, and heme oxygenase-1 proteins were observed. Moreover, APO promoted degradation of intracellular Aβ, increased activity of proteasome and insulin-degrading enzyme, protected against H2O2 toxicity, and decreased p53 protein levels in the cultured cells. Interpretation: 3xTg-AD mice show intraneuronal Aβ accumulation and memory disturbances before extracellular Aβ deposition. Our data demonstrating improvement of memory function of 3xTg-AD mice with decreases in intraneuronal Aβ and p-tau levels by APO treatment strongly suggest that intraneuronal Aβ is an important therapeutic target and APO will be a novel drug for AD.

    DOI： 10.1002/ana.22319

  • Tissue binding patterns and in vitro effects of Campylobacter jejuni DNA-binding protein from starved cells. Reviewed

    Ryo Yamasaki, Piao H, Minohara M, Kawamura N, Li W, Matsushita T, Mizunoe Y, Kira J

    Neurochem Res   2011.1

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  • Tissue binding patterns and in vitro effects of Campylobacter jejuni DNA-binding protein from starved cells. Reviewed International journal

    Hua Piao, Motozumi Minohara, Nobutoshi Kawamura, Wei Li, Takuya Matsushita, Ryo Yamasaki, Yoshimitsu Mizunoe, Jun-Ichi Kira

    Neurochemical research   36 ( 1 )   58 - 66   2011.1

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    DOI： 10.1007/s11064-010-0263-7

  • Tissue binding patterns and in vitro effects of campylobacter jejuni DNA-binding protein from starved cells Reviewed

    Hua Piao, Motozumi Minohara, Nobutoshi Kawamura, Wei Li, Takuya Matsushita, Ryo Yamasaki, Yoshimitsu Mizunoe, Jun Ichi Kira

    Neurochemical Research   36 ( 1 )   58 - 66   2011.1

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    DOI： 10.1007/s11064-010-0263-7

  • Activation of glutathione peroxidase and inhibition of p53-related apoptosis by apomorphine Reviewed

    Linqing Ma, Yasumasa Ohyagi, Norimichi Nakamura, Kyoko M. Iinuma, Katsue Miyoshi, Eri Himeno, Naoko Soejima, Yuki T. Yanagihara, Nobutaka Sakae, Ryo Yamasaki, Jun-Ichi Kira

    Journal of Alzheimer's Disease   27 ( 1 )   225 - 237   2011.1

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    DOI： 10.3233/JAD-2011-110140

  • Restoration of microglial function by granulocyte-colony stimulating factor in ALS model mice Reviewed

    Ryo Yamasaki, Masahito Tanaka, Mami Fukunaga, Takahisa Tateishi, Hitoshi Kikuchi, Kyoko Motomura, Takuya Matsushita, Yasumasa Ohyagi, Jun ichi Kira

    Journal of Neuroimmunology   229 ( 1-2 )   51 - 62   2010.12

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    DOI： 10.1016/j.jneuroim.2010.07.002

  • mSOD1-Tgマウスにおける神経細胞脆弱性とミクログリアの反応性

    福永 真実, 山崎 亮, 立石 貴久, 河村 信利, 大八木 保政, 吉良 潤一

    臨床神経学   50 ( 12 )   1265 - 1265   2010.12

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  • FUS遺伝子変異を認め好塩基性封入体を伴い多系統変性を呈した家族性ALSの病理学的検討

    立石 貴久, 鉾之原 敏博, 山崎 亮, 菊池 仁志, 長柄 祐子, 大八木 保政, 岩城 徹, 服巻 保幸, 吉良 潤一

    臨床神経学   50 ( 12 )   1084 - 1084   2010.12

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  • ALS患者末梢血における単球の表面抗原による亜群解析とその異常

    崔 訳文, 史 楠, 松下 拓也, 山崎 亮, 立石 貴久, 吉良 潤一

    臨床神経学   50 ( 12 )   1126 - 1126   2010.12

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  • ALS及びmSODG93Aマウスにおける脊髄前角細胞での核細胞質間輸送(karyopherinβ)の検討

