研究者データベース

白石 秀明(シライシ ヒデアキ)
北海道大学病院 小児・周産・女性科
講師

基本情報

所属

  • 北海道大学病院 小児・周産・女性科

職名

  • 講師

学位

  • 医学博士(北海道大学)

ホームページURL

J-Global ID

研究キーワード

  • てんかん   脳磁図   小児   てんかん症候群   空間フィルター法   環境化学物質   Angelmen症候群   神経発達   リスク評価   精神遅滞   母子保健   環境遺伝交互作用   ゲノム刷り込み現象   Prader-Willi症候群   次世代影響   

研究分野

  • ライフサイエンス / 胎児医学、小児成育学
  • ライフサイエンス / 医療管理学、医療系社会学
  • ライフサイエンス / 衛生学、公衆衛生学分野:実験系を含まない
  • ライフサイエンス / 衛生学、公衆衛生学分野:実験系を含む
  • ライフサイエンス / 衛生学、公衆衛生学分野:実験系を含まない
  • ライフサイエンス / 衛生学、公衆衛生学分野:実験系を含む
  • ライフサイエンス / 医療管理学、医療系社会学

職歴

  • 2020年06月 - 現在 北海道大学病院 小児科
  • 2015年10月 - 現在 北海道大学病院 小児科 講師
  • 2015年08月 - 現在 北海道大学病院 てんかんセンター 副部長
  • 2008年10月 - 2015年09月 北海道大学 大学病院 助教

学歴

  • 1986年04月 - 1992年03月   北海道大学   医学部   医学科

研究活動情報

論文

  • Tohru Okanishi, Yuji Fujii, Satoru Sakuma, Hideaki Shiraishi, Hirotaka Motoi, Kotaro Yazaki, Hideo Enoki, Ayataka Fujimoto
    Brain & development 44 6 380 - 385 2022年06月 
    OBJECTIVE: Childhood epilepsy with centrotemporal spikes (CECTS) is known as age-limited focal epilepsy syndrome in childhood. Lacosamide is a third-generation antiepileptic drug. This study aimed to evaluate the efficacy of lacosamide monotherapy for the treatment of CECTS. METHODS: We enrolled 18 patients (6 girls and 12 boys) who met the following criteria: 1) the age of onset of the seizures was between 3 and 13 years of age; 2) showing at least hemifacial and/or oropharyngeal seizures; 3) interictal discharges in central and/or middle temporal electrodes; 4) no intellectual disability; 5) treatment duration of lacosamide monotherapy over 6 months. We retrospectively collected and analyzed clinical data and treatment information. We evaluated the seizure occurrences during 0-3, 4-6, and 7-12 months from the treatment initiation and the last 6 months of the follow-up. We also evaluated the outcomes as seizure-free if the patients developed no seizures both over 6 months and 3 times of pretreatment mean seizure interval at the last follow-up. RESULTS: Of the patients, 39%, 67% and 72% were seizure-free during 0-3, 4-6, and 7-12 months from treatment initiation, respectively. Finally, 83% of the patients achieved seizure freedom. Seizure freedom was achieved in 72% during the first 4 months of treatment. All patients continued lacosamide monotherapy during the study, although four patients showed transient fatigue or somnolence. CONCLUSIONS: Lacosamide showed good efficacy for controlling seizures with fewer adverse effects, and therefore may be a good candidate as a first-line medication for the treatment of new-onset CECTS.
  • Naoto Kuroda, Takafumi Kubota, Toru Horinouchi, Naoki Ikegaya, Yu Kitazawa, Satoshi Kodama, Izumi Kuramochi, Teppei Matsubara, Naoto Nagino, Shuichiro Neshige, Temma Soga, Yutaro Takayama, Daichi Sone, Kousuke Kanemoto, Akio Ikeda, Kiyohito Terada, Hiroko Goji, Shinji Ohara, Koichi Hagiwara, Takashi Kamada, Koji Iida, Nobutsune Ishikawa, Hideaki Shiraishi, Osato Iwata, Hidenori Sugano, Yasushi Iimura, Takuichiro Higashi, Hiroshi Hosoyama, Ryosuke Hanaya, Akihiro Shimotake, Takayuki Kikuchi, Takeshi Yoshida, Hiroshi Shigeto, Jun Yokoyama, Takahiko Mukaino, Masaaki Kato, Masanori Sekimoto, Masahiro Mizobuchi, Yoko Aburakawa, Masaki Iwasaki, Eiji Nakagawa, Tomohiro Iwata, Kentaro Tokumoto, Takuji Nishida, Yukitoshi Takahashi, Kenjiro Kikuchi, Ryuki Matsuura, Shin-Ichiro Hamano, Ayataka Fujimoto, Hideo Enoki, Kyoichi Tomoto, Masako Watanabe, Youji Takubo, Toshihiko Fukuchi, Hidetoshi Nakamoto, Yuichi Kubota, Naoto Kunii, Yuichiro Shirota, Eiichi Ishikawa, Nobukazu Nakasato, Taketoshi Maehara, Motoki Inaji, Shunsuke Takagi, Takashi Enokizono, Yosuke Masuda, Takahiro Hayashi
    Epilepsia open 2022年05月28日 
    OBJECTIVE: The impact of the coronavirus disease 2019 (COVID-19) pandemic on epilepsy care across Japan was investigated by conducting a multicenter retrospective cohort study. METHODS: This study included monthly data on the frequency of (1) visits by outpatients with epilepsy, (2) outpatient electroencephalography (EEG) studies, (3) telemedicine for epilepsy, (4) admissions for epilepsy, (5) EEG monitoring, and (6) epilepsy surgery in epilepsy centers and clinics across Japan between January 2019 and December 2020. We defined the primary outcome as epilepsy-center-specific monthly data divided by the 12-month average in 2019 for each facility. We determined whether the COVID-19 pandemic-related factors (such as year [2019 or 2020], COVID-19 cases in each prefecture in the previous month, and the state of emergency) were independently associated with these outcomes. RESULTS: In 2020, the frequency of outpatient EEG studies (-10.7%, p<0.001) and cases with telemedicine (+2,608%, p=0.031) were affected. The number of COVID-19 cases was an independent associated factor for epilepsy admission (-3.75*10-3 % per case, p<0.001) and EEG monitoring (-3.81*10-3 % per case, p = 0.004). Further, the state of emergency was an independent factor associated with outpatient with epilepsy (-11.9%, p<0.001), outpatient EEG (-32.3%, p<0.001), telemedicine for epilepsy (+12,915%, p<0.001), epilepsy admissions (-35.3%; p<0.001), EEG monitoring (-24.7%: p<0.001), and epilepsy surgery (-50.3%, p<0.001). SIGNIFICANCE: We demonstrated the significant impact that the COVID-19 pandemic had on epilepsy care. These results support those of previous studies and clarify the effect size of each pandemic-related factor on epilepsy care.
  • Yushi Inoue, Shin-Ichiro Hamano, Masaharu Hayashi, Hiroshi Sakuma, Shinichi Hirose, Atsushi Ishii, Ryoko Honda, Akio Ikeda, Katsumi Imai, Kazutaka Jin, Akiko Kada, Akiyoshi Kakita, Mitsuhiro Kato, Kensuke Kawai, Tamihiro Kawakami, Katsuhiro Kobayashi, Toyojiro Matsuishi, Takeshi Matsuo, Shin Nabatame, Nobuhiko Okamoto, Susumu Ito, Akihisa Okumura, Akiko Saito, Hideaki Shiraishi, Hiroshi Shirozu, Takashi Saito, Hidenori Sugano, Yukitoshi Takahashi, Hitoshi Yamamoto, Tetsuhiro Fukuyama, Ichiro Kuki
    Epileptic disorders : international epilepsy journal with videotape 24 1 82 - 94 2022年02月01日 
    OBJECTIVE: To examine the current medical and psychosocial status of patients with epilepsy, aiming to facilitate appropriate application of the Intractable/Rare Diseases Act of Japan. METHODS: By analysing the cross-sectional data of patients registered in the tertiary hospital-based Epilepsy Syndrome Registry of Japan, we investigated the proportion of patients who met the severity criteria as defined by the Act (seizure frequency of at least once a month, or presence of intellectual/neurological/psychiatric symptoms, or both) and whether there are candidate syndrome/diseases to be added to the existing list in the Act. RESULTS: In total, 2,209 patients were registered. After excluding self-limited/idiopathic epilepsies, 1,851 of 2,110 patients (87.7%) met the severity criteria. The patients were classified into eight main epilepsy syndromes (594 patients), 20 groups based on aetiology (1,078 patients), and three groups without known aetiology (427 patients). Most of the groups classified by syndrome or aetiology had high proportions of patients satisfying the severity criteria (>90%), but some groups had relatively low proportions (<80%) resulting from favourable outcome of surgical therapy. Several small groups with known syndrome/aetiology await detailed analysis based on a sufficiently large enough number of patients registered, some of whom may potentially be added to the list of the Act. SIGNIFICANCE: The registry provides data to examine the usefulness of the severity criteria and list of diseases that are operationally defined by the Act. Most epilepsy patients with various syndromes/diseases and aetiology groups are covered by the Act but some are not, and the list of designated syndromes/diseases should be complemented by further amendments, as suggested by future research.
  • Mitsuhiro Kato, Akiko Kada, Hideaki Shiraishi, Jun Tohyama, Eiji Nakagawa, Yukitoshi Takahashi, Tomoyuki Akiyama, Akiyoshi Kakita, Noriko Miyake, Atsushi Fujita, Akiko M Saito, Yushi Inoue
    Annals of clinical and translational neurology 9 2 181 - 192 2022年02月 
    OBJECTIVE: To determine whether sirolimus, a mechanistic target of rapamycin (mTOR) inhibitor, reduces epileptic seizures associated with focal cortical dysplasia (FCD) type II. METHODS: Sixteen patients (aged 6-57 years) with FCD type II received sirolimus at an initial dose of 1 or 2 mg/day based on body weight (FCDS-01). In 15 patients, the dose was adjusted to achieve target trough ranges of 5-15 ng/mL, followed by a 12-week maintenance therapy period. The primary endpoint was a lower focal seizure frequency during the maintenance therapy period. Further, we also conducted a prospective cohort study (RES-FCD) in which 60 patients with FCD type II were included as an external control group. RESULTS: The focal seizure frequency reduced by 25% in all patients during the maintenance therapy period and by a median value of 17%, 28%, and 23% during the 1-4-, 5-8-, and 9-12-week periods. The response rate was 33%. The focal seizure frequency in the external control group reduced by 0.5%. However, the background characteristics of external and sirolimus-treated groups differed. Adverse events were consistent with those of mTOR inhibitors reported previously. The blood KL-6 level was elevated over time. INTERPRETATION: The reduction of focal seizures did not meet the predetermined level of statistical significance. The safety profile of the drug was tolerable. The potential for a reduction of focal seizures over time merit further investigations.
  • Hayato Watanabe, Atsushi Shimojo, Kazuyori Yagyu, Tsuyoshi Sonehara, Kazuyoshi Takano, Jared Boasen, Hideaki Shiraishi, Koichi Yokosawa, Takuya Saito
    PloS one 17 6 e0270090  2022年 
    Communication is one of the most important abilities in human society, which makes clarification of brain functions that underlie communication of great importance to cognitive neuroscience. To investigate the rapidly changing cortical-level brain activity underlying communication, a hyperscanning system with both high temporal and spatial resolution is extremely desirable. The modality of magnetoencephalography (MEG) would be ideal, but MEG hyperscanning systems suitable for communication studies remain rare. Here, we report the establishment of an MEG hyperscanning system that is optimized for natural, real-time, face-to-face communication between two adults in sitting positions. Two MEG systems, which are installed 500m away from each other, were directly connected with fiber optic cables. The number of intermediate devices was minimized, enabling transmission of trigger and auditory signals with almost no delay (1.95-3.90 μs and 3 ms, respectively). Additionally, video signals were transmitted at the lowest latency ever reported (60-100 ms). We furthermore verified the function of an auditory delay line to synchronize the audio with the video signals. This system is thus optimized for natural face-to-face communication, and additionally, music-based communication which requires higher temporal accuracy is also possible via audio-only transmission. Owing to the high temporal and spatial resolution of MEG, our system offers a unique advantage over existing hyperscanning modalities of EEG, fNIRS, or fMRI. It provides novel neuroscientific methodology to investigate communication and other forms of social interaction, and could potentially aid in the development of novel medications or interventions for communication disorders.
  • Yuki Ueda, Shuta Fujishige, Takeru Goto, Shuhei Kimura, Noriko Namatame, Masashi Narugami, Sachiko Nakakubo, Midori Nakajima, Kiyoshi Egawa, Naoya Kaneko, Kanako Nakayama, Nozomi Hishimura, Takeshi Yamaguchi, Akie Nakamura, Hideaki Shiraishi
    Epilepsia Open 2021年12月14日
  • Naoto Kuroda, Takafumi Kubota, Toru Horinouchi, Naoki Ikegaya, Yu Kitazawa, Satoshi Kodama, Teppei Matsubara, Naoto Nagino, Shuichiro Neshige, Temma Soga, Daichi Sone, Yutaro Takayama, Izumi Kuramochi, Kousuke Kanemoto, Akio Ikeda, Kiyohito Terada, Hiroko Goji, Shinji Ohara, Koichi Hagiwara, Takashi Kamada, Koji Iida, Nobutsune Ishikawa, Hideaki Shiraishi, Osato Iwata, Hidenori Sugano, Yasushi Iimura, Takuichiro Higashi, Hiroshi Hosoyama, Ryosuke Hanaya, Akihiro Shimotake, Takayuki Kikuchi, Takeshi Yoshida, Hiroshi Shigeto, Jun Yokoyama, Takahiko Mukaino, Masaaki Kato, Masanori Sekimoto, Masahiro Mizobuchi, Yoko Aburakawa, Masaki Iwasaki, Eiji Nakagawa, Tomohiro Iwata, Kentaro Tokumoto, Takuji Nishida, Yukitoshi Takahashi, Kenjiro Kikuchi, Ryuki Matsuura, Shin ichiro Hamano, Hideo Yamanouchi, Satsuki Watanabe, Ayataka Fujimoto, Hideo Enoki, Kyoichi Tomoto, Masako Watanabe, Youji Takubo, Toshihiko Fukuchi, Hidetoshi Nakamoto, Yuichi Kubota, Naoto Kunii, Yuichiro Shirota, Eiichi Ishikawa, Nobukazu Nakasato, Taketoshi Maehara, Motoki Inaji, Shunsuke Takagi, Takashi Enokizono, Yosuke Masuda, Takahiro Hayashi
    Epilepsy and Behavior 125 2021年12月 
    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%), 128 were included in the final analysis. As many as 35.2% 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.
  • Benedikt Schoser, Mark Roberts, Barry J Byrne, Sheela Sitaraman, Hai Jiang, Pascal Laforêt, Antonio Toscano, Jeff Castelli, Jordi Díaz-Manera, Mitchell Goldman, Ans T van der Ploeg, Drago Bratkovic, Srilakshmi Kuchipudi, Tahseen Mozaffar, Priya S Kishnani
    The Lancet. Neurology 20 12 1027 - 1037 2021年12月 
    BACKGROUND: Pompe disease is a rare disorder characterised by progressive loss of muscle and respiratory function due to acid α-glucosidase deficiency. Enzyme replacement therapy with recombinant human acid α-glucosidase, alglucosidase alfa, is the first approved treatment for the disease, but some patients do not respond, and many do not show a sustained benefit. We aimed to assess the safety and efficacy of an investigational two-component therapy (cipaglucosidase alfa, a novel recombinant human acid α-glucosidase, plus miglustat, an enzyme stabiliser) for late-onset Pompe disease. METHODS: We did a randomised, double-blind, parallel-group, phase 3 trial at 62 neuromuscular and metabolic medical centres in 24 countries in the Americas, Asia-Pacific, and Europe. Eligible participants were aged 18 years or older with late-onset Pompe disease, and had either been receiving alglucosidase alfa for at least 2 years or were enzyme replacement therapy-naive. Participants were randomly assigned (2:1) using interactive response technology software, stratified by 6-min walk distance and previous enzyme replacement therapy status, to intravenous cipaglucosidase alfa (20 mg/kg) plus oral miglustat or to intravenous alglucosidase alfa (20 mg/kg) plus oral placebo once every 2 weeks for 52 weeks. Patients, investigators, and outcome assessors were masked to treatment assignment. The primary endpoint was change from baseline to week 52 in 6-min walk distance, assessed using a mixed-effect model for repeated measures analysis for comparison of superiority in the intention-to-treat population (all patients who received at least one dose of study drug). This study is now complete and is registered with ClinicalTrials.gov, NCT03729362. FINDINGS: Between Dec 3, 2018, and Nov 26, 2019, 130 patients were screened for eligibility and 125 were enrolled and randomly assigned to receive cipaglucosidase alfa plus miglustat (n=85) or alglucosidase alfa plus placebo (n=40). Two patients in the alglucosidase alfa plus placebo group did not receive any dose due to absence of genotype confirmation of late-onset Pompe disease and were excluded from analysis. Six patients discontinued (one in the alglucosidase alfa plus placebo group, five in the cipaglucosidase alfa plus miglustat group), and 117 completed the study. At week 52, mean change from baseline in 6-min walk distance was 20·8 m (SE 4·6) in the cipaglucosidase alfa plus miglustat group versus 7·2 m (6·6) in the alglucosidase alfa plus placebo group using last observation carried forward (between-group difference 13·6 m [95% CI -2·8 to 29·9]). 118 (96%) of 123 patients experienced at least one treatment-emergent adverse event during the study; the incidence was similar between the cipaglucosidase alfa plus miglustat group (n=81 [95%]) and the alglucosidase alfa plus placebo group (n=37 [97%]). The most frequently reported treatment-emergent adverse events were fall (25 [29%] patients in the cipaglucosidase alfa plus miglustat group vs 15 [39%] in the alglucosidase alfa plus placebo group), headache (20 [24%] vs 9 [24%]), nasopharyngitis (19 [22%] vs 3 [8%]), myalgia (14 [16%] vs 5 [13%]), and arthralgia (13 [15%]) vs 5 [13%]). 12 serious adverse events occurred in eight patients in the cipaglucosidase alfa plus miglustat group; only one event (anaphylaxis) was deemed related to study drug. One serious adverse event (stroke) occurred in the alglucosidase alfa plus placebo group, which was deemed unrelated to study drug. There were no deaths. INTERPRETATION: Cipaglucosidase alfa plus miglustat did not achieve statistical superiority to alglucosidase alfa plus placebo for improving 6-min walk distance in our overall population of patients with late-onset Pompe disease. Further studies should investigate the longer-term safety and efficacy of cipaglucosidase alfa plus miglustat and whether this investigational two-component therapy might provide benefits, particularly in respiratory function and in patients who have been receiving enzyme replacement therapy for more than 2 years, as suggested by our secondary and subgroup analyses. FUNDING: Amicus Therapeutics.
  • Hyperscanningと脳活動 脳磁図を用いたコミュニケーション脳科学
    柳生 一自, 渡辺 隼人, 高野 一義, 下條 暁司, 白石 秀明, 横澤 宏一, 齊藤 卓弥
    精神神経学雑誌 2021特別号 S490 - S490 (公社)日本精神神経学会 2021年09月
  • Akira Kinoshita, Kaname Ohyama, Susumu Tanimura, Katsuya Matsuda, Tatsuya Kishino, Yutaka Negishi, Naoko Asahina, Hideaki Shiraishi, Kana Hosoki, Kiyotaka Tomiwa, Naoko Ishihara, Hiroyuki Mishima, Ryoichi Mori, Masahiro Nakashima, Shinji Saitoh, Koh-Ichiro Yoshiura
    Development (Cambridge, England) 148 16 2021年08月15日 
    Mutations in ITPR1 cause ataxia and aniridia in individuals with Gillespie syndrome (GLSP). However, the pathogenic mechanisms underlying aniridia remain unclear. We identified a de novo GLSP mutation hotspot in the 3'-region of ITPR1 in five individuals with GLSP. Furthermore, RNA-sequencing and immunoblotting revealed an eye-specific transcript of Itpr1, encoding a 218amino acid isoform. This isoform is localized not only in the endoplasmic reticulum, but also in the nuclear and cytoplasmic membranes. Ocular-specific transcription was repressed by SOX9 and induced by MAF in the anterior eye segment (AES) tissues. Mice lacking seven base pairs of the last Itpr1 exon exhibited ataxia and aniridia, in which the iris lymphatic vessels, sphincter and dilator muscles, corneal endothelium and stroma were disrupted, but the neural crest cells persisted after completion of AES formation. Our analyses revealed that the 218-amino acid isoform regulated the directionality of actin fibers and the intensity of focal adhesion. The isoform might control the nuclear entry of transcriptional regulators, such as YAP. It is also possible that ITPR1 regulates both AES differentiation and muscle contraction in the iris.
  • Katsuki Eguchi, Shinichi Shirai, Masaaki Matsushima, Takahiro Kano, Tomohiro Ichikawa, Kazuyoshi Yamazaki, Shuji Hamauchi, Toru Sasamori, Toshitaka Seki, Mayumi Kitagawa, Hideaki Shiraishi, Kiyohiro Houkin, Hidenao Sasaki, Ichiro Yabe
    Parkinsonism & Related Disorders 89 148 - 150 2021年08月
  • Akiko Kada, Jun Tohyama, Hideaki Shiraishi, Yukitoshi Takahashi, Eiji Nakagawa, Tomoyuki Akiyama, Akiko M Saito, Yushi Inoue, Mitsuhiro Kato
    The Kurume medical journal 66 2 115 - 120 2021年07月21日 
    Epileptic seizures are core symptoms in focal cortical dysplasia (FCD), a disease that often develops in infancy. Such seizures are refractory to conventional antiepileptic drugs (AED) and temporarily disappear in response to AED in only 17% of patients. Currently, surgical resection is an important option for the treatment of epileptic seizures in FCD. In 2015, Korean and Japanese groups independently reported that FCD is caused by somatic mosaic mutation of the MTOR gene in the brain tissue. Based on these results we decided to test a novel treatment using sirolimus, an mTOR inhibitor, for epileptic seizures in patients with FCD type II. A single arm open-label clinical trial for FCD type II patients is being conducted in order to evaluate the efficacy and safety of sirolimus. The dose of sirolimus is fixed for the first 4 weeks and dose adjustment is achieved to maintain a blood level of 5 to 15 ng/mL during 8 to 24 weeks after initiation of administration, and it is kept within this level during a maintenance therapy period of 12 weeks. Primary endpoint is a reduction in the rate of incidence of focal seizures (including focal to bilateral tonic-clonic seizures) per 28 days during the maintenance therapy period from the observation period. To evaluate the frequency of epileptic seizures, registry data will be used as an external control group. We hope that the results of this trial will lead to future innovative treatments for FCD type II patients.
  • Hidekazu Saito, Shogo Yazawa, Jun Shinozaki, Takashi Murahara, Hideaki Shiraishi, Masao Matsuhashi, Takashi Nagamine
    Journal of neuroscience methods 359 109213 - 109213 2021年07月15日 
    BACKGROUND: The baseline (BL) segment in the prestimulus period is generally assigned as a reference of evoked activities. However, an experimenter empirically defines its length in each condition. So far, the criterion for the length of a BL segment has not been established. NEW METHOD: We evaluated the effect of the length of the BL segment by recording somatosensory evoked magnetic fields (SEFs) under fixed stimulus onset asynchrony (SOA). For the evaluation of the length of the BL segment in the prestimulus period, five proportions in relation to SOA were used as the BL segment. In addition, we adopted other two types of BL segment which were the single data point measured from the value of stimulus onset (BL0) and the mean value of the whole raw data throughout the recording (DC mean). We investigated the influence of the BL segments on SEFs by utilizing two indicators: normalized N20 m amplitudes and estimated locations of corresponding equivalent current dipoles (ECDs). RESULTS: Both indicators did not show any significant differences, based on the factor of BL segments, in any SOA conditions. COMPARISON WITH EXISTING METHOD: The BL0 had by far the largest variation in the ECD locations.Therefore, utilizing stimulus onset as the BL segment should be avoided. In addition, considering that other BL segments provided comparable values by the two indicators, the DC mean can reasonably be adopted. CONCLUSIONS: We suggest that utilizing the DC mean could be employed as the BL segment.
  • Yuji Maruo, Kimiaki Uetake, Kiyoshi Egawa, Hideaki Shiraishi
    Pediatric neurology 120 1 - 2 2021年07月
  • Kazuyori Yagyu, Ryusaku Hashimoto, Atsushi Shimojo, Michiru Iwata, Keitaro Sueda, Ayumi Seki, Hideaki Shiraishi, Takuya Saito
    Brain & development 43 9 893 - 903 2021年05月26日 
    BACKGROUND: Dyslexia is a neurodevelopmental disorder which occurs in childhood but continues to influence academic and occupational function in adulthood. Recently, a Japanese dyslexia questionnaire and diagnostic procedure was established for primary school children. However, there is currently no procedure for the diagnosis or screening of dyslexia in individuals at or above junior high school age; accordingly, we aimed to develop a questionnaire to screen for reading difficulties in those individuals. METHODS: A questionnaire with various candidate items was developed from two English questionnaires, one Japanese questionnaire, and newly devised items focusing on the Japanese writing system and the most appropriate 28 items were selected. In total, 462 adults and 127 junior high to high school students were enrolled. Of those, 191 participants also took part in reading tests. After the exploratory factor analysis, reliability and validity were evaluated using the above control participants and 12 adolescents with dyslexia. RESULTS: The questionnaire included three factors, i.e., silent reading sub-scale (four items), writing sub-scale (four items), and aloud reading sub-scale (three items). Five were newly devised items focusing on the Japanese writing system. Cronbach's alphas of the three factors were 0.706, 0.638, and 0.568, respectively, and the interclass correlation coefficients (2,1) were 0.743, 0.609, and 0.695, respectively. The silent reading and aloud reading sub-scales were positively correlated with word, non-word, and passage reading time. DISCUSSION: The newly developed questionnaire correlated well with actual reading performance and may be used to screen reading difficulty in Japanese individuals at or above junior high school age.
  • Kiyoshi Egawa, Sachiko Nakakubo, Shuhei Kimura, Takeru Goto, Atsushi Manabe, Hideaki Shiraishi
    Brain & development 43 4 515 - 520 2021年04月 
    INTRODUCTION: Epilepsy is one of the main clinical problems in Angelman syndrome (AS). Seizures typically start in early childhood then decrease or are often alleviated by young adulthood. Several studies using AS model mice showed comparable seizure susceptibility during young adulthood. In contrast, the course of epilepsy post young adulthood differs from persistently relieved to rerising among reports. To elucidate this, we evaluated the seizure susceptibility of AS model mice of two different ages. METHODS: Mice lacking maternal Ube3a gene (Ube3am-/p+) of C57BL/6 background or their littermate wild type (WT) were divided into two groups by age, 2 to 3 months (2-3 M) and 6 to 12 months (6-12 M), corresponding to adolescent to young adult aged and middle aged humans, respectively. Seizure susceptibility was evaluated by flurothyl inhalation or intraperitoneal injection of pentylenetetrazole (PTZ IP)-induced acute seizure protocol. RESULTS: In the flurothyl-induced seizure paradigm, the latency to seizure occurrence had a significant interaction with genotype and age. Post-hoc analysis revealed that the latency was significantly shorter at 6-12 M than at 2-3 M in Ube3am-/p+ mice, and in Ube3am-/p+ mice than in WT mice at 6-12 M. No significant interaction or difference was observed by PTZ IP. CONCLUSION: The flurothyl-induced seizure paradigm revealed that seizure susceptibility of Ube3am-/p+ mice increased with age, similar to clinical studies reporting the reappearance of epilepsy in older age. The flurothyl-induced seizure paradigm applied to middle-aged Ube3am-/p+ mice could be a suitable protocol for screening drugs against seizures in AS.
  • Kiyoshi Egawa, Shinji Saitoh, Naoko Asahina, Hideaki Shiraishi
    Brain & development 43 4 521 - 527 2021年04月 
    INTRODUCTION: Angelman syndrome (AS) and Prader-Willi syndrome (PWS) are neurodevelopmental disorders caused by loss of function of maternally expressed UBE3A and paternally expressed contiguous genes on chromosome 15q11-13, respectively. A majority of these syndromes suffer from a large deletion of the relevant chromosome (AS Del or PWS Del), which includes biallelically expressed gamma-aminobutyric acid type A receptor subunit (GABAaR) genes, while remaining individuals present without the deletion (AS non-Del or PWS non-Del). We previously reported that AS Del, but not AS non-Del individuals, show aberrantly desynchronized somatosensory-evoked magnetic fields (SEFs) and speculated that it might reflect GABAergic dysfunction due to the hemizygosity of GABAaR genes. To verify its pathophysiological impact on PWS and AS, we analyzed the SEFs of PWS individuals. METHOD: SEFs were recorded from eight PWS Del and two PWS non-Del individuals. The latency and strength of the first peak (N1m) were compared with those of AS Del/non-Del individuals and controls, most of which were obtained earlier. RESULTS: In contrast to AS, both PWS Del and PWS non-Del showed normal SEF waveforms. Desynchronized response with delayed N1m peak latency was exclusively indicated in AS Del. N1m strength was statistically higher in AS Del and AS non-Del, but not in PWS Del and PWS non-Del. CONCLUSIONS: Our results indicate that the pathophysiological impact of the hemizygosity of GABAaR genes is lower in PWS than AS. UBE3A deficiency and the hemizygosity of GABAaR genes could synergistically deteriorate neuronal function, resulting in aberrant SEFs in AS Del.
  • Kiyoshi Egawa, Shinji Saitoh, Naoko Asahina, Hideaki Shiraishi
    eNeurologicalSci 22 100298 - 100298 2021年03月 
    Background: Angelman syndrome (AS) is neurodevelopmental disorder, causal gene of which is maternally expressed UBE3A. A majority of patients results from the large deletion of relevant chromosome which includes GABAA receptor subunit genes (GABARs) as well as UBE3A (AS Del). We previously reported aberrantly desynchronized primary somatosensory response in AS Del by using magnetoencephalography. The purpose of this study is to estimate cortical and subcortical involvement in the deficit of primary somatosensory processing in AS. Methods: We analyzed short-latency somatosensory-evoked potentials (SSEPs) in 8 patients with AS Del. SSEPs were recorded on a 4-channel system comprising of two cortical electrodes which were placed on the frontal and centro-parietal areas. The peak and onset latency of each component were measured to compare latency and interval times. Results: The first-cortical peak latency (N20, P20), and N13-N20 peak interval times were significantly prolonged in AS Del compared to healthy controls. In contrast, there was no difference in latencies between subcortical components up to N20 onset or for N11-N20 onset interval times. Conclusion: Highly desynchronized first-cortical SSEP components and normal latencies of subcortical components indicated cortical dysfunction rather than impairment of afferent pathways in AS Del patients, which might be attributed to GABAergic dysfunction due to loss of UBE3A function and heterozygosity of GABARs.
  • Yuji Maruo, Kiyoshi Egawa, Hidefumi Tonoki, Satoshi Terae, Yuki Ueda, Hideaki Shiraishi
    Pediatric neurology 116 55 - 56 2021年03月
  • Hideaki Shiraishi, Kenji Yamada, Kiyoshi Egawa, Mika Ishige, Fumihiro Ochi, Asami Watanabe, Sanae Kawakami, Kazuyo Kuzume, Kenji Watanabe, Koji Sameshima, Kiyotaka Nakamagoe, Akira Tamaoka, Naoko Asahina, Saki Yokoshiki, Keiko Kobayashi, Takashi Miyakoshi, Koji Oba, Toshiyuki Isoe, Hiroshi Hayashi, Seiji Yamaguchi, Norihiro Sato
    Brain & development 43 2 214 - 219 2021年02月 
    BACKGROUND: Very long-chain acyl-CoA dehydrogenase deficiency (VLCADD) is a mitochondrial fatty acid oxidation disorder that causes episodic attacks, such as general fatigue, hypotonia, myalgia, and rhabdomyolysis accompanied by lack of energy. As yet, there are no preventative drugs for these VLCADD-associated metabolic attacks. PATIENTS AND METHODS: We conducted an open-label, non-randomized, multi-center study into the effects of bezafibrate on five patients with VLCADD. Bezafibrate was administered for 4 years, and we analyzed the number of myopathic attacks requiring hospitalization and treatment infusions. RESULTS: The number of myopathic attacks requiring infusions of 24 h or longer significantly decreased during the study period. The patients' ability to conduct everyday activities was also improved by the treatment. CONCLUSION: Our findings show the potential long-term efficacy of bezafibrate in preventing myopathic attacks for patients with VLCADD.
  • Shuhei Kimura, Hideaki Shiraishi, Kiyoshi Egawa, Masaya Uchida, Michihiko Ueno
    Brain & development 43 2 361 - 362 2021年02月
  • ハイパースキャニングによる社会脳機能計測 Dual MEGを用いたコミュニケーション脳科学の可能性
    柳生 一自, 渡辺 隼人, 高野 一義, 下條 暁司, 白石 秀明, 横澤 宏一, 齊藤 卓弥
    日本生体磁気学会誌 34 1 108 - 108 日本生体磁気学会 2021年
  • MEGハイパースキャニングによる非言語コミュニケーション中の脳領域間の相関活動
    金 柱亨, 小野 弓絵, 高野 一義, 渡辺 隼人, 柳生 一自, 白石 秀明, 齋藤 卓弥, 横澤 宏一
    日本生体磁気学会誌 34 1 125 - 126 日本生体磁気学会 2021年
  • Shuhei Kimura, Hideaki Shiraishi, Kiyoshi Egawa, Masaya Uchida, Michihiko Ueno
    Brain & development 43 1 157 - 159 2021年01月 
    BACKGROUND: Leigh syndrome (LS) is a mitochondrial disorder that shows abnormal basal ganglia lesion and psychomotor regression. Although vitamins have been used for LS, we have not found any effective drug. CASE PRESENTATION: A 26-year-old man who showed psychomotor delay and short stature at the age of 1 year was diagnosed with LS according to the results of cerebrospinal fluid and high signal intensity in the bilateral striatum on T2-weighted magnetic resonance imaging. He demonstrated psychomotor delay and breathing disorders, but the progression was very slow. His symptoms suddenly worsened at the age of 24 years after acute epididymitis. He showed epileptic seizures simultaneously and his activities of daily living (ADL) significantly worsened. Several antiepileptic drugs were ineffective, but his seizures were suppressed by a low dose of perampanel and his ADL improved. CONCLUSION AND DISCUSSION: Our case showed that low-dose perampanel could be a drug for epileptic seizures and improvement of ADL in patients with LS.
  • Kosuke Otsuka, Kiyoshi Egawa, Noriyuki Fujima, Kohsuke Kudo, Satoshi Terae, Midori Nakajima, Tomoshiro Ito, Kazuyori Yagyu, Hideaki Shiraishi
    Epilepsy & behavior : E&B 114 Pt A 107516 - 107516 2021年01月 
    OBJECTIVE: This study examined whether the application of magnetoencephalography (MEG) to interpret magnetic resonance imaging (MRI) findings can aid the diagnosis of intractable epilepsy caused by organic brain lesions. METHODS: This study included 51 patients with epilepsy who had MEG clusters but whose initial MRI findings were interpreted as being negative for organic lesions. Three board-certified radiologists reinterpreted the MRI findings, utilizing the MEG findings as a guide. The degree to which the reinterpretation of the imaging results identified an organic lesion was rated on a 5-point scale. RESULTS: Reinterpretation of the MRI data with MEG guidance helped detect an abnormality by at least one radiologist in 18 of the 51 patients (35.2%) with symptomatic localization-related epilepsy. A surgery was performed in 7 of the 51 patients, and histopathological analysis results identified focal cortical dysplasia in 5 patients (Ia: 1, IIa: 2, unknown: 2), hippocampal sclerosis in 1 patient, and dysplastic neurons/gliosis in 1 patient. CONCLUSIONS: The results of this study highlight the potential diagnostic applications of MEG to detect organic epileptogenic lesions, particularly when radiological visualization is difficult with MRI alone.
  • Yuto Arai, Kiyotaka Kosugiyama, Takuya Tamura, Sasagu Matsumoto, Akira Sudo, Hideaki Shiraishi, Cammack Ivor, Akira Ohtake, Kiyoshi Nagumo
    Molecular genetics and metabolism reports 25 100684 - 100684 2020年12月 
    Hypertension is a rare complication of Leigh Syndrome (LS), but prognosis of patients with hypertension is poor and its presence is indicative of the terminal stage of the disease. Herein, we report a four-year-old girl case diagnosed with LS at 15 months of age who subsequently developed severe hypertension and respiratory failure. Physical examination and laboratory findings did not indicate a secondary cause of hypertension. Her respiratory failure was treated with non-invasive ventilation and hypertension controlled with enalapril, furosemide and spironolactone. To our knowledge, this is the first case of a patient with LS recovering from severe hypertension.
  • 江川 潔, 白石 秀明
    てんかんをめぐって 38 11 - 19 日本てんかん学会-北海道地方会 2020年12月 
    アンジェルマン症候群は、母性発現遺伝子UBE3Aを原因因子とする遺伝性疾患で、精神運動発達遅滞、言語障害、運動機能障害、特徴的な顔貌を主要症状とする。アンジェルマン症候群を発症する遺伝的メカニズムを紹介し、その病態生理の一つとして、GABA抑制系機能障害に関する著者らの研究を紹介した。また、アンジェルマン症候群のてんかんと治療に関する最近のトピックスを紹介した。
  • 柳生 一自, 渡辺 隼人, 高野 一義, 下條 暁司, 白石 秀明, 横澤 宏一, 齊藤 卓弥
    臨床神経生理学 48 5 440 - 440 (一社)日本臨床神経生理学会 2020年10月
  • Kazuhiro Koyanagawa, Yuta Kobayashi, Tadao Aikawa, Atsuhito Takeda, Hideaki Shiraishi, Satonori Tsuneta, Noriko Oyama-Manabe, Hiroyuki Iwano, Toshiyuki Nagai, Toshihisa Anzai
    Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine 20 3 320 - 324 2020年09月07日 
    To assess myocardial fibrosis associated with muscular dystrophy, T1-mapping and extracellular volume fraction (ECV) quantification was prospectively performed using cardiovascular MR (CMR) imaging in 6 male patients with muscular dystrophy and 5 female putative carriers of Duchenne or Becker muscular dystrophy. Five patients and all putative carriers had an elevated ECV (>29.5% for men and >35.2% for women), suggesting that ECV has a potential to detect diffuse fibrotic changes in patients and putative carriers of muscular dystrophy.
  • Kazuyoshi Takano, Hayato Watanabe, Kazuyori Yagyu, Atsushi Shimojo, Jared Boasen, Yui Murakami, Hideaki Shiraishi, Koichi Yokosawa, Takuya Saito
    Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference 2020 2893 - 2896 2020年07月 
    Face to face communication is interactive, and involves continuous feedforward and feedback of information, thoughts, and feelings to the opposite party. To accurately assess the neural processing underlying these interactions, synchronous and simultaneous recording of the brain activity from both parties is needed, a method known as hyperscanning. Here, we investigated the neural processing underlying nonverbal face-to-face communication using a magnetoencephalographic (MEG) hyperscanning system, comprising two fiber optically connected MEGs. Eight pairs of subjects participated. Each individual in each pair viewed a combined 80 randomized 20 s trials of 40 real-time and 40 recorded (hereafter, real and simulated, respectively) videos of the opposite party's face. Non-verbal communication through actions such as gaze, eye blinks, and facial expression was intrinsically only possible during real videos. After each trial, subjects individually subjectively discriminated whether the viewed video was real or simulated. Overall subjective discrimination accuracies were slightly but significantly above chance level. Statistical analysis of brain activity revealed a significant three way interaction between theta-band rhythm amplitude, video type, and subjective discrimination response in the right frontal cortex. Additionally, when subjects responded that videos were simulated, theta activity was significantly lower for real videos compared with simulated videos (p = 0.01). This result not only demonstrates the importance of right frontal theta activity during non-verbal communication, but also indicates the existence of unconscious, semi-automated neural processing during non-verbal communication that underlies one's ability to subjectively discriminate whether or not the opposite party is real.
  • Osamu Kawano, Kiyoshi Egawa, Hideaki Shiraishi
    Brain & development 42 5 389 - 392 2020年05月 
    BACKGROUND: Angelman syndrome (AS) is a neurodegenerative disorder caused by functional loss of the maternal ubiquitin-protein ligase 3A gene. Nonepileptic myoclonus, also described as tremulous movement, often occurs during puberty and increases in adulthood. The involuntary movement in AS has not been defined patho-physiologically and the drugs used such as levetiracetam and piracetam are not always effective. Recently, the alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionate (AMPA) receptor antagonist, perampanel (PER), was used to alleviate myoclonus in progressive myoclonus epilepsy. Herein, we tested the efficacy of PER for nonepileptic myoclonus. METHODS AND RESULTS: Four patients with AS, aged from 20 to 40 years at the beginning of treatment, were enrolled in our study. All patients reported disruption to their daily lives from the myoclonus movement. They experienced mild to moderate improvement with the starting dose of 2 mg. The dose was increased to 4 mg in one patient to achieve sufficient efficacy, while two had their dose reduced to 1 mg due to dizziness or possible exacerbation of myoclonus. The last patient continued to take the starting dose. Follow-up over 16-20 months revealed a significant reduction in the severity of nonepileptic myoclonus in all patients. CONCLUSION: Our study suggests that PER could be one of the promising drugs for nonepileptic myoclonus in AS.
  • 瀬戸 康貴, 上野 倫彦, 内田 雅也, 泉 岳, 白石 秀明, 武田 充人
    臨床小児医学 67 1-6 79 - 83 (財)小児愛育協会 2020年03月 [査読有り][通常論文]
     
