Shizuoka Institute of Epilepsy and Neurological Disorders

Shizuoka-shi, Japan

Shizuoka Institute of Epilepsy and Neurological Disorders

Shizuoka-shi, Japan
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Sato W.,Kyoto University | Kochiyama T.,Kyoto University | Uono S.,Kyoto University | Matsuda K.,Shizuoka Institute of Epilepsy and Neurological Disorders | And 3 more authors.
Journal of Cognitive Neuroscience | Year: 2012

Neuroimaging studies have reported greater activation of the human amygdala in response to faces than to nonfacial stimuli, yet little is known about the temporal profile of this activation. We investigated this issue by recording the intracranial field potentials of the amygdala in participants undergoing preneurosurgical assessment (n = 6). Participants observed faces, mosaics, and houses in upright and inverted orientations using a dummy target detection task. Time-frequency statistical parametric mapping analyses revealed that the amygdala showed greater gamma-band activity in response to faces than to mosaics at 200-300 msec, with a peak at 255 msec. Gamma-band activation with a similar temporal profile was also found in response to faces versus houses. Activation patterns did not differ between upright and inverted presentations of stimuli. These results suggest that the human amygdala is involved in the early stages of face processing, including the modulation of subjective perception of faces. © 2012 Massachusetts Institute of Technology.


Yamamoto Y.,Shizuoka Institute of Epilepsy and Neurological Disorders | Yamamoto Y.,University of Shizuoka | Inoue Y.,Shizuoka Institute of Epilepsy and Neurological Disorders | Matsuda K.,Shizuoka Institute of Epilepsy and Neurological Disorders | And 2 more authors.
Biological and Pharmaceutical Bulletin | Year: 2012

Lamotrigine (LTG) is an antiepileptic drug (AED) that was approved in Japan in 2008. We evaluated the influence of AEDs that induce hepatic enzymes (including phenytoin (PHT), phenobarbital (PB), carbamazepine (CBZ)), valproic acid (VPA), and various combinations of these drugs, on plasma LTG concentration in adult Japanese epilepsy patients. A total of 621 patients (mean age 34.4±11.8 years) were evaluated retrospectively. We calculated the concentration to dose ratio (CD ratio) for LTG with different AED regimens, and employed multiple regression analysis to determine factors influencing the LTG concentration. There was a linear correlation between the dose and concentration of LTG in patients treated with LTG (group I), LTG+VPA (group II), LTG+inducers (group III), or LTG+VPA+inducers (group IV). The mean CD ratio of patients on LTG monotherapy was 1.43±0.4 (μg/mL)/(mg/kg). When LTG was combined with VPA, the CD ratio increased about 2.2-fold, but there was no significant correlation between the CD ratio and VPA concentration. The mean CD ratios calculated in patients receiving LTG+PHT, LTG+PB, and LTG+CBZ were 0.56, 0.84, and 0.91, respectively. Addition of PHT significantly reduced the CD ratio in a concentration-dependent manner, in comparison with PB and CBZ (p<0.005 and p<0.001, respectively). Stepwise multiple regression analysis showed that the coefficient of determination of groups I, II, III, and IV were 0.94, 0.94, 0.90, and 0.91, respectively. In the clinical setting, these findings can help to estimate LTG concentrations and predict the inducing or inhibiting effects of concomitant AEDs. © 2012 The Pharmaceutical Society of Japan.


Sato W.,Kyoto University | Kochiyama T.,Japan Advanced Telecommunications Research Institute International | Uono S.,Kyoto University | Matsuda K.,Shizuoka Institute of Epilepsy and Neurological Disorders | And 3 more authors.
Neuropsychologia | Year: 2011

Neuroimaging studies have reported greater activation of the human amygdala in response to emotional facial expressions, especially for fear. However, little is known about how fast this activation occurs. We investigated this issue by recording the intracranial field potentials of the amygdala in subjects undergoing pre-neurosurgical assessment (n= 6). The subjects observed fearful, happy, and neutral facial expressions. Time-frequency statistical parametric mapping analyses revealed that the amygdala showed greater gamma-band activity in response to fearful compared with neutral facial expressions at 50-150. ms, with a peak at 135. ms. These results indicate that the human amygdala is able to rapidly process fearful facial expressions. © 2010 Elsevier Ltd.


