寒川 真 (サムカワ マコト)

  • 医学科 講師
Last Updated :2024/04/23

コミュニケーション情報 byコメンテータガイド

  • コメント

    ギラン・バレー症候群、CIDP、重症筋無力症、多発性硬化症、視神経脊髄炎等の原因・症状・治療法について研究しています。

研究者情報

学位

  • 博士(医学)(2014年03月 近畿大学大学院)

ホームページURL

J-Global ID

プロフィール

  • 日本内科学会認定内科医・総合内科専門医・指導医


    日本神経学会神経内科専門医・指導医


    日本頭痛学会認定頭痛専門医


    日本認知症学会専門医・指導医


    日本臨床神経生理学会専門医・指導医


    日本神経摂食嚥下・栄養学会代議員


    日本医師会認定産業医


    重症筋無力症/ランバート・イートン筋無力症候群診療ガイドライン協力医師


    Japan MG Registry study研究会会員

研究キーワード

  • 片頭痛   慢性炎症性多発性多発根ニューロパチー   ギラン・バレー症候群   重症筋無力症   

現在の研究分野(キーワード)

    ギラン・バレー症候群、CIDP、重症筋無力症、多発性硬化症、視神経脊髄炎等の原因・症状・治療法について研究しています。

研究分野

  • ライフサイエンス / 神経内科学 / 重症筋無力症
  • ライフサイエンス / 神経科学一般

経歴

  • 2018年04月 - 現在  近畿大学病院脳神経内科講師
  • 2015年04月 - 2018年03月  近畿大学病院脳神経内科医学部講師
  • 2014年04月 - 2015年03月  近畿大学医学部附属病院脳神経内科助教A
  • 2009年03月 - 2010年04月  近畿大学医学部附属病院神経内科助教B
  • 2007年04月 - 2009年03月  近畿大学医学部附属病院総合医学教育研修センター初期臨床研修医

学歴

  • 2010年04月 - 2014年03月   近畿大学   医学研究科
  • 2001年04月 - 2007年03月   近畿大学   医学部   医学科

所属学協会

  • 日本神経摂食嚥下・栄養学会   日本MGレジストリー多施設研究   日本末梢神経学会   日本臨床神経生理学会   日本神経免疫学会   日本認知症学会   日本頭痛学会   日本神経治療学会   日本神経学会   日本内科学会   

