|Hiroshi Shigeto||Last modified date：2022.11.09|
Professor / Medical technology / Department of Health Sciences / Faculty of Medical Sciences
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|Hiroshi Shigeto||Last modified date：2022.11.09|
|1.||Takahiko Mukaino, Taira Uehara, Jun Yokohama, Toshiki Okadome, Tomomi Arakawa, Setsu Yokoyama, Ayumi Sakata, Kei-Ichiro Takase, Osamu Togao, Naoki Akamatsu, Hiroshi Shigeto, Noriko Isobe, Jun-Ichi Kira, Atrophy of the hippocampal CA1 subfield relates to long-term forgetting in focal epilepsy., Epilepsia, 10.1111/epi.17378, 2022.07, OBJECTIVE: The mechanisms underlying accelerated long-term forgetting (ALF) in patients with epilepsy are still under investigation. We examined the contribution of hippocampal subfields and their morphology to long-term memory performance in patients with focal epilepsy. METHODS: We prospectively assessed long-term memory and performed magnetic resonance imaging in 80 patients with focal epilepsy (61 with temporal lobe epilepsy and 19 with extratemporal lobe epilepsy) and 30 healthy controls. The patients also underwent electroencephalography recording. Verbal and visuospatial memory was tested 30 s, 10 min, and 1 week after learning. We assessed the volumes of the whole hippocampus and seven subfields and deformation of the hippocampal shape. The contributions of the hippocampal volumes and shape deformation to long-term forgetting, controlling for confounding factors, including the presence of interictal epileptiform discharges, were assessed by multiple regression analyses. RESULTS: Patients with focal epilepsy had lower intelligence quotients and route recall scores at 10 min than controls. The focal epilepsy group had smaller volumes of both the right and left hippocampal tails than the control group, but there were no statistically significant group differences for the volumes of the whole hippocampus or other hippocampal subfields. Multiple regression analyses showed a significant association between the left CA1 volume and the 1-week story retention (β = 7.76; Bonferroni-corrected p = 0.044), but this was not found for the whole hippocampus or other subfield volumes. Hippocampal shape analyses revealed that atrophy of the superior-lateral, superior-central, and inferior-medial regions of the left hippocampus, corresponding to CA1 and CA2/3, was associated with the verbal retention rate. SIGNIFICANCE: Our results suggest that atrophy of the hippocampal CA1 region and its associated structures disrupts long-term memory consolidation in focal epilepsy. Neuronal cell loss in specific hippocampal subfields could be a key underlying cause of ALF in patients with epilepsy..|
|2.||Naoto Kuroda, Takafumi Kubota, Toru Horinouchi, Naoki Ikegaya, Yu Kitazawa, Satoshi Kodama, Izumi Kuramochi, Teppei Matsubara, Naoto Nagino, Shuichiro Neshige, Temma Soga, Yutaro Takayama, Daichi Sone, Kousuke Kanemoto, Akio Ikeda, Kiyohito Terada, Hiroko Goji, Shinji Ohara, Koichi Hagiwara, Takashi Kamada, Koji Iida, Nobutsune Ishikawa, Hideaki Shiraishi, Osato Iwata, Hidenori Sugano, Yasushi Iimura, Takuichiro Higashi, Hiroshi Hosoyama, Ryosuke Hanaya, Akihiro Shimotake, Takayuki Kikuchi, Takeshi Yoshida, Hiroshi Shigeto, Jun Yokoyama, Takahiko Mukaino, Masaaki Kato, Masanori Sekimoto, Masahiro Mizobuchi, Yoko Aburakawa, Masaki Iwasaki, Eiji Nakagawa, Tomohiro Iwata, Kentaro Tokumoto, Takuji Nishida, Yukitoshi Takahashi, Kenjiro Kikuchi, Ryuki Matsuura, Shin-Ichiro Hamano, Ayataka Fujimoto, Hideo Enoki, Kyoichi Tomoto, Masako Watanabe, Youji Takubo, Toshihiko Fukuchi, Hidetoshi Nakamoto, Yuichi Kubota, Naoto Kunii, Yuichiro Shirota, Eiichi Ishikawa, Nobukazu Nakasato, Taketoshi Maehara, Motoki Inaji, Shunsuke Takagi, Takashi Enokizono, Yosuke Masuda, Takahiro Hayashi, Impact of COVID-19 pandemic on epilepsy care in Japan: A national-level multicenter retrospective cohort study., Epilepsia open, 10.1002/epi4.12616, 2022.05, OBJECTIVE: The impact of the coronavirus disease 2019 (COVID-19) pandemic on epilepsy care across Japan was investigated by conducting a multicenter retrospective cohort study. METHODS: This study included monthly data on the frequency of (1) visits by outpatients with epilepsy, (2) outpatient electroencephalography (EEG) studies, (3) telemedicine for epilepsy, (4) admissions for epilepsy, (5) EEG monitoring, and (6) epilepsy surgery in epilepsy centers and clinics across Japan between January 2019 and December 2020. We defined the primary outcome as epilepsy-center-specific monthly data divided by the 12-month average in 2019 for each facility. We determined whether the COVID-19 pandemic-related factors (such as year [2019 or 2020], COVID-19 cases in each prefecture in the previous month, and the state of emergency) were independently associated with these outcomes. RESULTS: In 2020, the frequency of outpatient EEG studies (-10.7%, p<0.001) and cases with telemedicine (+2,608%, p=0.031) were affected. The number of COVID-19 cases was an independent associated factor for epilepsy admission (-3.75*10-3 % per case, p<0.001) and EEG monitoring (-3.81*10-3 % per case, p = 0.004). Further, the state of emergency was an independent factor associated with outpatient with epilepsy (-11.9%, p<0.001), outpatient EEG (-32.3%, p<0.001), telemedicine for epilepsy (+12,915%, p<0.001), epilepsy admissions (-35.3%; p<0.001), EEG monitoring (-24.7%: p<0.001), and epilepsy surgery (-50.3%, p<0.001). SIGNIFICANCE: We demonstrated the significant impact that the COVID-19 pandemic had on epilepsy care. These results support those of previous studies and clarify the effect size of each pandemic-related factor on epilepsy care..|
|3.||Katsuhiro Goto, Takafumi Shimogawa, Nobutaka Mukae, Tadahisa Shono, Fujio Fujiki, Atsuo Tanaka, Ayumi Sakata, Hiroshi Shigeto, Koji Yoshimoto, Takato Morioka, Implications and limitations of magnetic resonance perfusion imaging with 1.5-Tesla pulsed arterial spin labeling in detecting ictal hyperperfusion during non-convulsive status epileptics., Surgical neurology international, 10.25259/SNI_841_2021, 13, 147-147, 2022.03, Background: Recent our reports showed that 3-T pseudocontinuous arterial spin labeling (3-T pCASL) magnetic resonance perfusion imaging with dual post labeling delay (PLD) of 1.5 and 2.5 s clearly demonstrated the hemodynamics of ictal hyperperfusion associated with non-convulsive status epilepticus (NCSE). We aimed to examine the utility of 1.5-T pulsed arterial spin labeling (1.5-T PASL), which is more widely available for daily clinical use, for detecting ictal hyperperfusion. Methods: We retrospectively analyzed the findings of 1.5-T PASL with dual PLD of 1.5 s and 2.0 s in six patients and compared the findings with ictal electroencephalographic (EEG) findings. Results: In patients 1 and 2, we observed the repeated occurrence of ictal discharges (RID) on EEG. In patient 1, with PLDs of 1.5 s and 2.0 s, ictal ASL hyperperfusion was observed at the site that matched the RID localization. In patient 2, the RID amplitude was extremely low, with no ictal ASL hyperperfusion. In patient 3 with lateralized periodic discharges (LPD), we observed ictal ASL hyperperfusion at the site of maximal LPD amplitude, which was apparent at a PLD of 2.0 s but not 1.5 sec. Among three patients with rhythmic delta activity (RDA) of frequencies <2.5 Hz (Patients 4-6), we observed obvious and slight increases in ASL signals in patients 4 and 5 with NCSE, respectively. However, there was no apparent change in ASL signals in patient 6 with possible NCSE. Conclusion: The detection of ictal hyperperfusion on 1.5-T PASL might depend on the electrophysiological intensity of the epileptic ictus, which seemed to be more prominent on 1.5-T PASL than on 3-T pCASL. The 1.5-T PASL with dual PLDs showed the hemodynamics of ictal hyperperfusion in patients with RID and LPD. However, it may not be visualized in patients with extremely low amplitude RID or RDA (frequencies <2.5 Hz)..|
|4.||Ayako Motoki, Naoki Akamatsu, Tomoyuki Fumuro, Ayako Miyoshi, Hideaki Tanaka, Koichi Hagiwara, Shinji Ohara, Takashi Kamada, Hiroshi Shigeto, Hiroyuki Murai, Characteristics of olfactory dysfunction in patients with temporal lobe epilepsy., Epilepsy & behavior : E&B, 10.1016/j.yebeh.2021.108402, 125, 108402-108402, 2021.12, OBJECTIVE: To determine the characteristics of olfactory dysfunction in patients with temporal lobe epilepsy (TLE). METHODS: Odor identification was assessed using the odor stick identification test for Japanese (OSIT-J, full score 12 points) in 65 patients with TLE and in 74 controls. RESULTS: The mean OSIT-J score was significantly lower in patients with TLE (mean ± SD = 8.1 ± 2.8; median = 9) than in the control subjects (mean ± SD = 10.6 ± 1.1; median = 11) (P < 0.005). Olfactory dysfunction (hyposmia/anosmia) was associated with bilateral seizure foci and older age of onset in TLE. Patients who underwent temporal lobectomy for hippocampal sclerosis did not show significant decline after long-term recovery. The Indian ink part of OSIT-J was useful for the detection of olfactory deficits in patients with TLE (sensitivity = 47%, specificity = 93%). Patients with TLE tended to have preserved olfactory ability for stimulating odors and for familiar odors of daily life. SIGNIFICANCE: We observed characteristic odor identification deficits for individual odors used in OSIT-J. Our study findings provide deeper insight into the underlying mechanism of olfactory function in patients with TLE and may be beneficial in the clinical management of these patients..|
|5.||Ayako Motoki, Naoki Akamatsu, Tomoyuki Fumuro, Ayako Miyoshi, Hideaki Tanaka, Koichi Hagiwara, Shinji Ohara, Takashi Kamada, Hiroshi Shigeto, Hiroyuki Murai, Comparison of Acute Withdrawal and Slow Taper of Antiseizure Medications during Video Electroencephalographic Monitoring: Efficacy for Shortening of Hospital Stay., Journal of clinical medicine, 10.3390/jcm10245972, 10, 24, 2021.12, Antiepileptic medications (ASMs) are withdrawn at the epilepsy monitoring unit to facilitate seizure recordings. The effect of rapid tapering of ASMs on the length of hospital stay has not been well documented. We compared the mean length of hospital stay between patients who underwent acute ASM withdrawal and slow dose tapering during long-term video electroencephalography (EEG) monitoring. We retrospectively investigated 57 consecutive patients admitted to the epilepsy monitoring unit regarding the mean length of hospital stay in the acute ASM withdrawal group (n = 30) and slow-taper group (n = 27). In the acute-withdrawal group, all ASMs were discontinued once the patients were admitted. In the slow-taper group, the doses of ASMs were gradually reduced by 15-30% daily. We also evaluated the safety of the acute-withdrawal and slow-taper protocols. The mean lengths of hospital stay were 3.8 ± 1.92 and 5.2 ± 0.69 days in the acute-withdrawal and slow-taper groups, respectively (p < 0.005). No severe adverse events, including status epilepticus, were observed. Acute ASM withdrawal has the advantage of significantly reducing the length of hospital stay over slow tapering, without any severe adverse effects..|