    カテコラミン誘発多形性心室頻拍(catecholaminergic polymorphic ventricular tachycardia;CPVT)は遺伝性不整脈の一つで、心肺停止や突然死をきたす例も少なくない。今回、意識消失を主訴に当科受診歴のあった8歳女児で、自宅で心室細動となり、胸骨圧迫と自動体外式除細動器の使用にて救命された症例を経験した。当院搬送後も、局所麻酔刺激などで心室細動や多形性心室頻拍を繰り返したためCPVTと診断し、深鎮静ののち、β遮断薬、フレカイニドの導入と植え込み型除細動器の留置を行った。CPVTは多種多様な刺激により引き起こされるが、安静時心電図では特徴的所見に乏しく、疑うことができなければ事前診断や心事故の予防は難しい。安静時以外の意識消失をみた際には本疾患を考慮し鑑別診断を行う必要がある。また、本疾患症例を突然死から回避するためには市民救命救急の啓発を行うことが重要である。(著者抄録)
  • 柳生 一自, 白石 秀明
    脳神経内科 92 2 193 - 197 (有)科学評論社 2020年02月
  • 漢字熟語学習による脳磁場の変化
    下條 暁司, 柳生 一自, 白石 秀明
    日本生体磁気学会誌 33 1 130 - 131 日本生体磁気学会 2020年
  • 西野 瑛理, 生田目 紀子, 河野 修, 江川 潔, 小笠原 卓, 金田 眞, 白石 秀明
    脳と発達 52 1 38 - 40 (一社)日本小児神経学会 2020年01月 
    脊髄性筋萎縮症(spinal muscular atrophy;SMA)I型に対しアンチセンス核酸であるnusinersenを日齢55より開始した症例を経験した。症例は男児で、胎児期、生直後に異常を認めなかったが、1ヵ月健診で筋緊張低下を指摘された。日齢48に遺伝学的検査にて脊髄性筋萎縮症I型と診断し、日齢55よりnusinersenの投与を開始した。同剤導入4ヵ月時点で呼吸補助を必要とせず、全量経口哺乳、上下肢の抗重力運動が可能となった。しかし、生後4ヵ月時の上気道感染症罹患を契機に経口哺乳量は減少し、誤嚥性肺炎を来して再度全量経管栄養となった。呼吸に関しては奇異性呼吸の改善を認めず、生後6ヵ月頃より夜間の非侵襲的陽圧換気およびカフアシストを導入した。Nusinersen投与を乳児期極早期に施行し、四肢の運動発達に改善傾向が認められたが、哺乳、呼吸に関しては期待したほど大きな改善を認めなかった。本症例はSMAの亜分類においてSMAIa型と考えられ、nusinersenの治療効果はI型の中でも亜分類によって治療効果に差異があることが示唆された。(著者抄録)
  • Ryoji Naganuma, Ichiro Yabe, Megumi Takeuchi, Kirari Morishita, Shingo Nakane, Ryoken Takase, Ikuko Takahashi-Iwata, Masaaki Matsushima, Mika Otsuki, Hideaki Shiraishi, Hidenao Sasaki
    PloS one 15 9 e0232808  2020年 
    Studies on evoked responses in Parkinson's disease (PD) may be useful for elucidating the etiology and quantitative evaluation of PD. However, in previous studies, the association between evoked responses and detailed motor symptoms or cognitive functions has not been clear. This study investigated the characteristics of the visual (VEF), auditory (AEF), and somatosensory (SEF) evoked magnetic fields in patients with Parkinson's disease (PD), and the correlations between evoked fields and the patient's clinical characteristics, motor symptoms, and cognitive functions. Twenty patients with PD and 10 healthy controls (HCs) were recruited as participants. We recorded VEF, AEF, and SEF, collected clinical characteristics, performed physical examinations, and administered 10 cognitive tests. We investigated differences in the latencies of the evoked fields between patients with PD and HCs. We also evaluated the correlation of the latencies with motor symptoms and cognitive functioning. There were significant differences between the two groups in 6 of the cognitive tests, all of which suggested mild cognitive impairment in patients with PD. The latencies of the VEF N75m, P100m, N145m, AEF P50m, P100m, and SEF P60m components were greater in the patients with PD than in the HCs. The latencies mainly correlated with medication and motor symptoms, less so with cognitive tests, with some elements of the correlations remaining significant after Bonferroni correction. In conclusion, the latencies of the VEF, AEF, and SEF were greater in PD patients than in HCs and were mainly correlated with medication and motor symptoms rather than cognitive functioning. Findings from this study suggest that evoked fields may reflect basal ganglia functioning and are candidates for assessing motor symptoms or the therapeutic effects of medication in patients with PD.
  • 江川 潔, 白石 秀明
    てんかんをめぐって 37 49 - 54 日本てんかん学会-北海道地方会 2019年12月 
    アンジェルマン症候群(AS)は、母性発現遺伝子UBE3Aを原因遺伝子とする疾患で、その約8割に癲癇を合併するとされている。今回、ASモデルマウス(Ube3aを機能欠失させたC57/BL6系マウス)における癲癇発作の客観的評価を試みた。頭蓋内電極を埋め込み、24時間ビデオ脳波記録を行ったところ、癲癇性放電と徐波成分の増強が認められたものの、自発癲癇発作は認められず、自発発作の頻度による癲癇の評価は困難と考えられた。次にGABA受容体拮抗薬Pentylenetetrazolを腹腔内投与し、痙攣発作の観察を30分間行ったが、個体毎のバラツキが大きく、野生型マウス(WT)との有意差は認められなかった。そこで、揮発性のGABA受容体拮抗薬Fluorothylを密閉したチャンバー内に持続注入し、マウスが揮発ガスを吸入し痙攣に至るまでの潜時を調べることで発作閾値を評価した。結果、ASモデルマウスの発作閾値はWTに比べて有意に低く、本法はASモデルマウスにおける発作閾値評価法として優れていることが示唆された。
  • パーキンソン病患者における聴覚誘発脳磁場の検討
    長沼 亮滋, 竹内 恵, 森下 きらり, 中根 進児, 高橋 育子, 松島 理明, 大槻 美佳, 白石 秀明, 矢部 一郎, 佐々木 秀直
    臨床神経学 59 Suppl. S366 - S366 (一社)日本神経学会 2019年11月
  • Hideaki Shiraishi, Kenji Yamada, Eishin Oki, Mika Ishige, Toshiyuki Fukao, Yusuke Hamada, Norio Sakai, Fumihiro Ochi, Asami Watanabe, Sanae Kawakami, Kazuyo Kuzume, Kenji Watanabe, Koji Sameshima, Kiyotaka Nakamagoe, Akira Tamaoka, Naoko Asahina, Saki Yokoshiki, Takashi Miyakoshi, Koji Oba, Toshiyuki Isoe, Hiroshi Hayashi, Seiji Yamaguchi, Norihiro Sato
    Molecular genetics and metabolism reports 20 100496 - 100496 2019年09月 [査読有り][通常論文]
     
    Introduction: Fatty acid oxidation disorders (FAODs) are rare diseases caused by a defective mitochondrial fatty acid oxidation (FAO) enzyme. We recently reported that bezafibrate improved patient quality of life (QOL) based on the SF-36 questionnaire score in patients with FAODs during a 50-week, open-label, clinical trial. Herein we conducted further survey assessments of the trial patients to define the long-term efficacy and safety of bezafibrate. Materials and methods: This trial was an open-label, non-randomized, and multicenter study of bezafibrate treatment in five patients with very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency and one patient with carnitine palmitoyltransferase-II (CPT-2) deficiency (median age, 15.9 years; range, 5.8-26.4 years). The bezafibrate administration was continued for a further 102-174 weeks after the 24-week treatment described in our previous study. QOL was quantitated using the 36-Item Short Form Health Survey (SF-36) questionnaire, which constitutes eight components: physical functioning (PF), role limitation due to physical problems, bodily pain, general health perception, vitality, social functioning, role limitation due to emotional problems, and mental health. Results: PF was elevated in all patients and continued to rise during the study, with the total QOL scores increased from baseline in five of the six cases. In particular, three patients older than 20 years showed treatment efficacy, and all subcategories of QOL were elevated in two of these cases. Conclusion: Our findings supported one of the stated benefits of bezafibrate in improving QOL for patients with FAODs.
  • 収縮様式の異なる随意運動と運動関連脳磁場の関連
    菅原 和広, 齊藤 秀和, 臼井 桂子, 篠崎 淳, 佐々木 健史, 松橋 眞生, 白石 秀明, 長峯 隆
    理学療法学 46 Suppl.1 O9 - 6 (公社)日本理学療法士協会 2019年08月
  • 是松 聖悟, 宮本 雄策, 村松 一洋, 山中 岳, 白石 秀明, 吉永 治美, 中川 栄二, 稲垣 真澄, 金村 英秋, 小林 勝弘
    脳と発達 51 Suppl. S172 - S172 (一社)日本小児神経学会 2019年05月 [査読無し][通常論文]
  • リアルタイムコミュニケーションを計測するためのdual MEGシステムの構成
    渡辺 隼人, 下條 暁司, 柳生 一自, 曽根原 剛志, 白石 秀明, 横澤 宏一, 齊藤 卓弥
    日本生体磁気学会誌 32 1 158 - 159 日本生体磁気学会 2019年
  • 舌運動による舌・口蓋刺激誘発20-Hz脳磁場活動の変化
    前澤 仁志, 柳生 一自, 白石 秀明, 橋本 洋章, 平田 雅之
    日本生体磁気学会誌 32 1 196 - 197 日本生体磁気学会 2019年
  • リアルタイムコミュニケーションを計測するためのdual MEGシステムの構成
    渡辺 隼人, 下條 暁司, 柳生 一自, 曽根原 剛志, 白石 秀明, 横澤 宏一, 齊藤 卓弥
    日本生体磁気学会誌 32 1 158 - 159 日本生体磁気学会 2019年 [査読無し][通常論文]
  • 舌運動による舌・口蓋刺激誘発20-Hz脳磁場活動の変化
    前澤 仁志, 柳生 一自, 白石 秀明, 橋本 洋章, 平田 雅之
    日本生体磁気学会誌 32 1 196 - 197 日本生体磁気学会 2019年 [査読無し][通常論文]
  • 玉 珍, 矢澤 省吾, 村原 貴史, 齊藤 秀和, 篠崎 淳, 白石 秀明, 松橋 眞生, 松山 清治, 長峯 隆
    生体医工学 56 3 74 - 80 (公社)日本生体医工学会 2018年06月10日 [査読有り][通常論文]
     