研究活動情報

論文

  • Atsushi Terayama; Motoi Kuwahara; Keisuke Yoshikawa; Yuko Yamagishi; Makoto Samukawa; Shoko Yamashita; Kyohei Onishi; Tomoya Nagano; Chikao Tatsumi; Junko Ishii; Michi Kawamoto; Takashi Tokashiki; Shoko Deguchi; Kentaro Deguchi; Atsushi Ishida; Yasuhiko Baba; Shigeki Yamaguchi; Susumu Kusunoki; Yoshitaka Nagai
    Journal of neurology 2024年04月 
    BACKGROUND AND PURPOSE: Takotsubo cardiomyopathy (TCM) is a serious autonomic complication of Guillain-Barré syndrome (GBS). However, the association between TCM and GBS has not been investigated in detail. We investigated the characteristics of GBS patients with TCM (GBS-TCM). METHODS: Clinical features and anti-ganglioside antibody between the GBS-TCM patients and 62 classical GBS patients without TCM as control patients were compared. RESULTS: Eight GBS-TCM patients were identified, in whom TCM was diagnosed at a mean of 6.5 [range 3-42] days after the onset of GBS. The age at onset of GBS was elder in the GBS-TCM patients than in the control GBS patients (76.5 [56-87] vs. 52 [20-88] years, p < 0.01). Notably, cranial nerve deficits, particularly in the lower cranial nerves, were observed in all GBS-TCM patients (100% vs. 41.9%, p < 0.01). Additionally, the GBS-TCM patients showed a higher GBS disability score at nadir (5 [4-5] vs. 4 [1-5], p < 0.01), and lower Medical Research Council sum scores at admission and nadir (37 [30-44] vs. 48 [12-60] at admission, p < 0.05, and 20 [12-44] vs. 40 [0-60] at nadir, p < 0.05, respectively). Mechanical ventilation was more frequently required in the GBS-TCM patients (62.5% vs. 11.3%, p < 0.01). Three GBS-TCM patients were positive for anti-ganglioside antibodies. CONCLUSIONS: TCM occurred at a relatively early phase of GBS. The characteristics of GBS-TCM were the elder, lower cranial nerve involvements, severe limb weakness, and respiratory failure.
  • Makito Hirano; Motoi Kuwahara; Yuko Yamagishi; Makoto Samukawa; Kanako Fujii; Shoko Yamashita; Masahiro Ando; Nobuyuki Oka; Mamoru Nagano; Taro Matsui; Toshihide Takeuchi; Kazumasa Saigoh; Susumu Kusunoki; Hiroshi Takashima; Yoshitaka Nagai
    Scientific reports 13 1 17801 - 17801 2023年10月 
    Cerebellar ataxia, neuropathy, and vestibular areflexia syndrome (CANVAS) has recently been attributed to biallelic repeat expansions in RFC1. More recently, the disease entity has expanded to atypical phenotypes, including chronic neuropathy without cerebellar ataxia or vestibular areflexia. Very recently, RFC1 expansions were found in patients with Sjögren syndrome who had neuropathy that did not respond to immunotherapy. In this study RFC1 was examined in 240 patients with acute or chronic neuropathies, including 105 with Guillain-Barré syndrome or Miller Fisher syndrome, 76 with chronic inflammatory demyelinating polyneuropathy, and 59 with other types of chronic neuropathy. Biallelic RFC1 mutations were found in three patients with immune-mediated neuropathies, including Guillain-Barré syndrome, idiopathic sensory ataxic neuropathy, or anti-myelin-associated glycoprotein (MAG) neuropathy, who responded to immunotherapies. In addition, a patient with chronic sensory autonomic neuropathy had biallelic mutations, and subclinical changes in Schwann cells on nerve biopsy. In summary, we found CANVAS-related RFC1 mutations in patients with treatable immune-mediated neuropathy or demyelinating neuropathy.
  • Kazumasa Saigoh; Makito Hirano; Yoshiyuki Mitsui; Itsuki Oda; Atsuko Ikegawa; Makoto Samukawa; Keisuke Yoshikawa; Yuko Yamagishi; Susumu Kusunoki; Yoshitaka Nagai
    Journal of medical case reports 17 1 431 - 431 2023年10月 
    BACKGROUND: Huntington's disease is an autosomal dominant inherited disorder characterized by personality changes (such as irritability and restlessness) and psychotic symptoms (such as hallucinations and delusions). When the personality changes become noticeable, involuntary movements (chorea) also develop. The disease is caused by the CAG repeat expansion in the coding region of the HTT gene, and the diagnosis is based on the presence of this expansion. However, there is currently no effective treatment for the progression of Huntington's disease and its involuntary motor symptoms. Herein, we present a case in which memantine was effective in treating the chorea movements of Huntington's disease. CASE PRESENTATION: A 75-year-old Japanese woman presented to the hospital with involuntary movements of Huntington's disease that began when she was 73 years old. In a cerebral blood flow test (N-isopropyl-p-iodoamphetamine-single-photon emission computed tomography), decreased blood flow was observed in the precuneus (anterior wedge) and posterior cingulate gyrus. Usually, such areas of decreased blood flow are observed in patients with Alzheimer's-type dementia. So, we administered memantine for Alzheimer's-type dementia, and this treatment suppressed the involuntary movements of Huntington's disease, and the symptoms progressed slowly for 7 years after the onset of senility. In contrast, her brother died of complications of pneumonia during the course of Huntington's disease. CONCLUSIONS: We recorded changes in parameters such as the results of the N-isopropyl-p-iodoamphetamine-single-photon emission computed tomography and gait videos over 7 years. Treatment with memantine prevented the chorea movement and the progression of Huntington's disease. We believe this record will provide clinicians with valuable information in diagnosing and treating Huntington's disease.
  • 重症筋無力症における胸腺外悪性腫瘍の合併と免疫チェックポイント阻害薬使用例の検討
    吉積 一樹; 渡邊 将平; 木村 卓; 久保田 智哉; 鵜沢 顕之; 川口 直樹; 増田 眞之; 紺野 晋吾; 南 尚哉; 寒川 真; 渡辺 源也; 鈴木 靖士; 長根 百合子; 高橋 正紀; 鈴木 重明; 槍澤 公明
    臨床神経学 63 Suppl. S251 - S251 (一社)日本神経学会 2023年09月
  • Akiyuki Uzawa; Shigeaki Suzuki; Satoshi Kuwabara; Hiroyuki Akamine; Yosuke Onishi; Manato Yasuda; Yukiko Ozawa; Naoki Kawaguchi; Tomoya Kubota; Masanori P Takahashi; Yasushi Suzuki; Genya Watanabe; Takashi Kimura; Takamichi Sugimoto; Makoto Samukawa; Naoya Minami; Masayuki Masuda; Shingo Konno; Yuriko Nagane; Kimiaki Utsugisawa
    Journal of neurology, neurosurgery, and psychiatry 94 6 467 - 473 2023年01月 
    BACKGROUND: Early fast-acting treatment (EFT) is the aggressive use of fast-acting therapies such as plasmapheresis, intravenous immunoglobulin and/or intravenous high-dose methylprednisolone (IVMP) from the early phases of treatment. EFT is reportedly beneficial for early achievement of minimal manifestations (MM) or better status with ≤5 mg/day prednisolone (MM5mg), a practical therapeutic target for myasthenia gravis (MG). OBJECTIVE: The current study aimed to clarify which specific EFT regimen is efficacious and the patient characteristics that confer sensitivity to EFT. METHODS: We recruited a total of 1710 consecutive patients with MG who enrolled in the Japan MG Registry for this large-cohort study. Among them, 1066 with generalised MG who had received immunotherapy were analysed. Prognostic background factors were matched in a 1:1 ratio using propensity score matching analysis between patients treated with EFT (n=350) and those treated without EFT (n=350). The clinical course and time to first achieve MM5mg after starting immunotherapy was analysed in relation to treatment combinations and patient characteristics. RESULTS: Kaplan-Meier analyses showed that EFT had a significant effect on the achievement of MM5mg (p<0.0001, log-rank test; HR 1.82, p<0.0001). Notably, EFT was efficacious for any type of MG, and the inclusion of IVMP resulted in earlier and more frequent achievement of MM5mg (p=0.0352, log-rank test; HR 1.46, p=0.0380). In addition, early administration of calcineurin inhibitors also promoted MM5mg achievement. CONCLUSION: Early cycles of intervention with EFT and early use of calcineurin inhibitors provides long-term benefits in terms of achieving therapeutic targets for generalised MG, regardless of clinical subtype.
  • Akiyuki Uzawa; Shigeaki Suzuki; Satoshi Kuwabara; Hiroyuki Akamine; Yosuke Onishi; Manato Yasuda; Yukiko Ozawa; Naoki Kawaguchi; Tomoya Kubota; Masanori P Takahashi; Yasushi Suzuki; Genya Watanabe; Takashi Kimura; Takamichi Sugimoto; Makoto Samukawa; Naoya Minami; Masayuki Masuda; Shingo Konno; Yuriko Nagane; Kimiaki Utsugisawa
    Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics 20 2 518 - 523 2023年01月 
    The efficacy of intravenous high-dose methylprednisolone (IVMP) in ocular myasthenia gravis (MG) has not been fully established. This study aimed to elucidate the effects of early intervention with IVMP for achieving the therapeutic targets (minimal manifestations [MM] or MM or better status with prednisolone ≤ 5 mg/day [MM5mg]) in ocular MG. In this observational study, we included a total of 1710 consecutive patients with MG enrolled in the Japan MG Registry in 2021. Of these, 204 patients with ocular MG who received immunotherapy were analyzed. The clinical course and time to first achieve MM or MM5mg after starting immunotherapy were compared between the early IVMP group (treated with IVMP within 3 months of treatment initiation) and the non-early IVMP group. Despite having greater clinical severity before immunotherapy and lower oral prednisolone doses throughout the course, the early IVMP group (n = 55) showed a higher rate of achievement of MM (P = 0.0040, log-rank test; hazard ratio 1.58, 95% confidence interval [CI] 1.13-2.20, P < 0.0001) and MM5mg (P = 0.0005, log-rank test; hazard ratio 1.78, 95% CI 1.27-2.51, P < 0.0001) compared with the non-early IVMP group (n = 149). In conclusion, an early intervention with IVMP is likely to increase the probability of achieving a better long-term outcome and reducing the total dose of corticosteroids in ocular MG.
  • SCA8関連筋萎縮性側索硬化症の表現型変異と治療戦略(Phenotypic variation and therapeutic strategy of SCA8-associated amyotrophic lateral sclerosis)
    Hirano Makito; Takehara Toshiyuki; Murayama Shigeo; Izumi Yuishin; Samukawa Makoto; Matsubara Tomoyasu; Saito Yuko; Saigoh Kazumasa; Nakamura Yusaku; Fukuda Kanji; Kusunoki Susumu; Nagai Yoshitaka
    臨床神経学 62 Suppl. S216 - S216 2022年10月
  • 平野 牧人; 竹原 俊幸; 村山 繁雄; 和泉 唯信; 寒川 真; 松原 知康; 斎藤 祐子; 西郷 和真; 中村 雄作; 楠 進; 永井 義隆
    神経治療学 39 6 S232 - S232 (一社)日本神経治療学会 2022年10月
  • 平野 牧人; 竹原 俊幸; 村山 繁雄; 和泉 唯信; 寒川 真; 松原 知康; 斎藤 祐子; 西郷 和真; 中村 雄作; 楠 進; 永井 義隆