|6.||Nobutaka Mukae, Takafumi Shimogawa, Ayumi Sakata, Taira Uehara, Hiroshi Shigeto, Koji Yoshimoto, Takato Morioka, Reflection of the Ictal Electrocorticographic Discharges Confined to the Medial Temporal Lobe to the Scalp-Recorded Electroencephalogram., Clinical EEG and neuroscience, 10.1177/15500594211062702, 15500594211062702-15500594211062702, 2021.11, Objective: Previous reports on the simultaneous recording of electroencephalography (EEG) and electrocorticography (ECoG) have demonstrated that, in patients with temporal lobe epilepsy (TLE), ictal ECoG discharges with an amplitude as high as 1000 μV originating from the medial temporal lobe could not be recorded on EEG. In contrast, ictal EEG discharges were recorded after ictal ECoG discharges propagated to the lateral temporal lobe. Here, we report a case of TLE in which the ictal EEG discharges, corresponding to ictal ECoG discharges confined to the medial temporal lobe, were recorded. Case report: In the present case, ictal EEG discharges were hardly recognized when the amplitude of the ECoG discharges was less than 1500 μV. During the evolution and burst suppression phase, corresponding to highly synchronized ECoG discharges with amplitudes greater than 1500 to 2000 μV, rhythmic negative waves with the same frequency were clearly recorded both on the lateral temporal lobe and scalp. The amplitude of the lateral temporal ECoG was approximately one-tenth of that of the medial temporal ECoG. The amplitude of the scalp EEG was approximately one-tenth of that of the lateral temporal ECoG. Conclusions: Highly synchronized ictal ECoG discharges with high amplitude of greater than 1500 to 2000 μV in the medial temporal lobe could be recorded on the scalp as ictal EEG discharges via volume conduction..|
|7.||Koichi Hagiwara, Toshiki Okadome, Takahiko Mukaino, Taira Uehara, Hideaki Tanaka, Takashi Kamada, Ayako Miyoshi, Naoki Akamatsu, Shinji Ohara, Hiroshi Shigeto, Ictal asystole as a manifestation of pure insular epilepsy., Seizure, 10.1016/j.seizure.2021.06.026, 91, 192-195, 2021.10.|
|8.||Teppei Matsubara, Seppo P Ahlfors, Tatsuya Mima, Koichi Hagiwara, Hiroshi Shigeto, Shozo Tobimatsu, Yoshinobu Goto, Steven Stufflebeam, Bilateral Representation of Sensorimotor Responses in Benign Adult Familial Myoclonus Epilepsy: An MEG Study., Frontiers in neurology, 10.3389/fneur.2021.759866, 12, 759866-759866, 2021.10, Patients with cortical reflex myoclonus manifest typical neurophysiologic characteristics due to primary sensorimotor cortex (S1/M1) hyperexcitability, namely, contralateral giant somatosensory-evoked potentials/fields and a C-reflex (CR) in the stimulated arm. Some patients show a CR in both arms in response to unilateral stimulation, with about 10-ms delay in the non-stimulated compared with the stimulated arm. This bilateral C-reflex (BCR) may reflect strong involvement of bilateral S1/M1. However, the significance and exact pathophysiology of BCR within 50 ms are yet to be established because it is difficult to identify a true ipsilateral response in the presence of the giant component in the contralateral hemisphere. We hypothesized that in patients with BCR, bilateral S1/M1 activity will be detected using MEG source localization and interhemispheric connectivity will be stronger than in healthy controls (HCs) between S1/M1 cortices. We recruited five patients with cortical reflex myoclonus with BCR and 15 HCs. All patients had benign adult familial myoclonus epilepsy. The median nerve was electrically stimulated unilaterally. Ipsilateral activity was investigated in functional regions of interest that were determined by the N20m response to contralateral stimulation. Functional connectivity was investigated using weighted phase-lag index (wPLI) in the time-frequency window of 30-50 ms and 30-100 Hz. Among seven of the 10 arms of the patients who showed BCR, the average onset-to-onset delay between the stimulated and the non-stimulated arm was 8.4 ms. Ipsilateral S1/M1 activity was prominent in patients. The average time difference between bilateral cortical activities was 9.4 ms. The average wPLI was significantly higher in the patients compared with HCs in specific cortico-cortical connections. These connections included precentral-precentral, postcentral-precentral, inferior parietal (IP)-precentral, and IP-postcentral cortices interhemispherically (contralateral region-ipsilateral region), and precentral-IP and postcentral-IP intrahemispherically (contralateral region-contralateral region). The ipsilateral response in patients with BCR may be a pathologically enhanced motor response homologous to the giant component, which was too weak to be reliably detected in HCs. Bilateral representation of sensorimotor responses is associated with disinhibition of the transcallosal inhibitory pathway within homologous motor cortices, which is mediated by the IP. IP may play a role in suppressing the inappropriate movements seen in cortical myoclonus..|
|9.||Taira Uehara, Hiroshi Shigeto, Takahiko Mukaino, Jun Yokoyama, Toshiki Okadome, Ryo Yamasaki, Katsuya Ogata, Nobutaka Mukae, Ayumi Sakata, Shozo Tobimatsu, Jun-Ichi Kira, Rapidly spreading seizures arise from large-scale functional brain networks in focal epilepsy., NeuroImage, 10.1016/j.neuroimage.2021.118104, 237, 118104-118104, 2021.08, It remains unclear whether epileptogenic networks in focal epilepsy develop on physiological networks. This work aimed to explore the association between the rapid spread of ictal fast activity (IFA), a proposed biomarker for epileptogenic networks, and the functional connectivity or networks of healthy subjects. We reviewed 45 patients with focal epilepsy who underwent electrocorticographic (ECoG) recordings to identify the patients showing the rapid spread of IFA. IFA power was quantified as normalized beta-gamma band power. Using published resting-state functional magnetic resonance imaging databases, we estimated resting-state functional connectivity of healthy subjects (RSFC-HS) and resting-state networks of healthy subjects (RSNs-HS) at the locations corresponding to the patients' electrodes. We predicted the IFA power of each electrode based on RSFC-HS between electrode locations (RSFC-HS-based prediction) using a recently developed method, termed activity flow mapping. RSNs-HS were identified using seed-based and atlas-based methods. We compared IFA power with RSFC-HS-based prediction or RSNs-HS using non-parametric correlation coefficients. RSFC and seed-based RSNs of each patient (RSFC-PT and seed-based RSNs-PT) were also estimated using interictal ECoG data and compared with IFA power in the same way as RSFC-HS and seed-based RSNs-HS. Spatial autocorrelation-preserving randomization tests were performed for significance testing. Nine patients met the inclusion criteria. None of the patients had reflex seizures. Six patients showed pathological evidence of a structural etiology. In total, we analyzed 49 seizures (2-13 seizures per patient). We observed significant correlations between IFA power and RSFC-HS-based prediction, seed-based RSNs-HS, or atlas-based RSNs-HS in 28 (57.1%), 21 (42.9%), and 28 (57.1%) seizures, respectively. Thirty-two (65.3%) seizures showed a significant correlation with either seed-based or atlas-based RSNs-HS, but this ratio varied across patients: 27 (93.1%) of 29 seizures in six patients correlated with either of them. Among atlas-based RSNs-HS, correlated RSNs-HS with IFA power included the default mode, control, dorsal attention, somatomotor, and temporal-parietal networks. We could not obtain RSFC-PT and RSNs-PT in one patient due to frequent interictal epileptiform discharges. In the remaining eight patients, most of the seizures showed significant correlations between IFA power and RSFC-PT-based prediction or seed-based RSNs-PT. Our study provides evidence that the rapid spread of IFA in focal epilepsy can arise from physiological RSNs. This finding suggests an overlap between epileptogenic and functional networks, which may explain why functional networks in patients with focal epilepsy frequently disrupt..|
|10.||Nobutaka Mukae, Daisuke Kuga, Daisuke Murakami, Noritaka Komune, Yusuke Miyamoto, Takafumi Shimogawa, Ayumi Sakata, Hiroshi Shigeto, Toru Iwaki, Takato Morioka, Masahiro Mizoguchi, Endonasal endoscopic surgery for temporal lobe epilepsy associated with sphenoidal encephalocele., Surgical neurology international, 10.25259/SNI_542_2021, 12, 379-379, 2021.07, Background: Temporal lobe epilepsy (TLE) associated with temporal lobe encephalocele is rare, and the precise epileptogenic mechanisms and surgical strategies for such cases are still unknown. Although the previous studies have reported good seizure outcomes following chronic subdural electrode recording through invasive craniotomy, only few studies have reported successful epilepsy surgery through endoscopic endonasal lesionectomy. Case Description: An 18-year-old man developed generalized convulsions at the age of 15 years. Despite treatment with optimal doses of antiepileptic drugs, episodes of speech and reading difficulties were observed 2-3 times per week. Long-term video electroencephalogram (EEG) revealed ictal activities starting from the left anterior temporal region. Magnetic resonance imaging revealed a temporal lobe encephalocele in the left lateral fossa of the sphenoidal sinus (sphenoidal encephalocele). Through the endoscopic endonasal approach, the tip of the encephalocele was exposed. A depth electrode was inserted into the encephalocele, which showed frequent spikes superimposed with high-frequency oscillations (HFOs) suggesting intrinsic epileptogenicity. The encephalocele was resected 8 mm from the tip. Twelve months postoperatively, the patient had no recurrence of seizures on tapering of the medication. Conclusion: TLE associated with sphenoidal encephalocele could be controlled with endoscopic endonasal lesionectomy, after confirming the high epileptogenicity with analysis of HFOs of intraoperative EEG recorded using an intralesional depth electrode..|
|11.||Sakata A, Mukae N, Morioka T, Tanaka S, Shimogawa T, Shigeto H, Hotta T, Kang D, Mizoguchi M., Simultaneous Electroencephalographic and Electocorticographic Recordings of Lateralized Periodic Discharges in Temporal Lobe Epilepsy., Clin EEG Neurosci, 10.1177/1550059420972266., Online ahead of print, 2020.11, 片側性周期性放電を頭蓋内外で記録して比較した。.|
|12.||Mitsunori Shimmura, Taira Uehara, Katsuya Ogata, Hiroshi Shigeto, Tomoko Maeda, Ayumi Sakata, Ryo Yamasaki, Jun-Ichi Kira, Corrigendum to "Higher postictal parasympathetic activity following greater ictal heart rate increase in right- than left-sided seizures" [Epilepsy Behav 97 (2019) 161-168]., Epilepsy & behavior : E&B, 10.1016/j.yebeh.2019.106865, 103, Pt A, 106865-106865, 2020.02.|
|13.||Shimmura M, Uehara T, Ogata K, Shigeto H, Maeda T, Sakata A, Yamasaki R, Kira JI., Higher postictal parasympathetic activity following greater ictal heart rate increase in right- than left-sided seizures., Epilepsy Behav, 10.1016/j.yebeh.2019.05.026., 97, 161-168, 2019.08, 心拍から側頭葉てんかん性活動の側在性を推定できる。.|
|14.||Mitsunori Shimmura, Taira Uehara, Katsuya Ogata, Hiroshi Shigeto, Tomoko Maeda, Ayumi Sakata, Ryo Yamasaki, Jun-Ichi Kira, Higher postictal parasympathetic activity following greater ictal heart rate increase in right- than left-sided seizures., Epilepsy & behavior : E&B, 10.1016/j.yebeh.2019.05.026, 97, 161-168, 2019.08, OBJECTIVES: The objectives of this study were to determine how hemispheric laterality of seizure activity influences periictal heart rate variability (HRV) and investigate the ability of HRV parameters to discriminate right- and left-sided seizures. METHODS: Long-term video electroencephalogram-electrocardiogram recordings of 54 focal seizures in 25 patients with focal epilepsy were reviewed. Using linear mixed models, we examined the effect of seizure laterality on linear (standard deviation of R-R intervals [SDNN], root mean square of successive differences [RMSSD], low frequency [LF] and high frequency [HF] power of HRV, and LF/HF) and nonlinear (standard deviation [SD]1, SD2, and SD2/SD1 derived from Poincaré plots) periictal HRV parameters, the magnitude of heart rate (HR) changes, and the onset time of increased HR. Receiver operating characteristics (ROC) were used to determine the ability of these parameters to discriminate between right- and left-sided seizures. RESULTS: Postictal SDNN, RMSSD, LF, HF, SD1, and SD2 were higher in right- than left-sided seizures. Root mean square of successive difference and HF were decreased after left- but not right-sided seizures. Standard deviation of R-R intervals, LF, and SD1 were increased after right- but not left-sided seizures. Increased ictal HR was earlier and larger in right- than left-sided seizures. Postictal HF showed the greatest area under the ROC curve (AUC) (0.87) for discriminating right- and left-sided seizures. CONCLUSIONS: Our data suggest that postictal parasympathetic activity is higher, whereas ictal HR increase is greater, in right- than left-sided seizures. Involvement of the right hemisphere may be associated with postictal autonomic instability. Postictal HRV parameters may provide useful information on hemispheric laterality of seizure activity..|
