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

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.

DOI： 10.1111/epi.17378

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

Exercise-induced reflex seizures are a rare form of reflex seizures that are exclusively induced by a specific type of exercise. Many patients with exercise-induced reflex seizures exhibit drug-resistance, and are therefore advised to avoid the triggering exercise. Here, we describe a focal epilepsy patient with shadowboxing-induced reflex seizures. His semiology included focal aware seizures with speech and behavioral arrest that evolved to head version to the right, preceded by cephalic aura. We identified a specific motion that induced these seizures during shadowboxing using video-electroencephalographic recording, and the patient was able to continue boxing by avoiding this motion. We speculate that a broad brain network may be the pathological substrate of his exercise-induced reflex seizures. Identification of the specific motion that induces exercise-induced reflex seizures is useful for not only understanding the underlying pathophysiology, but also for minimizing the therapeutic restriction of the exercise.

DOI： 10.1016/j.ebr.2022.100543

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

BACKGROUND: Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a newly identified low-grade brain tumor with frequent epileptic presentation. Despite the facilitated use of invasive electroencephalography owing to the growing availability of stereo-electroencephalography (SEEG), intracranial features of tumor-related seizures are still scarcely described. This report provides the first description of SEEG-recorded seizures in PLNTY to provide an insight into its surgical strategy. OBSERVATIONS: Spontaneous clinical seizures were recorded with SEEG in a young adult patient with drug-resistant epilepsy associated with a PLNTY in the left lateral temporal cortex. The seizure onset was characterized by low-voltage fast activity (LVFA) and showed eccentric localization with respect to the tumor: LVFA was localized in the anterior portion of the tumor and spread toward the adjacent polar cortex. The language risks associated with the resection of the posterior temporal cortex could thus be minimized. LESSONS: PLNTY can show a focal and eccentric seizure-onset zone around the tumor. The present findings serve to improve the functional and seizure outcomes using the staged invasive approach in PLNTY.

DOI： 10.3171/CASE22106

Language：Others  

Basical approach of EEG diagnosis for epilepsy and progress in the field

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

BACKGROUND: Frontal disconnection surgery is a useful surgical option for patients with frontal epilepsy whose seizure onset zones are exceedingly large and thus are not amenable to conventional resective surgery. While it has the advantage of avoiding sequelae stemming from a large resection cavity, the impact of radical anatomofunctional disconnection of such a vast frontal region is not fully understood. OBSERVATIONS: The authors have identified secondary degeneration in the striatum ipsilateral to the frontal disconnection surgery in two adult patients who had otherwise favorable postoperative outcomes following the surgery. On serial postoperative magnetic resonance imaging, the striatum showed transient restricted diffusion in the caudate head and rostral putamen around several weeks postoperatively and subsequent atrophy in the caudate head. The affected striatal regions (i.e., the anterior portion of the striatum) were congruent with the known fronto-striatal connectivity corresponding to the disconnected frontal regions anterior to the primary and supplementary motor areas. Both patients achieved 1-year seizure freedom without apparent disability related to the surgery. LESSONS: The benign postoperative course despite the marked degenerative changes in the ipsilateral striatum supports the feasibility of the frontal disconnection surgery in otherwise inoperable patients with broad frontal epileptogenicity.

DOI： 10.3171/CASE21644

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

片側性周期性放電を頭蓋内外で記録して比較した。

DOI： 10.1177/1550059420972266

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

DOI： 10.1016/j.seizure.2021.06.026

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

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.

DOI： 10.3389/fneur.2021.759866

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

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.

DOI： 10.1016/j.neuroimage.2021.118104

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

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.

DOI： 10.25259/SNI_542_2021

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

心拍から側頭葉てんかん性活動の側在性を推定できる。

DOI： 10.1016/j.yebeh.2019.05.026.

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

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.

DOI： 10.1016/j.yebeh.2019.05.026

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

© 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.

DOI： 10.1016/j.clinph.2018.11.026

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

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.

DOI： 10.1016/j.yebeh.2018.08.036

Language：English  

DOI： 10.1016/j.jns.2017.07.040

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

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.

DOI： 10.1038/srep22991

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

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.

DOI： 10.1016/j.neuroimage.2015.09.006

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

Central pontine myelinolysis (CPM), which was originally considered to be the result of rapid correction of chronic hyponatremia, is not necessarily accompanied by hyponatremia or drastic changes in serum sodium level. Here, we report a case of an anorexic 55-year-old male with a history of pharyngo-laryngo-esophagogastrectomy, initially hospitalized with status epilepticus. Although his consciousness gradually recovered as we were controlling his convulsion, it deteriorated again with new onset of anisocoria, and magnetic resonance imaging (MRI) at this point revealed CPM. Rapid change of serum sodium or osmolarity, which is often associated with CPM, had not been apparent throughout his hospitalization. Instead, a review of the serum biochemistry test results showed that serum phosphate had drastically declined the day before the MRI first detected CPM. In this case, we suspect that hypophosphatemia induced by refeeding syndrome greatly contributed to the occurrence of CPM.

DOI： 10.1159/000440711

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

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.

DOI： 10.1016/j.clinph.2013.10.005

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

[Electroencephalography for patient with epilepsy].
Electroencephalography (EEG) is the primary means by which epileptic activity in the brain is measured. The frequency of epileptic discharges is influenced by vigilance and biological rhythms. When checking for epileptic activity using EEG, measurements must be repeated and recordings made during sleep are recommended if epileptic discharges do not readily appear. Epileptic discharges must be classified as generalized or focal discharges, and discriminated from non-epileptic discharges such as vertex sharp transients, positive occipital sharp transients, 14 & 6 positive spike discharge, and artifacts. Attention should be paid to small sharp spikes, 6Hz spike and slow wave, focal slow waves, and generalized rhythmic slow waves, which should all be considered variants of epileptic activity. EEG provides information regarding focal and generalized brain dysfunction in addition to epileptic activity. Because the misreading of EEG may negatively affect the lives of patients, reading the EEG correctly is quite important.

Language：Japanese  

[Spontaneous seizures in a rat models of multiple prenatal lesioning].
Focal cortical dysplasia (FCD) is an important cause of intractable epilepsy. Previous rat studies have utilized freeze lesioning of neonatal animals to model FCD; however, such models are unable to demonstrate spontaneous seizures without seizure-provoking events. Therefore, we created an animal model with multiple FCD, produced during embryonic development, and observed whether spontaneous seizures occurred. Furthermore, we examined the relationship between FCD and epileptogenesis using immunohistochemistry. At 18 days postconception, a frozen metal probe was placed bilaterally on the scalps of Sprague-Dawley rat embryos through the uterus wall to produce multiple FCD. Eleven of 16 rats showed spontaneous seizures arising in the hippocampus from postnatal day47. Movement cessation followed by sniffing and mastication, culminating in wet-dog shaking, was seen during the hippocampal EEG discharges. Alterations in the levels of glutamatergic and GABA-ergic receptors were investigated during growth. We created an animal model showing spontaneous seizures without a provoking event except for the existence of cortical dysplasia, and without a genetic or general systematic cause like MAM injection or irradiation. The seizures resembled human temporal lobe epilepsy both clinically and on EEG. This model should enable better clarification of the mechanisms underlying the development of human epilepsy.

DOI： 10.5692/clinicalneurol.54.1132

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

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.

DOI： 10.1016/j.jns.2013.09.025

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

The analysis of epileptic discharges in magnetoencephalography with minimum norm estimates (MNE) is expected to provide more precise localization of epileptic discharges compared with electroencephalographic estimations. However, the clinical feasibility of MNE remains unclear. In this study, we aimed to elucidate the onset and propagation patterns of interictal spikes using MNE. Seven patients with intractable epilepsy whose epileptogenicity was assumed to exist in the convexity of the cerebral cortex were studied. For MNE and electrocorticography (ECoG), we characterized the propagation patterns of interictal epileptic discharges according to the area in which they originated and where they extended; we then examined whether the propagation patterns observed in MNE were identified by ECoG. We also examined the relationship between the positions of spikes estimated by the equivalent current dipole (ECD) method and MNE. Among the seven patients, nine propagation patterns of epileptic discharges were observed by MNE, all of which were also identified by ECoG. In seven patterns, the epileptic activity propagated around the initial portion. However, in two patterns, the center of activities moved according to propagation with maintained activity of the initial portion. The locations of spikes identified by the ECD method were within the areas estimated by MNE when the epileptic activity propagated. However, the ECD method failed to detect onset activities identified by MNE in three of nine patterns. Thus, MNE is more useful as a means of presurgical evaluation for epilepsy than the ECD method because it can delineate the onset of epileptic activities as shown in ECoG.

DOI： 10.1016/j.nicl.2013.04.008

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

Focal cortical dysplasia (FCD) is an important cause of intractable epilepsy. Previous rat studies have utilized freeze lesioning of neonatal animals to model FCD; however, such models are unable to demonstrate spontaneous seizures without seizure-provoking events. Therefore, we created an animal model with multiple FCD, produced during embryonic development, and observed whether spontaneous seizures occurred. Furthermore, we examined the relationship between FCD and epileptogenesis using immunohistochemistry. At 18 days postconception, a frozen metal probe was placed bilaterally on the scalps of Sprague-Dawley rat embryos through the uterus wall to produce multiple FCD. Electroencephalogram (EEG) and video recording were performed from postnatal day (P) 35 to P77. Brain tissues were examined immunohistochemically at P28 and P78 using semiquantitative densitometry. Eleven of 16 rats (68.8%) showed spontaneous seizures arising in the hippocampus from P47. Movement cessation followed by sniffing and mastication, culminating in wet-dog shaking, was seen during the hippocampal EEG discharges. FCD was observed in the bilateral frontoparietal lobes. The expression levels of N-methyl-d-aspartate receptor (NMDAR) subunits 1, 2A, 2B, the glutamate/aspartate transporter and the glial glutamate transporter 1 (GLT1) at FCD sites were increased at P28 and P78. There were no major histological abnormalities in the hippocampi compared with those in the cortex. However, the expression levels of NMDAR 2A and 2B were increased at P28. Levels of NMDAR1, 2A and 2B, the glutamate/aspartate transporter and GLT1 were also increased at P78. We created an animal model showing spontaneous seizures without a provoking event except for the existence of cortical dysplasia, and without a genetic or general systematic cause like MAM injection or irradiation. The seizures resembled human temporal lobe epilepsy both clinically and on EEG. Alterations in the levels of glutamatergic and GABAergic receptors were investigated during growth. This model should enable better clarification of the mechanisms underlying the development of human epilepsy.