    神経治療学 39 6 S232 - S232 (一社)日本神経治療学会 2022年10月
  • Shigeaki Suzuki; Masayuki Masuda; Akiyuki Uzawa; Yuriko Nagane; Shingo Konno; Yasushi Suzuki; Tomoya Kubota; Takamichi Sugimoto; Makoto Samukawa; Genya Watanabe; Kei Ishizuchi; Hiroyuki Akamine; Yosuke Onishi; Kazuki Yoshizumi; Takafumi Uchi; Itaru Amino; Yuki Ueta; Naoya Minami; Naoki Kawaguchi; Takashi Kimura; Masanori P. Takahashi; Hiroyuki Murai; Kimiaki Utsugisawa
    Clinical and Experimental Neuroimmunology 2022年10月
  • Ituki Oda; Daisuke Danno; Kazumasa Saigoh; Johanna Wolf; Norihito Kawashita; Makito Hirano; Makoto Samukawa; Shigekazu Kitamura; Shoji Kikui; Takao Takeshima; Yoshiyuki Mitsui; Susumu Kusunoki; Yoshitaka Nagai
    Neuroscience research 2022年03月 
    We analyzed the clinical symptoms of hemiplegic migraine (HM) and their relevance in four Japanese patients considered to have ATP1A2 mutations as a cause. Sequencing of ATP1A2 was performed using the Sanger method in 43 blood samples from clinically suspected patients with familial HM. Subsequently, algorithm analysis, allele frequency determination, and three-dimensional structure analysis of the recognized variants were performed, and the recognized variants were evaluated. We found four heterozygous missense mutations in ATP1A2 (Case 1: p.R51C; Case 2: p.R65L; Case 3: p.A269P; Case 4: p.D999H), three of which had not been reported to date. These four mutations may also affect the structure of the protein products, as assessed using a three-dimensional structural analysis. In all four cases, the clinical symptoms included visual, sensory, motor, and verbal symptoms and the frequency and duration of headache attacks varied. Additionally, oral administration of a combination of lomerizine hydrochloride and topiramate had a partial effect in three cases. We report four missense mutations in ATP1A2. This report will be useful for the future analysis of mutations and clinical types in Asians, as well as Westerners, with migraine.
  • MELASにおける上腸間膜動脈症候群
    寒川 真; 桑原 基; 西郷 和真; 三井 良之; 楠 進
    臨床神経学 61 Suppl. S297 - S297 (一社)日本神経学会 2021年09月
  • Rino Inada; Makito Hirano; Nobuyuki Oka; Makoto Samukawa; Kazumasa Saigoh; Hidekazu Suzuki; Fukashi Udaka; Akihiro Hashiguchi; Hiroshi Takashima; Yukihiro Hamada; Yusaku Nakamura; Susumu Kusunoki
    Journal of neurology 268 8 2933 - 2942 2021年08月 
    BACKGROUND: We intended to clarify the phenotypic and molecular diversities of spinocerebellar ataxia type 2 (SCA2) in Japan. METHODS: DNA was extracted from the peripheral blood of 436 patients, including 126 patients with chronic neuropathy, 108 with amyotrophic lateral sclerosis, and 202 with cerebellar ataxia. We then PCR-amplified and sequenced the ATXN2 gene. The biopsied sural nerves of mutation-positive patients were subjected to light-microscopic and electron-microscopic analyses. Transfection analyses were performed using a Schwann cell line, IMS32. RESULTS: We found PCR-amplified products potentially corresponding to expanded CAG repeats in four patients. Two patients in the chronic neuropathy group had a full repeat expansion or an intermediate expansion (39 or 32 repeats), without limb ataxia. The sural nerve biopsy findings of the two patients included axonal neuropathy and mixed neuropathy (axonal changes with demyelination). Schwann cells harbored either cytoplasmic or nuclear inclusions on electron microscopic examination. Both patients recently exhibited pyramidal signs. In the third patient in the cerebellar ataxia group, we identified a novel 21-base duplication mutation near 22 CAG repeats (c.432_452dup). The transfection study revealed that the 21-base-duplication mutant Ataxin-2 proteins aggregated in IMS32 and rendered cells susceptible to oxidative stress, similar to a CAG-expanded mutant. The fourth patient, with 41 repeats, had ataxia and spasticity. The two patients with cerebellar ataxia also had peripheral neuropathy. CONCLUSIONS: Patients with expanded CAG repeats can exhibit a neuropathy-dominant phenotype not described previously. The novel 21-base-duplication mutant seems to share the aggregation properties of polyglutamine-expanded mutants.
  • James F. Howard; Vera Bril; Tuan Vu; Chafic Karam; Stojan Peric; Temur Margania; Hiroyuki Murai; Malgorzata Bilinska; Roman Shakarishvili; Marek Smilowski; Antonio Guglietta; Peter Ulrichts; Tony Vangeneugden; Kimiaki Utsugisawa; Jan Verschuuren; Renato Mantegazza; Jan De Bleeker; Kathy De Koning; Katrien De Mey; Annelien De Pue; Rudolf Mercelis; Maren Wyckmans; Caroline Vinck; Linda Wagemaekers; Jonathan Baets; Eduardo Ng; Jafar Shabanpour; Lubna Daniyal; Shabber Mannan; Hans Katzberg; Angela Genge; Zaeem Siddiqi; Jana Junkerová; Jana Horakova; Katerina Reguliova; Michaela Tyblova; Ivana Jurajdova; Iveta Novakova; Michala Jakubikova; Jiri Pitha; Stanislav Vohanka; Katerina Havelkova; Tomas Horak; Josef Bednarik; Mageda Horakova; Andreas Meisel; Dike Remstedt; Claudia Heibutzki; Siegfried Kohler; Sarah Hoffman; Frauke Stascheit; John Vissing; Lizzie Zafirakos; Kuldeep Kumar Khatri; Anne Autzen; Mads Peter Godtfeldt Stemmerik; Henning Andersen; Shahram Attarian; Alexander Tsiskaridze; Csilla Rózsa; Gedeonne Margo Jakab; Szilvia Toth; Gyorgyi Szabo; David Bors; Eniko Szabo; Angela Campanella; Fiammetta Vanoli; Rita Frangiamore; Carlo Antozzi; Silvia Bonanno; Lorenzo Maggi; Riccardo Giossi; Francesco Saccà; Angela Marsili; Tiziana Imbriglio; Giovanni Antonini; Girolamo Alfieri; Stefania Morino; Matteo Garibaldi; Laura Fionda; Luca Leonardi; Shingo Konno; Akiyuki Uzawa; Kaoru Sakuma; Chiho Watanabe; Yukiko Ozawa; Manato Yasuda; Yosuke Onishi; Makoto Samukawa; Tomoko Tsuda; Yasushi Suzuki; Sayaka Ishida; Genya Watanabe; Masanori Takahashi; Hiroko Nakamura; Erina Sugano; Tomoya Kubota; Tomihiro Imai; Suzuki Mari; Ayako Mori
    The Lancet Neurology 20 7 526 - 536 2021年07月 
    Background: There is an unmet need for treatment options for generalised myasthenia gravis that are effective, targeted, well tolerated, and can be used in a broad population of patients. We aimed to assess the safety and efficacy of efgartigimod (ARGX-113), a human IgG1 antibody Fc fragment engineered to reduce pathogenic IgG autoantibody levels, in patients with generalised myasthenia gravis. Methods: ADAPT was a randomised, double-blind, placebo-controlled, phase 3 trial done at 56 neuromuscular academic and community centres in 15 countries in North America, Europe, and Japan. Patients aged at least 18 years with generalised myasthenia gravis were eligible to participate in the study, regardless of anti-acetylcholine receptor antibody status, if they had a Myasthenia Gravis Activities of Daily Living (MG-ADL) score of at least 5 (>50% non-ocular), and were on a stable dose of at least one treatment for generalised myasthenia gravis. Patients were randomly assigned by interactive response technology (1:1) to efgartigimod (10 mg/kg) or matching placebo, administered as four infusions per cycle (one infusion per week), repeated as needed depending on clinical response no sooner than 8 weeks after initiation of the previous cycle. Patients, investigators, and clinical site staff were all masked to treatment allocation. The primary endpoint was proportion of acetylcholine receptor antibody-positive patients who were MG-ADL responders (≥2-point MG-ADL improvement sustained for ≥4 weeks) in the first treatment cycle. The primary analysis was done in the modified intention-to-treat population of all acetylcholine receptor antibody-positive patients who had a valid baseline MG-ADL assessment and at least one post-baseline MG-ADL assessment. The safety analysis included all randomly assigned patients who received at least one dose or part dose of efgartigimod or placebo. This trial is registered at ClinicalTrials.gov (NCT03669588); an open-label extension is ongoing (ADAPT+, NCT03770403). Findings: Between Sept 5, 2018, and Nov 26, 2019, 167 patients (84 in the efgartigimod group and 83 in the placebo group) were enrolled, randomly assigned, and treated. 129 (77%) were acetylcholine receptor antibody-positive. Of these patients, more of those in the efgartigimod group were MG-ADL responders (44 [68%] of 65) in cycle 1 than in the placebo group (19 [30%] of 64), with an odds ratio of 4·95 (95% CI 2·21–11·53, p<0·0001). 65 (77%) of 84 patients in the efgartigimod group and 70 (84%) of 83 in the placebo group had treatment-emergent adverse events, with the most frequent being headache (efgartigimod 24 [29%] vs placebo 23 [28%]) and nasopharyngitis (efgartigimod ten [12%] vs placebo 15 [18%]). Four (5%) efgartigimod-treated patients and seven (8%) patients in the placebo group had a serious adverse event. Three patients in each treatment group (4%) discontinued treatment during the study. There were no deaths. Interpretation: Efgartigimod was well tolerated and efficacious in patients with generalised myasthenia gravis. The individualised dosing based on clinical response was a unique feature of ADAPT, and translation to clinical practice with longer term safety and efficacy data will be further informed by the ongoing open-label extension. Funding: argenx.
  • 桑原 基; 福本 雄太; 吉川 恵輔; 寒川 真; 楠 進
    末梢神経 31 2 286 - 286 日本末梢神経学会 2020年12月
  • 病理学的に確認されたATXN8OS関連筋萎縮性側索硬化症の最初の患者についての報告(The first patient with pathologically-definite ATXN8OS-associated amyotrophic lateral sclerosis)
    Hirano Makito; Murayama Shigeo; Izumi Yuishin; Samukawa Makoto; Matsubara Tomoyasu; Saigoh Kazumasa; Nakamura Yusaku; Kusunoki Susumu
    臨床神経学 60 Suppl. S345 - S345 2020年11月
  • PNPLA2遺伝子関連中性脂肪蓄積病の臨床像
    寒川 真; 中村 尚子; 平野 牧人; 森川 みゆき; 坂田 花美; 西野 一三; 井泉 瑠美子; 鈴木 直輝; 黒田 宙; 滋賀 健介; 青木 正志; 楠 進
    臨床神経学 60 Suppl. S388 - S388 (一社)日本神経学会 2020年11月
  • 免疫チェックポイント阻害薬関連ニューロパチーの解析
    桑原 基; 福本 雄太; 吉川 恵輔; 寒川 真; 楠 進
    神経免疫学 25 1 163 - 163 (一社)日本神経免疫学会 2020年10月
  • 平野 牧人; 井上 貴美子; 伊藤 龍生; 寒川 真; 藤村 晴俊; 能勢 和宏; 楠 進; 中村 雄作
    神経治療学 37 6 S223 - S223 (一社)日本神経治療学会 2020年10月
  • Makito Hirano; Chiharu Isono; Makoto Samukawa; Kanji Fukuda; Susumu Kusunoki
    Parkinsonism & related disorders 78 98 - 99 2020年09月
  • Makoto Samukawa; Naoko Nakamura; Makito Hirano; Miyuki Morikawa; Hanami Sakata; Ichizo Nishino; Rumiko Izumi; Naoki Suzuki; Hiroshi Kuroda; Kensuke Shiga; Kazumasa Saigoh; Masashi Aoki; Susumu Kusunoki
    European neurology 83 3 1 - 6 2020年06月 [査読有り]
     