    健常成人11名(男性3名、女性8名、平均26.8±9.5歳)を対象とした。運動課題において、高頻度刺激への反応時間の個人平均は168〜267ms、低頻度刺激に対して運動中止の成功率は84〜96%であった。運動課題における高頻度刺激に対する聴覚反応は、右側頭葉聴覚皮質に11例、左側頭葉聴覚皮質に10例で等価電流双極子(ECD)を同定した。運動に関する活動として、9例が左一次運動野、1例が左の運動前野にECDを同定した。運動課題における低頻度刺激に対しては、聴覚に対する活動のほかに、1〜4個の電流源を同定した。左中心部には10例、前補足運動野に6例、右側には運動前野に3例、下前頭回に4例、側頭頭頂接合部に8例で認めた。高頻度刺激運動時に対してのECDは、10例中9例がBrodmann area 4(BA4)、1例がBA6、低頻度運動中止時は、10例中7例がBA6、2例がBA4に推定され、活動の頂点潜時は、高頻度刺激に対しての運動時は平均257ms、低頻度刺激に対しては平均196msで、筋電図反応の立ち上がりは平均211msであった。
  • 光ファイバで直結したdual脳磁計システムの構築
    渡辺 隼人, 柳生 一自, 下條 暁司, 曽根原 剛志, 品田 大成, 前田 珠希, 白石 秀明, 横澤 宏一, 齊藤 卓弥
    日本生体磁気学会誌 31 1 98 - 99 日本生体磁気学会 2018年06月 [査読無し][通常論文]
  • Kenji Yamada, Hideaki Shiraishi, Eishin Oki, Mika Ishige, Toshiyuki Fukao, Yusuke Hamada, Norio Sakai, Fumihiro Ochi, Asami Watanabe, Sanae Kawakami, Kazuyo Kuzume, Kenji Watanabe, Koji Sameshima, Kiyotaka Nakamagoe, Akira Tamaoka, Naoko Asahina, Saki Yokoshiki, Takashi Miyakoshi, Kota Ono, Koji Oba, Toshiyuki Isoe, Hiroshi Hayashi, Seiji Yamaguchi, Norihiro Sato
    Molecular genetics and metabolism reports 15 55 - 63 2018年06月 [査読有り][通常論文]
     
    Introduction: Fatty acid oxidation disorders (FAODs) are rare diseases caused by defects in mitochondrial fatty acid oxidation (FAO) enzymes. While the efficacy of bezafibrate, a peroxisome proliferator-activated receptor agonist, on the in vitro FAO capacity has been reported, the in vivo efficacy remains controversial. Therefore, we conducted a clinical trial of bezafibrate in Japanese patients with FAODs. Materials and methods: This trial was an open-label, non-randomized, and multicenter study of bezafibrate treatment in 6 patients with very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency and 2 patients with carnitine palmitoyltransferase-II (CPT-2) deficiency (median age, 8.2 years; ranging from 5.8 to 26.4 years). Bezafibrate was administered for 6 months following a 6-month observation period. The primary endpoint was the frequency of myopathic attacks, and the secondary endpoints were serum acylcarnitines (ACs, C14:1 or C16 + C18:1), creatine kinase (CK) levels, degree of muscle pain (VAS; visual analog scale) during myopathic attacks, and quality of life (QOL; evaluated using validated questionnaires). Results: The frequency of myopathic attacks after bezafibrate administration decreased in 3 patients, increased in 3, and did not change in 2. The CK, AC, and VAS values during attacks could be estimated in only three or four patients, but a half of the patients did not experience attacks before or after treatment. Changes in CK, AC, and VAS values varied across individuals. In contrast, three components of QOL, namely, physical functioning, role limitation due to physical problems (role physical), and social functioning, were significantly elevated. No adverse drug reactions were observed. Conclusion: In this study, the frequency of myopathic attacks and CK, AC, and VAS values during the attacks could not be evaluated due to several limitations, such as a small trial population. Our findings indicate that bezafibrate improves the QOL of patients with FAODs, but its efficacy must be examined in future investigations.
  • Suzuki H, Enatsu R, Kanno A, Ochi S, Murahara T, Yazawa S, Shiraishi H, Mikuni N
    NMC case report journal 4 4 127 - 130 2017年10月 [査読有り][通常論文]
     
    Reflex seizures are epileptic events triggered by specific external stimuli, or less commonly, internal mental stimuli. Understanding the characteristics of reflex seizures is important to elucidate the mechanisms underlying network abnormalities in epileptic conditions. This report details a patient with medically intractable reflex seizures provoked by sensory stimuli to the patient's right foot. Single-photon emission computed tomography (SPECT) during the seizure induced by sensory stimulation showed hyperperfusion in broad sensory-motor networks (dorsal column-medial lemniscus pathway, left thalamus, bilateral postcentral gyri and posterior parietal cortices, left supplementary motor area (SMA), and left paracentral lobule) and left caudateputamen. The irritative zones and ictal onset zone were localized to the left medial frontoparietal (SMA, anterior and middle cingulate gyrus, and paracentral lobule) and lateral posterior parietal cortices, as evidenced by amelioration of reflex seizures following intracranial electroencephalography and surgical resection of these areas. The neuroradiological and electrophysiological findings in our case study illustrate that the mechanism of reflex seizures may be associated with hyperexcitability of the broad sensory-motor networks, including the basal ganglia. Disconnection of these networks is necessary to treat reflex seizures.
  • Hideaki Shiraishi
    Neurological Surgery 45 10 933 - 942 2017年10月01日 [査読有り][通常論文]
  • Ikumi Hori, Takanobu Otomo, Mitsuko Nakashima, Fuyuki Miya, Yutaka Negishi, Hideaki Shiraishi, Yutaka Nonoda, Shinichi Magara, Jun Tohyama, Nobuhiko Okamoto, Takeshi Kumagai, Konomi Shimoda, Yoshiya Yukitake, Daigo Kajikawa, Tomohiro Morio, Ayako Hattori, Motoo Nakagawa, Naoki Ando, Ichizo Nishino, Mitsuhiro Kato, Tatsuhiko Tsunoda, Hirotomo Saitsu, Yonehiro Kanemura, Mami Yamasaki, Kenjiro Kosaki, Naomichi Matsumoto, Tamotsu Yoshimori, Shinji Saitoh
    Scientific reports 7 1 3552 - 3552 2017年06月14日 [査読有り][通常論文]
     
    Vici syndrome (VICIS) is a rare, autosomal recessive neurodevelopmental disorder with multisystem involvement characterized by agenesis of the corpus callosum, cataracts, cardiomyopathy, combined immunodeficiency, developmental delay, and hypopigmentation. Mutations in EPG5, a gene that encodes a key autophagy regulator, have been shown to cause VICIS, however, the precise pathomechanism underlying VICIS is yet to be clarified. Here, we describe detailed clinical (including brain MRI and muscle biopsy) and genetic features of nine Japanese patients with VICIS. Genetic dissection of these nine patients from seven families identified 14 causative mutations in EPG5. These included five nonsense, two frameshift, three splicing, one missense, and one multi-exon deletion mutations, and two initiation codon variants. Furthermore, cultured skin fibroblasts (SFs) from two affected patients demonstrated partial autophagic dysfunction. To investigate the function of EPG5, siRNA based EPG5 knock-down, and CRISPR/Cas9 mediated EPG5 knock-out HeLa cells were generated. EPG5-depleted cells exhibited a complete block of autophagic flux caused by defective autophagosome-lysosome fusion. Unexpectedly, endocytic degradation was normal in both VICIS SFs and EPG5 depleted cells, suggesting that EPG5 function is limited to the regulation of autophagosome-lysosome fusion.
  • 磁気共鳴画像がエメリー・ドレイフス型筋ジストロフィーの心臓病変の管理に有用と考えられた一例(Usefulness of cardiomagnetic resonance imaging in a patient with Emery-Dreifuss muscular dystrophy)
    山澤 弘州, 武田 充人, 泉 岳, 佐々木 理, 阿部 二郎, 佐々木 大輔, 白石 秀明, 西野 一三
    脳と発達 49 Suppl. S362 - S362 2017年05月 [査読有り][通常論文]
  • Hitoshi Maezawa, Hidetoshi Oguma, Yoshiyuki Hirai, Kazunari Hisadome, Hideaki Shiraishi, Makoto Funahashi
    NEUROSCIENCE RESEARCH 117 22 - 27 2017年04月 [査読有り][通常論文]
     
    Sophisticated tongue movements are coordinated finely via cortical control. We elucidated the cortical processes associated with voluntary tongue movement. Movement-related cortical fields were investigated during self-paced repetitive tongue protrusion. Surface tongue electromyograms were recorded to determine movement onset. To identify the location of the primary somatosensory cortex (S1), tongue somatosensory evoked fields were measured. The readiness fields (RFs) over both hemispheres began prior to movement onset and culminated in the motor fields (MFs) around movement onset. These signals were followed by transient movement evoked fields (MEFs) after movement onset. The MF and MEF peak latencies and magnitudes were not different between the hemispheres. The MF current sources were located in the precentral gyrus, suggesting they were located in the primary motor cortex (M1); this was contrary to the MEF sources, which were located in S1. We conclude that the RFs and MFs mainly reflect the cortical processes for the preparation and execution of tongue movement in the bilateral M1, without hemispheric dominance. Moreover, the MEFs may represent proprioceptive feedback from the tongue to bilateral S1. Such cortical processing related to the efferent and afferent information may aid in the coordination of sophisticated tongue movements. (C) 2016 Elsevier Ireland Ltd and Japan Neuroscience Society. All rights reserved.
  • ローレンツプロットを用いた体性感覚誘発脳磁場における活動の持続時間の評価
    齊藤 秀和, 矢澤 省吾, 篠崎 淳, 白石 秀明, 松橋 眞生, 長峯 隆
    日本生体磁気学会誌 30 1 152 - 153 日本生体磁気学会 2017年
  • Hideaki Shiraishi, Kiyoshi Egawa, Tomoshiro Ito, Osamu Kawano, Naoko Asahina, Shinobu Kohsaka
    Epilepsy and Behavior Case Reports 8 44 - 46 2017年 [査読有り][通常論文]
     
    We administered perampanel (PER) to a bedridden 13-year-old male patient with dentatorubral-pallidoluysian atrophy (DRPLA). The DRPLA diagnosis was based on the presence of a CAG trinucleotide repeat in the ATN1 gene. The patient experienced continuous myoclonic seizures and weekly generalized tonic–clonic seizures (GTCs). PER stopped the patient's myoclonic seizures and reduced the GTCs to fragmented clonic seizures. The patient recovered his intellectual abilities and began to walk again with assistance. We suggest that PER be considered as one of the key drugs used to treat patients with DRPLA.
  • T. Suzuki, N. Miyake, Y. Tsurusaki, N. Okamoto, A. Alkindy, A. Inaba, M. Sato, S. Ito, K. Muramatsu, S. Kimura, D. Ieda, S. Saitoh, M. Hiyane, H. Suzumura, K. Yagyu, H. Shiraishi, M. Nakajima, N. Fueki, Y. Habata, Y. Ueda, Y. Komatsu, K. Yan, K. Shimoda, Y. Shitara, S. Mizuno, K. Ichinomiya, K. Sameshima, Y. Tsuyusaki, K. Kurosawa, Y. Sakai, K. Haginoya, Y. Kobayashi, C. Yoshizawa, M. Hisano, M. Nakashima, H. Saitsu, S. Takeda, N. Matsumoto
    Clinical Genetics 90 6 526 - 535 2016年12月01日 [査読有り][通常論文]
     
    Joubert syndrome (JS) is rare recessive disorders characterized by the combination of hypoplasia/aplasia of the cerebellar vermis, thickened and elongated superior cerebellar peduncles, and a deep interpeduncular fossa which is defined by neuroimaging and is termed the ‘molar tooth sign’. JS is genetically highly heterogeneous, with at least 29 disease genes being involved. To further understand the genetic causes of JS, we performed whole-exome sequencing in 24 newly recruited JS families. Together with six previously reported families, we identified causative mutations in 25 out of 30 (24 + 6) families (83.3%). We identified eight mutated genes in 27 (21 + 6) Japanese families, TMEM67 (7/27, 25.9%) and CEP290 (6/27, 22.2%) were the most commonly mutated. Interestingly, 9 of 12 CEP290 disease alleles were c.6012-12T> A (75.0%), an allele that has not been reported in non-Japanese populations. Therefore c.6012-12T> A is a common allele in the Japanese population. Importantly, one Japanese and one Omani families carried compound biallelic mutations in two distinct genes (TMEM67/RPGRIP1L and TMEM138/BBS1, respectively). BBS1 is the causative gene in Bardet–Biedl syndrome. These concomitant mutations led to severe and/or complex clinical features in the patients, suggesting combined effects of different mutant genes.
  • Takanobu Toyoshima, Shogo Yazawa, Takashi Murahara, Masanori Ishiguro, Jun Shinozaki, Satoe Ichihara-Takeda, Hideaki Shiraishi, Masao Matsuhashi, Shun Shimohama, Takashi Nagamine
    NEUROSCIENCE RESEARCH 112 26 - 36 2016年11月 [査読有り][通常論文]
     
    We investigated the effect of load against self-paced movement on cortical involvement for motor execution. Ten right-handed healthy volunteers were requested to perform brisk extension of the right index finger at self-paced intervals exceeding 10 s for three load conditions: 0 g, 50 g and 100 g. Movement related magnetic fields were recorded using an MEG system. The signals were band-pass-filtered through 18-23 Hz and rectified before averaging with respect to EMG onset. We analyzed the time course and %change of peak amplitude with reference to the baseline amplitude in event-related desynchronization (ERD) or synchronization (ERS) in each hemisphere. Maximum response was observed around the left somatomotor area for all conditions. ERD did not show any significant difference before the movement onset among the three load conditions. For %change, ERS in the post-movement period was significantly larger for the 100 g load condition than for the 0 g load condition, and that was significantly greater over the left than over the right hemisphere. These findings indicate that the load has little effect on pre movement desynchronization, whereas it affects the post-movement synchronization on background rhythms. (C) 2016 Elsevier Ireland Ltd and Japan Neuroscience Society. All rights reserved.
  • Midori Nakajima, Elysa Widjaja, Shiro Baba, Yosuke Sato, Ryuhei Yoshida, Maya Tabei, Ayaka Okazaki, Satoru Sakuma, Stephanie A. Holowka, Ayako Ochi, O. Carter Snead, James T. Rutka, James M. Drake, Hideaki Shiraishi, Sam Doesburg, Hiroshi Otsubo
    EPILEPSIA 57 7 1169 - 1178 2016年07月 [査読有り][通常論文]
     
    ObjectiveTo investigate whether the magnetoencephalography (MEG) single moving dipole (SMD) method could delineate the epileptic zone of focal cortical dysplasia (FCD) at the bottom of sulcus (FCDB). MethodsWe retrospectively analyzed 17 children (11 male; mean age 8.8 years, range 3-17 years) with FCD type II who underwent epilepsy surgery. We compared spatial congruence between the following: (1) MEG cluster and FCDB and (2) MEG cluster and FCD at the brain surface (FCDS). We measured the volume and depth of magnetic resonance imaging (MRI)-visible lesions to investigate whether they affect spatial congruence between MEG cluster and MRI-visible lesion. ResultsEight children had FCDB and the other nine children had FCDS. The volume of MRI-visible lesions for FCDB ranged from 1,632 to 4,707 mm(3) (mean standard deviation [SD] 3,095 +/- 1,211 mm(3)). The depth of FCDB ranged from 19 to 33 mm (mean +/- SD 26 +/- 4 mm). The volume of MRI-visible lesion for FCDS ranged from 2,375 to 57,331 mm(3) (15,470 +/- 18,455 mm(3)). There was a tendency for a smaller volume of MRI-visible lesion for FCDB, relative to FCDS(p = 0.079). In FCDB, six children showed clusters of MEG dipoles and two children showed scattered MEG dipoles for interictal spikes. The spatial congruence between the MEG result and FCDB was partially overlapping in four children and discordant in another four children. In FCDS, eight children had MEG cluster and one child had MEG scatter alone. The spatial congruence between MEG result and FCDS was overlapping in eight of nine children (fully two; partially six) and discordant in one of nine children. Fifteen children (88%; FCDB eight; FCDS seven) became seizure-free after resective surgery. MEG spike dipole resection ratio in the cluster ranged from 4-100% (mean 67%) in 6 FCDB and 23-99% (mean 77%) in 8 FCDS. SignificanceThe SMD method may drift MEG spike dipoles for FCDB. Lesionectomy can control seizures for four of eight patients in FCDB despite the remote MEG dipoles. The FCDB or FCDS partially overlapped with MEG cluster may have an extending/invisible epileptogenic zone consecutive to the MRI-visible lesion.
  • Hirotomo Saitsu, Miho Watanabe, Tenpei Akita, Chihiro Ohba, Kenji Sugai, Winnie Peitee Ong, Hideaki Shiraishi, Shota Yuasa, Hiroshi Matsumoto, Khoo Teik Beng, Shinji Saitoh, Satoko Miyatake, Mitsuko Nakashima, Noriko Miyake, Mitsuhiro Kato, Atsuo Fukuda, Naomichi Matsumoto
    SCIENTIFIC REPORTS 6 30072  2016年07月 [査読有り][通常論文]
     
    Epilepsy of infancy with migrating focal seizures (EIMFS) is one of the early-onset epileptic syndromes characterized by migrating polymorphous focal seizures. Whole exome sequencing (WES) in ten sporadic and one familial case of EIMFS revealed compound heterozygous SLC12A5 (encoding the neuronal K+-Cl- co-transporter KCC2) mutations in two families: c.279 + 1G > C causing skipping of exon 3 in the transcript (p.E50_Q93del) and c.572C > T (p.A191V) in individuals 1 and 2, and c.967T > C (p.S323P) and c.1243A > G(p.M415V) in individual 3. Another patient (individual 4) with migrating multifocal seizures and compound heterozygous mutations [c.953G > C (p.W318S) and c.2242_2244del (p.S748del)] was identified by searching WES data from 526 patients and SLC12A5-targeted resequencing data from 141 patients with infantile epilepsy. Gramicidin-perforated patch-clamp analysis demonstrated strongly suppressed Cl- extrusion function of E50_Q93del and M415V mutants, with mildly impaired function of A191V and S323P mutants. Cell surface expression levels of these KCC2 mutants were similar to wildtype KCC2. Heterologous expression of two KCC2 mutants, mimicking the patient status, produced a significantly greater intracellular Cl- level than with wildtype KCC2, but less than without KCC2. These data clearly demonstrated that partially disrupted neuronal Cl- extrusion, mediated by two types of differentially impaired KCC2 mutant in an individual, causes EIMFS.
  • 山本 啓之, 柳生 一自, 鳴神 雅史, 伊藤 智城, 江川 潔, 眞鍋 智代, 高橋 香代子, 中根 進児, 白石 秀明
    日本生体磁気学会誌 29 1 12 - 13 日本生体磁気学会 2016年06月
  • Yu Kobayashi, Jun Tohyama, Mitsuhiro Kato, Noriyuki Akasaka, Shinichi Magara, Hideshi Kawashima, Tsukasa Ohashi, Hideaki Shiraishi, Mitsuko Nakashima, Hirotomo Saitsu, Naomichi Matsumoto
    BRAIN & DEVELOPMENT 38 3 285 - 292 2016年03月 [査読有り][通常論文]
     
    Objective: Recent studies have elucidated causative roles for genetic abnormalities in early-onset epileptic encephalopathies (EOEE). Accompanying characteristic features, in addition to seizures, have also been suggested to provide important clues for an early and accurate genetic diagnosis of affected patients. In this study, we investigated the underlying genetic causes in patients with EOEE associated with infantile movement disorders. Methods: We examined 11 patients with EOEE and involuntary movements (nine with West syndrome and two with nonsyndromic epileptic encephalopathy). All showed severe developmental delay, cognitive impairment, and involuntary movements such as chorea, ballism, dyskinesia or myoclonus, and hand stereotypies. We performed whole-exome sequencing of 10 patients, while the other patient underwent high-resolution melting analysis of candidate EOEE genes. Results: We identified mutations in CDKL5, SCN2A, SETD5, ALG13, and TBL1XR1 in seven patients with West syndrome, and in SCN1A and GRIN1 in the two patients with unclassified epileptic encephalopathy. All mutations were validated as de novo events. The genetic cause was undetermined in the remaining two patients. Conclusions: We found pathogenic mutations in seven genes, in nine of 11 patients with EOEE and involuntary movements. Although the results of our study are preliminary because of the small number of patients, they nevertheless suggest that specific accompanying phenotypes such as hyperkinetic movements or hand stereotypies could be important in narrowing the disease spectrum and identifying causative genetic abnormalities. (C) 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
  • Hitoshi Maezawa, Tatsuya Mima, Shogo Yazawa, Masao Matsuhashi, Hideaki Shiraishi, Makoto Funahashi
    NEUROIMAGE 128 284 - 292 2016年03月 [査読有り][通常論文]
     
    Tongue movements contribute to oral functions including swallowing, vocalizing, and breathing. Fine tongue movements are regulated through efferent and afferent connections between the cortex and tongue. It has been demonstrated that cortico-muscular coherence (CMC) is reflected at two frequency bands during isometric tongue protrusions: the beta (beta) band at 15-35 Hz and the low-frequency band at 2-10 Hz. The CMC at the beta band (beta-CMC) reflects motor commands from the primary motor cortex (M1) to the tongue muscles through hypoglossal motoneuron pools. However, the generator mechanism of the CMC at the low-frequency band (low-CMC) remains unknown. Here, we evaluated the mechanism of low-CMC during isometric tongue protrusion using magnetoencephalography (MEG). Somatosensory evoked fields (SEFs) were also recorded following electrical tongue stimulation. Significant low-CMC and beta-CMC were observed over both hemispheres for each side of the tongue. Time-domain analysis showed that the MEG signal followed the electromyography signal for low-CMC, which was contrary to the finding that the MEG signal preceded the electromyography signal for beta-CMC. The mean conduction time from the tongue to the cortex was not significantly different between the low-CMC (mean, 80.9 ms) and SEFs (mean, 71.1 ms). The cortical sources of low-CMC were located significantly posterior (mean, 10.1 mm) to the sources of beta-CMC in M1, but were in the same area as tongue SEFs in the primary somatosensory cortex (S1). These results reveal that the low-CMC may be driven by proprioceptive afferents from the tongue muscles to S1, and that the oscillatory interaction was derived from each side of the tongue to both hemispheres. Oscillatory proprioceptive feedback from the tongue muscles may aid in the coordination of sophisticated tongue movements in humans. (C) 2016 Elsevier Inc. All rights reserved.
  • Hideaki Shiraishi
    Clinical Applications of Magnetoencephalography 163 - 173 2016年01月01日 [査読有り][通常論文]
     
    Magnetoencephalography (MEG) is one of the best ways to analyze neural function. In particular, MEG is valuable for assessing brain activity in children with epilepsy, because it is noninvasive and can be used multiple times for the same patient, thus enabling changes in epileptogenicity to be monitored as a child’s growth. Conventional MEG analysis is not always able to define the epileptogenic area. To this end, the single-dipole analysis tools can resolve localized epileptic MEG discharges and demonstrate equivalent current dipoles (ECDs) in cerebral cortex. However, diffuse or multifocal epileptic activities are not suitable for such ECD analyses, because the formula underpinning the single-dipole method assumes a circumscribed epileptogenic area. In this chapter, we discuss an alternative MEG tool whereby spatial filtering analysis is used for widespread or multifocal epileptogenic areas. Furthermore, we performed time-frequency analysis on a patient with symptomatic localization-related epilepsy who showed rhythmic activities as subclinical electrical discharges. In conclusion, the single-dipole method, spatial filtering analysis, and time-frequency analysis could successfully resolve an epileptogenic area in patients with epilepsy. Thus, MEG analysis is potentially useful for presurgical evaluation or the diagnosis of epileptic syndromes for almost every patient with epilepsy.
  • Hitoshi Maezawa, Kaori Onishi, Kazuyori Yagyu, Hideaki Shiraishi, Yoshiyuki Hirai, Makoto Funahashi
    CLINICAL NEUROPHYSIOLOGY 127 1 698 - 705 2016年01月 [査読有り][通常論文]
     
    Objective: Modulation of 20-Hz activity in the primary sensorimotor cortex (SM1) may be important for oral functions. Here, we show that 20-Hz event-related desynchronization/synchronization (20-Hz ERD/ERS) is modulated by sensory input and motor output in the oral region. Methods: Magnetic 20-Hz activity was recorded following right-sided tongue stimulation during rest (Rest) and self-paced repetitive tongue movement (Move). To exclude proprioception effects, 20-Hz activity induced by right-sided hard palate stimulation was also recorded. The 20-Hz activity in the two conditions was compared via temporal spectral evolution analyses. Results: 20-Hz ERD/ERS was detected over bilateral temporoparietal areas in the Rest condition for both regions. Moreover, 20-Hz ERS was significantly suppressed in the Move condition for both regions. Conclusions: Detection of 20-Hz ERD/ERS during the Rest condition for both regions suggests that the SM1 functional state may be modulated by oral stimulation, with or without proprioceptive effects. Moreover, the suppression of 20-Hz ERS for the hard palate during the Move condition suggests that the stimulation-induced functional state of SM1 may have been modulated by the movement, even though the movement and stimulation areas were different. Significance: Sensorimotor function of the general oral region may be finely coordinated through 20-Hz cortical oscillation. (C) 2015 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.
  • 齊藤 秀和, 武田 真帆, 矢澤 省吾, 村原 貴史, 鈴木 鮎子, 白石 秀明, 松橋 眞生, 長峯 隆
    臨床神経生理学 43 5 461 - 461 (一社)日本臨床神経生理学会 2015年10月
  • Yuki Ueda, Kiyoshi Egawa, Tomoshiro Ito, Fumiya Takeuchi, Midori Nakajima, Kosuke Otsuka, Naoko Asahina, Kayoko Takahashi, Shingo Nakane, Shinobu Kohsaka, Hideaki Shiraishi
    EPILEPSY RESEARCH 114 141 - 146 2015年08月 [査読有り][通常論文]
     