DOI： 10.1016/j.eplepsyres.2013.03.003

Language：English  

DOI： 10.1038/npp.2013.56

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

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.

DOI： 10.1038/npp.2012.260

Language：English  

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

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.

DOI： 10.1016/j.eplepsyres.2012.10.002

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

OBJECTIVE: To establish the first evidence-based diagnostic criteria for atopic myelitis (AM) enabling it to be discriminated from myelitis-onset multiple sclerosis (MS), which is a difficult differential diagnosis. METHODS: Sixty-nine consecutive AM patients examined from 1996 to 2010 at Kyushu University hospital, who fulfilled the empirical definition of AM (2003), and 51 myelitis-onset MS patients in whom allergen-specific IgE was measured, were enrolled. The first available brain MRI findings were compared between the two. Then, we compared the clinical and laboratory features between the 16 AM cases who did not meet the Barkhof brain MRI criteria for MS after more than 5 years follow-up and 51 myelitis-onset MS cases. Based on the discriminative findings, we established diagnostic criteria for AM and calculated the sensitivity and specificity. RESULTS: AM patients had a significantly lower frequency of Barkhof brain lesions on baseline MRI than myelitis-onset MS patients. AM patients had a significantly higher frequency of present and/or past history of atopic disease and hyperIgEemia, and higher cerebrospinal fluid levels of interleukin 9 and CCL11/eotaxin, but a lower frequency of oligoclonal IgG bands than myelitis-onset MS patients. Our proposed diagnostic criteria for AM demonstrated 93.3% sensitivity and 93.3% specificity for AM against myelitis-onset MS, with 82.4% positive predictive value and 97.7% negative predictive value. CONCLUSION: Our first evidence-based criteria for AM show high sensitivity and specificity, and would be useful clinically.

DOI： 10.1016/j.jns.2012.02.007

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

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.

DOI： 10.1016/j.jocn.2011.03.021

Language：English  

DOI： 10.1016/j.neures.2011.07.1289

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

[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.

DOI： 10.5692/clinicalneurol.51.661

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

[Primary care of epilepsy and recent topics].

DOI： 10.15017/19872

Language：Japanese  

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.

DOI： 10.5692/clinicalneurol.51.43, 10.1002/ncn3.6_references_DOI_Dc0DC8QRi4N2FhXeZJON9hVnnrF, 10.5692/clinicalneurol.51.661_references_DOI_Dc0DC8QRi4N2FhXeZJON9hVnnrF

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

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.

DOI： 10.1016/j.brainres.2010.09.051

Language：English  

fosB-null mice exhibit impaired adult hippocampal neurogenesis and spontaneous epileptic seizures

DOI： 10.1016/j.neures.2010.07.1859

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

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.

DOI： 10.1016/j.neuroimage.2010.02.001

Language：Japanese  

てんかんのビデオ脳波モニタ

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

術前の評価だけではなく、てんかん診断という視点から局在関連てんかん患者85名の脳磁図結果を解析した。68名(80%)でてんかん性異常が検出され、うち7例(8.2%)では同時記録した脳波で捉えられなかった活動を検出できた。磁界から推定した電流源は、症状・神経放射線画像から推定されたてんかん原領域と一致する脳葉に存在した。17例(20%)で脳磁図、脳波とも1回の検査ではてんかん性異常を検出できなかったが、うち15例は複数回あるいは長時間脳波モニタリングでてんかん性異常が記録されていた。脳磁図も非服薬下での検査や複数回の検査を行うことが必要と考えられた。(著者抄録)

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

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.

DOI： 10.1016/j.jocn.2008.04.007

Language：Japanese  

当科てんかん外来を初診した86例中、複数回の全身痙攣発作を生じても脳波・脳MRIで異常がなく、てんかん診断に苦慮した4症例を検討した。2例で前兆としてあるいは前兆と関係なく既視感、記憶追想が存在し、うち1例で側頭葉内側のベンゾジアゼピン受容体の低下を認めた。他の1例で不安発作が存在した。4症例とも抗てんかん薬の投与で発作が消失・減少した。これらの症例では既視感、記憶追想、不安発作の有無の問診、脳MRI以外の画像検査の追加、抗てんかん薬の効果をみることが重要と考えられた。(著者抄録)

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

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.

DOI： 10.1111/j.1528-1167.2008.01558.x

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

OBJECTIVE: Malformations of cortical development are frequent causes of human refractory epilepsy. The freeze-lesion model in rats shows histopathological features similar to those found in human polymicrogyria. Previous studies reported in vitro hyperexcitability in this model, but in vivo epileptogenicity has not been confirmed. METHODS: Neocortical freeze lesions were induced in Sprague-Dawley rat pups (n = 10) on postnatal day 0 or 1 (P0/P1). Sham-operated animals served as controls (n = 10). On P60, animals were implanted with epidural electrodes for long-term video-EEG monitoring (4 weeks). The threshold for pentylenetetrazol-induced seizures was determined. Animals were sacrificed and brain sections processed for histological staining and in vitro electrophysiological recordings. Epileptiform field potential repetition rate, amplitude and integral were compared between slices containing a cortical freeze lesion, and slices from sham-operated rats. RESULTS: No interictal spikes and no electrographic or clinical seizures occurred in either group. The median threshold for pentylenetetrazol-induced seizures was 60 mg/kg for lesioned, and 45 mg/kg for control animals (difference not significant). No spontaneous epileptiform field potentials were recorded from either freeze-lesion or control slices bathed in normal, artificial cerebrospinal fluid (ACSF). Upon omission of Mg(2+) from the bath, epileptiform field potentials were elicited that showed a significantly higher burst integral in the freeze lesion slices compared to control slices. CONCLUSION: Neocortical freeze lesions induced in newborn rat pups show histological characteristics reminiscent of human cortical dysplasia. Brain slices containing neocortical freeze lesions display hyperexcitability in vitro, but the same lesion does not appear to show spontaneous epileptogenicity in vivo.

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

Status epilepticus (SE) is prolonged and repetitive epileptic seizure. SE is associated with widespread neuronal necrosis in vulnerable regions of the brain. We obtained diffusion-weighted image of magnetic resonance imaging (DWI-MRI) from two cases with SE. Case I is a 55-year-old man who showed repetitive tonic seizures on his left side evolving to generalized tonic seizure. Seizure occurred every 20 min at maximum rate. Todd's palsy was seen on his left side. Electroencephalogram (EEG) showed repetitive high-amplitude sharp waves over the right front-central region, which continued 10 days with diminishing repetitive rate of sharp waves. DWI-MRI showed high signal intensity at the right frontal cortex and dorsomedial portion of the thalamus. Apparent diffusion coefficient (ADC) was low at the same area. Case 2 is a 37-year-old woman. She initially stopped speech evolving to generalized tonic seizure. The seizure disappeared 30 min later. Todd's palsy appeared on her right side. EEG showed repetitive spikes, polyspikes and slow waves with high amplitude over the left centro-parieto-tempolo-occipital region, which disappeared next day. DWI-MRI showed high signal intensity at the left parieto-tempolo-occipital lobes without the thalamic involvement. ADC was low at the same area. Thalamus was involved in case 1, but not in case 2. The fact may depend on the intensity of epileptic activity of neocortex. (c) 2004 Elsevier B.V. All rights reserved.

DOI： 10.1016/j.ics.2004.11.075

Language：Japanese  

[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.

Language：Others  

Visual evoked cortical magnetic responses to checkerboard pattern reversal stimulation : a study on the neural generators of N75, P100 and N145

DOI： 10.1016/S0022-510X(98)00026-4

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

In order to localize the generator site of epilepfiform discharges, we applied the techniques of jerk-locked back averaging (JBA) of magnetoencephalographic (MEG) activities and dipole source localization in a patient with epilepsia partialis continua (EPC), who showed continuous, focal myoclonic jerks in the right arm. The myoclonic discharges in the right thenar muscle were used as a trigger pulse. JBA revealed consistent EEG and MEG transients that coincided consistently and constantly preceded the myoclonic jerks. The estimated dipoles of MEG were localized in a restricted area in the left precentral area, which closely correlated with the area of epileptic discharges recorded in electrocorticography. Therefore, JBA of MEG is considered to be a useful non-invasive method for localizing the epileptogenic area in EPC.

DOI： 10.1016/S0013-4694(97)00040-0

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

INTRODUCTION: NGLY1-associated congenital disorder of deglycosylation (CDDG1: OMIM #615273) is a rare autosomal recessive disorder caused by a functional impairment of endoplasmic reticulum in degradation of glycoproteins. Neurocognitive dysfunctions have been documented in patients with CDDG1; however, deteriorating phenotypes of affected individuals remain elusive. CASE PRESENTATION: A Japanese boy with delayed psychomotor development showed ataxic movements from age 5 years and myoclonic seizures from age 12 years. Appetite loss, motor and cognitive decline became evident at age 12 years. Electrophysiological studies identified paroxysmal discharges on myoclonic seizure and a giant somatosensory evoked potential. Perampanel was effective for controlling myoclonic seizures. Exome sequencing revealed that the patient carried compound heterozygous variants in NGLY1, NM_018297.4: c.857G > A and c.-17_12del, which were inherited from mother and father, respectively. A literature review confirmed that myoclonic seizures were observed in 28.5% of patients with epilepsy. No other patients had progressive myoclonic epilepsy or cognitive decline in association with loss-of-function variations in NGLY1. CONCLUSION: Our data provides evidence that a group of patients with CDDG1 manifest slowly progressive myoclonic epilepsy and cognitive decline during the long-term clinical course.

