Shizuoka Institute of Epilepsy and Neurological Disorders

Shizuoka-shi, Japan

Shizuoka Institute of Epilepsy and Neurological Disorders

Shizuoka-shi, Japan

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Wrench J.M.,University of Melbourne | Matsumoto R.,Kyoto University | Inoue Y.,Shizuoka Institute of Epilepsy and Neurological Disorders | Wilson S.J.,University of Melbourne | Wilson S.J.,Austin Health
Epilepsy and Behavior | Year: 2011

The accurate prediction of individual outcomes after epilepsy surgery represents a key challenge facing clinicians. It requires a precise understanding of surgical candidacy and the optimal timing of surgery to maximize a range of outcomes, including medical, psychosocial, cognitive, and psychiatric outcomes. We promote careful consideration of how epilepsy has affected an individual's developmental trajectory as key to constructing more differentiated profiles of postsurgical risk or resilience across multiple outcome measures. This life span approach conceives surgery as a crucial "turning point" in an individual's development from which varied outcome trajectories may follow. This helps clinicians understand the expectations patients and families bring to surgery, and emphasizes the interplay of factors that determine a patient's outcome. It also promotes comprehensive, longitudinal assessment of outcome using data analytical techniques that capture individual differences and identify subgroups with similar trajectories. An ongoing challenge facing clinicians is the development of an outcome classification system that incorporates outcomes other than seizures. We illustrate two emerging areas of research shaping how we define surgical candidacy and predict outcome: (1) using cortico-cortical evoked potentials to identify pathways of seizure propagation and cortico-cortical networks mediating cortical functions, and (2) predicting postoperative depression using a model that incorporates psychosocial and neurobiological factors. The latter research points to the importance of routine follow-up and postoperative psychosocial rehabilitation, particularly in patients deemed at "high risk" for poor outcomes so that early treatment interventions can be implemented. Significantly more research is needed to characterize those patients with poor outcomes who may require re-surgery. © 2011 Elsevier Inc.


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.


Fukao K.,Shizuoka Institute of Epilepsy and Neurological Disorders | Inoue Y.,Shizuoka Institute of Epilepsy and Neurological Disorders | Yagi K.,Shizuoka Institute of Epilepsy and Neurological Disorders
Epilepsia | Year: 2010

Purpose: To estimate magnetoencephalography (MEG) correlates of different types of aura in temporal lobe epilepsy (TLE). Methods: MEG study was performed on 57 patients (26 male and 31 female) with TLE, whose ages ranged from 14-46 years (mean 27 years). Interictal magnetoencephalograms showing discharges were analyzed, and spike-dipole clusters were categorized into left and right inferotemporal-horizontal (IH) and superotemporal-vertical (SV) types. Auras were classified into autonomic, auditory, and psychic seizures. The correlation between the four types of interictal spike-dipole and three types of aura was analyzed using Fisher's exact probability test. Results: IH type correlated with autonomic seizures (p = 0.0004), whereas SV type correlated with both auditory (p = 0.0002) and psychic seizures (p = 0.042). When subdivided into left and right, left IH type correlated with autonomic seizures (p = 0.046), but right IH type did not. Right SV type correlated with both auditory (p = 0.014) and psychic seizures (p = 0.002), but left SV did not correlate with either. Both types did not correlate with "no aura." Conclusions: Using our proposed classification of spike-dipoles, MEG distinguishes auras of mesial temporal origin from those of lateral temporal region. Furthermore, by adopting our classification, laterality of spike-dipoles is clearly demonstrated in auditory and psychic seizures. © 2010 International League Against Epilepsy.


Sato W.,Kyoto University | Kochiyama T.,Kyoto University | Uono S.,Kyoto University | Matsuda K.,Shizuoka Institute of Epilepsy and Neurological Disorders | And 4 more authors.
Cortex | Year: 2014

Neuroimaging studies have found greater activation in the inferior occipital gyrus (IOG), or occipital face area, in response to faces relative to non-facial stimuli. However, the temporal, frequency, and functional profiles of IOG activity during face processing remain unclear. Here, this issue was investigated by recording intracranial field potentials in the IOG during the presentation of faces, mosaics, and houses in upright and inverted orientations. Time-frequency statistical parametric mapping analyses revealed greater gamma-band activation in the IOG beginning at 110msec and covering 40-300Hz in response to upright faces relative to upright houses and mosaics. Phase-amplitude cross-frequency coupling analyses revealed more evident theta-gamma couplings at 115-256msec during the processing of upright faces as compared with that of upright houses and mosaics. Comparable gamma-band activity was observed during the processing of inverted and upright faces at about 100-200msec, but weaker activity and different coupling with theta-band activity after 200msec. These patterns of activity were more evident in the right than in the left IOG. These results, together with other evidence on neural communication, suggest that broadband gamma oscillations in the right IOG conduct rapid and multistage (i.e., both featural and configural) face processing in collaboration with theta oscillations transmitted from other brain regions. © 2014 Elsevier Ltd.


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.


Usui N.,Shizuoka Institute of Epilepsy and Neurological Disorders | Terada K.,Shizuoka Institute of Epilepsy and Neurological Disorders | Baba K.,Shizuoka Institute of Epilepsy and Neurological Disorders | Matsuda K.,Shizuoka Institute of Epilepsy and Neurological Disorders | And 4 more authors.
Annals of Neurology | Year: 2015

Objective We previously reported ictal very-high-frequency oscillations (VHFO) of 1,000 to 2,500Hz recorded by subdural macroelectrodes using a 10-kHz sampling rate. The purpose of this study was to clarify the clinical significance of ictal VHFO in neocortical epilepsy. Methods This study included 13 patients with neocortical epilepsy who underwent subdural electrode implantation and had at least 1 seizure recorded at a 10-kHz sampling rate and were followed for more than 2 years postoperatively. Extent of resection was determined considering the seizure onset zone (SOZ) and irritative zone, structural lesion, and functional areas. Areas showing VHFO and those with HFO were not taken into consideration. The presence or absence of VHFO (>1,000Hz), HFO (200-1,000Hz) and SOZ, and completeness of resection of these areas were compared with postoperative seizure outcome. Results Seven patients had favorable (Engel class Ia) and 6 had unfavorable outcomes (other classes). VHFO was recorded in 6 of 7 patients with a favorable outcome. On the contrary, VHFO was recorded in only 1 of 6 patients with unfavorable outcome. The presence of VHFO was significantly associated with favorable outcome. VHFO was recorded on a limited number of electrodes, and VHFO-generating areas were resected completely, whereas HFO-generating areas and/or SOZ were not always resected completely in both favorable and unfavorable outcome groups. Interpretation The presence of ictal VHFO may be predictive of favorable outcome. Ictal VHFO may be a more specific marker than ictal HFO or SOZ for identifying the core of epileptogenic zone. © 2015 American Neurological Association.

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