    Purpose: This study focused on the characteristic needle-like epileptic spikes of short duration and steep shape seen on magnetoencephalography (MEG) in patients diagnosed with focal cortical dysplasia (FCD) morphologically. We aimed to validate the analysis of MEG spike morphology as a noninvasive method of identifying the presence and location of FCD. Methods: MEG was collected by 204-channel helmet-shaped gradiometers. We analyzed MEG spike sources for 282 patients with symptomatic localization-related epilepsy. MEG showed clustered equivalent current dipoles when superimposed on their three-dimensional-magnetic resonance images (MRI) in 85 patients. Fifty-seven patients were excluded from our study, because they had destructive brain lesions or an insufficient number of spikes for statistical analysis. Twenty-eight patients (18 males, 10 females; aged 1-34 years) were finally matched to our inclusion criteria, and were categorized into three groups: FCD (7 patients), non-FCD (10 patients), and non-lesion (11 patients), based on the MRI findings. We measured the duration, amplitude, and tilt manually for at least 15 spikes per patient, and compared the three groups using a one-way analysis of variance, followed by the Tukey test when statistically significant (p <0.05). In 17 patients with visible MRI lesions, we investigated the correlation between the depth of the lesion and the tilt using the Pearson product moment correlation. Results: The average spike duration was significantly shorter in the FCD and non-lesion groups than in the non-FCD group (p <0.05). The average amplitude was not significantly different between the three groups. The average spike tilt was significantly steeper in the FCD group than in the non-FCD group (p = 0.0058). There was no significant difference between FCD and non-lesion patients in both duration and tilt. Our additional study revealed a significant negative correlation between the depth of the lesion and the average tilt (p = 0.0009). Significance: MEG epileptiform discharges of short duration and steep tilt characterize FCD, especially when located at the superficial neocortical gyrus. We speculate that this particular spike morphology results from the intrinsic epileptogenicity of FCD. Morphological analysis of MEG spikes can evaluate the etiology of epileptogenic lesions and detect a strong, localized epileptogenic focus such as that typically observed in FCD. (C) 2015 Elsevier B.V. All rights reserved.
  • Satoe Ichihara-Takeda, Shogo Yazawa, Takashi Murahara, Takanobu Toyoshima, Jun Shinozaki, Masanori Ishiguro, Hideaki Shiraishi, Nozomu Ikeda, Kiyoji Matsuyama, Shintaro Funahashi, Takashi Nagamine
    JOURNAL OF COGNITIVE NEUROSCIENCE 27 3 453 - 463 2015年03月 [査読有り][通常論文]
     
    Oscillatory brain activity is known to play an essential role in information processing in working memory. Recent studies have indicated that alpha activity (8-13 Hz) in the parieto-occipital area is strongly modulated in working memory tasks. However, the function of alpha activity in working memory is open to several interpretations, such that alpha activity may be a direct neural correlate of information processing in working memory or may reflect disengagement from information processing in other brain areas. To examine the functional contribution of alpha activity to visuospatial working memory, we introduced visuospatial distractors during a delay period and examined neural activity from the whole brain using magnetoencephalography. The strength of event-related alpha activity was estimated using the temporal spectral evolution (TSE) method. The results were as follows: (1) an increase of alpha activity during the delay period as indicated by elevated TSE curves was observed in parieto-occipital sensors in both the working memory task and a control task that did not require working memory; and (2) an increase of alpha activity during the delay period was not observed when distractors were presented, although TSE curves were constructed only from correct trials. These results indicate that the increase of alpha activity is not directly related to information processing in working memory but rather reflects the disengagement of attention from the visuospatial input.
  • Tomoshiro Ito, Hiroshi Otsubo, Hideaki Shiraishi, Kazuyori Yagyu, Yumi Takahashi, Yuki Ueda, Fumiya Takeuchi, Kayoko Takahashi, Shingo Nakane, Shinobu Kohsaka, Shinji Saitoh
    BRAIN & DEVELOPMENT 37 2 237 - 242 2015年02月 [査読有り][通常論文]
     
    Purpose: We evaluated whether magnetoencephalography (MEG), in addition to surgery, was valuable for the diagnosis and management of epileptic syndromes in patients with neocortical epilepsy (NE). Methods: We studied MEG in 73 patients (29 females; aged 1-26 years; mean 10.3 years) for the clinical diagnosis of epilepsy and for preoperative evaluation. MEG data were recorded by 204-channel whole head gradiometers with a 600 Hz sampling rate. MEG spike sources were localized on magnetic resonance images (MRI) using a single dipole model to project equivalent current dipoles. Results: MEG localized an epileptic focus with single clustered dipoles in 24 (33%) of 73 NE patients: 16 (25%) of 64 symptomatic localization-related epilepsy (SLRE) patients and eight (89%) of nine idiopathic localization-related epilepsy (ILRE) patients. MEG provided advantageous information in 12 (50%) of 24 patients with clustered dipoles and confirmed the diagnosis in the remaining 12 (50%). Furthermore, the use of MEG resulted in changes to surgical treatments in nine (38%) patients and in medical management in eight (33%). MEG confirmed the diagnosis in eight (16%) of 49 patients with scattered dipoles. MRI identified a single lesion (28 patients, 38%), multiple lesions (5, 7%), and no lesion (40, 55%). MRI provided confirming information in 19 of 28 patients with a single lesion and 18 of them required surgical resections. MRI did not provide any supportive information in 54 (74%) patients with a single (9), multiple (5) and no lesion (40). Conclusion: Our study shows that MEG provides fundamental information to aid the choice of diagnostic and therapeutic procedures including changes in medication in addition to surgical treatments for NE. (C) 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
  • 白石 秀明, 江川 潔, 細木 華奈
    日本臨床 別冊 神経症候群VI 436 - 440 (株)日本臨床社 2014年12月
  • Hitoshi Maezawa, Yoshiyuki Hirai, Hideaki Shiraishi, Makoto Funahashi
    JOURNAL OF THE NEUROLOGICAL SCIENCES 347 1-2 288 - 294 2014年12月 [査読有り][通常論文]
     
    Although oral sensory feedback is essential for mastication, whether the cortical activity elicited by oral stimulation is associated with the preferred chewing side (PCS) is unclear. Somatosensory evoked fields were measured in 12 healthy volunteers (6 with the right side as the PCS and 6 with the left side as the PCS) following tongue and hard palate stimulation. Three components were identified over the contralateral (P40m, P60m, and P80m) and ipsilateral [P40m(I), P60m(I), and P80m(I)] hemispheres. Since no component was consistently detected across subjects, we evaluated the cortical activity over each hemisphere using the activated root-mean-square (aRMS), which was the mean amplitude of the 18-channel RMS between 10 and 150 ms. For tongue stimulation, the aRMS for each hemisphere was 8.23 +/- 1.55 (contralateral, mean +/- SEM) and 4.67 +/- 0.88 (ipsilateral) fT/cm for the PCS, and 5.11 +/- 1.10 (contralateral) and 4.03 +/- 0.82 (ipsilateral) fT/cm for the non-PCS. For palate stimulation, the aRMS was 5.35 +/- 0.58 (contralateral) and 4.62 +/- 0.67 (ipsilateral) fT/cm for the PCS, and 4.63 +/- 0.56 (contralateral) and 4.14 +/- 0.60 (ipsilateral) fT/cm for the non-PCS. For hard palate stimulation, the aRMS did not differ between the PCS and non-PCS, whereas for tongue stimulation, the contralateral hemisphere aRMS was significantly greater for the PCS than for the non-PCS. Thus, our results show that lateralized cortical activation was associated with the PCS for tongue, but not hard palate, stimulation; a potential reason for this may be the different sensory-inputs between these two areas, specifically the presence or absence of fine motor function. (C) 2014 Elsevier B.V. All rights reserved.
  • Hitoshi Maezawa, Tatsuya Mima, Shogo Yazawa, Masao Matsuhashi, Hideaki Shiraishi, Yoshiyuki Hirai, Makoto Funahashi
    NEUROIMAGE 101 245 - 255 2014年11月 [査読有り][通常論文]
     
    Sophisticated tongue movements contribute to speech and mastication. These movements are regulated by communication between the bilateral cortex and each tongue side. The functional connection between the cortex and tongue was investigated using oscillatory interactions between whole-head magnetoencephalographic (MEG) signals and electromyographic (EMG) signals from both tongue sides during human tongue protrusion compared to thumb data. MEG-EMG coherence was observed at 14-36 Hz and 2-10 Hz over both hemispheres for each tongue side. EMG-EMG coherence between tongue sides was also detected at the same frequency bands. Thumb coherence was detected at 15-33 Hz over the contralateral hemisphere. Tongue coherence at 14-36 Hz was larger over the contralateral vs. ipsilateral hemisphere for both tongue sides. Tongue cortical sources were located in the lower part of the central sulcus and were anterior and inferior to the thumb areas, agreeing with the classical homunculus. Cross-correlogram analysis showed the MEG signal preceded the EMG signal. The cortex-tongue time lag was shorter than the cortex-thumb time lag. The cortex-muscle time lag decreased systematically with distance. These results suggest that during tongue protrusions, descending motor commands are modulated by bilateral cortical oscillations, and each tongue side is dominated by the contralateral hemisphere. (C) 2014 Elsevier Inc. All rights reserved.
  • 前澤 仁志, 美馬 達哉, 矢澤 省吾, 松橋 眞生, 白石 秀明, 舩橋 誠
    臨床神経生理学 42 5 298 - 298 (一社)日本臨床神経生理学会 2014年10月
  • Hiroaki Motegi, Shunsuke Terasaka, Hideaki Shiraishi, Kiyohiro Houkin
    CHILDS NERVOUS SYSTEM 30 7 1313 - 1315 2014年07月 [査読有り][通常論文]
     
    Artery of Percheron (AOP) is a rare variant of thalamoperforating artery with a single common trunk feeding both thalami and with or without contribution to the rostral midbrain. We report the first case of thalamic arteriovenous malformation (AVM) fed by AOP with hemorrhagic onset. A 12-month-old girl suddenly weakened and developed coma. Left thalamic hemorrhage was detected with the third and both lateral ventricles' hematoma. Thalamic AVM was discovered to be fed by an AOP originating from the contralateral posterior cerebral artery. Endovascular embolization of AVM was impossible due to a risk of bilateral thalamic infarction and anatomical inaccessibility. Thalamic AVM was removed by high superior parietal approach without new neurological symptoms. The first case of thalamic AVM fed by AOP originating from the contralateral posterior cerebral artery is reported. Surgical removal of AVM would be at the heart of treatment in order to avoid bilateral thalamic infarction.
  • Hideaki Shiraishi, Kazuhiro Haginoya, Eiji Nakagawa, Shinji Saitoh, Yutaka Kaneko, Nobukazu Nakasato, Derrick Chan, Hiroshi Otsubo
    BRAIN & DEVELOPMENT 36 1 21 - 27 2014年01月 [査読有り][通常論文]
     
    Rationale: Atypical benign partial epilepsy (ABPE) is characterized by centro-temporal electroencephalography (EEG) spikes, continuous spike and waves during sleep (CSWS), and multiple seizure types including epileptic negative myoclonus (ENM), but not tonic seizures. This study evaluated the localization of magnetoencephalography (MEG) spike sources (MEGSSs) to investigate the clinical features and mechanism underlying ABPE. Methods: We retrospectively analyzed seizure profiles, scalp video EEG (VEEG) and MEG in ABPE patients. Results: Eighteen ABPE patients were identified (nine girls and nine boys). Seizure onset ranged from 1.3 to 8.8 years (median, 2.9 years). Initial seizures consisted of focal motor seizures (15 patients) and absences/atypical absences (3). Seventeen patients had multiple seizure types including drop attacks (16), focal motor seizures (16), ENM (14), absences/atypical absences (11) and focal myoclonic seizures (10). VEEG showed centro-temporal spikes and CSWS in all patients. Magnetic resonance imaging (MRI) was reported as normal in all patients. MEGSSs were localized over the following regions: both Rolandic and sylvian (8), peri-sylvian (5), peri-Rolandic (4), parieto-occipital (1), bilateral (10) and unilateral (8). All patients were on more than two antiepileptic medications. ENM and absences/atypical absences were controlled in 14 patients treated with adjunctive ethosuximide. Conclusion: MEG localized the source of centro-temporal spikes and CSWS in the Rolandic-sylvian regions. Centro-temporal spikes, Rolandic-sylvian spike sources and focal motor seizures are evidence that ABPE presents with Rolandic-sylvian onset seizures. ABPE is therefore a unique, age-related and localization-related epilepsy with a Rolandic-sylvian epileptic focus plus possible thalamo-cortical epileptic networks in the developing brain of children. (C) 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
  • 柳生 一自, 下條 暁司, 板垣 俊, 岩田 みちる, 渡辺 隼人, 豊巻 敦人, 大塚 耕右, 白石 秀明, 室橋 春光
    臨床神経生理学 41 5 438 - 438 (一社)日本臨床神経生理学会 2013年10月
  • Keitaro Sueda, Fumiya Takeuchi, Hideaki Shiraishi, Shingo Nakane, Kotaro Sakurai, Kazuyori Yagyu, Naoko Asahina, Shinobu Kohsaka, Shinji Saitoh
    EPILEPSY RESEARCH 104 1-2 68 - 77 2013年03月 [査読有り][通常論文]
     
    Purpose: This study sought to demonstrate the origin and propagation of paroxysmal fast activity (PFA) in patients with epileptic spasms (ESs), using time-frequency analyses of magnetoencephalogram (MEG) PFA recordings. Methods: A 204-channel helmet-shaped MEG, with a 600 Hz sampling rate, was used to examine PEA in 3 children with ESs. We analyzed MEG recordings of PFA by short-time Fourier transform and the aberrant area or high-power spectrum was superimposed onto reconstructed three-dimensional magnetic resonance images as moving images. One ictal discharge was collected. One child and one adult with PFA due to Lennox-Gastaut syndrome were also examined for comparison. Results: All four PFAs in Patient 1 and five PFAs in Patient 3 were generated from one hemisphere. In Patient 2, four of seven PFAs were generated from one hemisphere and the remaining three were generated from both hemispheres. In Patient 3, one ictal MEG showed ictal discharges that were generated from the same area as the PEA, although the electroencephalogram showed no discharge. In Patients with Lennox-Gastaut syndrome, all 10 PFAs were generated from bilateral hemispheres simultaneously.
  • Kotaro Sakurai, Tsugiko Kurita, Youji Takeda, Hideaki Shiraishi, Ichiro Kusumi
    Epilepsy and Behavior Case Reports 1 1 74 - 76 2013年 [査読有り][通常論文]
     
    Akinetopsia is a rare syndrome in which a patient specifically loses the ability to perceive visual motion following bilateral cortical lesions outside the striate cortex. We describe a patient who showed akinetopsia recurrently as epileptic seizures.The patient was a 61-year-old man. At age 46, a cerebral arteriovenous malformation in the right parietal lobe was discovered. At age 58, he began to have a recurrent visual symptom by which smooth movements of objects suddenly appeared, resembling freeze frames in a motion picture. This symptom was paroxysmal and recurrent. Both EEG and magnetoencephalography showed repetitive right temporal spikes. We diagnosed his visual symptom as akinetopsia, which was aroused by hyperexcitability of the right temporal and parietal cortices, including area MT/V5. We administered carbamazepine 200. mg/day, which suppressed his akinetopsic symptom completely. © 2013 The Authors.
  • Isamu Ozaki, Hideaki Shiraishi, Kyousuke Kamada, Shigeki Kameyama, Naohiro Tsuyuguchi, Masato Yumoto, Yutaka Watanabe, Masayuki Hirata, Ryouhei Ishii, Yoshinobu Iguchi, Tomoaki Kimura, Ryosuke Takino, Isao Hashimoto
    CLINICAL NEUROPHYSIOLOGY 123 11 2116 - 2121 2012年11月 [査読有り][通常論文]
     
    Magnetoencephalography (MEG) is a record of the magnetic fields produced by the electrical activities of the brain using MEG systems. There are three types of sensors for MEG systems: magnetometer and two types of gradiometer. Among them, two types of gradiometer, axial and planar, have been used worldwide. Unfortunately, the waveforms recorded by the two types of gradiometer are often different from each other. This poses a serious problem in comparing and evaluating the data from the two gradiometers. We consider that the MEG study should be published in a way that allows other workers using different types of gradiometer to evaluate and replicate the results of MEG studies. There have been, however, no publication criteria for reports of studies on stimulus-evoked or event-related magnetic fields in human subjects. In this article, we propose publication criteria for evoked or event-related magnetic fields of the human brain: original waveforms of selected channels covering a region of interest, a root mean-squared (RMS) waveform and a contour map at an appropriate time. (C) 2012 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserved.
  • Yoshinori Tsurusaki, Shinji Saitoh, Kazuhiro Tomizawa, Akira Sudo, Naoko Asahina, Hideaki Shiraishi, Jun-ichi Ito, Hajime Tanaka, Hiroshi Doi, Hirotomo Saitsu, Noriko Miyake, Naomichi Matsumoto
    NEUROGENETICS 13 4 327 - 332 2012年11月 [査読有り][通常論文]
     
    Whole-exome sequencing of two affected sibs and their mother who showed a unique quadriceps-dominant form of neurogenic muscular atrophy disclosed a heterozygous DYNC1H1 mutation [p.H306R (c.917A > G)]. The identical mutation was recently reported in a pedigree with the axonal form of Charcot-Marie-Tooth disease. Three other missense mutations in DYNC1H1 were also identified in families with dominant spinal muscular atrophy with lower extremity predominance. Their clinical features were consistent with those of our family. Our study has demonstrated that the same DYNC1H1 mutation could cause spinal muscular atrophy as well as distal neuropathy, indicating pleotropic effects of the mutation.
  • Yumi Takahashi, Minako Sugiyama, Yuki Ueda, Tomoshiro Itoh, Kazuyori Yagyu, Hideaki Shiraishi, Yukayo Ukeba-Terashita, Masanori Nakanishi, Tetsuro Nagashima, Tomihiro Imai, Masakatsu Motomura, Shinji Saitoh
    BRAIN & DEVELOPMENT 34 9 784 - 786 2012年10月 [査読有り][通常論文]
     
    Anti-muscle-specific tyrosine kinase antibody (MuSK-Ab) is the second most frequent autoantibody identified in adult patients with myasthenia gravis (MG). Adult patients with MuSK-Ab demonstrate characteristic clinical features but very little information is available for childhood-onset patients with MuSK-positive MG. We report a childhood-onset female patient with MuSK-positive MG. This patient showed basic clinical features compatible with adult-onset MuSK-positive MG, but some features, including spontaneous improvement, are distinct from those in adult patients. Serial examination of MuSK-Ab titers revealed a gross correlation with clinical severity despite significantly high titers throughout the clinical course. Therefore, childhood-onset MuSK-positive MG may demonstrate a distinct clinical characteristics in the early period of illness. (C) 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
  • 尾崎 勇, 白石 秀明, 鎌田 恭輔, 亀山 茂樹, 露口 尚弘, 湯本 真人, 渡辺 裕貴, 平田 雅之, 石井 良平, 橋本 勲
    臨床神経生理学 40 5 493 - 493 (一社)日本臨床神経生理学会 2012年10月
  • 舌と硬口蓋の習慣性咀嚼側刺激により誘発される体性感覚野由来の神経活動 脳磁図を用いた検討
    前澤 仁志, 平井 喜幸, 白石 秀明, 舩橋 誠
    Journal of Oral Biosciences Supplement 2012 133 - 133 (一社)歯科基礎医学会 2012年09月
  • Takeshi Asano, Satoshi Kuroda, Kiyohiro Houkin, Daisuke Yoshida, Kazutoshi Cho, Hideaki Shiraishi, Shinji Saito
    Neurological Surgery 40 6 519 - 525 2012年06月10日 [査読有り][通常論文]
     
    We report a case of vein of Galen aneurysmal malformation (VGAM) with a newly developed dural arteriovenous fistula (AVF) subsequent to successful embolization. A male neonate diagnosed as VGAM with prenatal ultrasonography and MRI presented severe cardiac and respiratory failure soon after birth. Five sessions of transarterial embolization using NBCA were performed during the first 6 months of his life. The shunt flow was effectively reduced and heart failure was resolved after the treatment. Follow-up angiography performed 2.5 years after the last embolization revealed complete obliteration of VGAM and newly developed small dural AVF on the wall of the thrombosed falcorial sinus. We believe that the dural AVF in this case was caused by local venous hypertension or induction of angiogenic factor during the thrombosing process of VGAM.
  • Norimichi Higurashi, Xiuyu Shi, Sawa Yasumoto, Hirokazu Oguni, Masako Sakauchi, Kazuya Komi, Akie Miyamoto, Hideaki Shiraishi, Takeo Kato, Yoshio Makita, Shinichi Hirose
    EPILEPSY RESEARCH 99 1-2 28 - 37 2012年03月 [査読有り][通常論文]
     
    Purpose: To determine the significance of PCDH19 mutations in Japanese females with epilepsy and to delineate their phenotypes. Methods: PCDH19 sequencing analysis was performed in 116 females with various epilepsies, including 97 with Dravet syndrome (83.6%). They were referred for SCN1A analysis, and 52 carried SCN1A mutations. Results: Seven heterozygous mutations in exon 1 were identified in 7 patients (6.0%): 2 frameshift, 2 nonsense, and 3 missense mutations. One patient was a monozygotic twin, and her sister with mild phenotype carried the same mutation. The main clinical features among these 8 patients included early seizure onset (<= 25 months of age), seizure clusters (7/8), fever-associated seizures (7/8), single seizure type (6/8), and late deterioration of intellect (5/8). Seizure durations were generally up to a few minutes, and only one patient developed status epilepticus once. The main seizure types were generalized tonic clonic (4/8), tonic (3/8) and focal seizures, with (2/8) or without secondary generalization (3/8). Myoclonic, atonic and absence seizures were extremely rare. Two patients had Dravet syndrome (25%), and this proportion was significantly smaller than that in the total subjects (p < 0.01). Conclusion: PCDH19 mutation is a relatively frequent cause of epilepsy in Japanese females. Dravet syndrome was rare in our cohort. (C) 2011 Elsevier B.V. All rights reserved.
  • 小児ミトコンドリア心筋症の3例
    武田 充人, 須藤 章, 佐野 仁美, 白石 秀明, 村山 圭, 西野 一三, 山澤 弘州, 武井 黄太, 古川 卓郎, 上野 倫彦
    日本小児科学会雑誌 116 2 375 - 375 (公社)日本小児科学会 2012年02月 [査読有り][通常論文]
  • Kazuyori Yagyu, Keitaro Sueda, Hideaki Shiraishi, Naoko Asahina, Kotaro Sakurai, Shinobu Kohsaka, Yutaka Sawamura, Shinji Saitoh
    EPILEPSIA 52 12 E204 - E206 2011年12月 [査読有り][通常論文]
     
    A dysplastic neuronal lesion of the floor of the fourth ventricle (DNFFV) causes hemifacial seizures (HFS) from early infancy. However, it is still controversial whether HFS is generated by the facial nerve nucleus or cerebellar cortex. In this study, we confirm a direct correlation between the rhythmic activities in the DNFFV and HFS using intraoperative electroencephalography (EEG) and electromyography (EMG) monitoring. Our results support the theory that a DNFFV provokes ipsilateral HFS via the facial nerve nucleus.
  • 淺野 剛, 黒田 敏, 宝金 清博, 長 和俊, 吉田 大介, 白石 秀明, 斎藤 伸治
    JNET: Journal of Neuroendovascular Therapy 5 4 176 - 176 (NPO)日本脳神経血管内治療学会 2011年11月
  • Hideaki Shiraishi, Seppo P. Ahlfors, Steven M. Stufflebeam, Susanne Knake, Pal G. Larsson, Matti S. Haemaelaeinen, Kyoko Takano, Maki Okajima, Keisaku Hatanaka, Shinji Saitoh, Anders M. Dale, Eric Halgren
    JOURNAL OF CLINICAL NEUROPHYSIOLOGY 28 5 431 - 440 2011年10月 [査読有り][通常論文]
     
    Purpose: To compare three methods of localizing the source of epilepti-form activity recorded with magnetoencephalography: equivalent current dipole, minimum current estimate, and dynamic statistical parametric mapping (dSPM), and to evaluate the solutions by comparison with clinical symptoms and other electrophysiological and neuroradiological findings. Methods: Fourteen children of 3 to 15 years were studied. Magnetoencephalography was collected with a whole-head 204-channel helmetshaped sensor array. We calculated equivalent current dipoles and made minimum current estimate and dSPM movies to estimate the cortical distribution of interictal epileptiform discharges in these patients. Results: The results for four patients with localization-related epilepsy and one patient with Landau-Kleffner Syndrome were consistent among all the three analysis methods. In the rest of the patients, minimum current estimate and dSPM suggested multifocal or widespread activity; in these patients, the equivalent current dipole results were so scattered that interpretation of the results was not possible. For 9 patients with localization-related epilepsy and generalized epilepsy, the epileptiform discharges were wide spread or only slow waves, but dSPM suggested a possible propagation path of the interictal epileptiform discharges. Conclusion: Minimum current estimate and dSPM could identify the propagation of epileptiform activity with high temporal resolution. The results of dSPM were more stable because the solutions were less sensitive to background brain activity.
  • 非定型良性小児部分てんかん診断における脳磁図検査の有用性
    白石 秀明, 萩野谷 和裕, 中川 栄二, 香坂 忍, 金子 裕, 須貝 研司, 植松 貢, 柿坂 庸介, 中里 信和, 花谷 亮典, 秋山 倫之, 齋藤 伸治, 大坪 宏
    てんかん研究 29 2 337 - 337 (一社)日本てんかん学会 2011年09月 [査読有り][通常論文]
  • Hideaki Shiraishi
    BRAIN & DEVELOPMENT 33 3 276 - 281 2011年03月 [査読有り][通常論文]
     