DOI： 10.1016/j.ejmg.2023.104895

Language：Japanese  

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Seizure outcome of focus resection following evaluation using intracranial electrodes for pediatric epilepsy patients

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

BACKGROUND: We previously demonstrated the usefulness of periorbital electrodes in supplemental recording to detect epileptiform discharges in patients with mesial temporal lobe epilepsy (MTLE). However, eye movement may disturb periorbital electrode recording. To overcome this, we developed mandibular (MA) and chin (CH) electrodes and examined whether these electrodes could detect hippocampal epileptiform discharges. METHODS: This study included a patient with MTLE, who underwent insertion of bilateral hippocampal depth electrodes and video-electroencephalographic (EEG) monitoring with simultaneous recordings of extra- and intracranial EEG as part of a presurgical evaluation. We examined 100 consecutive interictal epileptiform discharges (IEDs) recorded from the hippocampus and two ictal discharges. We compared these IEDs from intracranial electrodes with those from extracranial electrodes such as MA and CH electrodes in addition to F7/8 and A1/2 of international EEG 10-20 system, T1/2 of Silverman, and periorbital electrodes. We analyzed the number, rate of laterality concordance, and mean amplitude of IEDs detected in extracranial EEG monitoring and characteristics of IEDs on the MA and CH electrodes. RESULTS: The MA and CH electrodes had nearly the same detection rate of hippocampal IEDs from other extracranial electrodes without contamination by eye movement. Three IEDs, not detected by A1/2 and T1/2, could be detected using the MA and CH electrodes. In two ictal events, the MA and CH electrodes detected the ictal discharges from the hippocampal onset as well as other extracranial electrodes. CONCLUSION: The MA and CH electrodes could detect hippocampal epileptiform discharges as well as A1/A2, T1/T2, and peri-orbital electrodes. These electrodes could serve as supplementary recording tools for detecting epileptiform discharges in MTLE.

DOI： 10.25259/SNI_1164_2022

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

BACKGROUND: Our recent report showed that 1.5-T pulsed arterial spin labeling (ASL) magnetic resonance (MR) perfusion imaging (1.5-T Pulsed ASL [PASL]), which is widely available in the field of neuroemergency, is useful for detecting ictal hyperperfusion. However, the visualization of intravascular ASL signals, namely, arterial transit artifact (ATA), is more remarkable than that of 3-T pseudocontinuous ASL and is easily confused with focal hyperperfusion. To eliminate ATA and enhance the detectability of (peri) ictal hyperperfusion, we developed the subtraction of ictal-interictal 1.5-T PASL images co-registered to conventional MR images (SIACOM). METHODS: We retrospectively analyzed the SIACOM findings in four patients who underwent ASL during both (peri) ictal and interictal states and examined the detectability for (peri) ictal hyperperfusion. RESULTS: In all patients, the ATA of the major arteries was almost eliminated from the subtraction image of the ictal-interictal ASL. In patients 1 and 2 with focal epilepsy, SIACOM revealed a tight anatomical relationship between the epileptogenic lesion and the hyperperfusion area compared with the original ASL image. In patient 3 with situation-related seizures, SIACOM detected minute hyperperfusion at the site coinciding with the abnormal electroencephalogram area. SIACOM of patient 4 with generalized epilepsy diagnosed ATA of the right middle cerebral artery, which was initially thought to be focal hyperperfusion on the original ASL image. CONCLUSION: Although it is necessary to examine several patients, SIACOM can eliminate most of the depiction of ATA and clearly demonstrate the pathophysiology of each epileptic seizure.

DOI： 10.25259/SNI_723_2022

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A surgical case of posterior cingulate epilepsy associated with cavernous hemangioma

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Diagnosis and treatment of focal epilepsy using stereotactic electroencephalography

Language：Japanese  

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A proposal report about clinical electroencephalography in Japan from the EEG subcommittee

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A proposal report about clinical electroencephalography in Japan from the EEG subcommittee

DOI： 10.11422/jscn.50.107

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

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&#37;, p<0.001) and cases with telemedicine (+2,608&#37;, p=0.031) were affected. The number of COVID-19 cases was an independent associated factor for epilepsy admission (-3.75*10-3 &#37; per case, p<0.001) and EEG monitoring (-3.81*10-3 &#37; per case, p = 0.004). Further, the state of emergency was an independent factor associated with outpatient with epilepsy (-11.9&#37;, p<0.001), outpatient EEG (-32.3&#37;, p<0.001), telemedicine for epilepsy (+12,915&#37;, p<0.001), epilepsy admissions (-35.3&#37;; p<0.001), EEG monitoring (-24.7&#37;: p<0.001), and epilepsy surgery (-50.3&#37;, 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.

DOI： 10.1002/epi4.12616

Language：Japanese  

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

Background: There is scarce evidence regarding focal resection surgery for super-refractory status epilepticus (SRSE), which is resistant to general anesthetic treatment over 24 h. We report two patients with SRSE, in whom good seizure outcomes were obtained following focal resection surgery. Case Description: Patient 1: A 58-year-old man who underwent left anterior temporal lobectomy with hippocampectomy at the age of 38 years after being diagnosed left medial temporal lobe epilepsy. After 19 years of surgery with no epileptic attacks, the patient developed SRSE. Electroencephalogram (EEG) demonstrated persistence of lateralized periodic discharges in the left frontotemporal region. On the 20th day after SRSE onset, resection of the frontal lobe and temporal lobe posterior to the resection cavity was performed. Patient 2: A 62-year-old man underwent craniotomy for anaplastic astrocytoma in the left frontal lobe at the age of 34 years. Since the age of 60 years, he developed SRSE 3 times over 1 and 1/12 years. On EEG, repeated ictal discharges were observed at the medial part of the left frontal region during the three SRSEs. Corresponding to the ictal EEG findings, high signals on diffusion-weighted magnetic resonance images and focal hypermetabolism on fluorodeoxyglucose-positron emission tomography were observed around the supplementary motor area, medial to the resection cavity. Resection surgery of the area was performed during the interictal period. Conclusion: Good seizure outcome was obtained in the two cases which provide additional support for the recent concept of focal resection surgery as an indication for SRSE.

DOI： 10.25259/SNI_152_2022

Language：English  

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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).

DOI： 10.25259/SNI_841_2021

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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&#37;, specificity = 93&#37;). 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.

DOI： 10.1016/j.yebeh.2021.108402

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

Objective: To identify the risk factors for psychological distress in electroencephalography (EEG) technicians during the coronavirus disease 2019 (COVID-19) pandemic. Method: In this national-level cross-sectional survey initiated by Japan Young Epilepsy Section (YES-Japan), a questionnaire was administered to 173 technicians engaged in EEG at four clinics specializing in epilepsy care and 20 hospitals accredited as (quasi-) epilepsy centers or epilepsy training facilities in Japan from March 1 to April 30, 2021. We collected data on participants’ profiles, information about work, and psychological distress outcome measurements, such as the K-6 and Tokyo Metropolitan Distress Scale for Pandemic (TMDP). Linear regression analysis was used to identify the risk factors for psychological distress. Factors that were significantly associated with psychological distress in the univariate analysis were subjected to multivariate analysis. Results: Among the 142 respondents (response rate: 82&#37;), 128 were included in the final analysis. As many as 35.2&#37; of EEG technicians have been under psychological distress. In multivariate linear regression analysis for K-6, female sex, examination for patients (suspected) with COVID-19, and change in salary or bonus were independent associated factors for psychological distress. Contrastingly, in multivariate linear regression analysis for TMDP, female sex, presence of cohabitants who had to be separated from the respondent due to this pandemic, and change in salary or bonus were independent associated factors for psychological distress. Conclusion: We successfully identified the risk factors associated with psychological distress in EEG technicians during the COVID-19 pandemic. Our results may help in understanding the psychological stress in EEG technicians during the COVID-19 pandemic and improving the work environment, which is necessary to maintain the mental health of EEG technicians.

DOI： 10.1016/j.yebeh.2021.108361

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

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&#37; 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.

DOI： 10.3390/jcm10245972

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

Reflection of the Ictal Electrocorticographic Discharges Confined to the Medial Temporal Lobe to the Scalp-Recorded Electroencephalogram.
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.

DOI： 10.1177/15500594211062702

Language：Japanese  

Successful treatment for sphenoidal myelomeningocele related temporal lobe epilepsy under endonasal endoscopic approach; a case report.

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Mandibular-Chin electrodes for detection of epileptiform discharges in medial temporal lobe: Verification with simultaneous recording of inra- & extra-cranial EEG

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

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

DOI： 10.1016/j.yebeh.2019.106865

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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.

DOI： 10.1016/j.yebeh.2019.05.026

Language：Japanese  

1997年より脳神経検査システムを導入し、オーダー連携による脳波検査、結果報告、管理を一元的に行っている。従来のレポート作成では技師による下書き、記載者および記載内容の履歴について表示されていなかった。一つのレポートに複数の技師が関与するにも関わらず、誰がどの所見を記載したのか詳細が不明であった。そこで、誰がどの所見を記載したのか履歴が残るレポート比較機能を構築し、導入した。医師にも判読研修中の医師と臨床評価だけでなく判読教育可能な医師がいるため、判読研修中医師は、中堅以上の技師の所見を参考にして入力する手立てとしても利用でき、判読教育可能な医師はそれぞれの技師や判読研修中医師の習熟度が把握できた。構築したレポート比較機能を使用し、日常的に脳波所見の目合わせを行っており、脳波検査における内部精度管理の一助としている。これに加え、外部精度管理の一環として他施設との医師主導型脳波カンファレンスに参加し、脳波判読の目合わせを行っている。それぞれの技師がどの程度脳波所見を認識しながら記録しているのか習熟度を把握でき、教育にも反映しやすくなった。

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DOI： 10.5692/clinicalneurol.cn-001044

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

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.