    Mutations in the PNPLA2 gene cause neutral lipid storage disease with myopathy (NLSDM) or triglyceride deposit cardiomyovasculopathy. We report a detailed case study of a 53-year-old man with NLSDM. The PNPLA2 gene was analyzed according to the reported method. We summarized the clinical, laboratory, and genetic information of 56 patients, including our patient and 55 other reported patients with homozygous or compound heterozygous mutations in the PNPLA2 gene. We found a novel homozygous mutation (c.194delC) in the PNPLA2 gene that resulted in frameshift. The patient suffered from normal-tension glaucoma and pulmonary cysts, symptoms that are relatively common in the elderly but were not previously reported for this disease. Our summary confirmed that Jordan's anomaly, polymorphonuclear leukocytes with lipid accumulation, was the most consistent finding of this disease. Because this disease is potentially treatable, our results may help rapid and correct diagnosis.
  • 桑原 基; 吉川 恵輔; 森川 みゆき; 稲田 莉乃; 寒川 真; 平野 牧人; 楠 進
    末梢神経 30 2 335 - 335 日本末梢神経学会 2019年12月
  • 寒冷刺激による頭痛と生体温度変化について
    西郷 和真; 米島 康平; 木川 和弥; 稲垣 拓武; 長谷川 隆典; 寒川 真; 濱田 征宏; 市橋 珠里; 三井 良之; 楠 進
    日本頭痛学会誌 46 2 486 - 486 (一社)日本頭痛学会 2019年11月
  • 仲間美奈; 西郷和真; 平野牧人; 濱田征宏; 金城ちなつ; 寒川真; 長谷川隆典; 北口正孝; 三井良之; 巽純子; 田村和朗; 楠進
    日本遺伝カウンセリング学会誌 40 3 95 - 100 日本遺伝カウンセリング学会 2019年10月 [査読有り]
     