    Rationale: Magnetoencephalography (MEG) is useful to localize epileptic foci in epilepsy as MEG has higher spatio-temporal resolution than conventional diagnostic imaging studies; positron emission computed tomography, single photon emission computed tomography and magnetic resonance imaging (MRI). Methods: We use 204-channel helmet-shaped MEG with a sampling rate of 600 Hz. A single dipole method calculates equivalent current dipoles to localize epileptic sources. The equivalent current dipoles are superimposed onto MRI as magnetic source imaging (MSI). fetal MEG data are analyzed using time frequency analysis. The power spectrum density is calculated using short-time Fourier transform and superimposed onto MRI results. Results: Clustered equivalent current dipoles represent epileptogenic zones in patients with localization-related epilepsy. The surgical plan is reliably developed from source localizations of dipoles and power spectrum of interictal spike discharges, and ictal frequency. Conclusion: MEG is indispensable in diagnosis and surgical resection for epilepsy to accurately localize the epileptogenic zone. (C) 2010 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
  • Naoko Asahina, Yukiko Matsunami, Keitaro Sueda, Hideaki Shiraishi, Shinji Saitoh
    PEDIATRICS INTERNATIONAL 52 5 838 - 841 2010年10月 [査読有り][通常論文]
  • Kazuyori Yagyu, Fumiya Takeuchi, Hideaki Shiraishi, Shingo Nakane, Keitaro Sueda, Naoko Asahina, Shinobu Kohsaka, Shuichi Umeoka, Naotaka Usui, Koichi Baba, Shinji Saitoh
    EPILEPSY RESEARCH 90 3 199 - 206 2010年08月 [査読有り][通常論文]
     
    Purpose: Ictal magenetoencephalographic (MEG) discharges convey significant information about ictal onset and propagation, but there is no established method for analyzing ictal MEG. This study sought to clarify the usefulness of time-frequency analyses using short-time Fourier transform (STFT) for ictal onset and propagation of ictal MEG activity in patients with neocortical epilepsy. Methods: Four ictal MEG discharges in two patients with perirolandic epilepsy and one with frontal lobe epilepsy (FLE) were evaluated by time-frequency analyses using STFT. Prominent oscillation bands were collected manually and the magnitudes of those specific bands were superimposed on individual 3D-magnetic resonance images. Results: SIFT showed specific rhythmic activities from alpha to beta bands at the magnetological onset in all four ictal MEG records. Those activities were located at the vicinity of interictal spike sources, as estimated by the single dipole method (SDM), and two of the four ictal rhythmic activities promptly propagated to ipsilateral or bilateral cerebral cortices. The patients with FLE and perirolandic epilepsy underwent frontal lobectomy and resection of primary motor area, respectively including the origin of high-magnitude areas of a specific band indicated by SIFT, and have been seizure free after the surgery. Conclusions: STFT for ictal MEG discharges readily demonstrated the ictal onset and propagation. These data were important for decisions on surgical procedure and extent of resection. Ictal MEG analyses using STFT could provide a powerful tool for noninvasive evaluation of ictal onset zone. (C) 2010 Elsevier B.V. All rights reserved.
  • Kotaro Sakurai, Youji Takeda, Naoaki Tanaka, Tsugiko Kurita, Hideaki Shiraishi, Fumiya Takeuchi, Shingo Nakane, Keitaro Sueda, Tsukasa Koyama
    EPILEPSY RESEARCH 89 2-3 176 - 184 2010年05月 [査読有り][通常論文]
     
    This study uses magnetoencephalography (MEG) to examine whether cortical regions that constitute a default mode network are involved during generalized spike-wave discharges (GSWs) in patients with juvenile absence epilepsy (JAE). We studied five JAE patients for whom MEG was recorded using a 204-channel, whole-head gradiometer system. Dynamic statistical parametric mapping (dSPM) was done to estimate the cortical source distribution of GSW. The dSPM results showed strong medial prefrontal activation in all patients, with activation in the posterior cingulate and precuneus in three of five patients simultaneously or slightly after medial prefrontal activation. Furthermore, dSPM showed that the initial activation of a GSW appears in the focal cortical regions. Cortical regions that constitute a default mode network are strongly involved in the GSW process in some patients with JAE. Results also show that focal cortical activation appears at the onset of a GSW. (C) 2009 Elsevier B.V. All rights reserved.
  • Keitaro Sueda, Furniya Takeuchi, Hideaki Shiraishi, Shingo Nakane, Naoko Asahina, Shinobu Kohsaka, Hideyuki Nakama, Taisuke Otsuki, Yutaka Sawamura, Shinji Saitoh
    EPILEPSY RESEARCH 88 2-3 100 - 107 2010年02月 [査読有り][通常論文]
     
    Purpose: To evaluate the effectiveness of surgery for epilepsy, we analyzed rhythmic fast activity by magnetoencephalography (MEG) before and after surgery using time-frequency analysis. To assess reliability, the results obtained by pre-surgical MEG and intraoperative electrocorticography were compared. Methods: Four children with symptomatic localization-related epilepsy caused by circumscribed cortical lesion were examined in the present study using 204-channel helmet-shaped MEG with a sampling rate of 600 Hz. One patient had dysembryoplastic neuroepithelial. tumor (DNT) and three patients had focal cortical dysplasia (FCD). Aberrant areas were superimposed, to reconstruct 3D MRI images, and illustrated as moving images. Results: In three patients, short-time Fourier transform (STFT) analyses of MEG showed rhythmic activities just above the lesion with FCD and in the vicinity of DNT. In one patient with FCD in the medial. temporal lobe, rhythmic activity appeared in the ipsilateral frontal lobe and temporal lateral aspect. These findings correlate well with the results obtained by intraoperative electrocorticography. After the surgery, three patients were relieved of their seizures, and the area of rhythmic MEG activity disappeared or become smaller. One patient had residual rhythmic MEG activity, and she suffered from seizure relapse. Conclusion: Time-frequency analyses using STFT successfully depicted MEG rhythmic fast activity, and would provide valuable information for pre- and post-surgical evaluations to define surgical strategies for patients with epilepsy. (C) 2009 Elsevier B.V. All rights reserved.
  • Jong Woo Lee, Naoaki Tanaka, Hideaki Shiraishi, Tracey A. Milligan, Barbara A. Dworetzky, Shahram Khoshbin, Steven M. Stufflebeam, Edward B. Bromfield
    JOURNAL OF CLINICAL NEUROPHYSIOLOGY 27 1 7 - 11 2010年02月 [査読有り][通常論文]
     
    EEGs obtained after craniotomy are difficult to read because of a breach rhythm consisting of unfiltered sharply contoured physiologic wave-forms that can mimic interictal epileptiform discharges. Magnetoencephalography (MEG) is less affected by the skull breach. The postcraniotomy EEG and MEG scans of 20 patients were reviewed by two experienced electroencephalographers. Larger interrater variability was found for EEG as compared with MEG. Review of patients who had postoperative seizures suggested that EEG was more sensitive but less specific than MEG in detecting interictal epileptiform discharges. Furthermore, several instances of sharp waveforms that were difficult to evaluate on EEG were found to be more easily interpretable on MEG. MEG may also help determine whether asymmetries in physiologic rhythms on EEG result from the skull defect or are pathologic. MEG should be considered as an adjunctive study in patients with a breach rhythm for evaluation of interictal epileptiform discharges and cerebral dysfunction.
  • てんかん診療における脳磁場解析の進歩
    白石 秀明, 竹内 文也, 末田 慶太朗, 朝比奈 直子, 柳生 一自, 香坂 忍, 中根 進児, 斉藤 伸治
    てんかんをめぐって XXVII 5 - 11 日本てんかん学会-北海道地方会 2009年01月
  • Naoko Asahina, Tohru Shiga, Kiyoshi Egawa, Hideaki Shiraishi, Shinobu Kohsaka, Shinji Saitoh
    JOURNAL OF PEDIATRICS 152 4 546 - 549 2008年04月 [査読有り][通常論文]
     
    Objective To evaluate the role of the gamma-aminobutyric acid type A (GABA(A)) receptor in Angelman syndrome (AS). Study design We performed [C-11]flumazenil positron emission tomography (PET) and examined GABA(A) receptor expression in 7 patients with AS of various genotypes (5 with the deletion, 1 with an imprinting defect [1D], and 1 with a UBE3A mutation) and 4 normal control healthy volunteers. Results Relative to the control subjects, the [C-11]flumazenil binding potentials (BPs) were significantly higher in the cerebral cortex and cerebellum in the 5 patients with the deletion and in the 1 patient with a UBE3A mutation, and were less frequently or barely increased in adult patients with the deletion and in the patient with 1Ds. Conclusions Total GABA(A) receptor expression was increased in patients with AS with various genotypes. We suggest that a developmental dysregulation of the GABA(A) receptor subunits occurs in patients with AS.
  • Kiyoshi Egawa, Naoko Asahina, Hideaki Shiraishi, Kyousuke Kamada, Fumiya Takeuchi, Shingo Nakane, Akira Sudo, Shinobu Kohsaka, Shinji Saitoh
    NEUROIMAGE 39 2 593 - 599 2008年01月 [査読有り][通常論文]
     
    A role for gamma-aminobutyric acid (GABA)ergic inhibition in cortical sensory processing is one of the principle concerns of brain research. Angelman syndrome (AS) is thought to be one of the few neurodevelopmental disorders with GABAergic-related genetic involvement. AS results from a functional deficit of the imprinted UBE3A gene, located at 15q11-q13, resulting mainly from a 4-Mb deletion that includes GABA(A) receptor subunit genes. These genes are believed to affect the GABAergic system and modulate the clinical severity of AS. To understand the underlying cortical dysfunction, we have investigated the primary somatosensory-evoked responses in AS patients. Subjects included eleven AS patients with a 15q11-q13 deletion (AS Del), two AS patients without a 15q11-q13 deletion, but with a UBE3A mutation (AS non-Del), six epilepsy patients (non-AS) and eleven normal control subjects. Somatosensory-evoked fields (SEFs) in response to median nerve stimulation were measured by magnetoencephalography. The N1m peak latency in AS Del patients was significantly longer (34.6 +/- 4.8 ms) than in non-AS patients (19.5 +/- 1.2 ms, P<0.001) or normal control subjects (18.4 +/- 1.8 ms, P<0.001). The next component, P1m, was prolonged and ambiguous and was only detected in patients taking clonazepam. In contrast, SEF waveforms of AS non-Del patients were similar to those of control individuals, rather than to AS Del patients. Thus, GABAergic dysfunction in AS Del patients is likely due to hemizygosity of GABAA receptor subunit genes, suggesting that GABAergic inhibition plays an important role in synchronous activity of human sensory systems. (C) 2007 Elsevier Inc. All rights reserved.
  • Hideaki Shiraishi, Kyoko Takano, Tohru Shiga, Maki Okajima, Akira Sudo, Naoko Asahina, Shinobu Kohsaka, Masakazu Fukuhara, Shinji Saitoh
    BRAIN & DEVELOPMENT 29 8 529 - 533 2007年09月 [査読有り][通常論文]
     
    Landau-Kleffner syndrome (LKS) is a childhood disorder of unknown etiology characterized by an acquired aphasia and epilepsy. We have performed comprehensive neurofunctional studies on an 8-year-old girl with typical LKS, with the aim of identifying lesions that may be responsible for her condition. 18F-fluoro-D-glUCOSe (FDG) positron emission computed tomography (PET), C-11-Flumazenil (FMZ) PET, Tc-99m- hexamethylpropylenearnine oxime single photon emission computed tomography (SPECT) and magnetoencephalography were performed before and after changes to the patient's medication led to a clinical improvement. Interictal SPECT showed hypoperfusion in the left frontal, left temporal, and left occipital lobes. F-18-FDG PET demonstrated a decrease in glucose metabolism medially in both temporal lobes and superiorly in the left temporal lobe. C-11-FMZ PET revealed a deficit in benzodiazepine receptor binding at the tip of the left temporal lobe. Magnetoencephalography demonstrated equivalent current dipoles located superiorly in the left temporal lobe. Our results suggest that the tip of the left temporal lobe plays an important role in the pathogenesis of LKS in our patient. (c) 2007 Elsevier B.V. All rights reserved.
  • Mitsuhiro Kato, Shinji Saitoh, Atsushi Kamei, Hideaki Shiraishi, Yuki Ueda, Manami Akasaka, Jun Tohyama, Noriyuki Akasaka, Kiyoshi Hayasaka
    AMERICAN JOURNAL OF HUMAN GENETICS 81 2 361 - 366 2007年08月 [査読有り][通常論文]
     
    Early infantile epileptic encephalopathy with suppression-burst pattern (EIEE) is one of the most severe and earliest forms of epilepsx,, often evolving into West syndrome; however, the pathogenesis of EIEE remains unclear. ARX is a crucial,gene for the development of interneurons in the fetal brain, and a polyalanine expansion mutation of APX causes mental retardation and seizures, including those of West syndrome, in males. We screened the ARX mutation and found a hemizygous, de novo, 33-bp duplication in exon 2, 298_330dupGCGGCA(GCG)(9), in two of three unrelated male patients with ElEE. This mutation is thought to expand the original 16 alanine residues to 27 alanine residues (A110_A111 insAAAAAAAAAAA) in the first polyalanine tract of the ARX protein. Although ElEE is mainly associated with brain malformations, ARX is the first gene found to be responsible for idiopathic EIEE. Our observation that ElEE had a longer expansion of the polyalanine tract than is seen in West syndrome is consistent with the findings of earlier onset and more-severe phenotypes in ElEE than in West syndrome.
  • Hideaki Shiraishi, Kiyoshi Egawa, Naoko Asahina, Shinji Nakane, Yasuyo Udo, Akira Satake, Shinobu Kohsaka, Shinji Saitoh
    International Congress Series 1300 689 - 692 2007年06月 [査読有り][通常論文]
     
    This study charted the magnetoencephalographic (MEG) findings with clinical course for atypical benign partial epilepsy in childhood (ABPE). We discuss the etiology and epileptogenesis of this condition. Three patients with ABPE (age: 8-10 years old) underwent MEG analysis using a 204ch helmet-shape MEG system. MEG showed unilateral epileptic activities over the frontal and temporal operculum and insular cortex during the worst seizures. The findings changed as the seizures were reduced by localized clustering of equivalent current dipoles (ECDs) at the unilateral temporal and frontal operculum near the primary motor area. We concluded that the localizations and directions of the ECDs in our patients during seizure remission resembled those of benign Rolandic epilepsy (BRE). We suggest a similar pathogenesis for ABPE and BRE. © 2007 Elsevier B.V. All rights reserved.
  • Watanabe Y, Yoshimura K, Shiraishi H
    No to hattatsu. Brain and development 39 174 - 179 3 2007年05月 [査読有り][通常論文]
  • てんかん症例における脳磁律動波に対する周波数解析の試み
    白石 秀明, 竹内 文也, 江川 潔, 末田 慶太朗, 朝比奈 直子, 香坂 忍, 中根 進児, 斉藤 伸治
    てんかんをめぐって XXVI 31 - 36 日本てんかん学会-北海道地方会 2007年03月 
    15歳女児。10歳時に初発したが顕在化せず、12歳時に発作が出現し抗てんかん薬治療を開始したが発作は持続し、14歳時のMRIで左後頭葉・頭頂葉に限局性病変を認め、Dysembryoplastic neuroepithelial tumor(DNT)と診断した。発作間歇時SPECTで腫瘍像と一致した部位に集積欠損領域を認め、左前頭葉集積は右側より低下していた。頭皮上脳波・脳磁図は左半球性に出現する磁場活動上の多棘波に対応し、周波数解析では棘律動が腫瘍部分周辺および腫瘍前方の前頭葉領域に拡延して出現した。腫瘍摘出術は皮質脳波所見の腫瘍縁より一脳回広く脳回切除を施行し、病理所見はDNTであった。術後皮質脳波で前頭葉方向の拡延する棘律動は残存したが術後1年発作症状は認めていない。以上より、律動性棘波は脳磁場計測においても位相、周波数ともにほぼ相同な波形を示し周波数解析法のみが解析可能な方法と考えられた。また、基礎活動としての律動波に対しても磁場解析の可能性が示唆された。
  • S Knake, E Halgren, H Shiraishi, K Hara, HM Hamer, PE Grant, VA Carr, D Foxe, S Camposano, E Busa, T Witzel, MS Hamalainen, SP Ahlfors, EB Bromfield, PM Black, BF Bourgeois, AJ Cole, GR Cosgrove, BA Dworetzky, Madsen, JR, PG Larsson, DL Schomer, EA Thiele, AM Dale, BR Rosen, SM Stufflebeam
    EPILEPSY RESEARCH 69 1 80 - 86 2006年04月 [査読有り][通常論文]
     
    Obective: To evaluate the sensitivity of a simultaneous whole-head 306-channel magnetoencephalography (MEG)/70-electrode EEG recording to detect interictal epileptiform activity (IED) in a prospective, consecutive cohort of patients with medically refractory epilepsy that were considered candidates for epilepsy surgery. Methods: Seventy patients were prospectively evaluated by simultaneously recorded MEG/EEG. All patients were surgical candidates or were considered for invasive EEG monitoring and had undergone an extensive presurgical evaluation at a tertiary epilepsy center. MEG and EEG raw traces were analysed individually by two independent reviewers. Results: MEG data could not be evaluated due to excessive magnetic artefacts in three patients (4%). In the remaining 67 patients, the overall sensitivity to detect IED was 72% (48/67 patients) for MEG and 61% for EEG (41/67 patients) analysing the raw data. In 13% (9/67 patients), MEG-only IED were recorded, whereas in 3% (2/67 patients) EEG-only IED were recorded. The combined sensitivity was 75% (50/67 patients). Conclusion: Three hundred and six-channel MEG has a similarly high sensitivity to record IED as EEG and appears to be complementary. In one-third of the EEG-negative patients, MEG can be expected to record IED, especially in the case of lateral neocortical epilepsy and/or cortical dysplasia. (c) 2006 Elsevier B.V. All rights reserved.
  • Angelman症候群の再発危険率と遺伝カウンセリング
    斉藤 伸治, 江川 潔, 朝比奈 直子, 白石 秀明, 須藤 章
    北海道医学雑誌 81 2 172 - 173 北海道医学会 2006年03月
  • Panayiotopoulos症候群の長期経過と治療効果
    須藤 章, 佐竹 明, 梶井 直文, 江川 潔, 朝比奈 直子, 白石 秀明, 石川 信義, 斉藤 伸治
    てんかんをめぐって XXV 26 - 35 日本てんかん学会-北海道地方会 2006年03月 
    Panayiotopoulos症候群と診断確実な症例の全経過の臨床的特徴を詳細に明らかにするため,けいれん性疾患で2年以上脳波フォローができた440例中,条件を全て満たし,抗てんかん薬中止後2年以上観察できた17例を対象として診療録をもとに後方視的に検討した.熱性けいれんの既往を6例に認めた.両親または兄弟が熱性けいれんまたはてんかんとされた家族歴を有するものを6例で認めた.発作時の年齢は,4歳をピークとして年齢とともに発作症例が減少し,10歳以後の発作症例はなかった.抗てんかん薬使用前後の発作回数は,治療前後で有意差なく,17例中12例で投薬後にも再発した.17例全例で投薬終了後2年間経過観察したが,発作の再発例はなく,脳波異常の再現もなかった
  • 非定型良性小児部分てんかん症例の経時的変化 脳磁図所見,治療経過を中心に
    白石 秀明, 江川 潔, 朝比奈 直子, 須藤 章, 香坂 忍, 斉藤 伸治, 中根 進児, 有働 康代
    てんかんをめぐって XXV 65 - 66 日本てんかん学会-北海道地方会 2006年03月
  • 小林 由典, 山崎 茂, 柳生 一自, 武井 黄太, 白石 秀明, 楠 幸博, 栃丸 博幸, 渡辺 徹, 崎山 幸雄, 横山 健, 岩波 悦勝, 片山 勝之
    臨床小児医学 53 5〜6 93 - 97 (財)小児愛育協会 2005年12月 
    症例1(1歳6ヵ月男児).40℃の発熱,咳嗽で近医を受診,X線にて右下肺野に浸潤影を認め紹介入院となった.白血球数増多,CRP上昇より細菌性肺炎と診断しCTMで解熱したが,再度の発熱でマイコプラズマ感染を疑いAZMを併用し,第14病日目に退院となった.しかし翌日夜に発熱で再診となり,X線で浸潤影の悪化を認め再入院となった.CTM・呼吸理学療法併用で解熱したが,CTM中止で再発熱しPAPM/BPを開始した.原因検索の気管支ファイバーで右主気管支に嵌頓した径5mm大のピーナッツを認め摘出したところ,症状の改善が認められ退院となった.症例2(1歳1ヵ月女児).ピーナッツ摂取後に咳き込み,喘鳴を認めたが,1分以内に改善した.その後,鼻汁で近医を受診,X線にて左肺野の透過性亢進,聴診で呼吸音低下を認め紹介入院となった.所見では,X線で吸気時に明らかな左肺野の透過性亢進,左横隔膜の低下,右肺への縦隔偏位を認め,気管支鏡により左主気管支に8.8×6.2mm大のピーナッツ片を確認,これを摘出した.以後,症状は改善し,第6病日目に退院となった
  • S Knake, C Triantafyllou, LL Wald, G Wiggins, GP Kirk, PG Larsson, SM Stufflebeam, MT Foley, H Shiraishi, AM Dale, E Halgren, PE Grant
    NEUROLOGY 65 7 1026 - 1031 2005年10月 [査読有り][通常論文]
     
    Background: Although detection of concordant lesions on MRI significantly improves postsurgical outcomes in focal epilepsy (FE), many conventional MR studies remain negative. The authors evaluated the role of phased array surface coil studies performed at 3 Tesla (3T PA MRI). Methods: Forty patients with medically intractable focal epilepsies were prospectively imaged with 3T PA-MRI including high matrix TSE T2, fluid attenuated inversion recovery, and magnetization prepared rapid gradient echo. All patients were considered candidates for epilepsy surgery. 3T PA-MRIs were reviewed by a neuroradiologist experienced in epilepsy imaging with access to clinical information. Findings were compared to reports of prior standard 1.5T MRI epilepsy studies performed at tertiary care centers. Results: Experienced, unblinded review of 3T PA-MRI studies yielded additional diagnostic information in 48% (19/40) compared to routine clinical reads at 1.5T. In 37.5% (15/40), this additional information motivated a change in clinical management. In the subgroup of patients with prior 1.5T MRIs interpreted as normal, 3T PA-MRI resulted in the detection of a new lesion in 65% (15/23). In the subgroup of 15 patients with known lesions, 3T PA-MRI better defined the lesion in 33% (5/15). Conclusion: Phased array surface coil studies performed at 3 Tesla read by an experienced unblinded neuroradiologist can improve the presurgical evaluation of patients with focal epilepsy when compared to routine clinical 1.5T studies read at tertiary care centers.
  • H Shiraishi, SP Ahlfors, SM Stufflebeam, K Takano, M Okajima, S Knake, K Hatanaka, S Kohsaka, S Saitoh, AM Dale, E Halgren
    EPILEPSIA 46 8 1264 - 1272 2005年08月 [査読有り][通常論文]
     
    Purpose: To examine whether magnetoencephalography (MEG) can be used to determine patterns of brain activity underlying widespread paroxysms of epilepsy patients, thereby extending the applicability of MEG to a larger population of epilepsy patients. Methods: We studied two children with symptomatic localization-related epilepsy. Case 1 had widespread spikes in EEG with an operation scar from a resection of a brain tumor; Case 2 had hemispheric slow-wave activity in EEG with sensory auras. MEG was collected with a 204-channel helmet-shaped sensor array. Dynamic statistical parametric maps ( dSPMs) were constructed to estimate the cortical distribution of interictal discharges for these patients. Equivalent current dipoles (ECDs) also were calculated for comparison with the results of dSPM. Results: In case 1 with widespread spikes, dSPM presented the major activity at the vicinity of the operation scar in the left frontal lobe at the peak of the spikes, and some activities were detected in the left temporal lobe just before the peak in some spikes. In case 2 with hemispheric slow waves, the most active area was located in the left parietal lobe, and additional activity was seen at the ipsilateral temporal and frontal lobes in dSPM. The source estimates correlated well with the ictal manifestation and interictal single-photon emission computed tomography (SPECT) findings for this patient. In comparison with the results of ECDs, ECDs could not express a prior activity at the left temporal lobe in case 1 and did not model well the MEG data in case 2. Conclusions: We suggest that by means of dSPM, MEG is useful for presurgical evaluation of patients, not only with localized epileptiform activity, but also with widespread spikes or slow waves, because it requires no selections of channels and no time-point selection.
  • H Shiraishi, SM Stufflebeam, S Knake, SP Ahlfors, A Sudo, N Asahina, K Egawa, K Hatanaka, S Kohsaka, S Saitoh, PE Grant, AM Dale, E Halgren
    JOURNAL OF CHILD NEUROLOGY 20 4 363 - 369 2005年04月 [査読有り][通常論文]
     