DOI： 10.1111/cen3.12332

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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.

DOI： 10.1038/srep27164

Language：Japanese  

[A case of paroxysmal sympathetic storm after acute disseminated encephalomyelitis and hypoxic encephalopathy responding to clonidine hydrochloride].
We report the case of a 17-year-old woman with paroxysmal sympathetic storm (PSS), which was successfully treated with clonidine hydrochloride. The patient was hospitalized for acute disseminated encephalomyelitis in June 2006. Dysphagia led to severe aspiration pneumonia in September 2006, and she suffered cardiopulmonary arrest. She survived but had severe brain damage, with her brain MRI showing diffuse hypoxic encephalopathy. From October 2006, she had several episodes of profound tachypnea (> 60/min), tachycardia (160 to 170 beats/min), hypertension (> 140 mmHg), hyperthermia (39°C), and decerebrate posturing. During the attacks, the levels of catecholamines in the patient's blood and urine were markedly elevated. Accordingly, a diagnosis of PSS associated with hypoxic encephalopathy was made. Her PSS clearly improved after the administration of clonidine hydrochloride (900 μg/day). This case suggests that clonidine hydrochloride, an α2 blocker, may be one therapeutic option for PSS.

DOI： 10.5692/clinicalneurol.cn-000793

Language：Japanese  

[A case of rhabdomyolysis after status epilepticus without stroke-like episodes in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes].
A 24-year-old man was referred to our hospital emergency department due to a sudden onset of convulsions after drinking. On arrival he presented status epilepticus and was managed by artificial ventilation. He had no brainstem signs or meningeal irritation. Head MRI showed an old infarction-like lesion in the left occipital lobe, but no abnormal signals on diffusion-weighted images. The patient showed acute rhabdomyolysis (CK 18,000 IU/l) and renal failure, and hemodialysis was started. On 18 day after admission, he was transferred to our department with mild proximal limb muscle weakness and bilateral sensorineural hearing impairment. Electroencephalography demonstrated diffuse intermittent slow wave activities. We suspected a mitochondrial disease because of a significant increase in the lactate/pyruvate ratio (24.1) in the spinal fluid, and identified A3243G mutations in mitochondrial DNA (heteroplasmy 20&#37;) in peripheral white blood cells. We diagnosed his illness as mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). This is a rare case presenting an acute onset of rhabdomyolysis following alcohol intake related to A3243G mitochondrial mutation without preceding stroke-like episodes.

DOI： 10.5692/clinicalneurol.cn-000834

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

[A case of primary central nervous system vasculitis diagnosed by second brain biopsy and treated successfully].
We report a case of primary central nervous system vasculitis (PCNSV) diagnosed by second brain biopsy. A 53-year-old man initially presented with left lateral gaze diplopia. Brain MRI revealed multiple enhanced lesions in the bilateral frontal lobe, bilateral basal ganglia, left cerebellum and brainstem. An initial brain biopsy of the right frontal lobe suggested immune-related encephalitis with angiocentric accumulation of chronic inflammatory cells, while malignant lymphoma could not be completely ruled out. The patient deteriorated despite being treated with repeated methylprednisolone pulse therapy, cyclophosphamide, and plasmapheresis. A second brain biopsy of the right temporal lobe was then performed. The biopsied specimens showed vascular wall disruption and fibrinoid necrosis with perivascular inflammatory infiltrates, mainly composed of CD8-positive T cells, and PCNSV was diagnosed. He was treated with high dose corticosteroids, in combination with methotrexate (8 mg/week), which reduced the brain lesions. As brain biopsy is an essential investigation for the histological diagnosis of PCNSV; subsequent biopsies may be required when a histopathological diagnosis has not been obtained by the first biopsy, and further aggressive therapy is being considered.

DOI： 10.5692/clinicalneurol.cn-000847

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A Case of Late Onset Myoclonic Epilepsy in Down Syndrome (LOMEDS)
Myoclonic epilepsy is being increasingly recognized as a late onset complication in middle-aged or elderly patients with Down syndrome, in association with acute cognitive decline or dementia. We report a case of late onset myoclonic epilepsy in Down syndrome (LOMEDS) with acute cognitive decline, aged 45 year-olds. Although her intelligence condition remained poor with one to two-words sentences and difficult in following commands even after treatment, induction of levetiracetam and lamotorigin resulted in rapid, sustained seizure freedom and upgrading social activity with no adverse events. A combination of levetiracetam and lamotorigin appear to be effective for LOMEDS, and could be considered as first line agents for this special condition.

DOI： 10.3805/jjes.32.564

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

[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.

DOI： 10.11477/mf.1416200170

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"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.

DOI： 10.1684/epd.2014.0695

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

[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.

DOI： 10.11477/mf.1436200049

Language：Japanese  

A case of non-convulsive status epilepticus after influenza virus B infection
A 24-year-old woman was referred to our hospital because of impaired consciousness after influenza virus B infection. Neurological examination revealed mild disturbance of consciousness without other neurological abnormalities. Laboratory tests showed elevated serum CRP, IL-6 and TNF-α levels. The level of IL-6 in the cerebrospinal fluid was also slightly elevated. Electroencephalography (EEG) disclosed almost continuous generalized spike and wave complexes and multiple spikes and wave complexes at 1.5–3 Hz. Brain MRI was normal. She was diagnosed as having influenza encephalopathy presenting non-convulsive status epilepticus (NCSE), and commenced methylprednisolone pulse therapy followed by prednisolone with gradual tapering. She was also treated with intravenous phenytoin and oral sodium valproate for NCSE. The next day, her consciousness level had improved. Although she became alert, epileptic discharges on EEG were still observed on the seventh hospital day, and levetiracetam was added. Then, her epileptic discharges almost completely disappeared on the twelfth hospital day. She was discharged without any neurological deficit. We consider this patient to be a case of transient NCSE due to influenza encephalopathy; alternatively, she may have epileptic traits and her NCSE may have been provoked by influenza virus infection.

DOI： 10.5692/clinicalneurol.54.227

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Epilepsy and functional brain networks

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

[Epilepsy and functional brain networks].
Recent resting-state functional MRI (rs-fMRI) studies have demonstrated that the human brain is composed of several essential large-scale functional brain networks, including the "default-mode network". We analyzed electrocorticogram data from four patients with intractable focal epilepsy and compared the extent of these large-scale functional brain networks with propagation pathways of ictal discharge. We found that large-scale functional brain networks had markedly similar spatial patterns compared with multisite ictal fast activity, suggesting that some epileptic activity propagates along large-scale functional brain networks. Given that decreased functional connectivity in large-scale functional brain networks has been reported in patients with focal epilepsy, ictal propagation may lead to chronic alteration of normal functional networks in the brain.

DOI： 10.5692/clinicalneurol.54.1139

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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.

DOI： 10.1016/j.neuroimage.2013.11.008

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Although there is no universally accepted definition, traumatic medial temporal lobe epilepsy (traumatic MTLE) can be generally defined as a type of post-traumatic epilepsy with medial temporal epileptogenicity. Previous studies have demonstrated that patients with traumatic MTLE frequently have hippocampal sclerosis (HS) coexisting with traumatic neocortical lesions. We report a 39-year-old MTLE patient with a subcortical lesion in the.internal capsule, suspected to be caused by mild diffuse axonal injury. The results of presurgical examinations and intraoperative electrocorticograms were consistent with a diagnosis of MTLE. Therefore, an anterior temporal lobectomy with hippocampectomy was performed. A 3D short tau inversion recovery 3-Tesla MRI scan clearly depicted possibly Wallerian degeneration in the temporal stem, which was continuous with the subcortical lesion. We speculate that Wallerian degeneration in the temporal stem could potentially involve the hippocampus, eventually leading to HS.

DOI： 10.3805/eands.6.1

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Age-related electrophysiological changes in the primary somatosensory cortex (SI) are well known. There is evidence that the amplitude of the N20 component of median nerve somatosensory evoked potentials typically increases with age, probably because of cortical disinhibition. The secondary somatosensory cortex (SII) receives dual input from the SI and the thalamus. We quantified age-related changes both in SI and SII using magnetoencephalography (MEG). We recorded somatosensory evoked magnetic fields (SEFs) to median nerve stimulation in 15 young adults (aged 22-36 years, mean age 29.0±4.1) and 15 older adults (aged 52-67 years, mean age 61.9±5.4), and analysed major SEF components in SI and SII. The amplitude and equivalent current dipole (ECD) strength of the N20m were significantly increased in the older adults, consistent with the well-known electrophysiological change for cortical disinhibition in SI. The latency of N20m showed a trend for increase in older subjects, possibly reflecting slowing of conduction velocity in the peripheral nerves. In contrast, SII response (response peak at around 80-120 ms) showed a different change in aging. Latencies of the contralateral SII responses showed a trend for shortening in the older adults. There were no significant age-related changes for the amplitudes and ECD strengths. Thus, SI and SII are differently affected by aging. The shortening of the SII latency suggests age-related plastic-adaptive change in SII, which is mediated by the direct thalamocortical pathway. © 2012 IEEE.

DOI： 10.1109/ICCME.2012.6275666

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Parainfectious or autoimmune striatal lesions have been repeatedly described in children. We report a 7-year-old girl with painful muscle spasms, leading to the diagnosis of childhood stiff-person syndrome (SPS). Striatal lesions were demonstrated by diffusion-weighted magnetic resonance imaging (MRI) and single-photon emission computed tomography but not by conventional MRI. Autoantibodies against glutamic acid decarboxylase (GAD) were absent. Steroid pulse therapy and high-dose intravenous immunoglobulin resolved all the symptoms with slight sequelae. Childhood SPS may be characterized by absent anti-GAD antibodies and a transient benign clinical course, and it may have a pathomechanism distinct from that in adult SPS.

DOI： 10.1016/j.braindev.2012.08.003

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DOI： 10.1136/jnnp-2012-302281

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[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.