|15.||Teppei Matsubara, Katsuya Ogata, Naruhito Hironaga, Taira Uehara, Takako Mitsudo, Hiroshi Shigeto, Toshihiko Maekawa, Shozo Tobimatsu, Monaural 40-Hz auditory steady-state magnetic responses can be useful for identifying epileptic focus in mesial temporal lobe epilepsy, CLINICAL NEUROPHYSIOLOGY, 10.1016/j.clinph.2018.11.026, 130, 3, 341-351, 2019.03, © 2018 International Federation of Clinical Neurophysiology Objective: Patients with mesial temporal lobe epilepsy (mTLE) often exhibit central auditory processing (CAP) dysfunction. Monaural 40-Hz auditory steady-state magnetic responses (ASSRs) were recorded to explore the pathophysiology of mTLE. Methods: Eighteen left mTLE patients, 11 right mTLE patients and 16 healthy controls (HCs) were examined. Monaural clicks were presented at a rate of 40 Hz. Phase-locking factor (PLF) and power values were analyzed within bilateral Heschl's gyri. Results: Monaural 40-Hz ASSR demonstrated temporal frequency dynamics in both PLF and power data. Symmetrical hemispheric contralaterality was revealed in HCs. However, predominant contralaterality was absent in mTLE patients. Specifically, right mTLE patients exhibited a lack of contralaterality in response to left ear but not right ear stimulation, and vice versa in left mTLE patients. Conclusion: This is the first study to use monaural 40-Hz ASSR with unilateral mTLE patients to clarify the relationship between CAP and epileptic focus. CAP dysfunction was characterized by a lack of contralaterality corresponding to epileptic focus. Significance: Monaural 40-Hz ASSR can provide useful information for localizing epileptic focus in mTLE patients..|
|16.||Teppei Matsubara, Katsuya Ogata, Naruhito Hironaga, Yoshikazu Kikuchi, Taira Uehara, Hiroshi Chatani, Takako Mitsudo, Hiroshi Shigeto, Shozo Tobimatsu, Altered neural synchronization to pure tone stimulation in patients with mesial temporal lobe epilepsy: An MEG study., Epilepsy & behavior : E&B, 10.1016/j.yebeh.2018.08.036, 88, 96-105, 2018.11, OBJECTIVE: Our previous study of monaural auditory evoked magnetic fields (AEFs) demonstrated that hippocampal sclerosis significantly modulated auditory processing in patients with mesial temporal lobe epilepsy (mTLE). However, the small sample size (n = 17) and focus on the M100 response were insufficient to elucidate the lateralization of the epileptic focus. Therefore, we increased the number of patients with mTLE (n = 39) to examine whether neural synchronization induced by monaural pure tone stimulation provides useful diagnostic information about epileptic foci in patients with unilateral mTLE. METHODS: Twenty-five patients with left mTLE, 14 patients with right mTLE, and 32 healthy controls (HCs) were recruited. Auditory stimuli of 500-Hz tone burst were monaurally presented to subjects. The AEF data were analyzed with source estimation of M100 responses in bilateral auditory cortices (ACs). Neural synchronization within ACs and between ACs was evaluated with phase-locking factor (PLF) and phase-locking value (PLV), respectively. Linear discriminant analysis was performed for diagnosis and lateralization of epileptic focus. RESULTS: The M100 amplitude revealed that patients with right mTLE exhibited smaller M100 amplitude than patients with left mTLE and HCs. Interestingly, PLF was able to differentiate the groups with mTLE, with decreased PLFs in the alpha band observed in patients with right mTLE compared with those (PLFs) in patients with left mTLE. Right hemispheric predominance was confirmed in both HCs and patients with left mTLE while patients with right mTLE showed a lack of right hemispheric predominance. Functional connectivity between bilateral ACs (PLV) was reduced in both patients with right and left mTLE compared with that of HCs. The accuracy of diagnosis and lateralization was 80%-90%. CONCLUSION: Auditory cortex subnormal function was more pronounced in patients with right mTLE compared with that in patients with left mTLE as well as HCs. Monaural AEFs can be used to reveal the pathophysiology of mTLE. Overall, our results indicate that altered neural synchronization may provide useful information about possible functional deterioration in patients with unilateral mTLE..|
|17.||Mitsunori Shimmura, Taira Uehara, Kenichiro Yamashita, Hiroshi Shigeto, Ryo Yamasaki, Kinya Ishikawa, Jun-Ichi Kira, Slowed abduction during smooth pursuit eye movement in episodic ataxia type 2 with a novel CACNA1A mutation, JOURNAL OF THE NEUROLOGICAL SCIENCES, 10.1016/j.jns.2017.07.040, 381, 4-6, 2017.10.|
|18.||Jun Yokoyama, Hiroo Yamaguchi, Hiroshi Shigeto, Takeshi Uchiumi, Hiroyuki Murai, Jun-Ichi Kira, A case of rhabdomyolysis after status epilepticus without stroke-like episodes in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes., Rinsho shinkeigaku = Clinical neurology, 10.5692/clinicalneurol.cn-001044, 57, 7, 400-401, 2017.07.|
|19.||Takashi Irie, Hiroshi Shigeto, Junpei Koge, Hiroo Yamaguchi, Hiroyuki Murai, Jun-Ichi Kira, Dermatomyositis complicated with asymmetric peripheral neuritis on exacerbation: A case report and literature review, Clinical and Experimental Neuroimmunology, 10.1111/cen3.12332, 7, 4, 373-380, 2016.11, We describe a 69-year-old woman with dermatomyositis who presented with asymmetric peripheral neuritis. The patient first developed dermatomyositis at 39 years-of-age
her symptoms improved several years later with oral prednisolone treatment. A year and a half before admission to our hospital, she developed a sensory disturbance in her feet that gradually expanded to both legs with left dominance. Six months later, bilateral leg muscle weakness and proximal muscle tenderness appeared. Neurological examination at admission revealed proximal muscle weakness and atrophy of all four limbs. Ankle jerk was absent bilaterally. There was sensory impairment in the left hand and both lower limbs, with more severe involvement of the left side and the medial side. Electrophysiological tests showed decreased sensory nerve action potentials in the left superficial fibular nerve and bilateral sural nerves, and decreased motor action potentials in the bilateral fibular nerves. We diagnosed mononeuritis multiplex as a complication of dermatomyositis. Her symptoms of mononeuritis multiplex and dermatomyositis were successfully treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone therapy. Although our literature search found nine cases of dermatomyositis with polyneuropathy/polyradiculoneuropathy, most of which involved axonal neuropathy, this is the first case report of dermatomyositis presenting with mononeuritis multiplex..
|20.||Yuki Matsushita, Yasunari Sakai, Mitsunori Shimmura, Hiroshi Shigeto, Miki Nishio, Satoshi Akamine, Masafumi Sanefuji, Yoshito Ishizaki, Hiroyuki Torisu, Yusaku Nakabeppu, Akira Suzuki, Hidetoshi Takada, Toshiro Hara, Corrigendum: Hyperactive mTOR signals in the proopiomelanocortin-expressing hippocampal neurons cause age-dependent epilepsy and premature death in mice., Scientific reports, 10.1038/srep27164, 6, 27164-27164, 2016.06.|
|21.||Yuki Matsushita, Yasunari Sakai, Mitsunori Shimmura, Hiroshi Shigeto, Miki Nishio, Satoshi Akamine, Masafumi Sanefuji, Yoshito Ishizaki, Hiroyuki Torisu, Yusaku Nakabeppu, Akira Suzuki, Hidetoshi Takada, Toshiro Hara, Hyperactive mTOR signals in the proopiomelanocortin-expressing hippocampal neurons cause age-dependent epilepsy and premature death in mice., Scientific reports, 10.1038/srep22991, 6, 22991-22991, 2016.03, Epilepsy is a frequent comorbidity in patients with focal cortical dysplasia (FCD). Recent studies utilizing massive sequencing data identified subsets of genes that are associated with epilepsy and FCD. AKT and mTOR-related signals have been recently implicated in the pathogenic processes of epilepsy and FCD. To clarify the functional roles of the AKT-mTOR pathway in the hippocampal neurons, we generated conditional knockout mice harboring the deletion of Pten (Pten-cKO) in Proopiomelanocortin-expressing neurons. The Pten-cKO mice developed normally until 8 weeks of age, then presented generalized seizures at 8-10 weeks of age. Video-monitored electroencephalograms detected paroxysmal discharges emerging from the cerebral cortex and hippocampus. These mice showed progressive hypertrophy of the dentate gyrus (DG) with increased expressions of excitatory synaptic markers (Psd95, Shank3 and Homer). In contrast, the expression of inhibitory neurons (Gad67) was decreased at 6-8 weeks of age. Immunofluorescence studies revealed the abnormal sprouting of mossy fibers in the DG of the Pten-cKO mice prior to the onset of seizures. The treatment of these mice with an mTOR inhibitor rapamycin successfully prevented the development of seizures and reversed these molecular phenotypes. These data indicate that the mTOR pathway regulates hippocampal excitability in the postnatal brain..|
|22.||Hiroshi Chatani, Koichi Hagiwara, Naruhito Hironaga, Katsuya Ogata, Hiroshi Shigeto, Takato Morioka, Ayumi Sakata, Kimiaki Hashiguchi, Nobuya Murakami, Taira Uehara, Jun-ichi Kira, Shozo Tobimatsu, Neuromagnetic evidence for hippocampal modulation of auditory processing, NEUROIMAGE, 10.1016/j.neuroimage.2015.09.006, 124, Pt A, 256-266, 2016.01, The hippocampus is well known to be involved in memory, as well as in perceptual processing. To date, the electrophysiological process by which unilateral hippocampal lesions, such as hippocampal sclerosis (HS), modulate the auditory processing remains unknown. Auditory-evoked magnetic fields (AEFs) are valuable for evaluating auditory functions, because M100, a major component of AEFs, originates from auditory areas. Therefore, AEFs of mesial temporal lobe epilepsy (mTLE, n = 17) with unilateral HS were compared with those of healthy (HC, n= 17) and disease controls (n= 9), thereby determining whether AEFs were indicative of hippocampal influences on the auditory processing. Monaural tone-burst stimuli were presented for each side, followed by analysis of M100 and a previously less characterized exogenous component (M400: 300-500 ms). The frequency of acceptable M100 dipoles was significantly decreased in the HS side. Beam-forming-based source localization analysis also showed decreased activity of the auditory area, which corresponded to the inadequately estimated dipoles. M400 was found to be related to the medial temporal structure on the HS side. Volumetric analysis was also performed, focusing on the auditory-related areas (planum temporale, Heschl's gyrus, and superior temporal gyrus), as well as the hippocampus. M100 amplitudes positively correlated with hippocampal and planum temporale volumes in the HC group, whereas they negatively correlated with Heschl's gyrus volume in the mTLE group. Interestingly, significantly enhanced M400 component was observed in the HS side of the mTLE patients. In addition, the M400 component positively correlated with Heschl's gyrus volume and tended to positively correlate with disease duration. M400 was markedly diminished after hippocampal resection. Although volumetric analysis showed decreased hippocampal volume in the HS side, the planum temporale and Heschl's gyrus, the two major sources of M100, were preserved. These results suggested that HS significantly influenced AEFs. Therefore, we concluded that the hippocampus modulates auditory processing differently under normal conditions and in HS. (C) 2015 The Authors. Published by Elsevier Inc..|
|23.||[Clinical and histological characteristics of ictal onset zone in cases of intractable epilepsy associated with dysembryoplastic neuroepithelial tumor].