    脊髄小脳変性症(spinocerebellar ataxia:SCA)は特に小脳性の運動失調症状を主体とする神経変性疾患の総称で、孤発性と遺伝性に大別される。遺伝性SCAのうち90%以上が常染色体優性遺伝を示し、原因遺伝子や臨床的特徴の違いにより40以上もの病型に分類される。疾患頻度は集団により異なるが、比較的頻度の多いSCA31が同定された2009年以降に近畿圏の病型別頻度は調査されていない。本研究では、近畿大学病院脳神経内科にて得られたSCA症例をもとに、近畿圏の病型別頻度を調査したところ、遺伝性SCA80例のうちSCA6が最も頻度が高く44%、次いでMJD/SCA3が12%、SCA31が10%であった。そのほかSCA8、DRPLA、SCA2、SCA1、SCA23、SCA36が認められ、病型未確定は14%であった。本解析を通じて本疾患の遺伝子診断の意義、pre-mutationの取り扱いについて考察したので報告する。(著者抄録)
  • 高齢で発症した抗neurofascin155(NF155)抗体関連ニューロパチーの1例
    坂田 花美; 寒川 真; 定金 秀爾; 緒方 英紀; 桑原 基; 竹内 啓喜; 岡 伸幸; 吉良 潤一; 楠 進
    臨床神経学 59 4 234 - 234 (一社)日本神経学会 2019年04月
  • Charcot-Marie-Tooth disease with a mutation in FBLN5 accompanying with the small vasculitis and widespread onion-bulb formations.
    Yamagishi Y; Samukawa M; Kuwahara M; Takada K; Saigoh K; Mitsui Y; Oka N; Hashiguchi A; Takashima H; Kusunoki S.
    J Neurol Sci 410 116623  2019年 [査読有り]
  • Hirano M; Itoh T; Fujimura H; Inoue K; Samukawa M; Nose K; Sakamoto H; Maekura S; Ueno S; Satou T; Nishioka T; Kusunoki S; Nakamura Y
    J Neuropathol Exp Neurol. 2019年 [査読有り]
     
    Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune encephalitis. The disease predominantly affects women (1:5-1:10), with only 3 reports of autopsy findings in women being published to date. The present study reports findings from the first autopsy performed on a man with anti-NMDAR encephalitis. The patient had some scattered lesions in the limbic system with neuronal loss, gliosis, and microglial activation. The temporal and frontal cortices showed additional patchy demyelination. T-lymphocyte infiltration was detectable in the fusiform gyrus lesion. These findings were partly similar to those reported in female patients. Although clinical differences based on the sex of the patient are reported for this disease, the observed pathological similarities potentially help to establish common therapeutic strategies for all patients. Severe testicular damage was additionally observed in the male patient in this study. Biopsy-proven severe testicular damage was also confirmed in another, previously fertile man who became azoospermic. Moreover, serum follicle-stimulating hormone levels, which often increased in response to disturbed spermatogenesis, were elevated, and testosterone/luteinizing hormone ratio reflecting Leydig cell function was low in all 5 male patients in this study. Overall, these findings suggest similar brain pathology in patients of both sexes and severe testicular damage in male patients.
  • Yoshikawa K; Kuwahara M; Saigoh K; Ishiura H; Yamagishi Y; Hamano Y; Samukawa M; Suzuki H; Hirano M; Mitsui Y; Tsuji S; Kusunoki S
    eNeurologicalSci 14 34 - 37 2019年 [査読有り]
     
    Spastic paraplegia 30 is a recently established autosomal recessive disease characterized by a complex form of spastic paraplegia associated with neuropathy. Homozygous mutations of KIF1A reportedly lead to hereditary spastic paraplegia or hereditary sensory and autonomic neuropathy type 2 (HSAN2), whereas heterozygous mutations can cause nonsyndromic and syndromic intellectual disability (MRD9). Here we report the case of a 37-year-old female who presented with gait disturbance complicated with moyamoya disease. Results: The patient exhibited hypotonia during infancy, after which intellectual disability, epileptic fits, spastic paraplegia, and cerebellar atrophy occurred. Genetic analysis revealed a novel de novo mutation (c.254C > A, p.A85D) in the motor domain of KIF1A.
  • 口腔内寒冷刺激における体温変動について
    西郷 和真; 米島 康平; 長谷川 隆典; 濱田 征宏; 寒川 真; 市橋 珠里; 三井 良之; 楠 進
    日本頭痛学会誌 45 2 422 - 422 (一社)日本頭痛学会 2018年11月
  • 【ニューロパチー-Basics & Updates】免疫介在性ニューロパチー Guillain-Barre症候群
    寒川 真; 楠 進
    Clinical Neuroscience 36 9 1035 - 1038 (株)中外医学社 2018年09月 [査読有り][招待有り]
  • Yuko Yamagishi; Kazumasa Saigoh; Yoshiro Saito; Ikuko Ogawa; Yoshiyuki Mitsui; Yukihiro Hamada; Makoto Samukawa; Hidekazu Suzuki; Motoi Kuwahara; Makito Hirano; Noriko Noguchi; Susumu Kusunoki
    Neuroscience research 128 58 - 62 2018年03月 [査読有り]
     
    Parkinson's disease (PD) is difficult to distinguish from progressive supranuclear palsy (PSP) and multiple system atrophy (MSA); in addition, biomarker studies in PD mostly focused on those found in the cerebrospinal fluid, and there are few reports of simple biomarkers identified by blood analysis. Previously, the DJ-1 gene was identified as a causative gene of familial PD. Oxidized DJ-1 protein (oxDJ-1) levels were reported to increase in the blood of patients with unmedicated PD. Therefore, we determined the levels of oxDJ-1 in the erythrocytes of patients with PD, PSP, and MSA using ELISA. The oxDJ-1 levels were 165±117, 96±78, and 69±40ng/mg protein in the PD, PSP, and MSA groups, respectively. The mean level in disease control group was 66±31, revealing significant differences between the PD and PSP groups, the PD and MSA groups, and the PD and disease control groups. Our results indicated that oxDJ-1 levels in erythrocytes can be used as a marker for the differential diagnosis of PD.
  • 森口幸太; 西郷和真; 山下翔子; 定金秀爾; 柳本諭志; 稲田莉乃; 岡崎真央; 寒川真; 鈴木秀和; 平野牧人; 三井良之; 楠 進
    大阪てんかん研究会雑誌 29 1 7 - 9 大阪てんかん研究会 2018年 [査読有り]
     