DOI： 10.5692/clinicalneurol.51.997

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DOI： 10.1159/000332033

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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.

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

[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.

DOI： 10.5692/clinicalneurol.51.595

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[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.

DOI： 10.5692/clinicalneurol.51.334

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Language：Japanese  

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.

DOI： 10.5692/clinicalneurol.50.461

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

[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.

DOI： 10.5692/clinicalneurol.50.482

Language：Japanese  

Language：Japanese  

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.

DOI： 10.5692/clinicalneurol.50.467

Language：Japanese  

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.

DOI： 10.5692/clinicalneurol.50.265

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

To categorize four types of sleep- and non-sleep-related hallucinations experienced by normal people and classify ghost or ghost-like stories by these categories. A total of 183 reliable tales of ghosts [41 from "Tohno Monogatari" (Tohno Folktales) and 142 from "Nihon Kaidan Shu" (Ghosts Tales of Japan)] are classified into hallucinations that are sleep-related hallucinations [hypnagogic hallucination-like (HyH) and REM sleep behavior disorder or somnambulism-like (RBDS) tales] and sleep-unrelated [vivid hallucination-like (VH) and highway hypnosis-like (HHy) tales] according to the criteria. Sixty to 70&#37; of these tales can be classified into these four types of hallucinations. Further, sleep-related hallucinations increased from 17.0&#37; to 36.6&#37; in about 40 years. Our criteria will be useful to classify hallucinations experienced by normal people and to elucidate the mechanisms of these kinds of hallucinations experienced in neurodegenerative or psychological disorders.

DOI： 10.1037/a0017611

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

Temporal lobe epilepsy associated with ‘triple pathology’ of hippocampal sclerosis, focal cortical dysplasia and cavernoma in the ipsilateral frontal lobe
The coexistence of hippocampal sclerosis (HS) and extrahippocampal lesions such as focal cortical dysplasia (FCD) and cavernoma (CA) in temporal lobe epilepsies (TLE) is termed ‘dual pathology’. We report a case of TLE having ‘triple pathology’ of HS, FCD and CA in the ipsilateral frontal lobe. Using chronic subdural electrode recording, an interictal electrocorticogram (ECoG) demonstrated that the medial temporal lobe lesion and the FCD lesion exhibited independent paroxysmal discharges, while an ictal ECoG demonstrated that the medial temporal lobe was the ictal on-set zone. We postulated that the FCD lesion caused repeated seizures, which in turn, caused secondary hippocampal damage and HS, and eventually medically intractable epilepsy. The coexistence of CA was considered incidental. As treatments, the epileptogenic HS was resected through an anterior temporal lobectomy, and the FCD lesion was biopsied, while no surgical intervention was performed for the CA lesion. The patient achieved good seizure control during 12 months after surgery. Chronic subdural electrode recording is important for the assessment of multiple epileptogenic lesions before epilepsy surgery.

DOI： 10.3805/eands.2.34

Language：Others  

Language：Others  

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Language：English  

Efficacy of mediodorsal thalamic nucleus stimulation in a rat model of cortical seizure
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)

DOI： 10.15017/15638

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

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&#37; vs. 20/84, 23.8&#37;, 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&#37; vs. 28/84, 33.3&#37;, 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.

DOI： 10.1016/j.jns.2009.02.371

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

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.

DOI： 10.1016/j.clinph.2009.01.020

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

DOI： 10.1212/WNL.0b013e3181a0fe74

Language：Japanese  

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

Language：Japanese  

デジタル脳波計 脳波検査技師と診療従事者で支えるてんかんのビデオ脳波モニタリング検査
脳波および同時記録ビデオのデジタル化に伴い、発作の起始や伝播、臨床発作との関連、頭蓋内と頭皮上脳波の相違などについて詳細に短時間に確認することが可能になった。当施設ではこれら脳波技師側からの情報と医師側からの臨床情報をお互いが授受する連携体制を構築し、モニター開始後一両日中に多くの情報を共有することができるようになった。今後もモニター時間短縮など患者さんの負担減少に貢献できるような運用をめざしている。(著者抄録)

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

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.

DOI： 10.1016/j.jns.2008.07.021

Language：Others  

Language：Others  

Language：Others  

Language：Others  

Language：Others  

Language：Others  

Language：Japanese  

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

Language：Japanese  

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.

DOI： 10.5692/clinicalneurol.48.36

Language：English  

Upper Motor Neuron Syndrome Associated with Subclinical Sjoegren's Syndrome
We present two patients with primary lateral sclerosis-like upper motor neuron disease accompanying subclinical Sjögren's syndrome. Both patients showed progressive spastic quadriparesis, but neither sensory involvement nor detrusor dysfunction was noted. Lower motor neuron signs were detected only in their late follow-up period. Although sicca symptom was nearly absent, salivary labial gland biopsy revealed marked sialoadenitis in both patients. They also displayed a constellation of findings that suggested an autoimmune etiology closely related to Sjögren's syndrome, including germinal center formation in one patient, and markedly elevated levels of anti-nuclear antibody with abnormal sialography in the other. Both patients showed significant neurological improvement after the initial course of intravenous immunoglobulin therapy. We suggest that the evidence for subclinical Sjögren's syndrome should be sought in patients presenting with selective upper motor neuron involvement.

DOI： 10.2169/internalmedicine.47.0846

Language：Japanese  

[Acute myelitis in a patient with ulcerative colitis].

DOI： 10.2169/naika.96.1703

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

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

DOI： 10.1016/j.neuroimage.2007.04.027

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

We report a 73-year-old man with SPG4. From aged 53 he had diabetes mellitus and at 64 he developed spastic paraparesis and urinary disturbance. At 70 years, he began to walk with a stick and noted abnormal sensations in bilateral feet. There was no relevant family history. Moderate spasticity with mild muscle weakness, markedly brisk tendon reflex with pathological reflexes, and mildly abnormal sensation in bilateral lower extremities, and markedly spastic gait were found. MRI showed mild C4-C7 spondylosis and L4-5 disk protrusion but no abnormality of the corpus callosum. Nerve conduction and needle EMG studies revealed various abnormalities in distal (MCV, SCV) and proximal (F-wave) peripheral nerves, but no neurogenic changes in limb muscles. We found a missense spastin gene mutation (1726T>C) that causes Leu534Pro substitution. This spastin gene mutation was novel in Japanese, but has been reported in an Italian family. The present case's neuropathy might be related to diabetes mellitus, because SPG4 is generally not associated with neuropathy. However, recent studies suggest that SPG4 patients sometimes have subclinical neuropathy, and longer disease duration may contribute to peripheral neuropathy. Further study of clinical characteristics associated with the Leu534Pro mutation will be necessary.

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

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&#37;) and of the 13 who underwent MEPs, four had abnormal CMCT (31&#37;). 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.

DOI： 10.1016/j.jns.2007.01.004

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

DOI： 10.1212/01.wnl.0000244489.65670.9f

Language：Japanese  

[An adult case of probable Bassen-Kornzweig syndrome, presenting resting tremor].
We report a 53-year-old woman with probable Bassen-Kornzweig syndrome. Her parents were a consanguineous marriage. At two years of age, she developed night blindness. During her childhood she had severe diarrhea that disappeared in adulthood. At 26 years of age, she was diagnosed as having retinitis pigmentosa and her visual acuity became worse thereafter. She noted tremor in the right hand at 37 years of age, gait ataxia at 42, and developed tremor in the bilateral lower extremities at 48. On admission, bilateral visual disturbance, resting and postural tremor, moderately poor coordination, mild distal dominant sensory impairment, an absence of tendon reflex in all four extremities, moderate to severe gait ataxia, and positive Romberg sign were found. Muscle rigidity and akinesia were not observed. Intelligence and muscle power were normal and pathological reflexes were absent. Acanthocytes were found in blood. Serum chemistry showed remarkable decreases in total cholesterol (54 mg/dl, normal 180-220), triglyceride (0 mg/dl, normal 30-150), beta-lipoprotein (3 mg/dl, normal 190-500), apoA-1 protein (66 mg/dl, normal 105-184), apoA-2 protein (11 mg/dl, normal 26-46), apoB protein (0 mg/dl, normal 38-104), apoC-2 protein (1.1 mg/dl, normal 1.2-6.4), vitamin A (297 ng/ml, normal 431-1,041), and vitamin E (0.19 ng/dl, normal 0.75-1.41). While, a marked increase in PIVKA II (703 mAU/ml, normal<40) due to a decrease in vitamin K was found. She was thus diagnosed as having Bassen-Kornzweig syndrome or hypo-betalipoproteinemia. Although brain MRI was normal, single-photon emission CT (SPECT) showed mildly decreased perfusion in the left parietal cortex and right striatum. Motor nerve conduction velocities were normal, but sensory nerve action potentials were not evoked in all four extremities. Surface EMG recorded on the right radial extensor and flexor carpi muscles at rest showed a 4.5 Hz tremor. Vitamin replacement therapy with vitamin A (10,000 IU/day), E (200 mg/day), and K (10 mg/day) was initiated. Several days after treatment, amplitude of resting tremor ameliorated mildly. Clonazepam was administered (0.5 mg/day) for further treatment. After one-month of treatment, vitamin A (656 ng/ml) and E (0.39 mg/dl) levels were elevated and PIVKA II level (29 mAU/ml) decreased. Only a mild right hand tremor remained, but sensory impairment and gait ataxia were not changed. The cause of Bassen-Kornzweig syndrome is a deletion of the microsomal triglyceride transfer protein (MTP) gene. While, familial hypo-betalipoproteinemia, due to a mutation of apolipoprotein B gene, is known to show the same phenotype. Because of the patient's refusal of genetic examination, which disease she has cannot be conclusively determined. Intention tremor was reported in Bassen-Kornzweig syndrome. However, her 4.5 Hz tremor was also present at rest, which resembled resting tremor in Parkinson's disease. Pathophysiology of Bassen-Kornzweig syndrome is known to be due to hypo-vitaminosis. Decreased [18F]-dopa uptake in striatum of patients with long-term hypo-vitamin E has been reported in PET study. Mild hypoperfusion was found in the striatum of the present cases: indicating that her tremor was associated with striatonigral damage. Thus, careful observation of extrapyramidal signs is necessary in abeta- or hypo-betalipoproteinemia.