Although the epileptogenic location of dysembryoplastic neuroepithelial tumors (DNTs) is controversial, it has recently been thought to be located within cortical dysplasia (CD) due to its frequent association with CD. Among the 84 resection surgeries for intractable epilepsy performed in our institution between January 2003 and April 2010, three patients had epileptogenic DNTs. In two cases, chronic subdural electrocorticography (ECoG) was performed, and the ictal onset zone was revealed to be in the cortex around the DNT. The ictal onset zone was resected along with the DNT, and good seizure outcome was achieved. Although histological examination of the ictal onset zone revealed mild gliosis, coexistence of CD was not noted. In the third case, the DNT was located in the left lateral temporal lobe and the intraoperative ECoG revealed frequent paroxysmal activity in the medial temporal lobe. Resection of the lateral temporal lobe involving the tumor did not result in good seizure control. The optimal surgical treatment of DNT is controversial. Some authors consider lesionectomy to be sufficient for good seizure control, whereas others advocate that additional resection of the epileptogenic zone beside the tumor improves outcome. Because the epileptogenic location of DNT varies among cases, it is important to identify its location by preoperative multimodal examinations, including chronic subdural ECoG recordings..
|24.||Nobutaka Mukae, Satoshi O Suzuki, Takato Morioka, Nobuya Murakami, Kimiaki Hashiguchi, Hiroshi Shigeto, Ayumi Sakata, Koji Iihara, ILAE focal cortical dysplasia type IIIc in the ictal onset zone in epileptic patients with solitary meningioangiomatosis., Epileptic disorders : international epilepsy journal with videotape, 10.1684/epd.2014.0695, 16, 4, 533-9, 2014.12, "Solitary" meningioangiomatosis (MA) is a rare, benign, hamartomatous lesion of the cerebral cortex and frequently leads to epilepsy. However, the source of the epileptogenicity in meningioangiomatosis remains controversial. We report two surgically-treated meningioangiomatosis cases with medically intractable epilepsy. In both cases, chronic subdural electrocorticogram (ECoG) recordings identified the ictal onset zone on apparently normal cortex, adjacent to and/or above the meningioangiomatosis lesion, not on the meningioangiomatosis lesion itself. The ictal onset zone was resected, along with the MA lesion, and good seizure outcome was achieved. Histological examination of the ictal onset zone revealed the presence of ILAE focal cortical dysplasia (FCD) type IIIc. Our case studies suggest that in the surgical management of epilepsy with meningioangiomatosis, it is important to identify undetected, but epileptogenic, ILAE FCD Type IIIc, using preoperative multimodal examinations, including chronic ECoG recordings..|
|25.||[Patients with intractable epilepsy who achieved good seizure control after craniotomy instead of vagal nerve stimulation].
Vagal nerve stimulation(VNS)is an effective adjunctive therapy for medically intractable epilepsy. However, VNS is a palliative therapy, and craniotomy should preferably be performed when complete seizure remission can be expected after craniotomy. We report here three patients who were referred for VNS therapy, but underwent craniotomy instead of VNS based on the results of a comprehensive preoperative evaluation, and achieved good seizure control. Case 1 was a 48-year-old woman with left temporal lobe epilepsy and amygdalar enlargement. Even though no left hippocampal sclerosis was observed on magnetic resonance imaging, she underwent left anterior temporal lobectomy and hippocampectomy. Case 2 was a 36-year-old woman with multiple bilateral subependymal nodular heterotopias, who underwent resection of the left medial temporal lobe including subependymal nodular heterotopias adjacent to the left inferior horn. Case 3 was a 25-year-old man with posttraumatic epilepsy. As the right hemisphere was most affected, multiple subpial transections were performed on the left frontal convexity. These three patients were referred to us for VNS therapy because there was a dissociation between the interictal electroencephalogram and magnetic resonance imaging findings, or because they had multiple or extensive epileptogenic lesions. Comprehensive preoperative evaluation including ictal electroencephalography can help to identify patients who are suitable candidates for craniotomy..
|26.||Koichi Hagiwara, Hiroshi Shigeto, Age-related changes across the primary and secondary somatosensory areas: an analysis of neuromagnetic oscillatory activities, Clin Neurophysiol, 2014.05.|
|27.||Koichi Hagiwara, Katsuya Ogata, Tsuyoshi Okamoto, Taira Uehara, Naruhito Hironaga, Hiroshi Shigeto, Jun-ichi Kira, Shozo Tobimatsu, Age-related changes across the primary and secondary somatosensory areas: An analysis of neuromagnetic oscillatory activities, CLINICAL NEUROPHYSIOLOGY, 10.1016/j.clinph.2013.10.005, 125, 5, 1021-1029, 2014.05, Objective: Age-related changes are well documented in the primary somatosensory cortex (SI). Based on previous somatosensory evoked potential studies, the amplitude of N20 typically increases with age probably due to cortical disinhibition. However, less is known about age-related change in the secondary somatosensory cortex (SII). The current study quantified age-related changes across SI and SII mainly based on oscillatory activity indices measured with magnetoencephalography.
Methods: We recorded somatosensory evoked magnetic fields (SEFs) to right median nerve stimulation in healthy young and old subjects and assessed major SEF components. Then, we evaluated the phase-locking factor (PLF) for local field synchrony on neural oscillations and the weighted phase-lag index (wPLI) for cortico-cortical synchrony between SI and SII.
Results: PLF was significantly increased in SI along with the increased amplitude of N20m in the old subjects. PLF was also increased in SII associated with a shortened peak latency of SEFs. wPLI analysis revealed the increased coherent activity between SI and SII.
Conclusions: Our results suggest that the functional coupling between SI and SII is influenced by the cortical disinhibition due to normal aging. Significance: We provide the first electrophysiological evidence for age-related changes in oscillatory neural activities across the somatosensory areas. (C) 2013 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved..
|28.||Hideaki Miyaji, Naruhito Hironaga, Toshiro Umezaki, Koichi Hagiwara, Hiroshi Shigeto, Motohiro Sawatsubashi, Shozo Tobimatsu, Shizuo Komune, Neuromagnetic detection of the laryngeal area: Sensory-evoked fields to air-puff stimulation, NEUROIMAGE, 10.1016/j.neuroimage.2013.11.008, 88, 162-169, 2014.03, The sensory projections from the oral cavity, pharynx, and larynx are crucial in assuring safe deglutition, coughing, breathing, and voice production/speaking. Although several studies using neuroimaging techniques have demonstrated cortical activation related to pharyngeal and laryngeal functions, little is known regarding sensory projections from the laryngeal area to the somatosensory cortex. The purpose of this study was to establish the cortical activity evoked by somatic air-puff stimulation at the laryngeal mucosa using magnetoencephalography. Twelve healthy volunteers were trained to inhibit swallowing in response to air stimuli delivered to the larynx. Minimum norm estimates was performed on the laryngeal somatosensory evoked fields (LSEFs) to best differentiate the target activations from non-task-related activations. Evoked magnetic fields were recorded with acceptable reproducibility in the left hemisphere, with a peak latency of approximately 100 ms in 10 subjects. Peak activation was estimated at the caudolateral region of the primary somatosensory area (S1). These results establish the ability to detect LSEFs with an acceptable reproducibility within a single subject and among subjects. These results also suggest the existence of laryngeal somatic afferent input to the caudolateral region of S1 in human. Our findings indicate that further investigation in this area is needed, and should focus on laryngeal lateralization, swallowing, and speech processing. (C) 2013 Elsevier Inc All rights reserved..|
|29.||Chikara Yamashita, Hiroshi Shigeto, Transient interhemispheric disconnection in a case of insulinoma-induced hypoglycemic encephalopathy, J Neurol Sci, 2013.12.|
|30.||Chikara Yamashita, Hiroshi Shigeto, Norihisa Maeda, Minako Kawaguchi, Mitsue Uryu, Satoru Motomura, Jun-ichi Kira, Transient interhemispheric disconnection in a case of insulinoma-induced hypoglycemic encephalopathy., Journal of the neurological sciences, 10.1016/j.jns.2013.09.025, 335, 1-2, 233-7, 2013.12, We report a case of a 22-year-old male who was transferred to our hospital in a comatose state following successive seizures. Low blood glucose had been detected upon his arrival at the previous hospital. He became responsive 12 days after the onset of coma. Upon regaining consciousness he exhibited severe dysarthria and several interhemispheric disconnection signs such as intermanual conflict, left-hand dysgraphia, left hemispatial neglect confined to the right hand, impaired interhemispheric transfer, and unilateral constructional apraxia of the right hand. Brain MRI disclosed T2-weighted and diffusion-weighted hyperintense lesions with reduced apparent diffusion coefficients in the bilateral centrum semiovale, splenium of the corpus callosum, right posterior limb of the internal capsule, and bilateral middle cerebellar peduncles. As the MRI findings vanished, his interhemispheric disconnection signs gradually resolved. Abdominal imaging studies revealed a pancreatic tumor, which was later endocrinologically diagnosed as an insulinoma. This is an extremely rare report of interhemispheric disconnection signs due to hypoglycemic encephalopathy. The lesions in the bilateral centrum semiovale likely contributed to the interhemispheric disconnection signs..|
|31.||Miyaji, Naruhito Hironaga, Hiroshi Shigeto, Neuromagnetic detection of the laryngeal area: Sensory-evoked fields to air-puff stimulation., Neuroimage, 88, 169-169, 2013.11.|
|32.||Hiroshi Shigeto, 鎌田 崇嗣, Spontaneous seizures in a rat model of multiple prenatal freeze lesioning, Epilepsy Res., 105, 3, 280-291, 2013.08.|
|33.||Noriko Yutsudo, Takashi Kamada, Kosuke Kajitani, Hiroko Nomaru, Atsuhisa Katogi, Yoko H. Ohnishi, Yoshinori N. Ohnishi, Kei Ichiro Takase, Sakumi Kunihiko, Hiroshi Shigeto, Yusaku Nakabeppu, Erratum FosB-null mice display impaired adult hippocampal neurogenesis and spontaneous epilepsy with depressive behavior (Neuropsychopharmacology (2013) 38 (1374-1375) DOI: 10.1038/npp.2013.56), Neuropsychopharmacology, 10.1038/npp.2013.56, 38, 7, 1374-1375, 2013.06.|