    ペランパネル(PER)を投与した難治性てんかん8例(男性4例、女性4例、平均45.5歳)を対象とした。知的能力障害を伴った症例が4例あり、幼少期からてんかん発作を認めた。比較的高齢使用例の2例は、特発性血小板減少性紫斑病や、多発血管炎性肉芽腫症を合併した。抗てんかん薬(AED)は、PERを含めて2~4剤(平均3.1剤)であった。併用AEDとして使用されていたのは、バルプロ酸(VPA)4例、レベチラセタム(LEV)3例、ラモトリギン(LTG)3例、カルバマゼピン(CBZ)3例、ゾニサミド(ZNS)2例、ラコサミド(LCM)1例、ガバペンチン(GBP)1例であった。知的能力障害を伴う2例は焦燥感、興奮性等の症状により投与を中止した。知的能力障害を伴わない成人発症てんかん6例は、比較的少量で有効性を認め、ふらつきで減量を要する症例もあったが、薬剤中止はなく忍容性は良好であった。CBZの併用は3例で、うち1例は最大容量に近い10mgまでPERが増量され、CBZにより血中濃度が低下していた可能性が考えられた。
  • Hirano M; Samukawa M; Isono C; Saigoh K; Nakamura Y; Kusunoki S
    Neurol Genet. 4 e252  2018年 [査読有り]
  • James F. Howard; Kimiaki Utsugisawa; Michael Benatar; Hiroyuki Murai; Richard J. Barohn; Isabel Illa; Saiju Jacob; John Vissing; Ted M. Burns; John T. Kissel; Srikanth Muppidi; Richard J. Nowak; Fanny O'Brien; Jing-Jing Wang; Renato Mantegazza
    LANCET NEUROLOGY 16 12 976 - 986 2017年12月 
    Background Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. Methods We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76 hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease, vaccination against Neisseria meningitides, and previous treatment with at least two immunosuppressive therapies or one immunosuppressive therapy and chronic intravenous immunoglobulin or plasma exchange for 12 months without symptom control. Patients with a history of thymoma or thymic neoplasms, thymectomy within 12 months before screening, or use of intravenous immunoglobulin or plasma exchange within 4 weeks before randomisation, or rituximab within 6 months before screening, were excluded. We randomly assigned participants (1:1) to either intravenous eculizumab or intravenous matched placebo for 26 weeks. Dosing for eculizumab was 900 mg on day 1 and at weeks 1, 2, and 3; 1200 mg at week 4; and 1200 mg given every second week thereafter as maintenance dosing. Randomisation was done centrally with an interactive voice or web-response system with patients stratified to one of four groups based on MGFA disease classification. Where possible, patients were maintained on existing myasthenia gravis therapies and rescue medication was allowed at the study physician's discretion. Patients, investigators, staff, and outcome assessors were masked to treatment assignment. The primary efficacy endpoint was the change from baseline to week 26 in MG-ADL total score measured by worst-rank ANCOVA. The efficacy population set was defined as all patients randomly assigned to treatment groups who received at least one dose of study drug, had a valid baseline MG-ADL assessment, and at least one post-baseline MG-ADL assessment. The safety analyses included all randomly assigned patients who received eculizumab or placebo. This trial is registered with ClinicalTrials.gov, number NCT01997229. Findings Between April 30, 2014, and Feb 19, 2016, we randomly assigned and treated 125 patients, 62 with eculizumab and 63 with placebo. The primary analysis showed no significant difference between eculizumab and placebo (least-squares mean rank 56.6 [SEM 4.5] vs 68.3 [4.5]; rank-based treatment difference -11.7, 95% CI -24.3 to 0.96; p=0.0698). No deaths or cases of meningococcal infection occurred during the study. The most common adverse events in both groups were headache and upper respiratory tract infection (ten [16%] for both events in the eculizumab group and 12 [19%] for both in the placebo group). Myasthenia gravis exacerbations were reported by six (10%) patients in the eculizumab group and 15 (24%) in the placebo group. Six (10%) patients in the eculizumab group and 12 (19%) in the placebo group required rescue therapy. Interpretation The change in the MG-ADL score was not statistically significant between eculizumab and placebo, as measured by the worst-rank analysis. Eculizumab was well tolerated. The use of a worst-rank analytical approach proved to be an important limitation of this study since the secondary and sensitivity analyses results were inconsistent with the primary endpoint result; further research into the role of complement is needed.
  • 若年頭痛患者における睡眠アンケート調査による関連性について
    西郷 和真; 竹下 真未; 佐藤 亜美; 濱田 征宏; 寒川 真; 楠 進
    日本頭痛学会誌 44 2 381 - 381 (一社)日本頭痛学会 2017年11月
  • 再生不良性貧血の同種骨髄移植後に中枢神経原発移植後リンパ増殖性疾患を発症した1例
    山名 正樹; 寺山 敦之; 森川 みゆき; 寒川 真; 鈴木 秀和; 田崎 貴之; 奥田 武司; 小森 舞子; 井上 宏昭; 楠 進
    神経免疫学 22 1 123 - 123 日本神経免疫学会 2017年10月
  • 経静脈的免疫グロブリン療法(IVIg)が奏効した抗ミトコンドリア抗体陽性筋炎の1例
    山名 正樹; 寒川 真; 三井 良之; 西野 一三; 楠 進
    臨床神経学 57 10 662 - 662 (一社)日本神経学会 2017年10月 [査読有り]
  • Motoi Kuwahara; Makoto Samukawa; Tae Ikeda; Miyuki Morikawa; Rino Ueno; Yukihiro Hamada; Susumu Kusunoki
    JOURNAL OF NEUROLOGY 264 3 467 - 475 2017年03月 [査読有り]
     
    Mycoplasma pneumoniae infection often causes various neurological complications of both the central nervous system (CNS) and the peripheral nervous system. We retrospectively investigated the IgM and IgG antibodies to nine glycolipids [GM1, GM2, GM3, GD1a, GD1b, GD3, GT1b, GQ1b, and Gal-C (galactocerebroside)] and clinical features in neurological diseases associated with M. pneumoniae infection diagnosed in multiple hospitals throughout Japan between September 2010 and March 2012. Of the 46 patients with neurological diseases associated with M. pneumoniae infection, 27 were diagnosed with Guillain-Barr, syndrome (GBS), 2 with Fisher syndrome (FS), 16 with CNS diseases, and 1 with both GBS and CNS disease. Anti-Gal-C IgM and IgG antibodies were most frequently detected (23/46, 50%). Patients with CNS diseases were younger than patients with GBS or FS, and IgM antibodies to Gal-C were more frequently detected in the patients with CNS diseases (41%) than in those with GBS or FS (13%). Of the nine patients who were positive for anti-Gal-C IgM antibody but lacked IgG antibody, we found the class-switch of anti-Gal-C antibody from IgM to IgG in two patients. The IgG antibodies appeared during their recovery phase, and the IgG belonged to the IgG1 subclass. Anti-Gal-C antibodies are closely associated with neurological diseases after M. pneumoniae infection. Particularly, anti-Gal-C IgM antibody is more frequently detected in younger patients affected with CNS involvement. The class-switch from IgM to IgG sometimes occurs in anti-Gal-C antibodies.
  • Miyuki Morikawa; Motoi Kuwahara; Rino Ueno; Makoto Samukawa; Yukihiro Hamada; Susumu Kusunoki
    Journal of Neuroimmunology 301 35 - 40 2016年12月 [査読有り]
     