    長柄 祐子, 立石 貴久, 福永 真実, 山崎 亮, 菊池 仁志, 吉良 潤一

    臨床神経学   50 ( 12 )   1264 - 1264   2010.12

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  • Restoration of microglial function by granulocyte-colony stimulating factor in ALS model mice. Reviewed International journal

    Ryo Yamasaki, Masahito Tanaka, Mami Fukunaga, Takahisa Tateishi, Hitoshi Kikuchi, Kyoko Motomura, Takuya Matsushita, Yasumasa Ohyagi, Jun-ichi Kira

    Journal of neuroimmunology   229 ( 1-2 )   51 - 62   2010.12

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    DOI： 10.1016/j.jneuroim.2010.07.002

  • A case of acute disseminated encephalomyelitis associated with Epstein-Barr virus reactivation during infliximab therapy Reviewed

    Maki Ueda, Takahisa Tateishi, Hiroshi Shigeto, Ryo Yamasaki, Yasumasa Ohyagi, Jun Ichi Kira

    Clinical Neurology   50 ( 7 )   461 - 466   2010.7

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    DOI： 10.5692/clinicalneurol.50.461

  • インフリキシマブ治療中にEpstein-Barrウイルス再活性化にともなって急性散在性脳脊髄炎を発症した1例

    上田 麻紀, 立石 貴久, 重藤 寛史, 山崎 亮, 大八木 保政, 吉良 潤一

    臨床神経学   50 ( 7 )   461 - 466   2010.7

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    症例は31歳女性である。クローン病に対してインフリキシマブ投与開始11ヵ月後に無菌性髄膜炎を発症し一時軽快したが、その後に体幹失調や球麻痺が出現した。髄液検査では単核球優位の細胞数増多、ミエリン塩基性蛋白とIgG indexが上昇しており血清のEpstein-Barrウイルス(EBV)抗体は既感染パターンを示し、髄液・血液PCRにてEBV-DNAを検出した。MRIにて脳幹、大脳皮質下白質、頸髄に散在性にT2高信号病変をみとめ急性散在性脳脊髄炎(ADEM)と診断した。各種免疫治療に抵抗性であったが、ステロイドパルス療法を反復し症状は改善した。抗TNF-α抗体製剤の副作用による脱髄が報告されているが、本症例は抗TNF-α抗体製剤投与中のEBV再活性化によって惹起されたADEMと考えられた。(著者抄録)

  • [A case of acute disseminated encephalomyelitis associated with Epstein-Barr virus reactivation during infliximab therapy]. Reviewed

    Maki Ueda, Takahisa Tateishi, Hiroshi Shigeto, Ryo Yamasaki, Yasumasa Ohyagi, Jun-ichi Kira

    Rinsho shinkeigaku = Clinical neurology   50 ( 7 )   461 - 6   2010.7

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    DOI： 10.5692/clinicalneurol.50.461

  • CSF chemokine alterations related to the clinical course of amyotrophic lateral sclerosis. Reviewed

    Tateishi T, Ryo Yamasaki, Tanaka M, Matsushita T, Kikuchi H, Isobe N, Ohyagi Y, Kira J

    J Neuroimmunol.   2010.5

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  • 高齢発症で多発筋炎との鑑別を要したmtDNA多重欠失症候群の1例

    小野 茉莉, 鳥居 孝子, 古田 興之介, 山崎 亮, 立石 貴久, 松岡 健, 大八木 保政, 吉良 潤一

    臨床神経学   50 ( 5 )   356 - 356   2010.5

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  • 銅欠乏による末梢神経障害、脊髄症、潜在的視神経症をきたした1例