Hirozawa D.,Shizuoka Institute of Epilepsy and Neurological Disorders
Journal of Clinical Neurophysiology | Year: 2017

PURPOSE:: Focal cortical dysplasia (FCD) is intrinsically epileptogenic, and an MRI-visible lesion typically constitutes the core part of the epileptogenic zone. We aimed to identify ictal EEG patterns that represent the epileptogenic zone by using subdural electrodes placed over the MRI-visible FCD lesion. METHODS:: We selected seven patients with frontal lobe epilepsy caused by pathologically proven FCD type IIb who underwent preoperative intracranial EEG evaluation with subdural electrodes followed by resection surgery with seizure-free outcome. The characteristics of ictal EEG patterns, interictal/ictal high-frequency oscillations, and ictal direct current shifts from intracranial electrodes placed over the MRI-visible lesion were analyzed. RESULTS:: Two seizure-onset patterns (low voltage fast activity and fast spike activity) were identified. Low voltage fast activity was seen in all patients with a lateral frontal lesion, and it was always preceded by preictal spikes. Fast spike activity occurred only in patients with a mesial frontal lesion. Interictal/ictal high-frequency oscillations and ictal direct current shifts were seen in all patients. CONCLUSIONS:: The epileptogenic zone of frontal FCD type IIb may be characterized by EEG seizure-onset patterns consisting of low voltage fast activity and fast spike activity accompanied by ictal high-frequency oscillations and ictal direct current shifts. Further study is needed to determine whether other seizure-onset patterns exist in patients with FCD type IIb. © 2017 by the American Clinical Neurophysiology Society


Nonaka T.,Tokyo Metropolitan Institute of Medical Science | Masuda-Suzukake M.,Tokyo Metropolitan Institute of Medical Science | Arai T.,Tokyo Metropolitan Institute of Medical Science | Arai T.,University of Tsukuba | And 8 more authors.
Cell Reports | Year: 2013

TDP-43 is the major component protein of ubiquitin-positive inclusions in brains of patients with frontotemporal lobar degeneration (FTLD-TDP) or amyotrophic lateral sclerosis (ALS). Here, we report the characterization of prion-like properties of aggregated TDP-43 prepared from diseased brains. When insoluble TDP-43 from ALS or FTLD-TDP brains was introduced as seeds into SH-SY5Y cells expressing TDP-43, phosphorylated and ubiquitinated TDP-43 was aggregated in a self-templating manner. Immunoblot analyses revealed that the C-terminal fragments of insoluble TDP-43 characteristic of each disease type acted as seeds, inducing seed-dependent aggregation of TDP-43 in these cells. The seeding ability of insoluble TDP-43 was unaffected by proteinase treatment but was abrogated by formic acid. One subtype of TDP-43 aggregate was resistant to boiling treatment. The insoluble fraction from cells harboring TDP-43 aggregates could also trigger intracellular TDP-43 aggregation. These results indicate that insoluble TDP-43 has prion-like properties that may play a role in the progression of TDP-43 proteinopathy. © 2013 The Authors.