    Our current purpose is to evaluate the applicability of dynamic statistical parametric mapping, a novel method for localizing epileptiform activity recorded with magnetoencephalography in patients with epilepsy. We report four pediatric patients with focal epilepsies. Magnetoencephalographic data were collected with a 306-channel whole-head helmet-shaped sensor array. We calculated equivalent current dipoles and dynamic statistical parametric mapping movies of the interictal epileptiform discharges that were based in the minimum-L2 norm estimate, minimizing the square sum of the dipole element amplitudes. The dynamic statistical parametric mapping analysis of interictal epileptiform discharges can demonstrate the rapid change and propagation of interical epileptiform discharges. According to these findings, specific epileptogenic lesion-focal cortical dysplasia could be found and patients could be operated on successfully. The presurgical analysis of interictal epileptiform discharges using dynamic statistical parametric mapping seems to be promising in patients with a possible underlying focal cortical dysplasia and might help to guide the placement of invasive electrodes.
  • 山崎 茂, 宮川 雅美, 柳生 一自, 白石 秀明, 楠 幸博, 栃丸 博幸, 渡辺 徹, 崎山 幸雄
    臨床小児医学 53 1〜2 23 - 27 (財)小児愛育協会 2005年04月 
    11歳6ヵ月,男児.気管支喘息の既往があった.1回目の生魚摂食後,大量下血を認め,ステロイドパルス療法により症状は軽快した.しかし血液検査,内視鏡,生検などからアレルギー所見は得られず,原因特定はできなかった.退院1ヵ月後,再度生魚摂取後に下血増量を認め,再入院となった.入院1日目,合計1000ml以上の下血が持続し,顔色不良,頭痛,嘔吐,眩暈感が出現した.MAP,FPP,25%アルブミンの補充を行い,絶飲絶食とした.入院5日目よりプレドニゾロン投与を開始したところ,下血は徐々に改善した.99mテクネチウムシンチグラフィー後,小腸造影にてメッケル憩室を認めたため,メッケル憩室切除術を行った.憩室内側に活動性潰瘍(UL-III)と異所性胃粘膜を認めた.術後,アレルギー検査ではIgE高値を認めたが,魚のプリックテストと皮内テストは陰性で,チャレンジテストでも下血は誘発されなかった.メッケル憩室による下血と診断した.プレドニゾロンを漸減,中止し,入院33日目,全身状態良好で退院した
  • Knake S, Grant PE, Stufflebeam SM, Wald LL, Shiraishi H, Rosenow F, Schomer DL, Fischl B, Dale AM, Halgren E
    Supplements to Clinical neurophysiology 57 494 - 502 2004年 [査読有り][通常論文]
  • 宮川 雅美, 田端 祐一, 武田 宏一郎, 柳生 一自, 白石 秀明, 佐々木 康, 楠 幸博, 栃丸 博幸, 渡辺 徹, 濱田 勇, 崎山 幸雄
    臨床小児医学 51 3〜4 79 - 81 (財)小児愛育協会 2003年08月 
    4ヵ月男児.発熱,発疹を主訴とした.第6病日に,両側眼球結膜充血,苺舌出現,発熱,不定形発疹,四肢先端の紅斑を含め,川崎病と診断した.γグロブリン 2.2g/kgを30時間かけて投与する超大量療法とアスピリンの内服を開始した.24時間後,臨床症状と炎症反応は改善を認めたが,高度肝機能障害が出現した.アスピリンによる副反応の可能性を考え,フルルビプロフェンに変更したが,検査値は改善しなかった.しかし,これらの所見は特に対処することなく次第に改善し,第16病日に退院した.その後,GOT,GPT,T.bilは正常範囲内に回復した.川崎病におけるγグロブリン大量療法は1g/kg日を2日間,または2g/kgの超大量療法が主流となりつつあるが,乳児早期例においては超大量療法はリスクが高いことを留意すべきであると考えられた
  • 柳生一自, 朝比奈直子, 田原泰夫, 宮川雅美, 白石秀明, 楠幸博, 栃丸博幸, 渡辺徹, 槙村浩一, 藤崎竜一, 山口英世, 崎山幸雄
    臨床小児医学 51 1-2 27 - 31 (財)小児愛育協会 2003年04月 [査読有り][通常論文]
     
    12歳男.発熱,咳嗽を主訴とした.2歳時にX連鎖慢性肉芽腫症(X-CGD)と診断さ,4歳時に左肺多発性肺アスペルギルス症と診断された.発熱は遅延し,肺アスペルギルス症が疑われた.喀痰細胞診ではAspergillus(-)であり,アスペルギルスDNA検査も陰性であった.好中球優位の白血球増多を認め,CRP陽性であった.ミカファンギンの点滴静注による治療を開始し,胸部レントゲン像の改善を認めた.第9病日以降は発熱なく経過した.ミカファンギン開始前は陰性であったβ-D-グルカンは第4病日に上昇し,アスペルギルスDNA検査も陽性になった.約2ヵ月間,ミカファンギンによる治療を続け,外来経過観察となった
  • S Kohsaka, S Mizukami, M Kohsaka, H Shiraishi, K Kobayashi
    NEUROSCIENCE 115 3 697 - 706 2002年 [査読有り][通常論文]
     
    The excitability change of the brainstem was investigated before and during the conspicuous epileptic discharge in six patients with generalized convulsive seizures. The discharge consisted of a short duration of recruiting rhythm, which was considered equivalent to the seizure discharge on electroencephalogram. The excitability of the brainstem was measured with the parameters (amplitude and area) of component waves (wave-III and -V) of brainstem auditory evoked potentials. The theoretical background of the analysis is that brainstem auditory evoked potentials are 'far-field' potentials, by which they convey the information on the activity change of the brainstem even during the paroxysmal discharge within the cortex. The excitability of both the ventral (parameters of wave-III) and the dorsal brainstem (parameters of wave-V) exhibited a synchronized change (activation-inactivation). They were enhanced from -2.4 +/- 0.4 s, reaching the maxima before the onset of the seizure discharge, and decayed corresponding to the emergence of the recruiting rhythm. The results suggest the possibility that the widespread (ventral and dorsal) and synchronized activation of the brainstem triggers the seizure discharge in human generalized epilepsy. During the widespread activation of the brainstem, both the thalamus and the cortex probably undergo a suppressed inhibitory state through the cholinergic activation, precipitating the seizure discharge. (C) 2002 IBRO. Published by Elsevier Science Ltd. All rights reserved.
  • H Shiraishi, Y Watanabe, M Watanabe, Y Inoue, T Fujiwara, K Yagi
    EPILEPSIA 42 7 875 - 882 2001年07月 [査読有り][通常論文]
     
    Purpose: To determine whether magnetoencephalography (MEG) has any clinical value for the analysis of seizure discharges in patients with medial frontal lobe epilepsy (FLE). Methods: Four patients were studied with 74-channel MEG. Interictal and ictal electroencephalographic (EEG) and MEG recordings were obtained. The equivalent current dipoles (ECDs) of the MEG spikes were calculated. Results: In two patients with postural seizures, interictal EEG spikes occurred at Cz or Fz. The ECDs of interictal MEG spikes were localized around the supplementary motor area. In the other two patients with focal motor or oculomotor seizures, interictal EEG spikes occurred at Fz or Cz. The ECDs of interictal MEG spikes were localized at the top of the medial frontal region. The ECDs detected at MEG ictal onset were also localized in the same area as those of the interictal discharges. Conclusions: In medial FLE patients, interictal and ictal MEG indicated consistent ECD localization that corresponded to the semiology of clinical seizures. Our findings demonstrate that MEG is a useful tool for detecting epileptogenic focus.
  • Danon病1家系における臨床的,筋病理学的,分子遺伝学的検討
    高橋 真紀, 高野 亨子, 白石 秀明, 和田 敬仁, 久保田 健夫, 後藤 雄一, 山本 彩香, 西野 一三, 斉藤 伸治
    脳と発達 33 Suppl. S139 - S139 (一社)日本小児神経学会 2001年05月 [査読有り][通常論文]
  • H Shiraishi, T Fujiwara, Y Inoue, K Yagi
    EPILEPSIA 42 3 393 - 397 2001年03月 [査読有り][通常論文]
     
    Purpose: We examined the incidence and distribution of photosensitivity among the different age groups and different types of epilepsies and epileptic syndromes. Furthermore, we considered the influence of ethnic and geographic factors on the incidence of photoparoxysmal response (PPR) in epilepsy patients. Methods: We analyzed the responses to intermittent photic stimulation (TPS) by using a Grass PS22 or PS33 photic stimulator for in 2,187 unselected patients with epilepsy who were treated in our center. Results: The classic PPR was elicited in 37 (1.7%) patients. The mean age of these 37 patients was 17.0 years. The subpopulation of patients having PPR included 2.0% of all patients with symptomatic generalized epilepsy, 5.6% (p < 0.01) of those with idiopathic generalized epilepsy, 0.7% of those with symptomatic localization-related epilepsy, and 2.9% of those with undetermined epilepsy. PPR accounted for 17.4 % (p < 0.01) of the patients with juvenile myoclonic epilepsy, 7.6% (p < 0.01) of those with grand mal on awakening, and 6.1% (p < 0.01) of those with symptomatic occipital lobe epilepsy. The incidence of PPR increased in patients up to age 15 years, and suddenly decreased after age 20 years. Conclusion: The present study presents the first report from eastern Asia, analyzing the incidence of PPR with a restricted definition comparable to the other studies, and the rate of PPR was relatively low compared with the studies performed in the European countries. We could confirm the clear relation between age and positive PPR.
  • 酸性マルターゼが正常なリソソーム性糖原病 Danon病1家系における臨床的,筋病理学的,分子遺伝学的検討
    高橋 真紀, 高野 亨子, 白石 秀明, 和田 敬仁, 斉藤 伸治, 久保田 健夫, 後藤 雄一, 山本 彩香, 西野 一三
    小児科診療 65 1 139 - 140 (株)診断と治療社 2001年01月 [査読有り][通常論文]

その他活動・業績

  • 渡辺隼人, 渡辺隼人, 下條暁司, 下條暁司, 白石秀明, 横澤宏一 日本生体磁気学会誌 35 (1) 2022年
  • 柳生一自, 渡辺隼人, 渡辺隼人, 高野一義, 下條暁司, 白石秀明, 横澤宏一, 齊藤卓弥 臨床神経生理学(Web) 48 (5) 2020年
  • 渡辺隼人, 渡辺隼人, 下條暁司, 柳生一自, 高野一義, 横澤宏一, 村上優衣, 村上優衣, 白石秀明, 齊藤卓弥 日本生体磁気学会誌 33 (1) 2020年
  • 医療安全に関するWGからの提言 脳波等神経生理検査時の鎮静における医療安全に関する提言・指針の作成
    是松 聖悟, 宮本 雄策, 村松 一洋, 山中 岳, 白石 秀明, 吉永 治美, 中川 栄二, 稲垣 真澄, 金村 英秋, 小林 勝弘 脳と発達 51 (Suppl.) S172 -S172 2019年05月 [査読無し][通常論文]
  • 関節運動を伴う筋弛緩時の脳磁場変化
    菅原 和広, 齊藤 秀和, 臼井 桂子, 篠崎 淳, 松橋 眞生, 白石 秀明, 長峯 隆 臨床神経生理学 46 (5) 435 -435 2018年10月 [査読無し][通常論文]
  • 関節運動を伴う筋弛緩時の脳磁場変化
    菅原 和広, 齊藤 秀和, 臼井 桂子, 篠崎 淳, 松橋 眞生, 白石 秀明, 長峯 隆 臨床神経生理学 46 (5) 435 -435 2018年10月 [査読無し][通常論文]
  • Midori Nakajima, Simeon Wong, Elysa Widjaja, Shiro Baba, Tohru Okanishi, Lynne Takada, Yosuke Sato, Hiroki Iwata, Maya Sogabe, Hikaru Morooka, Robyn Whitney, Yuki Ueda, Tomoshiro Ito, Kazuyori Yagyu, Ayako Ochi, O Carter Snead 3rd, James T Rutka, James M Drake, Sam Doesburg, Fumiya Takeuchi, Hideaki Shiraishi, Hiroshi Otsubo Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology 129 (6) 1182 -1191 2018年06月 [査読有り][通常論文]
     
    OBJECTIVE: To investigate whether advanced dynamic statistical parametric mapping (AdSPM) using magnetoencephalography (MEG) can better localize focal cortical dysplasia at bottom of sulcus (FCDB). METHODS: We analyzed 15 children with diagnosis of FCDB in surgical specimen and 3 T MRI by using MEG. Using AdSPM, we analyzed a ±50 ms epoch relative to each single moving dipole (SMD) and applied summation technique to estimate the source activity. The most active area in AdSPM was defined as the location of AdSPM spike source. We compared spatial congruence between MRI-visible FCDB and (1) dipole cluster in SMD method; and (2) AdSPM spike source. RESULTS: AdSPM localized FCDB in 12 (80%) of 15 children whereas dipole cluster localized six (40%). AdSPM spike source was concordant within seizure onset zone in nine (82%) of 11 children with intracranial video EEG. Eleven children with resective surgery achieved seizure freedom with follow-up period of 1.9 ± 1.5 years. Ten (91%) of them had an AdSPM spike source in the resection area. CONCLUSION: AdSPM can noninvasively and neurophysiologically localize epileptogenic FCDB, whether it overlaps with the dipole cluster or not. SIGNIFICANCE: This is the first study to localize epileptogenic FCDB using MEG.
  • Tomoshiro Ito, Masashi Narugami, Kiyoshi Egawa, Hiroyuki Yamamoto, Naoko Asahina, Shinobu Kohsaka, Atsushi Ishii, Shinichi Hirose, Hideaki Shiraishi Brain & development 40 (3) 226 -228 2018年03月 [査読有り][通常論文]
     
    Alternating hemiplegia of childhood (AHC) is a rare neurological disease mainly caused by mutations in the ATP1A3 gene and showing varied clinical severity according to genotype. Patients with a p.Gly755Ser (p.G755S) mutation, one of minor genotypes for AHC, were recently described as having a mild phenotype, although their long-term outcomes are still unclear due to the lack of long-term follow up. Here, we demonstrate the full clinical course of a 43-year-old female AHC patient with p.G755S mutation. Although her motor dysfunction had been relatively mild into her 30 s, she showed a subsequent severe aggravation of symptoms that left her bedridden, concomitant with a recent recurrence of seizure status. The seizures were refractory to anti-epileptic drugs, but administration of flunarizine improved seizures and the paralysis. Our case suggests that the phenotype of AHC with p.G755S mutation is not necessarily mild, despite such a presentation during the patient's younger years.
  • 下條暁司, 柳生一自, 曽根原剛志, 渡辺隼人, 品田大成, 前田珠希, 白石秀明, 横澤宏一, 齊藤卓弥 日本生体医工学会大会プログラム・抄録集(Web) 57th 2018年
  • Tetsuo Onda, Masaya Uchida, Kiyotaka Kosugiyama, Michihiko Ueno, Hideaki Shiraishi Journal of the Japan Epilepsy Society 35 (3) 715 -721 2018年 [査読有り][通常論文]
     
    Previous studies have estimated that one medial temporal lobe epilepsy (MTLE) patient per 100,000 population is a good candidate for surgery, but these patients are easily overlooked if the attendant physician and/or the patient's guardian do not recognize the specific seizure symptoms. Over a period of 9 years in the western Iburi district of Hokkaido prefecture (population 205,000), we have encountered three surgical cases of MTLE patients who have become seizure free. All three patients had been diagnosed initially as having drug-resistant unclassifiable symptomatic localization-related epilepsy. All three patients were eventually diagnosed as having MTLE because of an awareness of the characteristic clinical symptoms of MTLE and results of neuroimaging and electrophysiological studies however, it took 5-11 years (mean 7.3 years) from the onset of the epilepsy attacks until a diagnosis of MTLE was made and 6-11 years (mean 8.3 years) until the surgeries were performed. Because the initial seizures in MTLE generally occur in patients around 5-10 years of age, pediatricians are the attendant physicians for the initial diagnosis and treatment. Thus, it is important that general pediatricians are aware of the clinical features of MTLE to ensure a favorable prognosis for their patients.
  • S. Kohsaka, M. Kohsaka, H. Shiraishi EPILEPSIA 58 S7 -S7 2017年12月 [査読無し][通常論文]
  • 限局性皮質異形成に対して準緊急的てんかん手術を施行した新生児例
    信田 大喜子, 鎌田 晃嘉, 辻岡 孝郎, 内田 雅也, 伊藤 智城, 植松 貢, 岩崎 真樹, 白石 秀明, 上野 倫彦 臨床小児医学 65 (1-6) 42 -46 2017年12月 [査読無し][通常論文]
     
    背景:限局性皮質異形成(focal cortical dysplasia:FCD)は、難治性てんかんの原因の一つで、時に胎児期・新生児早期より発作が発現する。FCDによる難治性てんかんは、乳児期早期の手術介入を必要とする場合がある。症例:日齢1より覚醒時に両眼瞼のちく溺から始まり、両口角の痙攣・左右不定に非対称の四肢強直を来す発作を認め、ほぼ重積状態となった。頭部MRI・脳波・核医学検査で右前頭・頭頂部葉に跨がる皮質異形成が疑われた。発作は複数の抗てんかん薬内服によっても抑制されず、重積状態が持続した。日齢61に脳回異常部を準緊急的に切除した。病理診断はFCD IIaおよび多小脳回であった。術後に発作は消失し、明らかな麻痺なく発達伸長も認めている。結語:本症例は新生児早期からFCDを基盤とする発作が重積し、薬物治療効果が乏しく、病変切除術を施行し発作の抑制を得た。(著者抄録)
  • 木村修平, 信田大喜子, 辻岡孝郎, 内田雅也, 上野倫彦, 白石秀明, 笹森徹, 寳金清博, 江夏怜, 越智さと子, 三國信啓, 佐野仁美 日本小児科学会雑誌 121 (11) 1890 -1890 2017年11月01日 [査読無し][通常論文]
  • 難治性てんかんに対して迷走神経刺激療法を導入した3例
    木村 修平, 信田 大喜子, 辻岡 孝郎, 内田 雅也, 上野 倫彦, 白石 秀明, 笹森 徹, 寳金 清博, 江夏 怜, 越智 さと子, 三國 信啓, 佐野 仁美 日本小児科学会雑誌 121 (11) 1890 -1890 2017年11月 [査読無し][通常論文]
  • 中高生における読字障害スクリーニング、診断のための質問紙および音読検査の開発
    柳生 一自, 須山 聡, 前田 珠希, 白石 秀明, 齊藤 卓弥 日本児童青年精神医学会総会抄録集 58回 166 -166 2017年10月 [査読無し][通常論文]
  • 幻視を有するparkinsonism患者6例における視覚誘発脳磁場の検討
    長沼 亮滋, 高橋 育子, 松島 理明, 矢部 一郎, 白石 秀明, 森下 きらり, 高橋 香代子, 中根 進児, 佐々木 秀直 臨床神経生理学 45 (5) 454 -454 2017年10月 [査読無し][通常論文]
  • 磁気共鳴画像がエメリー・ドレイフス型筋ジストロフィーの心臓病変の管理に有用と考えられた一例(Usefulness of cardiomagnetic resonance imaging in a patient with Emery-Dreifuss muscular dystrophy)
    山澤 弘州, 武田 充人, 泉 岳, 佐々木 理, 阿部 二郎, 佐々木 大輔, 白石 秀明, 西野 一三 脳と発達 49 (Suppl.) S362 -S362 2017年05月 [査読無し][通常論文]
  • ローレンツプロットを用いた体性感覚誘発脳磁場における活動の持続時間の評価
    齊藤 秀和, 矢澤 省吾, 篠崎 淳, 白石 秀明, 松橋 眞生, 長峯 隆 日本生体磁気学会誌 30 (1) 152 -153 2017年 [査読無し][通常論文]
  • 環境省「エコチル調査」開始4年を経ての集計結果の報告 全国と北海道の参加者の喫煙・食物アレルギー等について
    土川 陽子, 西條 泰明, 伊藤 善也, 吉岡 英治, 川西 康之, 堤 裕幸, 遠藤 俊明, 馬場 剛, 小林 正樹, 水上 尚典, 有賀 正, 長 和俊, 白石 秀明, 千石 一雄, 宮本 敏伸, 東 寛, 荒木 敦子, 池野 多美子, 宮下 ちひろ, 伊藤 佐智子, 湊屋 街子, 小林 澄貴, アイツバマイ ゆふ, 山崎 圭子, 西原 進吉, 田村 菜穂美, 岸 玲子 北海道母性衛生学会誌 45 33 -38 2016年08月 [査読無し][通常論文]
  • Kazuyori Yagyu, Takuya Saitoh, Atsushi Shimojo, Hideaki Shiraishi, Satoshi Suyuma INTERNATIONAL JOURNAL OF NEUROPSYCHOPHARMACOLOGY 19 174 -174 2016年06月 [査読無し][通常論文]
  • 乳児期早期に発症した皮質形成異常に伴う難治てんかん7例の検討
    植松 有里佳, 植松 貢, 及川 善嗣, 大久保 幸宗, 岩崎 真樹, 柿坂 庸介, 白石 秀明, 赤坂 真奈美, 亀井 淳, 鈴木 雄一, 加藤 朝子, 呉 繁夫 脳と発達 48 (2) 138 -138 2016年03月 [査読無し][通常論文]
  • 北海道のエコチル調査の集計結果 生後2歳までの食物アレルギーの頻度および食品の摂食状況について
    アイツバマイ ゆふ, 西條 泰明, 伊藤 善也, 村林 宏, 吉岡 英治, 川西 康之, 水上 尚典, 有賀 正, 長和 俊, 白石 秀明, 千石 一雄, 宮本 敏伸, 東 寛, 堤 裕幸, 遠藤 俊明, 馬場 剛, 小林 正樹, 荒木 敦子, 池野 多美子, 宮下 ちひろ, 伊藤 佐智子, 土川 陽子, 湊屋 街子, 小林 澄貴, 山崎 圭子, 西原 進吉, 田村 菜穂美, 岸 玲子 北海道公衆衛生学雑誌 29 (1特別付録) 36 -36 2015年11月 [査読無し][通常論文]
  • 環境省「エコチル調査」開始3年を経ての集計データの報告と調査予定
    土川 陽子, 西條 泰明, 伊藤 善也, 吉岡 英治, 川西 康之, 堤 裕幸, 遠藤 俊明, 馬場 剛, 小林 正樹, 水上 尚典, 有賀 正, 長 和俊, 白石 秀明, 千石 一雄, 宮本 敏伸, 東 寛, 荒木 敦子, 池野 多美子, 宮下 ちひろ, 伊藤 佐智子, 岸 玲子 北海道母性衛生学会誌 44 25 -30 2015年08月 [査読無し][通常論文]
  • 齊藤 秀和, 矢澤 省吾, 篠崎 淳, 白石 秀明, 松橋 眞生, 長峯 隆 日本生体磁気学会誌 28 (1) 136 -137 2015年06月 [査読無し][通常論文]
  • 運動遂行における荷重負荷の影響 緩徐脳磁場による検討
    豊島 貴信, 矢澤 省吾, 村原 貴史, 長峯 隆, 白石 秀明, 松橋 眞生, 下濱 俊 臨床神経学 53 (12) 1418 -1418 2013年12月 [査読無し][通常論文]
  • 脳磁図の臨床応用:虚血性脳血管障害領域での最近の動向と将来展望
    池田英敏, 坂本真一, 尾崎 勇, 井口義信, 平田雅之, 亀山茂樹, 石井良平, 白石秀明, 渡辺裕貴, 橋本 勲露口尚弘, 鎌田恭輔, 中里信和, 宇田武弘 第28回日本生体磁気学会 2013年06月 [査読無し][招待有り]
  • 脳磁図を用いた小児疾患診断の臨床応用:最近の動向と将来展望
    白石秀明, 尾崎 勇, 井口義信, 石井良平, 鎌田恭輔, 亀山茂樹, 露口尚弘, 中里信和, 平田雅之, 渡辺裕貴, 橋本 勲 第28回日本生体磁気学会 2013年06月 [査読無し][招待有り]
  • 脳磁図の臨床応用: 精神科疾患・認知症領域での最近の動向と将来展望
    石井良平, 渡辺裕貴, 青木保典, 平田雅之, 白石秀明, 尾崎 勇, 井口義信, 露口尚弘, 鎌田恭輔, 亀山茂樹, 中里信和, 橋本 勲, 武田雅俊 第28回日本生体磁気学会 2013年06月 [査読無し][招待有り]
  • 脳磁図の臨床応用:脳腫瘍治療に関する歴史的背景と最近の動向
    鎌田恭輔, 露口尚弘, 中里信和, 尾崎 勇, 池田英敏, 井口義信, 平田雅之, 亀山茂樹, 石井良平, 白石秀明, 渡辺裕貴, 橋本 勲 第28回日本生体磁気学会 2013年06月 [査読無し][招待有り]
  • 脳磁図の臨床応用:神経内科・リハビリテーション領域での最近の動向と将来展望
    尾崎 勇, 井口義信, 白石秀明, 石井良平, 平田雅之, 露口尚弘, 鎌田恭輔, 渡辺裕貴, 亀山茂樹, 橋本 勲 第28回日本生体磁気学会 2013年06月 [査読無し][招待有り]
  • 末田 慶太朗, 竹内 文也, 白石 秀明, 中根 進児, 櫻井 高太郎, 柳生 一自, 朝比奈 直子, 香坂 忍, 斉藤 伸治 北海道醫學雜誌 = Acta medica Hokkaidonensia 88 (2) 109 -109 2013年04月01日
  • 石井 良平, 渡辺 裕貴, 青木 保典, 平田 雅之, 白石 秀明, 尾﨑 勇, 井口 義信, 露口 尚弘, 鎌田 恭輔, 亀山 茂樹, 中里 信和, 橋本 勲, 武田 雅俊 臨床神経生理学 41 (1) 29 -45 2013年02月 [査読無し][通常論文]
     