    西口 明子, 篠田 紘司, 鳥居 孝子, 古田 興之介, 山崎 亮, 重藤 寛史, 大八木 保政, 吉良 潤一

    神経治療学   27 ( 3 )   466 - 466   2010.5

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  • 孤発性片麻痺性片頭痛を呈した全身性エリテマトーデスの1例

    土井 光, 立石 貴久, 磯部 紀子, 山崎 亮, 大八木 保政, 吉良 潤一

    臨床神経学   50 ( 5 )   332 - 334   2010.5

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    症例は39歳の女性である。誘因なく左半身の異常感覚・不全片麻痺に続き視野欠損が出現し、3時間以内に消失する発作を反復性にみとめ、その発作の中で2回は嘔気をともなう左側頭部痛をともなった。頭部MR検査で明らかな異常所見なく、孤発性片麻痺性片頭痛(SHM)に合致した。また再発性の皮疹や関節痛も発作2年前よりみとめ、全身性エリテマトーデス(SLE)と診断した。くりかえす発作に対しアスピリンおよび塩酸ロメリジンの投与をおこない、その後発作はすみやかに消失した。SLEにSHMをともなうことはきわめてまれであるが、SHMの鑑別診断としてSLEも考慮する必要がある。(著者抄録)

  • [Sporadic hemiplegic migraine-like headache in a patient with systemic lupus erythematosus]. Reviewed

    Hikaru Doi, Takahisa Tateishi, Noriko Isobe, Ryo Yamasaki, Yasumasa Ohyagi, Jun-ichi Kira

    Rinsho shinkeigaku = Clinical neurology   50 ( 5 )   332 - 4   2010.5

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    DOI： 10.5692/clinicalneurol.50.332

  • CSF chemokine alterations related to the clinical course of amyotrophic lateral sclerosis. Reviewed International journal

    Takahisa Tateishi, Ryo Yamasaki, Masahito Tanaka, Takuya Matsushita, Hitoshi Kikuchi, Noriko Isobe, Yasumasa Ohyagi, Jun-ichi Kira

    Journal of neuroimmunology   222 ( 1-2 )   76 - 81   2010.5

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    DOI： 10.1016/j.jneuroim.2010.03.004

  • Sporadic hemiplegic migraine-like headache in a patient with systemic lupus erythematosus Reviewed

    Hikaru Doi, Takahisa Tateishi, Noriko Isobe, Ryo Yamasaki, Yasumasa Ohyagi, Jun Ichi Kira

    Clinical Neurology   50 ( 5 )   332 - 334   2010.5

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    DOI： 10.5692/clinicalneurol.50.332

  • CSF chemokine alterations related to the clinical course of amyotrophic lateral sclerosis Reviewed

    Takahisa Tateishi, Ryo Yamasaki, Masahito Tanaka, Takuya Matsushita, Hitoshi Kikuchi, Noriko Isobe, Yasumasa Ohyagi, Jun ichi Kira

    Journal of Neuroimmunology   222 ( 1-2 )   76 - 81   2010.5

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    DOI： 10.1016/j.jneuroim.2010.03.004

  • Reappraisal of brain MRI features in patients with multiple sclerosis and neuromyelitis optica according to anti-aquaporin-4 antibody status. Reviewed

    Matsushita T, Isobe N, Piao H, Matsuoka T, Ishizu T, Doi H, Masaki K, Yoshiura T, Ryo Yamasaki, Ohyagi Y, Kira J

    J Neurol Sci.   2010.4

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  • ダプソンの併用により副腎皮質ステロイド薬の減量が可能になった神経Sweet病の1例

    柴田 憲一, 立石 貴久, 山崎 亮, 大八木 保政, 吉良 潤一

    臨床神経学   50 ( 4 )   257 - 261   2010.4

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    症例は40歳男性である。2001年から髄膜炎をくりかえし、2005年から紅斑をみとめ、皮膚病理所見とHLA B54/Cw1陽性から神経Sweet病と診断した。今回、発熱、口腔内アフタ多発のため入院した。神経学的には四肢腱反射亢進、両側病的反射、