|34.||Noriko Yutsudo, Takashi Kamada, Kosuke Kajitani, Hiroko Nomaru, Atsuhisa Katogi, Yoko H Ohnishi, Yoshinori N Ohnishi, Kei-ichiro Takase, Kunihiko Sakumi, Hiroshi Shigeto, Yusaku Nakabeppu, fosB-null mice display impaired adult hippocampal neurogenesis and spontaneous epilepsy with depressive behavior., Neuropsychopharmacology : official publication of the American College of Neuropsychopharmacology, 10.1038/npp.2012.260, 38, 5, 895-906, 2013.04, Patients with epilepsy are at high risk for major depression relative to the general population, and both disorders are associated with changes in adult hippocampal neurogenesis, although the mechanisms underlying disease onset remain unknown. The expression of fosB, an immediate early gene encoding FosB and ΔFosB/Δ2ΔFosB by alternative splicing and translation initiation, is known to be induced in neural progenitor cells within the subventricular zone of the lateral ventricles and subgranular zone of the hippocampus, following transient forebrain ischemia in the rat brain. Moreover, adenovirus-mediated expression of fosB gene products can promote neural stem cell proliferation. We recently found that fosB-null mice show increased depressive behavior, suggesting impaired neurogenesis in fosB-null mice. In the current study, we analyzed neurogenesis in the hippocampal dentate gyrus of fosB-null and fosB(d/d) mice that express ΔFosB/Δ2ΔFosB but not FosB, in comparison with wild-type mice, alongside neuropathology, behaviors, and gene expression profiles. fosB-null but not fosB(d/d) mice displayed impaired neurogenesis in the adult hippocampus and spontaneous epilepsy. Microarray analysis revealed that genes related to neurogenesis, depression, and epilepsy were altered in the hippocampus of fosB-null mice. Thus, we conclude that the fosB-null mouse is the first animal model to provide a genetic and molecular basis for the comorbidity between depression and epilepsy with abnormal neurogenesis, all of which are caused by loss of a single gene, fosB..|
|35.||Yuji Kanamori, Hiroshi Shigeto, Minimum norm estimates in MEG can delineate the onset of interictal epileptic discharges: A comparison with ECoG findings, Neuroimage Clin., 2013.04.|
|36.||Hiroshi Shigeto, Atthaporn Boongird, Kenneth Baker, Christoph Kellinghaus, Imad Najm, Hans Lüders, Systematic study of the effects of stimulus parameters and stimulus location on afterdischarges elicited by electrical stimulation in the rat., Epilepsy research, 10.1016/j.eplepsyres.2012.10.002, 104, 1-2, 17-25, 2013.03, Electrical brain stimulation is used in a variety of clinical situations, including cortical mapping for epilepsy surgery, cortical stimulation therapy to terminate seizure activity in the cortex, and in deep brain stimulation therapy. However, the effects of stimulus parameters are not fully understood. In this study, we systematically tested the impact of various stimulation parameters on the generation of motor symptoms and afterdischarges (ADs). Focal electrical stimulation was delivered at subdural cortical, intracortical, and hippocampal sites in a rat model. The effects of stimulus parameter on the generation of motor symptoms and on the occurrence of ADs were examined. The effect of stimulus irregularity was tested using random or regular 50Hz stimulation through subdural electrodes. Hippocampal stimulation produced ADs at lower thresholds than neocortical stimulation. Hippocampal stimulation also produced significantly longer ADs. Both in hippocampal and cortical stimulation, when the total current was kept constant with changing pulse width, the threshold for motor symptom or AD was lowest between 50 and 100Hz and higher at both low and high frequencies. However, if the pulse width was fixed, the threshold did not increase above 100Hz and it apparently continued to decrease through 800Hz even if the difference did not reach statistical significance. There was no significant difference between random and regular stimulation. Overall, these results indicate that electrode location and several stimulus parameters including frequency, pulse width, and total electricity are important in electrical stimulation to produce motor symptoms and ADs..|
|37.||Sanefuji M, Hiroshi Shigeto, Toshiro Hara, A case of childhood stiff-person syndrome with striatal lesions: A possible entity distinct from the classical adult form, Brain Dev, 2012.08.|
|38.||Noriko Isobe, Hiroshi Shigeto, Jun-ichi Kira, First diagnostic criteria for atopic myelitis with special reference to discrimination from myelitis-onset multiple sclerosis., J Neurol Sci., 316, 30-35, 2012.05.|
|39.||Saiga, Hiroshi Shigeto, Tateishi, Ohyagi, Inflammatory radiculoneuropathy in an ALS4 patient with a novel SETX mutation., J Neurol Neurosurg Psychiatry, 83, 763-764, 2012.05.|
|40.||Tomonori Iwata, Hiroshi Shigeto, Katsuya Ogata, Ko-ichi Hagiwara, Yuji Kanamori, Taira Uehara, Yasumasa Ohyagi, Shozo Tobimatsu, Jun-ichi Kira, Hyperexcitability restricted to the lower limb motor system in a patient with stiff-leg syndrome., Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia, 10.1016/j.jocn.2011.03.021, 18, 12, 1720-2, 2011.12, We report a 29-year-old man who presented with a 2-year history of progressive stiffness and painful spasms limited to the bilateral lower limbs, exaggerated by auditory and tactile stimuli. His deep tendon reflexes were slightly increased in both lower extremities. His plantar response was flexor. His serum and cerebrospinal fluid were negative for anti-glutamic acid decarboxylase antibodies. Electromyography of antagonist muscle pairs in his distal lower limbs revealed a failure of reciprocal inhibition. We used transcranial magnetic stimulation with a paired-pulse paradigm, delivered to the cortical area of the upper and lower limbs, and revealed significantly enhanced facilitation only in the area of his lower limbs, but not that representing his upper limbs. His symptoms were improved substantially by 20mg/day of oral diazepam. To our knowledge this is the first report of a patient with hyperexcitability limited to the lower limb motor system in a patient with stiff-leg syndrome..|
|41.||[An adult neurologist's update on epilepsy therapy].
Adult neurologists routinely encounter cases of epilepsy. Appropriate therapy based on a correct diagnosis is very important, and is aided by knowledge of seizure semiology and the correct reading of EEG findings. Many factors need to be considered when deciding upon a treatment regime for adult epilepsy patients, such as employment, marriage, child bearing status, and co-existent disease in elderly patients. Four new antiepileptic drugs (AEDs), which have been used in other countries for more than 10 years, have been authorized for use over the past few years in Japan. Because new AEDs also have interactions and side effects, administration to patients must be carried out based on an understanding of drug actions and interaction mechanisms. Surgical treatment should be considered for drug resistant patients, especially for those suffering from temporal lobe epilepsy with hippocampal sclerosis. For drug resistant patients who are not candidates for resection therapy, we can undertake vagus nerve stimulation therapy, which has recently been authorized for use in Japan. Other electrical stimulation therapies, targeting the anterior nucleus of thalamus, hippocampus and epileptic neo-cortex, have been investigated and are now under study in the USA. Neurologists should be aware of such newly introduced therapies in giving a better quality of life for epilepsy patients..
|42.||Kei-ichiro Takase, Hiroshi Shigeto, Kohnosuke Furuta, Nobutaka Sakae, Yasumasa Ohyagi, Jun-ichi Kira, Transient vocal cord palsy caused by hypoperfusion of unilateral hemisphere., Fukuoka igaku zasshi = Hukuoka acta medica, 102, 9, 273-6, 2011.09, We report a 68-year-old man who exhibited mild dysarthria and mild right hemiparesis resulted from hypoperfusion of the left hemisphere. An MR angiography showed a severe stenosis at the second portion of left middle cerebral artery (MCA). After the beginning of treatment, the patient suffered from hoarseness, followed by breathing failure. The laryngeal fiber exhibited right vocal cord paresis. Unilateral cortico-bulbar tract dysfunction does not typically cause vocal cord palsy. However, several cases indicate the involvement of a dominant projection from the contralateral cortico-bulbar tract to the vocal cord. In the present case, hypoperfusion of the left hemisphere might have temporarily produced right vocal cord palsy, considering the stenosis of the left MCA..|
|43.||[Epilepsy practice for neurologists].
Epilepsy is a common disease with a high incidence of about one percent. Knowledge of seizure semiology and correct reading of EEG findings are important for diagnosis of epilepsy. Because the primary therapy for epilepsy is antiepileptic drugs (AEDs), including several ones that are newly permitted in Japan, we need to prescribe them based on an understanding of their actions and interaction mechanisms. However, we also need to consider early surgical treatment for temporal lobe epilepsy with hippocampal sclerosis. In the therapeutic decision for adult epilepsy patients many factors such as employment, marriage, child bearing, and co-existent disease need to be considered. The present review provides an overview of the basis of epilepsy practice for neurologists treating adults with epilepsy, including a discussion of new AEDs, epilepsy surgery, women with epilepsy, and epilepsy in the elderly..
|44.||[A case of Charles Bonnet syndrome following syphilitic optic neuritis].
Charles Bonnet syndrome refers to visual hallucinations in patients with visual acuity loss or visual field loss without dementia. We report a case of Charles Bonnet syndrome following syphilitic optic neuritis. A 62-year-old man was admitted to our hospital suffering acute bilateral visual loss in a few months. On admission, he was almost blind and his optic discs were found to be atrophic on fundoscopy. In addition to increased cell counts and protein concentration in cerebrospinal fluid (CSF), serum and CSF rapid plasma reagin tests were positive. A diagnosis of syphilitic optic neuritis was made and he was treated with intravenous penicillin G (24 million units per day for 14 days) without any recovery. After treatment finished, he began to experience complex, vivid, elaborate and colored visual hallucinations. He recognized these visions as unreal and felt distressed by them. No cognitive impairment was observed on several neuropsychological tests. We diagnosed the patient as suffering from Charles Bonnet syndrome. Brain MRI revealed diffuse mild atrophy of the cerebral cortex and multiple T2 high signal intensity lesions in the deep cerebral white matter. Single photon emission computed tomography revealed decreased regional cerebral blood flow in bilateral medial occipital lobes. Administration of olanzapine resulted in a partial remission of visual hallucinations. Charles Bonnet syndrome following syphilitic optic neuritis is rare. In the present case, visual loss and dysfunction of bilateral medial occipital lobes may have triggered the visual hallucinations, which were alleviated by olanzapine..
|45.||[Primary care of epilepsy and recent topics]..|
|46.||A case of Borrelia brainstem encephalitis presenting with severe dysphagia
We report the case of a 30-year-old man who developed severe dysphagia owing to neuroborreliosis. He showed dysphagia, diplopia, hiccups, and walking difficulty Neurological examination revealed mild disturbance of consciousness, diplopia on left lateral gaze, left-side-dominant blephaloptosis, gaze-evoked horizontal nystagmus on left lateral gaze, mild bilateral muscle weakness, palatoplegia, dysphagia, dysarthria, and truncal ataxia. An increased pharyngeal reflex caused dysphagia in this patient. An EEG revealed intermittent high amplitude slow wave activity. However, head MRI, blood count, serum chemistry, and cerebrospinal fluid examination showed no abnormality. Initially, brainstem encephalitis with unknown etiology was diagnosed. The hiccups, diplopia, and ptosis were improved by corticosteroid therapy, but other symptoms were refractory to corticosteroid therapy and IVIg. After these immunotherapies, anti-Borrelia IgG and IgM antibodies were found to be positive, and symptoms, including dysphagia, were improved by doxycycline and cefotaxime. Because the clinical symptoms of Borrelia infection are widely variable, neuroborreliosis should be considered in patients with brainstem encephalitis refractory to conventional immunotherapies.
|47.||[A case of chronic inflammatory demyelinating polyradiculoneuropathy concomitant with acquired von Willebrand syndrome].
