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

DOI： 10.3389/fnins.2018.00894

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

DOI： 10.1111/nan.12123

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

DOI： 10.1097/WNR.0000000000000068

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of the Neurological Sciences  

The epidemiology and etiology of facial onset sensory and motor neuronopathy (FOSMN), a rare syndrome that initiates with facial sensory disturbances followed by bulbar symptoms, remain unknown. To estimate the prevalence of FOSMN in Japan and establish the characteristics of this disease, we conducted a nationwide epidemiological survey. In the primary survey, we received answers from 604 facilities (49.8%), leading to an estimated number of 35.8 (95% confidential interval: 21.5–50.2) FOSMN cases in Japan. The secondary survey collected detailed clinical and laboratory data from 21 cases. Decreased or absent corneal and pharyngeal reflexes were present in over 85% of the cases. Electrophysiological analyses detected blink reflex test abnormalities in 94.1% of the examined cases. Immunotherapy was administered in 81% of cases and all patients received intravenous immunoglobulin. Among them, 35.3% were judged to have temporary beneficial effects evaluated by the physicians in charge. Immunotherapy tended to be effective in the early stage of disease. The spreading pattern of motor and sensory symptoms differed between cases and the characteristics of the motor-dominant and sensory-dominant cases were distinct. Cases with motor-dominant progression appeared to mimic amyotrophic lateral sclerosis. This is the first nationwide epidemiological survey of FOSMN in Japan. The clinical course of FOSMN is highly variable and motor-dominant cases developed a more severe condition than other types of cases. Because clinical interventions tend to be effective in the early phase of the disease, an early diagnosis is desirable.

DOI： 10.1016/j.jns.2024.122957

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PubMed

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

DOI： doi: 10.1016/j.jns.2024.122957.

Language：Japanese   Publisher：日本神経治療学会  

DOI： 10.15082/jsnt.41.6_s98

CiNii Research

Web of Science

Language：Japanese   Publisher：(株)日本プランニングセンター  

神経筋疾患を対象とした臨床試験における患者報告アウトカム(Patient-Reported Outcome:PRO)の利用状況を明らかにすることを目的に、グローバルな臨床試験登録データベースであるClinicalTrials.govを用いて、筋萎縮性側索硬化症など神経筋疾患の中でも運動機能障害をきたす代表的な9疾患を対象に検索を行い、評価項目にPROを含む臨床試験(PRO関連臨床試験)の割合について、疾患および期間別に比較した。その結果、最もPRO関連臨床試験数の割合が高い疾患は重症筋無力症で、2017～2021年に開始された臨床試験の約8割で評価項目にPROが使用されていた。一方、PRO関連臨床試験の割合が低い疾患は、筋ジストロフィーおよびハンチントン病で、2017～2021年に開始された臨床試験におけるPRO使用の割合は、いずれも3割以下であった。また、筋萎縮性側索硬化症に対するPRO関連臨床試験について、期間別に検討した結果、2007年～2011年の5年間に比べ2017～2021年の5年間では、PRO関連臨床試験は1.7倍に増加しており、前者の期間ではほとんどの臨床試験で包括的(疾患特異的ではない)PRO尺度が使用されていたが、2012年以降に開始された臨床試験では、約7割で疾患特異的PRO尺度が使用されていることが分かった。

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

DOI： https://doi.org/10.3390/ijms232416046

Language：English   Publishing type：Research paper (scientific journal)   Publisher：International Journal of Molecular Sciences  

Connexin 30 (Cx30), which forms gap junctions between astrocytes, regulates cell adhesion and migration, and modulates glutamate transport. Cx30 is upregulated on activated astroglia in central nervous system inflammatory lesions, including spinal cord lesions in mutant superoxide dismutase 1 (mSOD1) transgenic amyotrophic lateral sclerosis (ALS) model mice. Here, we investigated the role of Cx30 in mSOD1 mice. Cx30 was highly expressed in the pre-onset stage in mSOD1 mice. mSOD1 mice with knockout (KO) of the Cx30 gene (Cx30KO-mSOD1 mice) showed delayed disease onset and tended to have an extended survival period (log-rank, p = 0.09). At the progressive and end stages of the disease, anterior horn cells were significantly preserved in Cx30KO-mSOD1 mice. In lesions of these mice, glial fibrillary acidic protein/C3-positive inflammatory astroglia were decreased. Additionally, the activation of astrocytes in Cx30KO-mSOD1 mice was reduced compared with mSOD1 mice by gene expression microarray. Furthermore, expression of connexin 43 at the pre-onset stage was downregulated in Cx30KO-mSOD1 mice. These findings suggest that reduced expression of astroglial Cx30 at the early disease stage in ALS model mice protects neurons by attenuating astroglial inflammation.

DOI： 10.3390/ijms232416046

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

DOI： https://doi.org/10.1136/bmjopen-2022-061225

Language：English   Publisher：BMJ Open  

Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) is a complication of prior pulmonary thromboembolism (PE), caused by incomplete clot dissolution after PE. In patients with CTEPH, lifelong anticoagulation is mandatory to prevent recurrence of PE and secondary in situ thrombus formation. Warfarin, a vitamin K antagonist, is commonly used for anticoagulation in CTEPH based on historical experience and evidence. The anticoagulant activity of warfarin is affected by food and drug interactions, requiring regular monitoring of prothrombin time. The lability of anticoagulant effect often results in haemorrhagic and thromboembolic complications. Thus, lifelong warfarin is a handicap in terms of safety and convenience. Currently, the use of direct oral anticoagulants (DOACs) in CTEPH has increased with the advent of four DOACs. The safety of DOACs is superior to warfarin, with less intracranial bleeding in patients with non-valvular atrial fibrillation and venous thromboembolism. Edoxaban, the latest DOAC, also has proven efficacy and safety for those diseases in two large clinical trials; the ENGAGE-AF trial and HOKUSAI-VTE trial. The present trial seeks to evaluate whether edoxaban is non-inferior to warfarin in preventing worsening of CTEPH. Methods and analysis The KABUKI trial (is an investigator-initiated, multicentre, phase 3, randomised, single-blind, parallel-group, warfarin-controlled, non-inferiority trial to evaluate the efficacy and safety of edoxaban versus warfarin (vitamin K Antagonist) in subjects with chronic thromBoembolic pUlmonary hypertension taking warfarin (vitamin K antagonIst) at baseline) is designed to prove the non-inferiority of edoxaban to warfarin in terms of efficacy and safety in patients with CTEPH. Ethics and dissemination This study is approved by the Institutional Review Board of each participating institution. The findings will be published in a peer-reviewed journal, including positive, negative and inconclusive results. Trial registration number NCT04730037. Protocol version This paper was written per the study protocol V.4.0, dated 29 January 2021.

DOI： 10.1136/bmjopen-2022-061225

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

DOI： 10.1038/s41598-021-96064-6

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

Event date： 2021.5

Language：Japanese  

Country：Japan  

Event date： 2024.4

Language：Japanese  

Country：Japan  

Event date： 2024.4

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Country：Japan  

Event date： 2024.4

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Country：United States  

Event date： 2024.4

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Country：Switzerland  

Event date： 2024.3

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Venue：日本（大阪）   Country：Japan  

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Venue：日本（名古屋）   Country：Japan  

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Venue：千葉   Country：Japan  

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Country：Japan  

Event date： 2022.5

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