Shimojima K.,Tokyo Women's Medical University | Imai K.,Shizuoka Institute of Epilepsy and Neurological Disorders | Yamamoto T.,Tokyo Women's Medical University
American Journal of Medical Genetics, Part A | Year: 2010

The recent development of high-throughput analysis for genomic copy numbers has enabled to identify microscopic chromosomal duplications that had never been recognized before. Microarray-based comparative genomic hybridization (aCGH) identified a de novo 2.1-Mb microduplication in the 22q11.22q11.23 region surrounded by low copy repeats (LCRs) LCR22E and LCR22H in a 5-year-old boy with developmental delay, hyperactivity, epilepsy, and distinctive facial features, which were within the wide range of the clinical manifestations of the patients with the same duplication pattern. Fiber-fluorescent in situ hybridization (FISH) analysis confirmed that the duplicated segments were aligned in a tandem configuration. Familial single nucleotide polymorphism (SNP) typing determined that the duplication was derived from paternal interchromosomal non-allelic homologous recombination (NAHR) during the first meiotic process of spermatogenesis. Although no patient with the deletions of the distal 22q11.2 has been reported as showing epilepsy, at least five patients including the presenting patient having the duplication between LCR22E and LCR22G showed epilepsy. Thus, the gain of the genomic copy number of this region may have epileptogenesis. © 2010 Wiley-Liss, Inc.


Takayama R.,Shizuoka Institute of Epilepsy and Neurological Disorders | Fujiwara T.,Shizuoka Institute of Epilepsy and Neurological Disorders | Shigematsu H.,Shizuoka Institute of Epilepsy and Neurological Disorders | Imai K.,Shizuoka Institute of Epilepsy and Neurological Disorders | And 3 more authors.
Epilepsia | Year: 2014

Objective This study attempted to clarify the long-term course of Dravet syndrome (DS). Methods Sixty-four patients diagnosed with DS (44 with typical DS, and 20 with atypical DS) were studied. The long-term outcomes of clinical seizures, electroencephalographic findings, neuropsychological findings, and social situation were analyzed. The follow-up period ranged from 11 to 34 years 5 months (median 24 years). Results At the last visit, the ages ranged from 19 years to 45 years (median 30 years). Fifty-nine patients continued to have generalized tonic-clonic seizures (GTCS). Status epilepticus and unilateral seizures were not observed and myoclonic seizures, atypical absence seizures, and photosensitive seizures were resolved in most patients. The frequency of complex partial seizures was equally low, with five patients at presentation and six patients at the last visit, respectively. Five patients achieved seizure remission (seizure-free for 1 year or longer). Only 1 of 44 patients with typical DS had seizure remission, whereas 4 of 20 patients with atypical DS remitted, with a statistically significant difference between the two phenotypes (p = 0.03). Intellectual disability was found in all patients; especially, severe intellectual disability was prevalent. Patients with atypical DS tended to have milder intellectual disability compared to those with typical DS (p = 0.0283). Occipital alpha rhythm in the basic activity was associated with milder intellectual disability (p = 0.0085). The freedom from seizures correlated with appearance of occipital alpha rhythms (p = 0.0008) and disappearance of epileptic discharges (p = 0.0004). Two patients with GTCS died. Mutations of the neuronal voltage-gated sodium channel alpha subunit type 1 gene were detected at a high frequency (33 of 36 patients examined). Seizure remission was found only in the missense mutation group. Significance The long-term seizure and intellectual outcomes are extremely poor in patients with typical DS compared to those with atypical DS. Epilepsy phenotype may influence long-term course of DS. © 2014 International League Against Epilepsy.


Miya K.,University of Toyama | Takahashi Y.,Shizuoka Institute of Epilepsy and Neurological Disorders | Mori H.,University of Toyama
Brain and Development | Year: 2014