We report a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) concomitant with acquired von Willebrand syndrome. A 33-year-old man developed motor and sensory polyneuropathy with electrophysiological conduction slowing. At this time, M-protein was absent He was diagnosed with CIDP and received intravenous immunoglobulin and subsequent oral corticosteroids, which resulted in almost complete remission for over 10 years. At the age of 44, he presented with chronic anemia. Laboratory tests and colonoscopy revealed that he had acquired von Willebrand syndrome with monoclonal gammopathy of undetermined significance (IgG lambda type) and colon cancer. Bleeding symptoms were.resolved with intravenous immunoglobulin, but not with supplementation of factor VIII. Shortly after successful excision of the cancer, CIDP and acquired von Willebrand syndrome simultaneously recurred. Intravenous immunoglobulin produced rapid improvement of both neurological and hematological abnormalities. Concurring CIDP and acquired von Willebrand syndrome in the present case may indicate that the conditions have a partly common immunological background including monoclonal gammopathy and a potential common autoantibody-mediated mechanism. Alternatively, dysfunction of von Willebrand factor may increase blood-nerve barrier permeability, inducing the recurrence of CIDP..
|48.||A late-onset case of nonconvulsive status epilepticus of generalized epilepsy
We report a 78-year-old woman who had episodes of nonconvulsive status epilepticus (NCSE) with dizziness. At 75 years of age, she had first seizure, but was not well examined. At 78 years of age, she had brief myoclonic jerks of her arms, soon after awakening, in May. She suffered from strong dizziness and was admitted in our hospital at the end of June. The symptoms regressed with bed rest in few days and she was discharged. However, she was admitted again with dizziness in the middle of July. There were no myoclonic jerks of her arms or legs and she could converse and interact normally, but was slightly disoriented (JCS: 2). Blood test, Cerebrospinal fluid analyses and brain MRI were normal. An EEG showed frequent intermittent generalized multiple spikes and slow wave complexes and a 3-4Hz generalized spike and slow wave complexes every 2-4 seconds during whole 20 minutes record. Intravenous injection of 5mg diazepam terminated status immediately. Thereafter, she was treated with sodium valproate (400mg/day). Her symptoms improved, and interictal epileptic discharges extremely decreased. Late-onset NCSE of generalized epilepsy is rare. We discussed this case as an important case for diagnosis of NCSE with subtle symptom of dizziness.
|49.||Madoka Noriuchi, Yoshiaki Kikuchi, Takashi Yoshiura, Ryutaro Kira, Hiroshi Shigeto, Toshiro Hara, Shozo Tobimatsu, Yoko Kamio, Altered white matter fractional anisotropy and social impairment in children with autism spectrum disorder, BRAIN RESEARCH, 10.1016/j.brainres.2010.09.051, 1362, 141-149, 2010.11, Individuals with autism spectrum disorder (ASD) have severe difficulties in social interaction and communication, as well as restricted and/or stereotyped patterns of behavior. Previous studies have suggested that abnormal neural connectivity might be associated with higher information processing dysfunction involving social impairment. However, the white matter structure in ASD is poorly understood. To explore this, we conducted a voxel-based, whole-brain diffusion tensor imaging (DTI) analysis to determine fractional anisotropy (FA), lambda(1), lambda(2) and lambda(3) in high-functioning children with ASD compared with age-, gender-, and handedness-matched healthy control participants. We then investigated whether DTI parameters were associated with behaviorally measured social function. We found that FA and lambda(1) were significantly lower in the ASD group than in the control group in the white matter around left dorsolateral prefrontal cortex (DLPFC), posterior superior temporal sulcus/temporo-parietal junction, right temporal pole, amygdala, superior longitudinal fasciculus, occipitofrontal fasciculus, mid- and left anterior corpus callosum, and mid- and right anterior cingulate cortex. The FA value in the left DLPFC was negatively correlated with the degree of social impairment in children with ASD. Higher values were observed in the cerebellar vermis lobules in the ASD group. The white matter alterations in children with ASD were around cortical regions that play important roles in social cognition and information integration. These DTI results and their relationship to social impairment add to evidence of cerebral and cerebellar white matter structural abnormalities in ASD. (C) 2010 Elsevier B.V. All rights reserved..|
|50.||A case of acute disseminated encephalomyelitis associated with Epstein-Barr virus reactivation during infliximab therapy
A 31-year-old woman with Crohn's disease that had been refractory to drug therapies for 7 years had been treated with infliximab for a year. She was admitted to our hospital because of truncal ataxia and bulbar palsy, which presented following aseptic meningitis. Neurological examination revealed abducens paresis on the left, gaze-evoked nystagmus on upward and rightward gaze, right facial muscle weakness, bulbar palsy, weakness in the right upper extremity, limb ataxia predominantly on the left side, diminished sense in the lower extremities predominantly on the right, diffuse hyperreflexia in all extremities. Antibodies to Epstein-Barr virus (EBV) in serum demonstrated a previous infection pattern, and EBV-DNA was detected in peripheral blood and cerebrospinal fluid (CSF) by PCR. CSF analysis indicated pleocytosis, an elevation of IgG index and a marked increase in the level of myelin basic protein. FLAIR MRI images revealed multiple hyperintense lesions in the brainstem, subcortical white matter, and cervical spinal cord. Accordingly, we diagnosed her as having acute disseminated encephalomyelitis (ADEM) , associated with reactivated EBV infection. Although gancyclovir, plasma exchange and intravenous high dose immunoglobulins were not effective, repetitive use of methylprednisolone pulse therapy alleviated her symptoms and the abnormal MRI lesions. It is suggested that the reactivated EBV infection caused by infliximab may have contributed to the development of ADEM in this case. Besides the demyelinating event directly induced by anti-TNF-α therapy, we should pay attention to the occurrence of reactivated EBV-triggered ADEM during anti-TNF-α therapy.
|51.||A case of anti-Hu antibody- and anti-GluR.EPSILON.2 antibody-positive paraneoplastic neurological syndrome presenting with limbic encephalitis and peripheral neuropathy
We report a case of paraneoplastic neurological syndrome with anti-neuronal antibodies, namely anti-Hu and anti-GluRε2 antibodies in sera. A 72-year-old male had a transient history of eye movement disorder and sensory neuropathy, which improved spontaneously. Two years later, he was admitted to another hospital because of gait disturbance, numbness of the hands and an attack of unconsciousness with generalized convulsion. He was admitted to our hospital with prolonged consciousness disturbance and muscular weakness of all extremities. On admission his consciousness deteriorated slightly without neck stiffness. His cranial nervous system was normal except for incomplete abduction and elevation of both eyes. The patient had severe distal dominant weakness and atrophy in the muscles of all four limbs. Muscle tonus was decreased and hyporeflexia was noted in the four extremities. Plantar response was extensor. Neither sensory disturbance nor ataxia was observed. Cranial MRI showed T2-weighted high intensity lesions in the bilateral mesial temporal lobes, including the hippocampi. A nerve conduction study revealed motor-dominant peripheral neuropathy with prolonged latency; the amplitudes of compound muscle action potentials were severely reduced in all four limbs and those of sensory nerve action potentials were moderately reduced in the right upper and lower extremities. We also found a left hilar lymphadenopathy showing accumulation of FDG on PET, suggesting a possibility of malignancy. Anti-Hu and anti-GluRε2 antibodies were detected in sera but not in CSF. We diagnosed him with limbic encephalitis and peripheral neuropathy due to paraneoplastic neurological syndrome and treated him with two courses of intravenous immunoglobulin (IVIg) (400mg/kg, 5 days) . The consciousness disturbance, and prolonged distal latency revealed by motor nerve conduction studies improved slightly. Although the roles of anti-neuronal antibodies in paraneoplastic conditions remain unknown, we consider that IVIg may be worth using to treat cases with anti-Hu and anti-GluRε2 antibodies.
|52.||[A case of mononeuropathy multiplex associated with idiopathic thrombocytopenic purpura].
We report a case of mononeuropathy multiplex with idiopathic thrombocytopenic purpura (ITP). A 78-year-old man developed patches of purpura on his left forearm. His platelet count was 11,000/microl and platelet-associated IgG was elevated. He was diagnosed as having ITP. At the beginning of the following month, he noticed dysesthesia and weakness of his left finger and left lower limb, as well as dysesthesia of his bilateral lower thighs. Neurological examination revealed weakness in the area of the left ulnar nerve and of the left anterior tibial muscle. Dysesthesia presented in the area of the left ulnar nerve and bilateral superficial peroneal nerves. Nerve conduction studies revealed asymmetric axonal sensorimotor neuropathy (mononeuropathy multiplex). A cerebrospinal fluid specimen showed a normal cell count and normal protein level Serum anti-ganglioside antibody was negative. The platelet count gradually increased after the introduction of corticosteroid therapy. His neurological deficits and electrophysiological findings also improved. Immune-mediated neuropathy was suggested as the cause of his mononeuropathy multiplex with ITP..