    脳磁図は, 脳電気生理活動を直接測定できる, 高時間空間分解能を有する, 基準電極が必要無いため誤ったセンサー間の相関を生じない等優れた特性を持ち, この脳磁図を用いることにより脳電気生理活動を即時的でより精密に測定できると考えられている。また, 脳磁図測定データに対する解析手法も発展してきており, 電流源密度分布推定法, 脳部位間の相関解析, 脳磁図波形複雑性解析等が実用化されている。この脳磁図と脳磁図解析法を用いて精神科疾患の病態解明を目的に臨床研究も盛んに行われるようになってきており, 様々な研究成果が報告されている。本論文では, 2012年3月までの間に発表された文献検索に基づき主要な精神科疾患・認知症疾患に関する論文を渉猟し, 脳磁図臨床研究の動向を調べた。その結果, 統合失調症95本, 気分障害23本, 発達障害29本, てんかん精神病13本, アルツハイマー病と軽度認知機能障害を含む認知症71本の論文をリストアップし, 各疾患で代表的な論文についてその研究成果を概説した。精神科疾患・認知症に対する臨床的有用性を強調した論文も散見されたが, 米国医療政策研究局のエビデンスレベル分類で2以上の評価を得た論文は, 認知症を対象とした2編のみであった。今後は, 精神科疾患・認知症に対して, 被験者数をより多く集め, 多施設間の共同研究による, より客観的な脳磁図の臨床研究が行われていくことが期待される。
  • 鎌田 恭輔, 露口 尚弘, 中里 信和, 尾﨑 勇, 池田 英敏, 井口 義信, 平田 雅之, 亀山 茂樹, 石井 良平, 白石 秀明, 渡辺 裕貴, 橋本 勲 臨床神経生理学 41 (1) 46 -53 2013年02月 [査読無し][通常論文]
     
    脳腫瘍の術前診断として, 腫瘍の性状, 局在に加え腫瘍近傍の脳機能マッピングが重要である。しかし, 開頭術前に脳機能局在を確認する方法は未だに十分に確立していない。現在脳磁図は脳神経外科手術に際して保険適応となっている唯一の脳機能画像方法であるが, その有用性などに関するエビデンスレベルはあまり論じられていない。今後の脳磁図診断の臨床応用の方向性を見極めるために, 現在の脳腫瘍に対する脳磁図の診断的エビデンスレベルを明らかにする。MEDLINE にて (brain tumor OR neoplasm) AND (MEG OR magnetoencephalography) を1985年から2012年7月まで検索した。その結果920件の論文が検索され, エビデンスレベル, 原著論文に基づいて56論文に絞りこみ現在までの脳腫瘍に対する脳磁図の臨床応用についてまとめた。1993年に体性感覚誘発脳磁界を用いて脳腫瘍術前診断で中心溝を同定した報告は初めての臨床応用であった。その後脳磁図は同様の目的で脳腫瘍患者に使用され続け, 多くのエビデンスレベル1の論文が報告された。また1999年より言語優位半球の同定のために脳磁図の応用が始まり, 現在までエビデンスレベルの高い6編の論文があった。しかし, その同定率が90%を超える論文は少なく, さらなる手法の工夫が必要と考えられた。さらに近年はResting state脳磁図のように課題負荷の少ない検査法の報告も認められ, 今後患者負担の少ない検査法への発展も期待できる。中心溝同定, 言語機能局在に関しては, 脳磁図は脳腫瘍術前診断として推奨グレードの高い有用な検査法である。
  • 柳生一自, 下條暁司, 板垣俊, 岩田みちる, 渡辺隼人, 豊巻敦人, 大塚耕右, 白石秀明, 室橋春光 臨床神経生理学 41 (5) 2013年
  • 白石 秀明 睡眠医療 : 睡眠医学・医療専門誌 7 (2) 195 -201 2013年
  • 尾﨑 勇, 橋本 勲, 井口 義信, 白石 秀明, 石井 良平, 平田 雅之, 露口 尚弘, 鎌田 恭輔, 渡辺 裕貴, 亀山 茂樹 臨床神経生理学 41 (2) 57 -70 2013年 [査読無し][通常論文]
     
    脳磁図の科学的エビデンスは, てんかん以外の疾患において明らかでない。本研究では文献検索に基づき神経内科疾患と神経リハビリテーションの脳磁図臨床研究の動向を調べることを目的とした。MEDLINE にて (amyotrophic lateral sclerosisなど個々の対象疾患名) AND (MEG OR magnetoencephalography) を検索して2011年11月までに発表された論文を抽出した。この中から原著論文をえらび, エビデンスレベル, 抄録内容に基づいて, 神経内科疾患ではALS 3編, Parkinson病8編, 多発性硬化症4編について概要をまとめた。神経リハビリテーションについては虚血性脳血管障害からの回復に関わる研究に焦点を絞り, 運動感覚障害9編, 失語症3編について概要をまとめた。27編のうち診断・治療方針の決定に関するエビデンスレベルはグレード1: 0編, 2: 18編, 3: 3編, 4: 6編, 5: 0編, 6: 0編であった。脳磁図は局所の自発活動を非侵襲的に計測できることから, 少数症例の検討ながら, 複数の領域間の機能的連絡あるいは視床下核などの深部組織の活動と脳局所のコヒーレンスなど, 疾患や回復過程に伴う脳機能変化や疾患固有の病態生理を理解する上で新しい知見をもたらすことがわかった。
  • 運動準備から遂行に移行する瞬間における制御機構の研究 音欠落に対する反応運動を用いて
    豊島 貴信, 矢澤 省吾, 村原 貴史, 白石 秀明, 松橋 眞生, 長峯 隆, 下濱 俊 臨床神経生理学 40 (5) 441 -441 2012年10月 [査読無し][通常論文]
  • 運動イメージ中の脳活動 脳磁場による検討
    齊藤 秀和, 矢澤 省吾, 村原 貴史, 玉 珍, 豊島 貴信, 白石 秀明, 松橋 眞生, 竹田 里江, 長峯 隆, 松山 清治 臨床神経生理学 40 (5) 493 -493 2012年10月 [査読無し][通常論文]
  • Colored Density Spectral Array(CDSA)を用いたCSWSをもつてんかんの評価(第2報) 正常脳波との比較
    伊藤 智城, 最上 友紀子, 高山 留美子, 大谷 英之, 秋山 倫之, 池田 浩子, 今井 克美, 白石 秀明, 重松 秀夫, 高橋 幸利, 井上 有史 てんかん研究 30 (2) 417 -417 2012年09月 [査読無し][通常論文]
  • 白石 秀明, 尾崎 勇, 井口 義信, 石井 良平, 鎌田 恭輔, 亀山 茂樹, 露口 尚弘, 中里 信和, 平田 雅之, 渡辺 裕貴, 橋本 勲 臨床神経生理学 : Japanese journal of clinical neurophysiology 40 (4) 203 -208 2012年08月01日 [査読無し][通常論文]
     
    脳磁図検査は, てんかん, 脳血管障害, 認知機能異常などの神経疾患に対して行われて来た。しかし, 小児疾患の中で, てんかん以外は科学的根拠が現状において証明されていない。本研究では, 文献検索に基づき, 小児における神経疾患において, 脳磁図検査がどのように行われているのかを調査した。検索は, MEDLINEを用いて, child AND (MEG OR magnetoencephalography) で行った。検索により, 2010年6月までで, 93論文が検索された。この中で, てんかん, 一症例のみの症例報告, 総説を除外すると, 14論文が見出された。それらの対象疾患は, Dioxin暴露, 脳室周囲白質軟化症, 多小脳回, 白皮症, もやもや病, Angelman症候群, 書字障害, 注意欠陥多動障害, 広汎性発達障害であった。これら研究は全て後ろ向きの対照比較研究で, エビデンスレベルは2aが10論文, 2bが4論文で, レベル1はなかった。推奨グレードは全てBであった。脳磁図検査は小児神経疾患研究において, てんかん研究以外では一般的ではないが, 非侵襲的検査であり, 乳幼児から繰り返し何度も検査を施行できるという利点を持つことから, 発達脳における変化を考察する上で, 脳磁図検査は有力な検査法となることが期待される。
  • 露口 尚弘, 鎌田 恭輔, 中里 信和, 宇田 武弘, 池田 英敏, 坂本 真一, 尾崎 勇, 井口 義信, 平田 雅之, 亀山 茂樹, 石井 良平, 白石 秀明, 渡辺 裕貴, 橋本 勲 臨床神経生理学 : Japanese journal of clinical neurophysiology 40 (4) 195 -202 2012年08月01日 [査読無し][通常論文]
     
    PETやPerfusion CTのような様々な脳検査法で脳の循環代謝を測定することは, 虚血巣をふくむ領域の脳機能の評価に有用である。しかし, 脳の循環動態は直接的に脳の神経活動を反映しているものではない。容積伝導した神経活動を表す頭皮脳波において, 急性期の脳虚血巣での徐波の出現が知られているが, 空間分解能, 定量性において脳機能の客観的指標とするには問題があった。脳磁図はこの脳波の短所をカバーできるものと期待され, 虚血性脳疾患への臨床応用がなされつつある。しかし, 脳磁図は, てんかん以外の疾患においては科学的エビデンスは明らかでない。本研究では文献検索に基づき虚血性脳疾患の脳磁図臨床研究の動向を調べMEDLINE にて (stroke OR cerebral ischemia) AND (MEG OR magnetoencephalography) を検索2010年7月までで58論文が検索された。この中から原著論文をえらび, エビデンスレベル, 抄録内容に基づいて25論文に絞りこみ現在脳磁図がどのように利用されているかを調べた。さらにエビデンスレベルはグレード2以上の12編について検討してまとめた。総じて虚血脳での脳磁図の変化を報告した文献が多く, 診断・治療方針の決定に関してはエビデンスレベルの高い論文もあるもののごく少数の報告に限られており, 今後の研究に期待しなければならない。しかし, 虚血に伴う脳の障害程度を脳循環代謝以外の方法で明らかにできることは意義深いと考えられる。
  • 突発刺激に対し高頻度反応課題遂行を中断する際の脳活動
    玉 珍, 矢澤 省吾, 村原 貴史, 斎藤 秀和, 白石 秀明, 松橋 眞生, 松山 清治, 長峯 隆 日本生体磁気学会誌 25 (1) 110 -111 2012年06月 [査読無し][通常論文]
  • 白石 秀明, 尾崎 勇, 井口 義信, 石井 良平, 鎌田 恭輔, 亀山 茂樹, 露口 尚弘, 中里 信和, 長峯 隆, 平田 雅之, 湯本 真人, 渡辺 裕貴, 橋本 勲 臨床神経生理学 : Japanese journal of clinical neurophysiology 40 (3) 119 -130 2012年06月01日 [査読無し][通常論文]
     
    脳磁図検査を施行している国内36施設に対して, 脳磁図検査がどのような症例に対して行われ, どのような検査が行われているのかに関して, 平成21年5月に, 郵送による質問紙調査を行った。また, これらの施設で, 検査を施行している医師, 検査技師が施行上, あるいは財政的にどんな問題に直面しているのかに関しても質問した。24施設 (回答率67%) より回答を得た。年間150~200症例の検査を施行している施設が多い一方, 50例以下の施設も多くあった。脳磁図検査を依頼された患者の臨床診断では, てんかんと脳腫瘍がほぼ半数を占め, 認知症, 脳血管障害, 変性疾患がこれに次いでいた。患者の年齢層は幅広かったが, 60歳以上の症例が16%に達していた。脳神経外科医と精神科医が脳磁図の記録と解析の主体を担っており, 約半数の施設では専任臨床検査技師が配置されていなかった。脳磁図記録のプロトコルには, 自発脳磁場解析, 体性感覚誘発脳磁場, 聴覚誘発脳磁場, 視覚誘発脳磁場, 運動関連脳磁場, 言語優位半球同定が含まれていた。多くの施設で, てんかん患者の自発脳磁場解析の為にてんかん性棘波の自動解析ソフトウエアの開発が望まれていた。年間の維持経費は500~2,000万円の施設が多い一方, 83%の施設で赤字を計上していた。健康保険による脳磁図検査の適応拡大が, これらの負債の軽減に必要であることが示唆された。
  • 平田 雅之, 亀山 茂樹, 後藤 哲, 柳澤 琢史, 貴島 晴彦, 押野 悟, 吉峰 俊樹, 井口 義信, 石井 良平, 尾崎 勇, 鎌田 恭輔, 白石 秀明, 露口 尚弘, 渡辺 裕貴, 橋本 勲 臨床神経生理学 : Japanese journal of clinical neurophysiology 40 (3) 140 -146 2012年06月01日 [査読無し][通常論文]
     
    脳磁図は2004年に保険適用検査となったが, 科学的エビデンスは必ずしも明らかでない。本研究では文献検索にもとづきてんかんの脳磁図臨床研究の動向を調べた。MEDLINE にてepilepsy AND (MEG OR magnetoencephalography OR (magnetic source imaging)) を検索, 2010年7月までで961論文が検索された。これをエビデンスレベル, 抄録内容にもとづいて65論文に絞りこみ内容を調べた。エビデンスレベルはグレード1: 0編, 2: 3編, 3: 20編, 4: 22編, 5: 14編, 6: 6編であった。総じて有用性を報告したものが多く, 特に診断・治療方針の決定に関してはエビデンスレベルの高い論文もあり, てんかん焦点源検索や頭蓋内電極留置部位決定に関して頭皮脳波より優れていると考えられた。現在, 脳磁図の保険適用は手術適応例に対する術前検査であるが, 手術適応評価法としての位置づけがむしろ適切と考えられる。
  • 対側優位の脳波所見を有する片側広汎性萎縮性病変の難治てんかんに対し大脳半球離断術が著効した2例
    高山 留美子, 今井 克美, 須藤 章, 白石 秀明, 南 弘一, 伊藤 智城, 九鬼 一郎, 秋山 麻里, 最上 友紀子, 秋山 倫之, 池田 浩子, 重松 秀夫, 馬場 好一, 高橋 幸利, 井上 有史 脳と発達 44 (Suppl.) S269 -S269 2012年05月 [査読無し][通常論文]
  • 小児神経筋疾患における心筋症の初期病変に関する考察
    武田 充人, 須藤 章, 白石 秀明, 齋藤 伸治 脳と発達 44 (Suppl.) S239 -S239 2012年05月 [査読無し][通常論文]
  • 鷲見 佳泰, 村元 恵美子, 中村 博彦, 溝渕 雅広, 白石 秀明, 臼居 礼子 Japanese Journal of Neurosurgery 21 (9) 736 -740 2012年 [査読無し][通常論文]
     
    症例は34歳の男性. 6歳時に数分間意識減損する発作で初発し, 難治性に経過した. 発作は, 軽度右ジストニアを伴う両上肢自動症で, 発作時頭皮脳波は左前側頭部に律動性θ波が出現し, 左側頭葉起始を示唆した. 一方, MRIは右海馬硬化, 18F-FDG PETは右側頭葉代謝低下, 11C-FMZ PETは右側頭葉内側の集積低下を認め, 右側頭葉起始を示唆した. 頭蓋内脳波記録は, 発作発射が右側頭葉内側に起始し早期に左側頭葉へ移行して左優位に経過した. 選択的右扁桃体海馬切除術を行い, 術後発作は消失した. 頭皮脳波・発作症候と画像所見が一致しない例でも, MRI・PET所見が一致している場合は, 手術側の決定に有用である.
  • 反応課題遂行を中断する際の脳磁場活動 解析法の検討
    矢澤 省吾, 村原 貴史, 玉 珍, 齋藤 秀和, 白石 秀明, 松橋 眞生, 長峯 隆 日本生理学雑誌 73 (12) 277 -277 2011年12月 [査読無し][通常論文]
  • 反応課題遂行を中断する際の運動関連領野の関与 背景脳活動の検討
    矢澤 省吾, 村原 貴史, 玉 珍, 齋藤 秀和, 白石 秀明, 松橋 眞生, 長峯 隆 臨床神経生理学 39 (5) 445 -445 2011年10月 [査読無し][通常論文]
  • 白石 秀明 脳と発達 43 (5) 2011年09月01日 [査読無し][通常論文]
  • 反応課題遂行を中断する際の運動関連領野の関与
    矢澤 省吾, 村原 貴史, 石黒 雅敬, 長峯 隆, 竹田 里江, 豊島 貴信, 白石 秀明, 松橋 眞生 日本生理学雑誌 72 (12) 256 -256 2010年12月 [査読無し][通常論文]
  • Prader-Willi症候群を疑われる児の鑑別診断
    斉藤 伸治, 細木 華奈, 植田 佑樹, 伊藤 智城, 高橋 有美, 柳生 一自, 白石 秀明, 太田 亨, 新川 詔夫 脳と発達 42 (6) 474 -474 2010年11月 [査読無し][通常論文]
  • S. Kohsaka, H. Shiraishi, S. Saitoh, M. Kohsaka EPILEPSIA 51 96 -96 2010年06月 [査読無し][通常論文]
  • 白石 秀明 臨床脳波 52 (3) 127 -133 2010年03月 [査読無し][通常論文]
  • Hideaki Shiraishi, K. Haginoya, N. Nakasato, Y. Kakisaka, E. Nakagawa, K. Sugai, D. Chan, H. Otsubo EPILEPSIA 50 178 -178 2009年11月 [査読無し][通常論文]
  • 運動遂行における荷重負荷の影響 脳磁場計測による検討
    豊島 貴信, 矢澤 省吾, 村原 貴史, 竹田 里江, 石黒 雅敬, 白石 秀明, 松橋 眞生, 下濱 俊, 長峯 隆 臨床神経生理学 37 (5) 340 -340 2009年10月 [査読無し][通常論文]
  • S. Kohsaka, M. Kohsaka, H. Shiraishi, S. Saitoh EPILEPSIA 50 8 -9 2009年10月 [査読無し][通常論文]
  • 白石 秀明 日本小児科学会雑誌 113 (4) 667 -676 2009年04月01日 [査読無し][通常論文]
  • 柳生 一自, 櫻井 高太郎, 伊藤 智城, 高橋 有美, 末田 慶太朗, 朝比奈 直子, 白石 秀明, 武田 洋司, 澤村 豊, 中根 進児, 斉藤 伸治 てんかん研究 26 (3) 2009年01月31日 [査読無し][通常論文]
  • 白石 秀明 Epilepsy 2 (2) 84 -86 2008年11月 [査読無し][通常論文]
  • 白石 秀明, 伊藤 智城, 高橋 有美, 柳生 一自, 朝比奈 直子, 香坂 忍, 佐竹 明, 中根 進児, 斉藤 伸治 てんかん研究 26 (2) 309 -309 2008年09月30日 [査読無し][通常論文]
  • 櫻井 高太郎, 武田 洋司, 栗田 紹子, 白石 秀明, 竹内 文也, 中根 進児, 小山 司 てんかん研究 26 (2) 343 -343 2008年09月30日 [査読無し][通常論文]
  • Hideaki Shiraishi, Shinji Saitoh NEUROSCIENCE RESEARCH 61 S34 -S34 2008年 [査読無し][通常論文]
  • Derrick Chan, Kazuhiro Haginoya, H. Shiraishi, Eiji Nakagawa, Kenji Sugai, H. Otsubo EPILEPSIA 49 211 -212 2008年 [査読無し][通常論文]
  • 白石 秀明, 末田 慶太朗, 竹内 文也, 朝比奈 直子, 中根 進児, 香坂 忍, 澤村 豊, 斉藤 伸治 てんかん研究 25 (3) 2007年09月30日 [査読無し][通常論文]
  • 白石 秀明, 竹内 文也, 江川 潔, 末田 慶太朗, 朝比奈 直子, 香坂 忍, 中根 進児, 斉藤 伸治 CI研究 : progress in computed imaging 29 (1) 43 -49 2007年06月30日 [査読無し][通常論文]
  • 渡辺 裕貴, 芳村 勝城, 白石 秀明 脳と発達 = OFFICIAL JOURNAL OF THE JAPANESE SOCIETY OF CHILD NEUROLOGY 39 (3) 174 -179 2007年05月01日 [査読無し][通常論文]
     
    脳磁図はてんかん棘波の発生源を等価電流双極子 (ECD) として表示することができる.てんかん棘波の大きさをあらわすECDの電流モーメントは症候性部分てんかんでは診断にかかわらず同様の値を示すが, 良性小児てんかん (BECT) の電流モーメントはそれらよりも明らかに大きく, また, 焦点内での分布にも特徴がある. MRI画像にみられる病変とECDとの位置関係にはそれぞれ特徴があり, 各病変がもつてんかん原性の強さを示していると考えられるため, 外科的な治療を考える上で参考となる.
  • 伊藤智成, 土畠智幸, 西間木敦子, 武井黄太, 楠幸博, 渡辺徹, 崎山幸雄, 白石秀明, 寺江聡 てんかんをめぐって 26 69-70 2007年03月31日 [査読無し][通常論文]
  • 脊髄動静脈奇形を原因とするクモ膜下出血の1乳児例
    佐藤 泰征, 江川 潔, 朝比奈 直子, 白石 秀明, 斉藤 伸治, 有賀 正 日本小児科学会雑誌 110 (4) 593 -593 2006年04月 [査読無し][通常論文]
  • HASHIMOTO Isao, KAKIGI Ryusuke, NAGAMINE Takashi, NAKASATO Nobukazu, SHIRAISHI Hideaki, WATANABE Yutaka 臨床神経生理学 : Japanese journal of clinical neurophysiology 33 (4) 231 -252 2005年08月01日
  • 臨床脳磁図検査解析指針
    橋本 勲, 柿木 隆介, 白石 秀明, 中里 信和, 長峯 隆, 渡辺 裕貴, 日本臨床神経生理学会脳磁図ガイドライン作成委員会 臨床神経生理学 33 (2) 69 -86 2005年04月 [査読無し][通常論文]
  • H Shiraishi, SP Ahlfors, K Takano, M Okajima, N Asahina, A Sudo, S Kohsaka, S Knake, SM Stufflebeam, E Halgren, K Hatanaka, S Saitoh EPILEPSIA 46 18 -18 2005年 [査読無し][通常論文]
  • A Sudo, A Satake, A Kajii, K Egawa, N Asahina, H Shiraishi, N Ishikawa, S Saitoh EPILEPSIA 46 22 -22 2005年 [査読無し][通常論文]
  • H Shiraishi, K Egawa, N Asahina, A Sudo, S Nakane, Y Udo, A Satake, S Kohsaka, S Saitoh EPILEPSIA 46 175 -175 2005年 [査読無し][通常論文]
  • 臨床脳磁図検査解析指針試案(2004)
    橋本 勲, 柿木 隆介, 白石 秀明, 中里 信和, 長峯 隆, 渡辺 裕貴, 日本臨床神経生理学会脳磁図ガイドライン作成委員会 臨床神経生理学 32 (4) (1) -(19) 2004年08月 [査読無し][通常論文]
  • 柳生 一自, 宮川 雅美, 白石 秀明 臨床小児医学 52 (1) 27 -32 2004年04月 [査読無し][通常論文]
     
    6歳男児,9歳男児,12歳男児.症例は3例ともに発熱,白血球減少,網状赤血球の低下を認め,2例で血小板減少を認めた.また,2例では体幹部を中心とした出血斑を認めたが,特発性血小板減少性紫斑病やアレルギー性紫斑病とは臨床像が異なっていた.他の1例では骨髄検査で血球貪食像を認め,更に肝脾腫,肝機能障害もみられた.それぞれ発熱から5,5,8日目にHPV-B19-IgM抗体の出現を認め,ヒトパルボウイルスB19感染症の確定診断に至った
  • 白石 秀明, 高野 亨子, 岡嶋 真紀, 須藤 章, 香坂 忍, 畑中 啓作, 斉藤 伸治 てんかん研究 22 (1) 2004年02月29日 [査読無し][通常論文]
  • 臨床脳磁図検査解析指針試案
    渡辺 裕貴, 中里 信和, 今井 克巳, 白石 秀明, 二宮 宏智, MEGガイドライン作成グル 臨床神経生理学 32 (1) (1) -(13) 2004年02月 [査読無し][通常論文]
  • S Kohsaka, S Mizukami, H Shiraishi, M Kohsaka EPILEPSIA 45 75 -75 2004年 [査読無し][通常論文]
  • K Takano, M Takahashi, H Shiraishi, A Sudo, S Saitoh, S Kohsaka, T Shiga, M Fukuhara EPILEPSIA 45 64 -64 2004年 [査読無し][通常論文]
  • 白石 秀明, Ahlfors Seppo P, 高野 亨子, 岡嶋 真紀, 朝比 奈直子, 須藤 章, 香坂 忍, Knake Susanne, Stufflebeam Steven M, Halgren Eric, 畑中 啓作, 斉藤 伸治 日本てんかん学会プログラム・予稿集 0 (37) 173 -173 2003年10月30日 [査読無し][通常論文]
  • 光感受性てんかん 突発反応の脳波・脳磁図による検討
    白石 秀明, 渡辺 裕貴, 井上 有史, 藤原 建樹, 八木 和一 厚生省精神・神経疾患研究委託費研究報告書 新技術を用いたてんかん等の診断法と治療法の開発 平成12年度 111 -117 2001年10月 [査読無し][通常論文]
     
    2187名の癲癇症例を対象に,二つのPPR定義(Binnieの基準,Waltsの基準)を用い,定義の違いによる,癲癇類型,癲癇症候群分類,臨床発作,光誘発発作を比較検討し,又,これらの脳波反応に一致して出現した脳磁反応の変化について検討した.そのうちPPR陽性症例は広義の基準であるWaltsらの基準の2倍までを含めて4.2%であった.類型別検討では特発性全般癲癇症例で陽性率が高かった.PPR陽性症例の平均年齢はclassic PPRで一番低く,Waltsの2倍で一番高かった.光感受性が強いとされるBickford A群ではclassicPPR陽性群症例ではBickford群症例が多かった.脳磁図による検討では癲癇類型別,PPR波形別に脳磁反応の出現様式が異なることが示された
  • 香坂 忍, 水上 晋, 白石 秀明, 香坂 雅子 日本てんかん学会プログラム・予稿集 0 (35) 141 -141 2001年09月27日 [査読無し][通常論文]
  • 高野 亨子, 高橋 真紀, 白石 秀明, 須藤 章, 斎藤 伸治, 香坂 忍, 志賀 哲, 福原 正和 日本てんかん学会プログラム・予稿集 0 (35) 164 -164 2001年09月27日 [査読無し][通常論文]
  • 新技術を用いたてんかん等の診断法と治療法の開発 てんかん外科とMEG 内側側頭葉てんかんにおけるてんかん原性焦点の側方性とMEG dipoleの相関
    三原 忠紘, 渡辺 裕貴, 白石 秀明, 松田 一己, 鳥取 孝安, 大坪 俊昭, 松山 望, 井上 有史, 八木 和一 厚生省精神・神経疾患研究委託費による研究報告集 平成11年度 22 -22 2000年12月 [査読無し][通常論文]
     