The N-methyl- d-aspartate receptor (NMDAR) is involved in normal physiological and pathological states in the brain. Anti-NMDAR encephalitis is characterized by memory deficits, seizures, confusion, and psychological disturbances in males and females of all ages. This type of encephalitis is often associated with ovarian teratoma in young women, but children are less likely to have tumors. Anti-NMDAR encephalitis is a neuroimmune syndrome in patients with autoantibodies recognizing extracellular epitopes of NMDAR, and the autoantibodies attenuate NMDAR function through the internalization of NMDAR. Following the initial symptoms of inflammation, the patients show the various symptoms such as memory loss, confusion, emotional disturbances, psychosis, dyskinesis, decrease in speech intelligibility, and seizures. About half of these patients improved with immunotherapy including high-dose intravenous corticosteroids and intravenous immunoglobulins is administrated to these patients, but the patients who had no improvement with these therapy require further treatments with rituximab or cyclophosphamide. It is necessary to detect anti-NMDAR antibodies at early stages, because the prognosis of these patients may be improved by early treatment. Recovery is slow, and the patients may have some disturbances in their motor function and cognition. The pathologic mechanism underlying the development of anti-NMDAR encephalitis has been elucidated gradually, but the optimal treatment has not yet been clarified. Further studies are required to clarify in detail the mechanism underlying anti-NMDA encephalitis and to develop effective treatments. © 2013 The Japanese Society of Child Neurology.


Usui N.,Shizuoka Institute of Epilepsy and Neurological Disorders
Neurologia Medico-Chirurgica | Year: 2016

This article reviews the current topics in the field of epilepsy surgery. Each type of epilepsy is associated with a different set of questions and goals. In mesial temporal lobe epilepsy (MTLE) with hippocampal sclerosis (HS), postoperative seizure outcome is satisfactory. A recent meta-analysis revealed superior seizure outcome after anterior temporal lobectomy compared with selective amygdalohippocampectomy; in terms of cognitive outcome; however, amygdalohippocampectomy may be beneficial. In temporal lobe epilepsy with normal magnetic resonance imaging (MRI), postoperative seizure outcome is not as favorable as it is in MTLE with HS; further improvement of seizure outcome in these cases is necessary. Focal cortical dysplasia is the most common substrate in intractable neocortical epilepsy, especially in children, as well as in MRI-invisible neocortical epilepsy. Postoperative seizure-free outcome is approximately 60-70%; further diagnostic and therapeutic improvement is required. Regarding diagnostic methodology, an important topic currently under discussion is wideband electroencephalogram (EEG) analysis. Although high-frequency oscillations and ictal direct current shifts are considered important markers of epileptogenic zones, the clinical significance of these findings should be clarified further. Regarding alternatives to surgery, neuromodulation therapy can be an option for patients who are not amenable to resective surgery. In addition to vagus nerve stimulation, intracranial stimulation such as responsive neurostimulation or anterior thalamic stimulation is reported to have a modest seizure suppression effect. Postoperative management such as rehabilitation and antiepileptic drug (AED) management is important. It has been reported that postoperative rehabilitation improves postoperative employment status. Pre- and post-operative comprehensive care is mandatory for postoperative improvement of quality of life. © 2016, Japan Neurosurgical Society. All rights reserved.


Kubota H.,Shizuoka Institute of Epilepsy and Neurological Disorders | Awaya Y.,Seibo International Catholic Hospital
Epilepsy and Behavior | Year: 2010

The aim of epilepsy treatment is not only to eliminate seizures, but also to improve health-related quality of life (HRQOL). We conducted a postal self-administered survey of HRQOL for Japanese patients using the Quality of Life in Epilepsy inventory (QOLIE-31), Version 1.0, and analyzed factors influencing their quality of life (QOL). Data from 599 analyzable patients were evaluated and a number of factors influencing QOL were identified, including severity and frequency of seizures, seizure control, type of epilepsy, contributing events such as injuries and falls during seizures, number of antiepileptic drugs, employment status, and surgical outcome. These findings suggest that comprehensive management of the patient should be emphasized. Consideration of all the results led to classification of these factors as one of two types: "all or nothing" and "linear." With respect to "all or nothing" factors (e.g. "daytime remarkable seizures"), patients may not be able to improve their QOL unless these factors can be completely controlled. Comparison of each score on the QOLIE-31 subscales with published data revealed that the scores for the subscale Medication Effects were markedly low. © 2010 Elsevier Inc.

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