|54.||Koichi Hagiwara, Tsuyoshi Okamoto, Hiroshi Shigeto, Katsuya Ogata, Yuko Somehara, Takuya Matsushita, Jun-ichi Kira, Shozo Tobimatsu, Oscillatory gamma synchronization binds the primary and secondary somatosensory areas in humans, NEUROIMAGE, 10.1016/j.neuroimage.2010.02.001, 51, 1, 412-420, 2010.05, Induced gamma activity has a key role in the temporal binding of distributed cortico-cortical processing. To elucidate the neural synchronization in the early-stage somatosensory processing, we studied the functional connectivity between the primary and secondary somatosensory cortices (SI and 511) in healthy subjects using magnetoencephalography (MEG) with excellent spatiotemporal resolution. First, somatosensory-evoked magnetic fields were recorded to determine the locations of each cortical activity. Then we analyzed the phase-locking values (PLVs) of the induced gamma activity to assess neural synchrony within the somatosensory cortical network. We also assessed PLVs in patients with multiple sclerosis (MS) to validate our PLV analysis in evaluating the inter-areal functional connectivity, which can often be impaired in MS. The PLVs of the induced gamma activity were calculated for each pair of unaveraged MEG signals that represented the activities of the contralateral SI and bilateral SII areas. Analysis of PLVs between the SI and SII areas showed significantly increased PLVs for gamma-band activities, starting at an early post-stimulus stage in normal controls, whereas this increase in PLVs was apparently diminished in MS. The PLV analysis provided evidence for early-latency, gamma-band neuronal synchronization between the SI and SII areas in normal controls. Our study first demonstrates the gamma-band synchrony in the early-stage human somatosensory processing. (C) 2010 Elsevier Inc. All rights reserved..|
|55.||高瀬 敬一郎, 重藤 寛史, 鎌田 崇嗣, 大八木 保政, 吉良 潤一, 皮質てんかんラットモデルでの視床背内側核刺激の効果(Efficacy of Mediodorsal Thalamic Nucleus Stimulation in a Rat Model of Cortical Seizure), 福岡医学雑誌, 100, 8, 274-280, 2009.08, 背景 視床背内側核は前頭葉と辺縁系とに強く結合している。この研究では、前頭葉への電気刺激で励起されるてんかん発作に対し、視床背内側核電気刺激が影響を与えるか否かを検討した。対象と方法 生後28日目の成熟雄ラット(n=7)の脳表(両側前頭葉)にネジ電極を、両側視床背内側核に深部電極を埋め込み、10日間休ませた後38日目から1日1回前頭葉の電極に電気刺激を行い、全身けいれん発作を出現させた。刺激は明らかな後放電(前頭葉の脳表からのてんかん性放電)が出現するまで、徐々に上昇させ、後放電が出現した時点で刺激を終了した。また、一定の刺激電圧(0.1mA)で視床背内側核電気刺激を0Hz、1Hz、5Hz、10Hzの周波数で各ラットに前頭葉刺激と同時に1日おきに行った。それぞれの群で後放電の閾値と持続時間を計測し比較した。結果 けいれん発作は両側前頭葉の棘波とそれに伴う全身強直間代性けいれんであった。後放電の閾値には4群間で有意な差を認めなかった。持続時間に関しては10Hz刺激でコントロール群より軽度短かったものの、全体として有意差を認めなかった。結論 視床背内側核の電気刺激は、今回のような低頻度刺激ではてんかん発作を抑制することは難しいと思われる。今後視床刺激の部位や頻度を変更する必要がある。(著者抄録).|
|56.||Akihiro Watanabe, Takuya Matsushita, Hikaru Doi, Takashi Matsuoka, Hiroshi Shigeto, Noriko Isobe, Yuji Kawano, Shozo Tobimatsu, Jun-ichi Kira, Multi modality-evoked potential study of anti-aquaporin-4 antibody-positive and -negative multiple sclerosis patients, JOURNAL OF THE NEUROLOGICAL SCIENCES, 10.1016/j.jns.2009.02.371, 281, 1-2, 34-40, 2009 Jun 15;281(1-2):34-40. Epub 2009 Apr 1.
, 2009.06, Neuromyelitis optica (NMO) is claimed to be a distinct disease entity from multiple sclerosis (MS) because of its strong association with NMO-IgG/anti-AQP4 antibody; however, the in vivo role of the antibody remains unknown. Therefore, we aimed to clarify whether the presence of anti-AQP4 antibody is associated with any abnormalities in multimodality-evoked potentials in 111 patients with relapsing-remitting or relapsing-progressive MS, including the opticospinal form of MS, 18 of whom were seropositive for anti-AQP4 antibody. More patients with anti-AQP4 antibody showed a lack of the P100 component on visual-evoked potentials (VEPs) than those without the antibody (11/17, 64.7% vs. 20/84, 23.8%, p = 0.003), whereas the frequency of delayed P100 latency was significantly higher in the latter group than in the former (1/17, 5.9% vs. 28/84, 33.3%, p = 0.021). The frequencies of non-responses and delayed central sensory conduction times in median and posterior tibial nerve somatosensory-evoked potentials (SEPs) were not significantly different between anti-AQP4 antibody-positive and -negative patients. In terms of upper and lower limb motor-evoked potentials (MEPs), the frequencies of non-responses and delayed central motor conduction times did not differ significantly based on the presence or absence of anti-AQP4 antibody. The frequency of optic nerve lesions on MRI was significantly higher in anti-AQP4 antibody-positive patients than in anti-AQP4 antibody-negative patients (p=0.0137). Multiple logistic analyses revealed that anti-AQP4 antibody positivity (OR=8.406, p=0.02) and unevoked VEP responses (OR=35.432, p<0.001) were significantly related to development of severe visual impairment. Such an association of anti-AQP4 antibody with disability was not found for either severe motor or sensory impairment. These findings suggest a distinctive nature of optic nerve lesions between anti-AQP4 antibody-positive and -negative patients; lesions are supposed to be more necrotic in the former group and more demyelinating in the latter. (C) 2009 Elsevier B.V. All rights reserved..
|57.||Katsuya Ogata, Tsuyoshi Okamoto, Takao Yamasaki, Hiroshi Shigeto, Shozo Tobimatsu, Pre-movement gating of somatosensory-evoked potentials by self-initiated movements: The effects of ageing and its implication, CLINICAL NEUROPHYSIOLOGY, 10.1016/j.clinph.2009.01.020, 120, 6, 1143-1148, 2009 Jun;120(6):1143-8. Epub 2009 May 10., 2009.06, Objective: To study whether the gating effect of the self-initiated movements on the cortical somatosensory-evoked potentials (SEPs) is affected by ageing.
Methods: The SEPs elicited by stimulating the right median nerve were recorded in 14 young and 16 older healthy Subjects, while self-initiated movements of the right fingers were performed at 5-10 s intervals. The amplitudes of the major components of the SEPs at F3 and C3' (2 cm posterior to 0) during the pre-movement period were analysed as the resting condition subserving the baseline.
Results: The amplitudes at rest were significantly greater in the elderly than in the Younger subjects. The amplitudes of P27, N35 and P45 at C3' as well as N30 at F3 decreased significantly during the pre-movement period. However, the ratio of amplitudes in the pre-movement period to the resting period in the elderly was not significantly different from that in the younger subjects, except for the interaction of N30.
Conclusions: The effect of age on the gating of N30 at F3 may indicate an altered preparatory processing of self-initiated movement in the elderly. The gating effect of older subjects at C3' is almost comparable to that of young ones, which appears to be a compensatory mechanism to maintain the precise movements.
Significance: Ageing affects the SEPs differently at rest and pre-movement gating. (C) 2009 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved..
|58.||Hagiwara K, Ochi H, Suzuki S, Shimizu Y, Tokuda T, Murai H, Shigeto H, Ohyagi Y, Iwata M, Iwaki T, Kira JI., Highly selective leptomeningeal amyloidosis with transthyretin variant Ala25Thr., Neurology. 2009, Apr 14;72(15):1358-60, 2009.04.|
|59.||話題の医療 脳波デジタルファイリングシステム ビデオ脳波モニタリングシステムの構築 てんかん診療において.|
|61.||Kei-ichiro Takase, Hiroshi Shigeto, Satoshi O. Suzuki, Hitoshi Kikuchi, Yasumasa Ohyagi, Jun-ichi Kira, Cortical kindling in a focal freeze lesion rat model, JOURNAL OF CLINICAL NEUROSCIENCE, 10.1016/j.jocn.2008.04.007, 16, 1, 94-98, 2009 Jan;16(1):94-8. Epub 2008 Nov 18.
, 2009.01, Focal cortical dysplasia (FCD) is of increasing interest as a cause of focal epilepsy. We aimed to determine whether the existence of FCD influences the epileptogenicity induced by electrical kindling stimulation of the cortices. We created an FCD rat model by focal contact of a frozen metal probe on the scalp immediately after birth. To produce afterdischarges (ADs), electrical stimulation was applied to the frontal cortices once daily for 20 consecutive days from postnatal day 38 (P38). Thresholds and durations of ADs were measured. Brains were exposed and examined histologically at P58. We observed mild FCD, which consisted of disorganized cortices with extra sulci: however, there was no statistical difference in the thresholds or durations of ADs between FCD rats and control animals. These results suggest that FCD might not influence vulnerability to epileptogenicity, at least in some patients with fluid FCD. (c) 2008 Elsevier Ltd. All rights reserved..
|62.||Toshihiro Hokonohara, Hiroshi Shigeto, Yuji Kawano, Yasumasa Ohyagi, Michiya Uehara, Jun-ichi Kira, Facial onset sensory and motor neuronopathy (FOSMN) syndrome responding to immunotherapies, JOURNAL OF THE NEUROLOGICAL SCIENCES, 10.1016/j.jns.2008.07.021, 275, 1-2, 157-158, 2008 Dec 15;275(1-2):157-8. , 2008.12, We report the first non-Caucasian case of facial onset sensory and motor neuronopathy (FOSMN) syndrome partially responding to various immunotherapies. A 55-year-old man had first felt paresthesia on his right cheek at age 45. This gradually extended to the scalp. Paresthesia of bilateral fingers and dysphagia appeared 6 years later. On admission, facial sensory impairment and bulbar palsy were found. There were no sensory or motor deficits evident in any limb, except for decreased deep tendon reflex and vibratory sensation. Videofluorography (VF) revealed decreased pharyngeal clearance. The sensory nerve action potential (SNAP) amplitudes of median and ulnar nerves were decreased. Intravenous immunoglobulin therapy and plasma exchange ameliorated his dysesthesia and dysphagia after several weeks, and resulted in improvements in VF and SNAP abnormalities. These observations suggest that FOSMN syndrome maybe, in part, immune-mediated. (C) 2008 Elsevier B.V. All rights reserved..|
|63.||Kei-ichiro Takase, Hiroshi Shigeto, Satoshi O. Suzuki, Hitoshi Kikuchi, Yasumasa Ohyagi, Jun-ichi Kira, Prenatal freeze lesioning produces epileptogenic focal cortical dysplasia, EPILEPSIA, 10.1111/j.1528-1167.2008.01558.x, 49, 6, 997-1010, Epilepsia. 2008 Mar 4
, 2008.06, Purpose: Focal cortical dysplasia (FCD) is thought to be an important cause of intractable epilepsy. However, its epileptogenicity remains unclear. Therefore, we created a novel rat model by freeze lesioning during the late embryonic stage to verify whether FCD influences seizure activities.
Methods: At 18 days postconception, a frozen probe was placed on the left scalp of a Sprague-Dawley rat embryo through the uterus wall. For 40 consecutive days from postnatal day 38 (P38), electrical kindling stimulation was applied to the frontal lobes of male rat pups. Afterdischarges (ADs) were measured in both the cortex and hippocampus. Brain tissues were examined by immunohistochemistry.
Results: All brains from prenatally freeze-lesioned rats displayed severe disorganization of the cortical layers with randomly oriented dendrites/axons. In addition, heterotopic cortices were observed in 42.1% of cases. ADs in the cortex and hippocampus were significantly prolonged in freeze-lesioned rats compared with those in sham-operated and control rats. FCD rats also revealed early development of hippocampal kindling and spontaneous cortico-hippocampal spikes, even in the chronic EEG recordings. Immunoreactivities for N-methyl-D-aspartate receptor (NMDAR) subunit 2B and glutamate/aspartate transporter in the lesions were significantly enhanced compared with the nonlesioned side, even in the absence of electrical stimulation. After electrical stimulation, NMDAR1 and 2B were markedly upregulated not only in the FCD, but also in the hippocampus.