    側頭葉内側構造に限局する棘波はMEGでは検出されないが,外科治療の適応となる難治症例の棘波は,かなりの強さを持ち広い範囲が同期しやすい傾向にあるのでMEGで検出される.MEGで検出された場合の所見は,てんかん原性焦点側の推定に有用である.特に両側の側頭葉にMEG dipoleの集積を認める症例では,bitemporal lobe epilesyを疑って,頭蓋内脳波を行った法が良い
  • 新技術を用いたてんかん等の診断法と治療法の開発 光感受性てんかん 光刺激と光突発反応の位相差について
    渡辺 裕貴, 白石 秀明, 井上 有史, 藤原 建樹, 八木 和一 厚生省精神・神経疾患研究委託費による研究報告集 平成11年度 5 -5 2000年12月 [査読無し][通常論文]
     
    MEGの反応は主に皮質興奮を表すと考えられていることから,全般てんかんでclassic PPRが出現しない時,また局在関連てんかんでclassic PPRが出現するときは,IPSと皮質反応は互いに同期して出現しているが,全般てんかんでclassic PPRが出現するときは,これらの同期性が認められなくなることが示された
  • 山田 康一郎, 白石 秀明, 渡辺 裕貴, 八木 和一, 滝川 守国 てんかん研究 = Journal of the Japan Epilepsy Society 18 (3) 204 -211 2000年10月31日 [査読無し][通常論文]
     
    視覚発作を有する10例の症候性後頭葉てんかん患者に対して脳波、脳磁図同時記録を施行し、脳磁図上の発作間歇時発射を解析して得られた単一等価電流双極子 (single equivalent current dipole: 以下ECD) の局在位置について検討した。ECDの局在位置と視覚発作症状との間に相関があり、後頭葉内側にECDが局在する症例では要素性幻視を、後頭葉外側に局在する症例では複雑性幻視やそれに続いて他葉起源と思われる症状を呈する傾向があった。この違いは後頭葉各部位の機能と関連していると思われた。後頭葉てんかんにおける脳磁図の有用性が確認されたとともに、後頭葉てんかんの診断をする上で視覚発作の内容の吟味がてんかん焦点の局在位置を推定する際に重要であることが示唆された。
  • 脳波・筋電図の臨床 光突発反応の脳波学的検討
    白石 秀明, 井上 有史, 藤原 建樹, 八木 和一 臨床脳波 42 (5) 306 -311 2000年05月 [査読無し][通常論文]
     
    代表的な光突発反応(PPR)の定義であるBinnieによるclassic PPR,およびWaltzらによるPPRの定義を用い,PPRと癲癇類型・癲癇症候群分類,臨床発作,光誘発発作との関係を比較検討した.2187例中37例にclassic PPRを認めた.光刺激時間を4秒間とし,また反応が出現した時点でただちに刺激を中止するという手法を用い,光刺激と関係なく生じる自発性癲癇性異常波の混入をできるだけ排除した.この基準を用いたPPR陽性症例の年齢群による分布は11歳から15歳にピークを持つ曲線となった.個々の症例における経年変化は検討していないが,PPR陽性症例においてPPRの波形が加齢とともに軽微な反応に変化している可能性が示唆された
  • てんかん外科とMEG Cortical Dysplasiaの外科治療におけるMEGの役割
    三原 忠紘, 渡辺 裕貴, 山田 康一郎, 白石 秀明, 深尾 憲二朗, 松田 一己, 鳥取 孝安, 大坪 俊昭, 馬場 好一, 臼井 直敬, 井上 有史, 八木 和一 厚生省精神・神経疾患研究委託費による研究報告集 平成10年度 113 -113 1999年12月 [査読無し][通常論文]
     
    多数のMEG双極子が外側皮質に限局して集積している場合,この所見の信憑性は高いと考えられるので,これらの症例では,MEG所見に基づいて外科戦略を組み立てる,即ち,頭蓋内脳波を省略できるか,電極をどこに配備するか,また双極子の集積部位をtargetにした切除外科が可能になると期待される
  • 光感受性てんかん 光感受性てんかんの症候学・脳波学的検討
    渡邊 裕貴, 白石 秀明, 井上 有史, 藤原 建樹, 八木 和一 厚生省精神・神経疾患研究委託費による研究報告集 平成10年度 117 -117 1999年12月 [査読無し][通常論文]
     
    てんかん患者2187例についてPPRの陽性率を求めた.てんかん全体のPPR陽性率は1.7%で,てんかん類型別ではIGEとUDEが,症候群別にみると全般てんかんでは若年性ミオクロニーてんかんと覚醒時大発作てんかんが,部分てんかんでは後頭葉てんかんのみが高い陽性率を示した.またPPR陽性率には強い年齢依存性を認めた
  • 白石 秀明, 山田 康一郎, 深尾 憲二朗, 渡邉 雅子, 渡邉 裕貴, 藤原 建樹, 八木 和一 日本てんかん学会プログラム・予稿集 0 (33) 121 -121 1999年10月22日 [査読無し][通常論文]
  • 藤原 建樹, 白石 秀明, 渡辺 宏雄, 小澤 武司, 川崎 峰雄, 高橋 幸利, 八木 和一 日本てんかん学会プログラム・予稿集 0 (33) 153 -153 1999年10月22日 [査読無し][通常論文]
  • 白石 秀明, 渡邉 裕貴, 渡邉 雅子, 重松 秀夫, 山田 康一郎, 藤原 建樹, 八木 和一 てんかん研究 = Journal of the Japan Epilepsy Society 17 (3) 200 -204 1999年10月01日 [査読無し][通常論文]
     
    前頭葉内側表面に限局性皮質病変を持つ、前頭葉てんかん患者について、発作問欠時、発作時の脳磁図所見が、この病変近傍のてんかん原性を示唆した症例を経験した。症例は17歳女性で、1歳2ヵ月時より両口角が下に引かれ、四肢の伸展強直を来たす発作が日単位で持続していた。発作間欠時脳波ではFz~Czの棘波、あるいは両側広汎性に出現する棘波を認めた。発作時脳波では上記の発作問欠時のてんかん波が増加した後、全野で活動が抑制され、その後広汎性の棘律動が出現し、脳波上発作起始部位の同定は不可能であった。MRI上、左前頭葉内側皮質に限局性病変を認め、脳磁図において発作問欠時に出現する棘波の等価電流双極子を求めたところ、病変近傍に双極子の局在が認められた。脳磁図は空間分解能に優れる特性から、大脳半球内側表面でのてんかん原性焦点の検索において有用であると考えた。
  • 光突発反応の脳波学的検討
    白石 秀明, 高橋 幸利, 渡邊 裕貴, 井上 有史, 藤原 建樹, 八木 和一 脳波と筋電図 27 (2) 211 -211 1999年04月 [査読無し][通常論文]
  • 側頭葉てんかんの磁気棘双極子分布型と手術成績
    深尾 憲二朗, 渡辺 裕貴, 白石 秀明, 山田 康一郎, 三原 忠紘, 井上 有史, 藤原 建樹, 八木 和一 脳波と筋電図 27 (2) 215 -215 1999年04月 [査読無し][通常論文]
  • てんかん類型・症候群別の光突発反応の頻度
    白石 秀明, 井上 有史, 藤原 建樹, 八木 和一 てんかん研究 17 (1) 51 -51 1999年02月 [査読無し][通常論文]
  • 白石 秀明, 井上 有史, 藤原 建樹, 八木 和一 日本てんかん学会プログラム・予稿集 0 (32) 111 -111 1998年10月09日 [査読無し][通常論文]
  • 深尾 憲二朗, 渡辺 裕貴, 鳥辺 泰久, 白石 秀明, 山田 康一郎, 松田 一己, 三原 忠紘, 井上 有史, 藤原 建樹, 八木 和一 日本てんかん学会プログラム・予稿集 0 (32) 148 -148 1998年10月09日 [査読無し][通常論文]
  • 白石秀明 BRAIN MEDICAL 6 145 -152 1998年
  • 深尾 憲二朗, 渡辺 裕貴, 鳥辺 泰久, 白石 秀明, 松田 一己, 井上 有史, 三原 忠紘, 藤原 建樹, 八木 和一 日本てんかん学会プログラム・予稿集 0 (31) 143 -143 1997年09月18日 [査読無し][通常論文]
  • 太田 八千雄, 南部 春生, 浜田 勇, 岡嶋 覚, 植竹 公明, 服部 哲夫, 三浦 正次, 白石 秀明 日本小児科学会雑誌 100 (5) 941 -945 1996年05月01日 [査読無し][通常論文]
  • 白石 秀明, 山田 諭, 服部 哲夫, 三浦 正次, 太田 八千雄, 南部 春生, 浜田 勇 日本小児科学会雑誌 99 (6) 1154 -1158 1995年06月01日 [査読無し][通常論文]

共同研究・競争的資金等の研究課題

  • 胎児期環境の神経発達障害と脳の性分化および肥満への影響解明:出生コーホート研究
    日本学術振興会:科学研究費助成事業 基盤研究(A)
    研究期間 : 2019年04月 -2023年03月 
    代表者 : 岸 玲子, 荒木 敦子, 小林 澄貴, 齊藤 卓弥, 三浦 りゅう, 湊屋 街子, 中島 そのみ, 宮下 ちひろ, 白石 秀明, アイツバマイ ゆふ, 山崎 圭子
  • 子どもの言語機能はどのように形成され修復されるのか?-脳磁図による検討-
    日本学術振興会:科学研究費助成事業 基盤研究(C)
    研究期間 : 2017年04月 -2020年03月 
    代表者 : 白石 秀明, 竹内 文也, 柳生 一自
     
    言語遅滞児に対し、言語機能発達段階を脳磁図によって検討し定量化・比較を行う為に、昨年度に引き続き5名の患児に対して、複数回の検討を行なった。北海道大学病院に設置されている306ch脳磁計を使用し、独自に開発した文字情報混合装置を用いて、患児の好む任意のDVD画像に文字情報を組み込み、これを約10分間の時間を単位として約40分間視聴することによって、文字刺激を行った。刺激情報は、遠距離焦点の強化プロジェクターを用いて眼前に置いたスクリーンに直接投影し、この画像を患児に見せた。 言語刺激に対する、事象関連脱同期反応を検討した。独自に開発した、Matlab toolを用いた、解析ソフトウエアを用いて、任意の時間範囲、周波数、脳の部位における、事象関連脱同期反応を、図示した。この検討の上で、ヒトの基本的な基礎活動である、10Hz帯域の基礎活動(α律動)周辺の5~15Hzの基礎活動に特に注目した。 経年的変化を観察している急性脳症後に言語を獲得しつつある患児、Angelman症候群で発語はないものの、言語理解が進んでいる兄弟例、先天性筋症で発語はないものの言語理解が予想される患児に関して、検討を行なった。いずれかの時期で、言語優位半球の分化が得られてくるのではとの予想を立てていたが、急性脳症後の11歳患児(脳症罹患後10年)において、昨年から左半球優位の、10Hz帯域における有意な事象関連脱同期が見出されるようになったが、この結果は安定していた。他の症例に関しては、まだ、両側性の反応があるが、検査に順応して来たため、安定した結果が得られるようになった。今後も、縦断的な研究の継続が望まれた。
  • 読字障害における中間表現型:認知・知覚機能のサブグループ化と介入支援方法の開発
    日本学術振興会:科学研究費助成事業 基盤研究(C)
    研究期間 : 2016年04月 -2020年03月 
    代表者 : 柳生 一自, 橋本 竜作, 関 あゆみ, 齊藤 卓弥, 須山 聡, 白石 秀明
     
    H29年度までは、北海道大学病院小児科・精神科外来にて延べ30名程度の限局性学習症と診断される児童をリクルートしてきた。全例について臨床表現系としての各種検査(WISC-IV、音読検査など)を行ってきた。また認知・知覚的表現系については音韻処理課題の準備を行い、眼球運動機能検査についてもGazefinderを用いた課題を作成した。またDTVP-3を海外から購入し、今後は標準データの取得を含めて検討していく予定である。またタブレット機器とタッチペンを用いた書字評価ソフトウェアの開発を行った。今後は微細運動困難を原因とする書字障害児の評価方法を確立する予定である。 脳神経基盤研究については20名以上のディスレクシア群および14名の対照群で音読時の脳活動について脳磁図を用いて測定した。現在これによって得られた結果からは、無意味単語音読時の左側頭葉上側頭部の活動低下、有意味単語音読時の左下前頭葉の活動低下、さらに絵の呼称時に右下前頭葉の活動亢進が明らかとなった。特に絵の呼称時の脳活動の違いは、読字障害をもつ子どもたちが、文字だけでなく視覚刺激に対する反応が異なることを示しており、視覚刺激にて通常と異なる脳内経路を使用していることが示唆された。一部の研究では読字障害をもつ人は、視覚的・空間的な把握能力に優れているという報告もなされており、本研究で得られた結果は、こうした脳活動のそもそもの違いを示しているのかもしれない。 H30年度、研究代表者は1年間米国Massachusetts General Hospitalに勤務し、主に脳磁図および機能的MRIの解析について研鑽を積んだ一方で、国内臨床におけるデータ解析については遅れかが生じたため、研究期間の延長を申請した。今後、米国での研究を生かして臨床データを用いた解析を行うことで、より精緻な研究成果が見込める。
  • 日本学術振興会:科学研究費助成事業 基盤研究(C)
    研究期間 : 2014年04月 -2017年03月 
    代表者 : 白石 秀明, 竹内 文也, 柳生 一自
     
    小児の言語機能はどのように形成され、発達に伴って変化していくのかに関して、脳磁図を用いて6年間に渡って、変化を検討してきた。これまで、サルの研究では当初右側大脳半球が言語機能を司り、3歳頃に左側に移っていくのではないかとされていたが、私達の研究では、当初、両方の大脳半球が同時に活動した後に、数年で言語優位半球が決定されていくのではないか、ということが初めてわかった。 特に急性脳炎など、中途障害を来した子どもたちの、リハビリテーションの為の指標を、脳磁図を用いて指し示す事が出来る可能性があることが示された。これらの研究を継続することにより、脳の発達過程が解明されることを今後の目標としたい。
  • 日本学術振興会:科学研究費助成事業 基盤研究(A)
    研究期間 : 2013年04月 -2016年03月 
    代表者 : 岸 玲子, 池野 多美子, 荒木 敦子, 宮下 ちひろ, 中島 そのみ, 佐々木 成子, 花岡 知之, 梶原 淳睦, 松浦 英幸, 室橋 春光, 白石 秀明, 佐田 文宏, 西條 泰明, 吉岡 英治, 安住 薫, 安住 薫
     
    母児20000組を対象とした北海道内40産院コホートで、妊娠中の化学物質、特に喫煙・ダイオキシン類・有機フッ素化合物に対する曝露と、学童期までの児の発育、神経行動発達および免疫アレルギーとの関連を検討した。妊娠中の化学物質曝露が(1) PPARβ遺伝子多型を介して血清脂質に、(2) 母のDNA修復(XRCC1)遺伝子多型を介して児の出生体重に、(3) 児のIGF2のDNAメチル化に、そして(4) 1.5歳児の神経行動発達に影響することを明らかにした。加えて、学童期のADHDに関するリスク要因が検討され、妊娠中の飲酒や喫煙がリスクとなることが明らかにされた。
  • 日本学術振興会:科学研究費助成事業 挑戦的萌芽研究
    研究期間 : 2013年04月 -2015年03月 
    代表者 : 岸 玲子, 室橋 春光, 白石 秀明, 池野 多美子, 宮下 ちひろ, 花岡 知之, 佐々木 成子
     
    大規模出生コホートの8歳児に、調査票を用いADHD症状をConners3PとADHD-RSで評価し、胎児期の喫煙や葉酸摂取など環境因子のリスクを検討した。回答者2,545名について解析した結果、妊娠中の喫煙曝露によりADHD傾向が強く胎児期の喫煙のリスクが有意に高かった(OR=2.2;95%CI,1.002-4.631)。さらにコホート内症例対照研究デザインで、ADHD疑い症例群138名と対照群について、EP1システム(Fludigm社製)で神経伝達関連遺伝子(DRD4,DAT1,HTR1B,COMT,SNAP25)と脳由来神経栄養因子(BDNF)についてSNPs解析を200名分終了した。
  • 日本学術振興会:科学研究費助成事業 基盤研究(C)
    研究期間 : 2012年04月 -2015年03月 
    代表者 : 池野 多美子, 室橋 春光, 白石 秀明, 佐々木 成子, 岸 玲子, 荒木 敦子, 安住 薫
     
    2万人規模の出生コーホート参加者8歳児を対象に、注意欠如多動性障害(ADHD)発症の環境リスク要因を明らかにすることを目的とした。1)8歳児2,545名でADHD症状得点への喫煙曝露の影響を解析した結果、胎児期喫煙曝露より学童期の養育環境や母の抑うつが影響していた。2) Conners3の各指標から症例と対照計179名に対しWISC-Ⅳ検査など対面調査を実施した。3) 統計学パワーが最大になるよう計算した390名(症例:対照=1:2)のうちADHD疑い群138名を含む200名分について、神経伝達関連遺伝子(DRD4、DAT1など)と脳由来神経栄養因子(BDNF)の SNPs解析を行った。
  • 日本学術振興会:科学研究費助成事業 基盤研究(A)
    研究期間 : 2010年04月 -2014年03月 
    代表者 : 岸 玲子, 吉岡 英治, 佐々木 成子, 室橋 春光, 中島 そのみ, 白石 秀明, 安住 薫, 池野 多美子, 荒木 敦子, 花岡 知之, 梶原 淳睦, 斉藤 貢一, 佐田 文宏, 西條 泰明, 伊藤 佐智子, 宮下 ちひろ
     
    妊婦514名とその児を対象とした1産院コホートで、ベースライン時、1歳半、3歳半、7歳児の両親または児の食習慣・生活習慣や育児環境などを詳細に調べた。児の神経行動発達検査は、生後6か月と1歳半にBSID-II、3歳半にK-ABC、3歳7か月にCBCL、7歳にWISC-III、CBCLなどを実施した。環境化学物質として母体血および母乳中のダイオキシン・PCB異性体類、母体血中PFOS/PFOA等を測定した。
  • 日本学術振興会:科学研究費助成事業 基盤研究(C)
    研究期間 : 2011年 -2013年 
    代表者 : 白石 秀明, 竹内 文也, 齋藤 伸治
     
    急性脳症などに由来する脳機能障害に伴う、言語機能障害児に関して、脳磁図を用いて、言語機能回復、獲得を客観的に評価した。小児に言語刺激を有効に提供できる刺激作成装置、プロジェクターを開発し、言語発達遅滞児に対して経年的評価を施行した。5例に対して検討を行い、経年的変化を3例で確認した。対象症例においては、言語優位半球前頭部において、α帯域の律動性基礎波活動が減弱し、事象関連脱同期反応が見出され、この範囲は、経年的に拡大していた。言語機能発達を客観的に表現する事が可能であった。 本研究の成果により、これまで困難とされていた、年少小児おける言語機能獲得過程の客観的評価が可能となった。
  • 日本学術振興会:科学研究費助成事業 挑戦的萌芽研究
    研究期間 : 2011年 -2012年 
    代表者 : 岸 玲子, 安住 薫, 池野 多美子, 吉岡 英治, 白石 秀明, 佐々木 成子
     
    出生前向きコホートで8才児の注意欠陥/多動性障害(ADHD)への喫煙影響を明らかにするため,Conners3を用い調査した。8歳児1,192名から調査票を回収した。アウトカムはConners3の「不注意」「多動衝動」「総合指標」得点で評価した。妊娠前の母親,7歳時両親が喫煙している場合、各得点が高かった(p=0.014~0.006)。Conners3得点からADHD疑い群8名と対照群28名に対面調査を実施した。WISC-IVによる知能検査は両群に差がなかった。ADHD発症に関わる化学物質曝露と代謝関連遺伝子多型,環境遺伝交互作用を総説にまとめた。
  • 日本学術振興会:科学研究費助成事業 基盤研究(C)
    研究期間 : 2010年 -2012年 
    代表者 : 長峯 隆, 矢澤 省吾, 篠崎 淳, 原田 邦明, 白石 秀明
     
    運動を随意的に停止する機構は未解明である。特に歩行の停止困難などは転倒を伴い、安全性を検討するうえで重要である。音に対して反応運動を行わせ、音が予期せず欠落した場合に運動を中断させる課題を開発した。歩行の記録に先立ってまず単純な運動による検討を行った。右手、左手、右足の運動を行わせて脳磁計を用いて脳活動を計測すると、運動を中断する際にその運動に対応する一次運動野が活動し、賦活される運動領野は動かす部位で異なった。歩行の際の脳活動記録を試みたが雑音の影響が排除できなかった。新たに運動イメージの課題を追加し、指の運動をイメージする際の脳活動を記録できた。歩行への応用を検討中である。
  • 日本学術振興会:科学研究費助成事業 基盤研究(C)
    研究期間 : 2009年 -2011年 
    代表者 : 齋藤 伸治, 白石 秀明
     
    アンジェルマン症候群(AS)およびプラダー・ウイリー症候群(PWS)の中枢神経機能障害の成因を明らかにすることを目的に、遺伝学的解析を実施した。AS 168名、PWS 202名の遺伝学的解析を行い、ASの54名(32.1%)、PWSの82名(40.6%)の確定診断を行うことができた。遺伝学的に確定されなかったAS疑い例の中に、SLC9A6遺伝子変異1例および、微細染色体異常2例、PWS疑い例のなかに、微細染色体異常22例を同定した。これらの例はASもしくはPWSと共通した分子経路に変異を持つ可能性が示唆された。
  • 小児てんかん症例における空間フィルター法を用いた脳磁場解析
    日本学術振興会:科学研究費助成事業 基盤研究(C)
    研究期間 : 2006年 -2007年 
    代表者 : 白石 秀明, 竹内 文也, 齋藤 伸治
     
    109例のてんかん症例に対して脳磁場解析を行った。脳磁場測定には、306ch全頭型脳磁場計測装置(Vector View system, Neuromag Oy., Stockholm, Sweden)を用いた。記録周波数は600Hzを用いた。 症例におけるてんかん症候群分類は、国際抗てんかん連盟による発作型・てんかん症候群分類(1987)に従うと、症候性局在関連てんかん85例、特発性局在関連てんかん15例、症候性全般てんかん5例、特発性全般てんかん2例、未決定てんかん2例であった。全症例に対して、従来まで用いられてきた等価性電流双極子(Equivalent current dipole: ECD)の推定を行った。この推定により、電流源が単一脳回に局在し、ECDの局在に集積傾向を示したものが、症候性局在関連てんかん65例、特発性局在関連てんかん12例、症候性全般てんかん3例、特発性全般てんかん1例、未決定てんかん2例であった。これらの症例は、限局、あるいは単純な磁場分布を示しているもので、それらの結果と臨床発作対応が得られた。一方、単一双極子法にて解が求められない症例に対して、dynamic statistical parametric mapping(dSPM)を用いた解析を行った。dSPMでは、脳磁場分布が広汎に存在する、16例(症候性局在関連てんかん10例、特発性局在関連てんかん3例、症候性全般てんかん2例、特発性全般てんかん1例)の検討が可能であった。更に、これらの解析によっても解が得られない、低振幅棘波律動、広汎性棘波律動、棘・徐波律動、徐波律動を磁場反応として持つ症候性局在関連てんかん10例に対して、短時間フーリエ変換を用いて、特異的に出現する周波数帯域の局在を求めた。これらの所見は、後に施行した頭蓋内脳波所見と一致し、前頭葉てんかん症例では一側半球より先行して出現する棘律動の出現が見出され、確定診断に至った。これらの手法を用いることにより、全てのてんかん症候群に対して、脳磁図検査が可能になった。

教育活動情報

主要な担当授業

  • 基本医学研究
    開講年度 : 2021年
    課程区分 : 修士課程
    開講学部 : 医学院
    キーワード : 発達、小児医療、周産期医療、がんプロフェッショナル development, pediatric care, perinatal care, cancer professional
  • 基本医学総論
    開講年度 : 2021年
    課程区分 : 修士課程
    開講学部 : 医学院
    キーワード : 発達、小児医療、周産期医療、がんプロフェッショナル development, pediatric care, perinatal care, cancer professional
  • 大学院共通授業科目(教育プログラム):脳科学入門
    開講年度 : 2021年
    課程区分 : 修士課程
    開講学部 : 大学院共通科目
    キーワード : 脳, 機能イメージング, fMRI, MEG
  • 基盤医学研究
    開講年度 : 2021年
    課程区分 : 博士後期課程
    開講学部 : 医学院
    キーワード : 発達、小児医療、周産期医療、がんプロフェッショナル
  • 臨床医学研究
    開講年度 : 2021年
    課程区分 : 博士後期課程
    開講学部 : 医学院
    キーワード : 発達、小児医療、周産期医療、がんプロフェッショナル
  • 医学総論
    開講年度 : 2021年
    課程区分 : 博士後期課程
    開講学部 : 医学院
    キーワード : 発達、小児医療、周産期医療、がんプロフェッショナル
  • 発達障害学
    開講年度 : 2021年
    課程区分 : 学士課程
    開講学部 : 医学部
    キーワード : 小児科,発達の特徴,疾病と障害,病態と治療,保健と予防、救急救命、栄養
  • 小児科学・小児外科学
    開講年度 : 2021年
    課程区分 : 学士課程
    開講学部 : 医学部
    キーワード : 小児科学


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