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

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

DOI： 10.1016/j.neuroimage.2005.12.032

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

[Venous infarction associated with carotid-cavernous fistula].
We report an 88-year-old woman who developed a hemorrhagic venous infarction in the left cerebral hemisphere and brainstem, in association with left carotid-cavernous fistula (CCF). Without aura the patient noticed diplopia due to left abducens palsy, and exophthal mos and congestion of the left eye. Brain CT revealed extrusion of the left eye, and dilatation of left superior orbital vein and cerebral cortical veins. She received diagnosis of CCF. Brain CT also revealed a small mass in the left ethmoidal sinus, which was not attached to the CCF. Biopsy of the mass was done under local anesthesia. On the following she had high fever. Her consciousness level deteriorated and she developed right hemiparesis FLAIR images of MRI showed, extensive high signal lesions in the left frontal and temporal cortices, basal ganglia, thalamus, midbrain and pons. These findings were consisted with venous infarction, possibly associated with peri-operative infection and hypovolemia. Intracranial hemorrhage occur in 3&#37; of cases with CCF, but venous infarction was much rarer. The patients with CCF, who show dilatation of cortical veins in CT or MRI, have a higher risk of cerebral hemorrhage or infarction, and should be carefully observed.

Language：Japanese  

[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.

DOI： 10.11477/mf.1406100134

Language：Japanese  

[Fibromuscular dysplasia of the basilar artery presenting as cerebral infarction in a young female].
We reported a 20-year-old woman with fibromuscular dysplasia (FMD) of the basilar artery presenting multiple cerebral infarctions. A sudden onset of consciousness disturbance and right hemiparesis was experienced. A neurological examination on day 2 revealed an absence of light and corneal reflexes on the left side, homonymous left upper quadrant anopsia and right hemiparesis with Babinski sign: she was also somnolent. On head MRI, multiple high signal intensity lesions were seen in the right occipital lobe, bilateral thalami and left pons on T2- and diffusion weighted images. Brain angiogram revealed the string of beads sign of the basilar artery, suggesting FMD. Neurological deficits gradually improved in the 2 months that followed, leaving slight hemiparesis and homonymous left upper quadrant anopsia In the following 3 years, no recurrence was seen with aspirin (81 mg/day). FMD in the head and neck usually affects extracranial segments of the carotid and vertebral arteries, while FMD of the basilar artery is extremely rare. To the best of our knowledge, 12 cases with FMD of the basilar artery have been reported; of these, 11 were symptomatic and 5 died. Since FMD of the basilar artery has poor prognosis, attention needs to be paid for FMD in young adults as a differential diagnosis of cerebral infarction in the territory of the basilar artery.

Language：Japanese  

[Relapsing Wernicke's encephalopathy after gastrectomy].

DOI： 10.2169/naika.94.1606

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

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

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.

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

A patient with mitochondrial encephalomyopathy presenting gynecomastia with elevation of serum estriol level
We report a 23-year-old man with mitochondrial encephalomyopathy. At 21 years of age, he noted speech distubance. Since his dysarthria did not improve thereafter, he was admitted to our hospital. On admission, he showed mild gynecomastia. Neurological examination revealed mild decrease in performance IQ in WAIS-R, mild scanning speech, mild left hearing disturbance, mild to moderate muscle weakness in proximal four extremities, mild bilateral limb ataxia, and mild to moderate truncal ataxia. While, no brisk deep tendon reflex, pathological reflex, aberrant muscle tonus, sensory disturbance, retinopathy, myoclonus or autonomic disorder was found. Serum levels of lactate (23.2 mg/dl, normal≤18.7) and pyruvate (1.23 mg/dl, normal&lt
0.94) were elevated, and serum lactate levels were markedly elevated (118.1 mg/dl) after 15-minute exercise (15 Watts/minute). CSF levels of lactate (31.2 mg/dl, normal≤12.5) and pyruvate (1.48 mg/dl, normal&lt
0.75) were also elevated. Head MRI showed mild cerebral and cerebellar atrophy, but 1H-MRS showed no lactate peak. Moreover, muscle biopsy from left biceps muscle showed lots of ragged-red fibers, and he was thus diagnosed as having mitochondrial encephalomyopathy. However, nt3243 mutation of mitochondria DNA was not present. Next, we confirmed gynecomastia by mammography, and checked serum levels of estrogens. Mildly decreased estradiol (19.9 pg/ml
normal, 20-59), normal estrone (24.0 pg/ml, normal&lt
30.0) and mildly increased estriol (6.03 pg/ml, normal≤5.0) were found. While, the serum levels of cortisol, dehydroepiandrosterone-sulfate (DHEA-S), androstenedione, testosterone, luteinizing hormone (LH) and follicle stimulating hormone (FSH) were all within normal limits. Since the steroid hormone synthesis system and hypothalamus-pituitary system seem to be normal, 16α-hydroxylase that converts estradiol to estriol may be upregulated. While, aromatase (P-450arom) is well known to convert androgens to estrogens. In addition, 16α-hydroxylase and P-450arom convert DHEA-S to estriol. Since it is recently reported that P-450arom is considerably expressed in muscle tissues as well as fat tissues and that muscle tissue may be a major organ to produce estrogens in men and postmenopausal women, estriol production may be increased in the present patient's muscle. Although hypogonadism due to hypothalamus-pituitary disorders was sometimes reported, there have been no reports that suggest an increased estrogen production in skeletal muscles in mitochondrial encephalomyopathies. Recently, estrogen has been known to protect muscle fibers from oxidative damages due to exercise. Thus, it is of potential that estrogens increased locally in muscle tissues of the patients with mitochondrial encephalomyopathies protect muscle fibers from oxidative damage due to mitochondrial dysfunction.

Language：Japanese  

[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.

Language：Japanese  

[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.

DOI： 10.11477/mf.1406901888

Language：Others  

Clinical abnormalities disclosed by Bender Gestalt test in patients with progressive supranuclear paralysis.

Language：Others  

Parkinson disease is more frequent in female in Japan?

Language：Others  

Application of QOL evaluation scale, PDQ-39 to patients with Parkinson disease.

Language：Japanese  

Spontaneous improvement of subacute exacerbation in a case of sensory ataxic neuropathy associated with Sjögren's syndrome
We reported a 66-year-old man with Sjögren's syndrome (sicca syndrome) presenting a sensory ataxic neuropathy, which showed spontaneous remission. He developed difficulty in standing and walking during recent several months. Neurological examinations showed sensory ataxia with areflexia in all extremities and mild distal-dominant decrease in the superficial sensation. Laboratory examinations of blood, urine, and cerebrospinal fluid were all unremarkable; the blood levels of vitamin B1, B2, B6 and B12 were low normal. Sensory nerve action potentials and somatosensory evoked potentials were absent. Cervical cord MRI revealed no abnormal signals. Severe loss of myelinated fibers and scattered myelin ovoids were seen in sural nerve biopsy. Tentative diagnosis at admission was subacute sensory neuropathy associated with malignancy. Screening examinations for malignancy were undertaken and all revealed negative. Because of coexisting sicca symptoms and positive Shirmer test (0 mm), a lip biopsy was performed and a diagnosis of Sjögren's syndrome was confirmed. Sensory ataxia improved gradually. Two years later, blood B1 levels were low but he remained able to walk. This case is an uncommon example of spontaneous symptomatic remission in sensory ataxic neuropathy associated with Sjögren's syndrome.

Language：Others  

Language：Others  

Language：Others  

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

We report a case of a 63-year-old man suffering from anti-Ri-associated paraneoplastic cerebellar degeneration (PCD) with gastric cancer. The neurologic presentation was limited to severe cerebellar ataxia without opsoclonus. The gastric cancer was composed of both poorly differentiated adenocarcinoma and neuro-endocrine carcinoma. The patient's serum reacted with recombinant Ri antigen and the neuroendocrine tumor component. It is thus considered that PCD without opsoclonus in the present case was related to the gastric neuroendocrine tumor and anti-Ri antibody.

Language：Others  

Feasibility and Limitation of Magnetoencephalography in Epilepsy Surgery.

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

A positron emission tomography (PET) study on the regional cerebral glucose metabolism (rCMRglc) was performed in six patients with corticobasal degeneration (CBD). The clinical features included asymmetrical parkinsonism with apraxia, were related to the cerebral cortical and basal ganglionic dysfunction. An MRI study showed all cases to have asymmetrical atrophy in the front-parietal cortex contralateral to the dominantly affected limb; however, no case was pathologically verified. A PET study revealed three cases to have asymmetrical glucose hypometabolism in the parietal lobe and thalamus, which was compatible with the results of previous reports. However, two patients demonstrated symmetrical glucose hypometabolism in the frontal lobe, striatum and parietal lobe while one case had a diffuse hypometabolism, in spite of a marked asymmetry of the neurological findings. These results therefore suggest the heterogeneity of the glucose hypometabolism in CBD based on the PET findings.

DOI： 10.1016/S0022-510X(98)00269-X

Language：Others  

Motor evoked potentials with transcranial magnetic stimulation in SMON. IV. Analysis of soleus late response.

Language：Others  

Epileptogenicity of middle fossa arachnoid cyst.

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

Language：Others  

Visual evoked cortical magnetic responses: a non-invasive test of the brain function in patients with Minamata disease.