Conclusions: Prenatal freeze lesioning of the brain produces a severe neuronal migration disorder, closely mimicking human FCD. Our model suggests that FCD is associated with vulnerability to epilepsy, and may augment hippocampal epileptogenicity..
|64.||A case of eosinophilic myositis in continuum from localized nodular myositis
We report a 72-year-old man with eosinophilic myositis (EM). At age 71 he noticed a painful nodule in his left calf. A biopsy (first biopsy) showed marked infiltration of mononucleated cells and necrotic muscle fibers. Several phagocytosed fibers were also seen. He was diagnosed as having myositis. The painful nodule disappeared spontaneously. At age 72, he again had a painful nodule, but this time in his right calf; again, this disappeared spontaneously on the first admission. Just after discharge, he noted painful nodules in the left thigh and right anterior tibial muscles and was again admitted (second admission). Neurological examination revealed mild proximal-dominant weakness in all four extremities but no other abnormalities. Laboratory studies showed elevated creatine kinase (CK) level (38,803U/l; normal 62-287) and positive Jo-1 antibody, but no eosinophilia. Needle electromyography of the limb muscles showed myogenic patterns. Magnetic resonance imaging of the lower limbs demonstrated several T2-high and gadolinium (Gd)-enhanced lesions. Muscle biopsy (second biopsy) from the left quadriceps femoris showed marked infiltration of eosinophils; he was diagnosed as having EM. Administration of prednisolone was initiated at 60 mg/day and then gradually tapered. After starting treatment with steroids, his muscle weakness gradually ameliorated, CK level dramatically decreased, and the nodules disappeared. Clinically, the patient had developed localized nodular myositis (LNM), but pathologically it was EM without peripheral blood eosinophilia and positive Jo-1 antibody that is occasionally found in polymyositis (PM). Thus, this patient demonstrated overlapping characteristics of EM, LNM, and possibly PM, suggesting that a common mechanism underlay these conditions. As discussed, the involvement of eosinophils in three inflammatory myopathies was indicated.
|65.||Hagiwara K, Murai H, Ochi H, Osoegawa M, Shigeto H, Ohyagi Y, Kira J., Upper motor neuron syndrome associated with subclinical Sjögren's syndrome., Intern Med., 2008;47(11):1047-51. Epub 2008 Jun 2., 2008.06.|
|66.||[Acute myelitis in a patient with ulcerative colitis]..|
|67.||Age-related alterations of the functional interactions within the basal ganglia and cerebellar motor loops in vivo.
Taniwaki T, Okayama A, Yoshiura T, Togao O, Nakamura Y, Yamasaki T, Ogata K, Shigeto H, Ohyagi Y, Kira J, Tobimatsu S, NeuroImage, 2007, vol. 36, no. 4, pp. 1263-1276, 2007.
|68.||Arnold Angelo M. Pineda, Katsuya Ogata, Manabu Osoegawa, Hiroyuki Murai, Hiroshi Shigeto, Takashi Yoshiura, Shozo Tobimatsu, Jun-ichi Kira, A distinct subgroup of chronic inflammatory demyelinating polyneuropathy with CNS demyelination and a favorable response to immunotherapy, JOURNAL OF THE NEUROLOGICAL SCIENCES, 10.1016/j.jns.2007.01.004, 255, 1-2, 1-6, 15;255(1-2):1-6.
, 2007.04, To explore subclinical central nervous system (CNS) involvement in chronic inflammatory demyelinating polyneuropathy (CIDP), we recorded somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) using transcranial magnetic stimulation, to measure central sensory conduction time (CSCT) and central motor conduction time (CMCT) and examined brain and spinal cord MRI in patients with probable CIDP based on the American Academy of Neurology AIDS Task Force criteria. Eighteen patients with probable CIDP (12 males and 6 females; mean age at examination +/- SD, 45.8 +/- 17.0 years; range, 17-72) were included in the study. Of the 13 patients who underwent SEPs, one had prolonged CSCT (8%) and of the 13 who underwent MEPs, four had abnormal CMCT (31%). Cranial MRI revealed five of 18 patients had abnormal scans, only one of which showed multiple ovoid periventricular lesions suggestive of demyelination while none showed any intramedullary lesion on spinal cord MRI. Thus, 6 of the 18 patients were considered to have subclinical demyelinative CNS involvement which had lower disability on Global Neurological Disability Score (GNDS) (p=0.0061), a male preponderance (0.0537) and a larger compound muscle action potential (CMAP) amplitude in the median nerve (p=0.005) than those without. The decrease of GNDS with immunologic therapies was nearly significant in the former (p=0.0556) but not in the latter. The results of the present study suggest that subclinical CNS involvement in CIDP is not uncommon in Japanese patients and that CIDP with subclinical CNS involvement is more demyelinative thus responsive to immunotherapies while those without have more axonal damage and less responsive to immunotherapies. (c) 2007 Elsevier B.V. All rights reserved..
|69.||Kellinghaus C, Moddel G, Shigeto H, Ying Z, Jacobsson B, Gonzalez-Martinez J, Burrier C, Janigro D, Najm IM., Dissociation between in vitro and in vivo epileptogenicity in a rat model of cortical dysplasia., Epileptic Disord, 9(1):11-9.
|70.||Takayuki Taniwaki, Akira Okayama, Takashi Yoshiura, Osamu Togao, Yasuhiko Nakamura, Takao Yamasaki, Katsuya Ogata, Hiroshi Shigeto, Yasumasa Ohyagi, Jun-ichi Kira, Shozo Tobimatsu, Functional network of the basal ganglia and cerebellar motor loops in vivo: Different activation patterns between self-initiated and externally triggered movements, NEUROIMAGE, 10.1016/j.neuroimage.2005.12.032, 31, 2, 745-753, 31:745-753, 2006.06, Taniwaki T, Okayama A, Yoshiura T, Togao O, Nakamura Y, Yamasaki T, Ogata K, Shigeto H, Ohyagi Y, Kira J, Tobimatsu S, NeuroImage, 2006, vol. 31, no. 2, pp. 745-753, 2006.|
|71.||[A case of adult-onset Sydenham chorea accompanied with psychiatric symptoms].
We report a 56-year-old man with adult-onset Sydenham chorea. Since January 2003, he had often troubled other persons, and in October 2003, following an episode of fever in August of the same year, he noticed left shoulder joint pain and involuntary movements of his limbs, especially on the left side. These involuntary movements gradually worsened and he became unable to converse due to psychiatric symptoms. On admission, neurological examination revealed dementia, emotional incontinence, abnormal behavior and chorea in four limbs. Brain MRI disclosed swelling of bilateral caudate heads that was more marked on the right side. Hypermetabolism in bilateral caudate nuclei, especially on the right, was found on FDG-PET study, which was compatible with his left side-dominant chorea and might reflect inflammation as a nature. A gallium scintigram demonstrated excess accumulations in the plural joints of his extremities, which gradually decreased in parallel with joint pain relief. The present case was diagnosed as Sydenham chorea, because of the presence of arthritis, chorea, fever, increased erythrocyte sedimentation rate and elevated CRP. We believe that this is a first report of adult-onset Sydenham chorea accompanied with psychiatric symptoms..
|72.||[Relapsing Wernicke's encephalopathy after gastrectomy]..|
|73.||Hiroshi Shigeto, Taira Uehara, Kazuki Uchida, Takuo Nomura, Takayuki Taniwaki, Jun-ichi Kira, Thalamic involvement of status epilepticus: diffusion-weighted image of MRI in two cases of status epilepticus, International congress series, 1278, 193-196, 2005.01.|
|74.||Yuko Tsuruta, Koji Ikezoe, Hideaki Nakagaki, Hiroshi Shigeto, Masakazu Kawajiri, Yasumasa Ohyagi, Jun-Ichi Kira, A case of dermato-fasciitis: amyopathic dermatomyositis associated with fasciitis., Clinical rheumatology, 23, 2, 160-2, 2004.04, Amyopathic dermatomyositis (ADM) is characterized by the typical cutaneous features of dermatomyositis and minor involvement of the skeletal muscles. A 50-year-old woman had fever, reddening and pain in the distal part of all four limbs, and cutaneous findings such as Gottron's papules and periorbital heliotrope. She showed no muscle weakness or atrophy, and her serum creatine kinase was within the normal range. Electromyography showed no myopathic pattern. Magnetic resonance imaging (MRI) recorded abnormal hyperintensity in the fascia and muscle of the tibialis anterior. A biopsy from the tibialis anterior muscle showed fasciitis and mild myopathic changes with focal perivascular infiltration. This patient also presented with interstitial pneumonitis, although evaluation for malignancy was negative. With steroid therapy, her symptoms and MRI abnormality disappeared within 2 months. This case is therefore considered to be a variant of ADM, presenting as dermato-fasciitis..|
|75.||[A case of severe parkinsonism induced by failure of ventriculo-peritoneal shunt for aqueductal stenosis].
A 26-year-old man, who had received a ventriculo-peritoneal shunt for obstructive hydrocephalus after possible encephalitis, complained of disturbance of upward gaze and difficulty in movement seven months after the shunt implantation. One month later, neurological examination revealed upward gaze paresis and rigidity of all four limbs, but the neuroimaging studies revealed no ventricular dilatation. His symptoms deteriorated, and tremor of the extremities appeared. He was admitted to our hospital 10 months after the shunt implantation. He developed akinetic mutism soon after admission. Cerebrospinal fluid protein was elevated (62 mg/dl). At that time, the shunt reservoir was found to be insufficiently filled, and neuroimaging showed dilatation of the lateral and third ventricles with no dilatation of the fourth ventricle. A neuroendoscopic third ventriculostomy with removal of the previous shunt system gradually resolved the parkinsonism within two months, and the patient became capable of walking. The dilatation of the ventricles improved on neuroimaging. The present report suggests that shunt malfunction should be suspected when parkinsonism occurs in patients who have undergone a shunt placement, even though hydrocephalus on neuroimaging is not observed..
|76.||[Facioscapulohumeral muscular dystrophy with sinus dysfunction].
We report a 47-year-old man with facioscapulohumeral muscular dystrophy (FSHD) presenting with sinus dysfunction. He became unable to roll over and stand up at the age of 42, but he could still walk. Facial muscle involvement, scapular winging, asymmetrical involvement, funnel chest, and the absence of contractures were typical of FSHD. Electrocardiogram (ECG) and cardiac echogram showed the overload of both right atrium and ventricle. On Holter ECG, transient P wave inversion and P-P interval elongation (maximally 2.4 seconds) repeatedly appeared mainly during sleep. There was no bundle branch block, atrioventricular junctional rhythm, or increase of premature ventricular beats. Vital capacity was decreased (0.62 L, 16% of the predicted value). Arterial blood gas analysis showed hypercapnia and hypoxia which aggravated during sleep (PaCO2 87.3Torr, PaO2 41.5Torr). Sleep apnea was not observed. Intracardiac ECG was not performed and he died 2 weeks later. In FSHD, sinus node dysfunction may become distinct especially in the setting of respiratory failure..
|77.||[Juvenile-onset multiple brain infarcts localized in the posterior circulation: a case report].
We report a 37-year-old male patient with multiple brain infarcts due to arterial lesions localized in the posterior circulation, who developed a paramedian pontine infarct on the left side. He had been treated as schizophrenia for 20 years. A cranial CT performed one year before showed old small infarcts in the territories of the bilateral thalamo-perforating and left thalamo-geniculate arteries and the right posterior inferior cerebellar artery. The vertebral and basilar arteries were small in diameter on MRI and MR angiography(MRA). Cerebral angiography revealed a narrow smooth basilar artery. In addition, the P2 segments of the bilateral posterior cerebral arteries were markedly narrow with irregular walls. Carotid arteriograms were normal and no atherosclerosis was found. The nature of these arterial lesions remains unknown in this case. Even if MRA shows vertebrobasilar artery hypoplasia, a known congenital risk factor of a posterior circulation infarct, we must rule out a possibility that some arterial pathology is going on..