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

DOI： 10.1111/j.1528-1157.1996.tb01834.x

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

To clarify the topographical relationship between peri-Rolandic lesions and the central sulcus, we carried out presurgical functional mapping by using magnetoencephalography (MEG), functional magnetic resonance imaging (f- MRI), and motor evoked potentials (MEPs) on 5 patients. The sensory cortex was identified by somatosensory evoked magnetic fields using MEG (magnetic source imaging (MSI)). The motor area of the hand region was identified using f-MRI, during a hand squeezing task. In addition, transcranial magnetic stimulation localized the hand motor area on the scalp, which was mapped onto the MRI. In all cases, the sensory cortical vein or the lack of any functional activation in the area of peri-lesional edema. MEPs were also unable to localize the entire motor strip. Therefore, at present, MSI is considered to be the most reliable method to localize peri-Rolandic lesions. [Neurol Res 1995; 17: 361-367] cortical vein or the lack of any functional activation in the area of peri-lesional edema. MEPs were also unable to localize the entire motor strip. Therefore, at present, MSI is considered to be the most reliable method to localize peri-Rolandic lesions.

DOI： 10.1080/01616412.1995.11740343

Language：Others  

Clinical possibilities of SQUID application.

Language：Others  

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

A hypothalamic hamartoma associated with true precocious puberty in a 7-month-old girl is hereby reported. Hormonal studies disclosed elevated serum levels of luteinizing hormone (LH) and follicle stimulating hormone, both of which responded well to LH-releasing hormone stimulation. Following a subtotal removal of the tumor, the clinical manifestations of precocious puberty as well as associated endocrinological abnormalities returned to normal. The role of surgery for this lesion, which appears to be safe when a planned microsurgical course is employed, is discussed. © 1993 Walter de Gruyter & Co.

DOI： 10.1007/BF00258250

Language：Others  

A case of hyperornithinemia-hyperammonemia-homocitrullinuria(HHH) syndrome with spastic paraparesis and severe distal muscle atrophy of lower limbs.

Language：Japanese  

16歳男,両親はいとこ婚。幼少時より知能遅延および嘔吐がしばしばあったが,意識消失発作はなかった。15歳時より進行性の歩行障害をきたし,大腿四頭筋の高度の痙縮と下肢遠位筋に著明な筋萎縮と脱力を認め,筋電図および神経生検所見から筋萎縮の原因は脊髄前角細胞障害が考えられた。これら神経徴候の出現に高オルニチンのみならず高グルタミン酸などアミノ酸異常が関与している可能性が示唆された

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：General book, introductory book for general audience

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Responsible for pages：総ページ数：87p   Language：Others  

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Language：Japanese   Book type：Scholarly book

Responsible for pages：総ページ数：84p   Language：Others  

Language：English   Book type：Scholarly book

Event date： 2023.6

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：九州 WEB   Country：Japan  

Event date： 2023.5 - 2023.6

Language：Japanese   Presentation type：Symposium, workshop panel (public)  

Venue：幕張メッセ国際会議場   Country：Japan  

Event date： 2023.5 - 2023.6

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：幕張メッセ国際会議場   Country：Japan  

Event date： 2021.6

Language：English   Presentation type：Oral presentation (general)  

Venue：WEB   Country：Ireland  

Event date： 2020.11

Language：Japanese   Presentation type：Symposium, workshop panel (public)  

Venue：国立京都国際会館   Country：Japan  

Event date： 2020.11

Language：Japanese   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Venue：国立京都国際会館   Country：Japan  

Event date： 2020.11

Language：Japanese   Presentation type：Symposium, workshop panel (public)  

Venue：国立京都国際会館   Country：Japan  

Event date： 2020.8 - 2020.9

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：岡山コンベンションセンター   Country：Japan  

Event date： 2020.1

Language：Japanese  

Venue：アクトシティ浜松コングレスセンター   Country：Japan  

Event date： 2019.12

Language：English  

Venue：Baltimore   Country：United States  

Event date： 2019.10 - 2019.11

Language：Japanese   Presentation type：Symposium, workshop panel (public)  

Venue：神戸国際会議場   Country：Japan  

Event date： 2019.6

Language：English   Presentation type：Oral presentation (general)  

Venue：Centara Grand & Bangkok Convention Centre at Centralworld in Bangkok, Thailand   Country：Thailand  

Objective:
To assess prognostic factors predicting seizure recurrence after resective surgery for temporal lobe epilepsy patients who had chance to use new antiepileptic drugs (AEDs).

Methods:
We retrospectively investigated surgical outcomes in patients with neocortical or medial temporal lobe epilepsy who underwent temporal lobe resection between May 2014 and June 2017, and were observed for a minimum of one and half year after the surgery. To identify independent predictors for seizure relapse, we first used the univariate analysis (gender, seizure-onset age, using preoperative AEDs treatment, surgical side, pathology, etc.) to find significance differences and then used Cox’s proportional hazards analyses for multivariate analysis using results of univariate analyses.

Results:
A total of 41 consecutive patients were included in this study. Estimated epileptogenic zone in neocortex, existence of inter-ictal epileptiform discharges after operation, and duration of disease before surgery were significantly associated with seizure relapse, whereas preoperative treatment of new AEDs did not change the outcome: (1) Neocortical temporal lobe epilepsy compared to medial temporal lobe epilepsy (hazard ratio [HR] 0.363, 95&#37; confidence interval [CI] 0.162-0.809; p=0.013), (2) Postoperative inter-ictal epileptiform discharges on EEG (HR 0.475, 95&#37; CI 0.240-0.939, p=0.032), (3) Shorter duration of disease (HR 0.965, 95&#37; CI 0.942-0.990, p=0.005).

Conclusions:
Our study disclosed that the patients with estimated epileptogenic zone in neocortex, postoperative inter-ictal epileptiform discharges, and shorter duration of disease before surgery are more likely to experience seizure recurrence postoperatively. Our results may be useful for selecting candidates for resective surgery in patients with temporal lobe epilepsy.

Other Link： https://www.ilae.org/congresses/33rd-international-epilepsy-congress

Event date： 2014.5

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Event date： 2010.12

Language：Others  

Venue：San Antonio, TX   Country：United States  

Event date： 2010.11

Language：Others   Presentation type：Oral presentation (general)  

Venue：神戸   Country：Japan  

Event date： 2010.10 - 2010.11

Language：Others  

Venue：Kobe   Country：Japan  

Event date： 2010.10

Language：Others   Presentation type：Oral presentation (general)  

Venue：岡山   Country：Japan  

Event date： 2010.6

Language：Others   Presentation type：Oral presentation (general)  

Venue：久留米   Country：Japan  

Event date： 2010.5 - 2011.5

Language：Others  

Venue：東京国際フォーラム   Country：Japan  

Event date： 2010.5 - 2011.5

Language：Others  

Venue：東京国際フォーラム   Country：Japan  

Event date： 2010.3

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Event date： 2010.2

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Event date： 2009.12

Language：Others  

Venue：Boston   Country：United States  

Event date： 2009.11

Language：Others   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Venue：北九州   Country：Japan  

Event date： 2009.10

Language：Others   Presentation type：Oral presentation (general)  

Venue：弘前   Country：Japan  

Event date： 2009.9 - 2010.9

Language：Others  

Venue：Athena   Country：Greece  

Event date： 2009.8

Language：Others   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2009.6

Language：Others   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2009.5

Language：Others   Presentation type：Oral presentation (general)  

Venue：仙台   Country：Japan  

Event date： 2009.4

Language：Others   Presentation type：Oral presentation (general)  

Venue：Seou   Country：Korea, Republic of  

Event date： 2008.11

Language：Others   Presentation type：Oral presentation (general)  

Venue：神戸   Country：Japan  

Event date： 2008.8

Language：Others   Presentation type：Symposium, workshop panel (public)  

Venue：札幌   Country：Japan  

Event date： 2008.6

Language：Others   Presentation type：Symposium, workshop panel (public)  

Venue：東京   Country：Japan  

Event date： 2008.4

Language：Others   Presentation type：Symposium, workshop panel (public)  

Venue：Chicago   Country：United States  

Language：Others   Presentation type：Oral presentation (general)  

Venue：宇都宮   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：東京   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：金沢   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：横浜   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：名古屋   Country：Japan  

Event date： 2024.7 - 2023.7

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：WEB 福岡市   Country：Japan  

Event date： 2024.4

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：TKP ガーデンシティお茶の水   Country：Japan  

Event date： 2024.3

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：神戸国際会館   Country：Japan  

Event date： 2024.3

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：徳島　あわぎんホール   Country：Japan  

Event date： 2024.2

Language：Japanese   Presentation type：Symposium, workshop panel (public)  

Venue：ニューオータニイン札幌   Country：Japan  

Event date： 2024.1

Language：Japanese  

Venue：WEB（獨協医科大学）   Country：Japan  

Event date： 2023.12

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2023.12

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：ＪＲ博多シティ会議室, WEB ハイブリット   Country：Japan  

Event date： 2023.11 - 2023.12

Language：Japanese  

Venue：福岡国際会議場   Country：Japan  

Event date： 2023.11 - 2023.12

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：福岡国際会議場   Country：Japan  

Event date： 2023.11 - 2023.12

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：福岡国際会議場   Country：Japan  

Event date： 2023.11 - 2023.12

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：福岡国際会議場   Country：Japan  

Event date： 2023.10

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：パシフィコ横浜   Country：Japan  

Event date： 2023.10

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：京王プラザホテル   Country：Japan  

Event date： 2023.10

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：京王プラザホテル   Country：Japan  

Event date： 2023.9

Language：English   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Venue：Karolinska Institute   Country：Sweden  

Event date： 2023.8

Language：Japanese   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Venue：グランド日航台場   Country：Japan  

Event date： 2023.8

Language：Japanese   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Venue：京都大学紫蘭会館   Country：Japan  

Event date： 2023.8

Language：English   Presentation type：Oral presentation (general)  

Venue：仙台国際センター   Country：Japan  

The 46th Annual Meeting of the Japan Neuroscience Society

Event date： 2023.6

Language：Japanese  

Venue：ライトキューブ宇都宮   Country：Japan  

Event date： 2023.5 - 2023.6

Language：Japanese  

Venue：幕張メッセ国際会議場   Country：Japan  

Event date： 2023.5 - 2023.6

Language：Japanese  

Venue：幕張メッセ国際会議場   Country：Japan  

Event date： 2023.5 - 2023.6

Language：English  

Venue：幕張メッセ国際会議場   Country：Japan  

Event date： 2023.4

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：WEB 福岡市   Country：Japan  

Event date： 2023.4

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2023.1

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2023.1

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：山口   Country：Japan  

Event date： 2021.6

Language：English  

Venue：WEB   Country：Ireland  

Event date： 2021.5

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：国立京都国際会館 WEB   Country：Japan  

Event date： 2021.5

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：国立京都国際会館   Country：Japan  

Event date： 2021.3

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：九州大学医学部 薬学部第一講堂   Country：Japan  

Event date： 2021.2

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：WEB   Country：Japan  

Event date： 2021.1

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：WEB   Country：Japan  

Event date： 2020.11

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：国立京都国際会館   Country：Japan  

Event date： 2020.7

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：WEB   Country：Japan  

Event date： 2019.11

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：ザ・セレクトン福島   Country：Japan  

Event date： 2019.10 - 2019.11

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：神戸国際会議場   Country：Japan  

Event date： 2019.3

Language：Japanese   Presentation type：Oral presentation (general)  

Venue：福岡大学病院 メディカルホール   Country：Japan  

Event date： 2013.11

Language：Japanese   Presentation type：Symposium, workshop panel (public)  

Venue：高知   Country：Japan  

Event date： 2013.10

Language：Japanese   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Venue：北九州   Country：Japan  

Event date： 2010.11

Language：Others  

Venue：神戸   Country：Japan  

Event date： 2010.10

Language：Others   Presentation type：Oral presentation (general)  

Venue：岡山   Country：Japan  

Event date： 2010.10

Language：Others  

Venue：岡山   Country：Japan  

Event date： 2010.10

Language：Others  

Venue：岡山   Country：Japan  

Event date： 2010.5 - 2011.5

Language：Others   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Venue：九州医療センター   Country：Japan  

Event date： 2010.5

Language：Others  

Venue：東京国際フォーラム   Country：Japan  

Event date： 2010.4

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡センタービル   Country：Japan  

Event date： 2010.2

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Event date： 2010.2

Language：Others  

Venue：福岡   Country：Japan  

Event date： 2009.2

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Event date： 2008.10

Language：Others   Presentation type：Oral presentation (general)  

Venue：東京   Country：Japan  

Event date： 2008.6

Language：Others   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2008.5

Language：Others   Presentation type：Oral presentation (general)  

Venue：横浜   Country：Japan  

Language：Others   Presentation type：Symposium, workshop panel (public)  

Venue：Boston, MA   Country：United States  

Language：Others  

Venue：熊本   Country：Japan  

Language：Others  

Venue：熊本   Country：Japan  

Language：Others  

Venue：東京   Country：Japan  

Language：Others   Presentation type：Symposium, workshop panel (public)  

Venue：宮崎   Country：Japan  

Language：Others  

Venue：静岡   Country：Japan  

Language：Others  

Venue：福岡   Country：Japan  

Language：Others  

Venue：東京   Country：Japan  

Language：Others  

Venue：東京   Country：Japan  

Language：Others  

Venue：鹿児島   Country：Japan  

Language：Others  

Venue：鹿児島   Country：Japan  

Language：Others  

Venue：旭川   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Language：Others  

Venue：東京   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：東京   Country：Japan  

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Venue：福岡   Country：Japan  

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Venue：横浜   Country：Japan  

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Venue：福岡   Country：Japan  

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Venue：東京   Country：Japan  

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Venue：金沢   Country：Japan  

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Venue：三重   Country：Japan  

Language：Others   Presentation type：Public lecture, seminar, tutorial, course, or other speech  

Venue：福岡   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

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Venue：福岡   Country：Japan  

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Venue：福岡   Country：Japan  

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Country：Japan  

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Venue：福岡   Country：Japan  

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Venue：佐賀   Country：Japan  

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Venue：福岡   Country：Japan  

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Venue：福岡   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

Language：Others   Presentation type：Oral presentation (general)  

Venue：福岡   Country：Japan  

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Venue：福岡   Country：Japan  

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症例は24歳の男性。夜間飲酒した翌朝に痙攣を認め当院救急部に搬送された。到着後に痙攣重積を呈して人工呼吸器管理となった。脳幹反射の異常や病的反射、髄膜刺激徴候は認めなかった。頭部MRIの拡散強調画像で異常信号はなく、左後頭葉に陳旧性梗塞様の所見を認めた。入院直後より横紋筋融解症による高CK血症と急性腎不全を呈し持続血液透析濾過法を開始した。髄液中L/P比の著明な増加よりミトコンドリア病を疑い、末梢血にてミトコンドリアDNAのA3243G変異(ヘテロプラスミー20&#37;)が判明しmitochondrial myopathy、encephalopathy、lactic acidosis、and stroke-like episodes(MELAS)と診断した。本症例はMELASとしては非典型的な経過を辿ったため貴重な症例と考えられた。(著者抄録)

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Country：Japan  

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A case of paroxysmal sympathetic storm after acute disseminated encephalomyelitis and hypoxic encephalopathy responding to clonidine hydrochloride

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Monaural auditory stimulation can be useful for identifying epileptic focus in patients with mesial temporal lobe epilepsy: MEG studies.

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Country：Japan  

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Transient strial MRI abnormality ipsilateral to the frontal disconnection surgery

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Country：Japan  

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EEG and long-term video EEG as clinical tools and research topics in the field of neurology

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Language related brain region～From an epileptologist’s point of view
Study of the localization of brain language areas started with findings obtained from patients with brain injury, however functional localization studies in epileptology began with findings on brain functional mapping using cortical electrical stimulation during brain surgery. Knowledge of language area identification and knowledge of language sequelae due to brain resection is essential for those engaged in epilepsy surgery. In recent years, many non-invasive examination and analysis methods have been developed, and it has become possible to carry out measurements on not only pathological brains such as epilepsy and cerebrovascular disease but also normal brains. In this educational lecture, I will introduce the language areas related to epilepsy treatment and actual brain functional mapping. In addition, recent findings on the language areas that are becoming clear by use of various examinations and analysis methods will be discussed, that is, the language areas related to vision, the language areas related to hearing, and the white matter tracts that connect cortical language areas. These language areas will be discussed together with some cases.

Language：Japanese  

Country：Japan  

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臨床神経生理学会が主催し,臨床脳波の専門的知識・技術習得を目的とした脳波セミナー・アドバンスコースは2020年で5回を数えた。この経験を踏まえ,本邦の臨床神経生理学の臨床・教育・研究の発展に係る問題点と方向性をまとめた。未だ臨床実用段階ではないが,今後,臨床脳波は情報通信技術とAI(artificial intelligence)の発展・活用が期待される。同時に,脳波判読にかかる各科の医師,技師の養成を継続しつつ,脳波報告書の解釈の標準化や,ICU(intensive care unit)での長時間脳波モニタを含む脳波保険点数加算の要望など,脳波学を一般臨床へ還元できる体制を整えていく必要がある。脳波教育・研究の発展に関し,国内外の関連学会と協力する余地がある。そして,基礎と実践(ハンズオン)の教育機会の均てん化のため,Webの活用や各地域で講師育成を行って最適化する必要がある。また,生涯教育,ICU専従医師などさまざまなキャリアを持つ医療者向けに教育対象を広げるのが望ましい。(著者抄録)

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Country：Japan  

Investigation of structural MRI features in incomplete hippocampal inversion

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Country：Japan  

Automatic estimation of epileptogenic zone using convolutional neural network on chronic subdural electrocorticogram recordings

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Country：Japan  

”de novo psychosis” following epilepsy surgery: three case reports

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Country：Japan  

Detection of ictal hyperperfusion during non-convulsive status epileptics using magnetic resonance perfusion imaging with 1.5-Tesla pulsed arterial spin labeling

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Country：Japan  

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Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)  

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EEG and long-term video EEG as clinical tools and research topics in the field of neurology
EEG can detect brain dysfunction which is difficult to be detected by neuroimages and blood tests, therefore it is effective in diagnosing epilepsy, sleep medicine, and disturbance of consciousness. It is clinically significant that EEG makes it possible to investigate the relationship between symptoms and EEG by recording videos simultaneously. Long-term video EEG monitoring is useful to detect abnormalities that could not be extracted by short-time conventional EEG recording. Due to development of digital technologies and higher performance amplifiers, EEG became possible to record high-frequency and low-frequency EEG activities with a long-time scale, and several new knowledges have been obtained in the research fields. New research fields have been expanding; such as utilizing EEG as long-term biological signal sensor, introduction of machine learning for EEG data analysis, and trial use as a telemedicine and educational tool. The usefulness of EEG in the field of neurology and several examples of EEG/long-term video EEG monitoring as promising research tools will be introduced in this section.

DOI： 10.11422/jscn.49.152

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痙攣

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DOI： 10.11422/jscn.48.81

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症例は53歳女性。原発性胆汁性肝硬変にて入院中に, 1分間程度の焦点運動発作の後から, 高度意識障害が遷延し, 脳波で一側性周期性放電 (lateralized periodic discharges: LPDs) を呈した。持続脳波モニタリングを行いながら, ミダゾラムと抗てんかん薬による治療を行い, LPDsは消失したが意識障害は改善しなかった。ミダゾラム中止後, 次第にLPDsに代わって, 三相性波形 (triphasic morphology) を伴う全般性周期性放電 (generalized periodic discharges: GPDs) が顕在化し, その解釈が治療上の問題となった。形態的特徴や, 刺激に対する反応性, 臨床情報などから, LPDsは非けいれん性重積状態に伴う周期性放電, GPDsは肝性脳症に伴う三相波と考えられた。両者の鑑別はしばしば問題となるが, 相違点を理解する上で示唆に富む症例であった。

DOI： 10.11422/jscn.48.102

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