    We performed a serological investigation using glycoarray in Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and multifocal motor neuropathy (MMN). Antibodies to 10 glycolipids and 45 glycolipid complexes were tested. Anti-GM1/sulfatide and anti-GA1/sulfatide IgG antibodies were common in GBS (20.0% and 19.0%, respectively). Anti-GQ1b/sulfatide IgG antibody was detected in 14.0% of GBS patients. IgG antibodies to antigens containing GQ1b were significantly correlated with ophthalmoplegia in GBS (p < 0.01). IgM antibodies to antigens containing GM1 or GalNAc-GD1a were in 50% and 37.5% of MMN patients, respectively. Glycoarray is efficient for detecting antibodies against numerous glycolipid complexes in immune-mediated neuropathies.
  • Makoto Samukawa; Motoi Kuwahara; Miyuki Morikawa; Rino Ueno; Yukihiro Hamada; Kazuo Takada; Makito Hirano; Yoshiyuki Mitsui; Masahiro Sonoo; Susumu Kusunoki
    Journal of Neuroimmunology 301 61 - 64 2016年12月 [査読有り]
     
    Whether patients who have GBS with antibodies to galactocerebroside (Gal-C) and gangliosides (Gal-C-GS-GBS) more often have demyelinating or axonal neuropathy remains controversial. We assessed the electrophysiological data from 16 patients with Gal-C-GS-GBS based on the two established criteria to clarify this issue. In this largest cohort of Gal-C-GS-GBS, eight patients had demyelinating neuropathy and none exhibited axonal neuropathy on either criterion. These data indicated that antibodies to Gal-C, a myelin antigen, might predominantly be associated with demyelinating neuropathy, even in the presence of concomitant antibodies to gangliosides.
  • わが国におけるGBSの予後予測マーカーの検討
    山岸 裕子; 鈴木 秀和; 寒川 真; 桑原 基; 濱田 征宏; 福本 雄太; 山名 正樹; 油原 佳子; 吉川 恵輔; 森川 みゆき; 上野 莉乃; 河合 滋; 岡崎 真央; 西郷 和真; 宮本 勝一; 三井 良之; 楠 進
    臨床神経学 56 Suppl. S425 - S425 (一社)日本神経学会 2016年12月
  • Samukawa M; Kuwahara M; Morikawa M; Ueno R; Hamada Y; Takada K; Hirano M; Mitsui Y; Sonoo M; Kusunoki S
    Journal of neuroimmunology 301 61 - 64 2016年12月 [査読有り]
     
    Whether patients who have GBS with antibodies to galactocerebroside (Gal-C) and gangliosides (Gal-C-GS-GBS) more often have demyelinating or axonal neuropathy remains controversial. We assessed the electrophysiological data from 16 patients with Gal-C-GS-GBS based on the two established criteria to clarify this issue. In this largest cohort of Gal-C-GS-GBS, eight patients had demyelinating neuropathy and none exhibited axonal neuropathy on either criterion. These data indicated that antibodies to Gal-C, a myelin antigen, might predominantly be associated with demyelinating neuropathy, even in the presence of concomitant antibodies to gangliosides. (C) 2016 Elsevier B.V. All rights reserved.
  • Miyuki Morikawa; Motoi Kuwahara; Rino Ueno; Makoto Samukawa; Yukihiro Hamada; Susumu Kusunoki
    JOURNAL OF NEUROIMMUNOLOGY 301 35 - 40 2016年12月 [査読有り]
     
    We performed a serological investigation using glycoarray in Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and multifocal motor neuropathy (MMN). Antibodies to 10 glycolipids and 45 glycolipid complexes were tested. Anti-GM1/sulfatide and anti-GA1/sulfatide IgG antibodies were common in GBS (20.0% and 19.0%, respectively). Anti-GQ1b/sulfatide IgG antibody was detected in 14.0% of GBS patients. IgG antibodies to antigens containing GQ1b were significantly correlated with ophthalmoplegia in GBS (p < 0.01). IgM antibodies to antigens containing GM1 or GaINAc-GD1a were in 50% and 37.5% of MMN patients, respectively. Glycoarray is efficient for detecting antibodies against numerous glycolipid complexes in immune-mediated neuropathies. (C) 2016 Elsevier B.V. All rights reserved.
  • 睡眠中に頭痛発作にて中途覚醒する頭痛の1例
    西郷 和真; 濱田 征宏; 油原 佳子; 寒川 真; 三井 良之; 大森 隆; 楠 進
    日本頭痛学会誌 43 2 319 - 319 (一社)日本頭痛学会 2016年10月
  • Hiroshi Ureshino; Takero Shindo; Hiroyoshi Nishikawa; Nobukazu Watanabe; Eri Watanabe; Natsuko Satoh; Kazutaka Kitaura; Hiroaki Kitamura; Kazuko Doi; Kotaro Nagase; Hiromi Kimura; Makoto Samukawa; Susumu Kusunoki; Masaharu Miyahara; Tadasu Shin-; Ryuji Suzuki; Shimon Sakaguchi; Shinya Kimura
    CANCER IMMUNOLOGY RESEARCH 4 8 644 - 649 2016年08月 [査読有り]
     
    The regulatory T cells (Treg) with the most potent immunosuppressive activity are the effector Tregs (eTreg) with a CD45RA -Foxp3(++)CCR4(+) phenotype. Adult T-cell leukemia (ATL) cells often share the Treg phenotype and also express CCR4. Although mogamulizumab, a monoclonal antibody to CCR4, shows marked antitumor effects against ATL and peripheral T-cell lymphoma, concerns have been raised that it may induce severe autoimmune immunopathology by depleting eTregs. Here, we present case reports for two patients with ATL who responded to mogamulizumab but developed a severe skin rash and autoimmune brainstem encephalitis. Deep sequencing of the T-cell receptor revealed that ATL cells and naturally occurring Tregs within the cell population with a Treg phenotype can be clearly distinguished according to CADM1 expression. The onset of skin rash and brainstem encephalitis was coincident with eTreg depletion from the peripheral blood, whereas ATL relapses were coincident with eTreg recovery. These results imply that eTreg numbers in the peripheral blood sensitively reflect the equilibrium between antitumor immunity and autoimmunity, and that mogamulizumab might suppress ATL until the eTreg population recovers. Close monitoring of eTreg numbers is crucial if we are to provide immunomodulatory treatments that target malignancy without severe adverse events. (C) 2016 AACR.
  • RCVS、片麻痺性片頭痛の両者の病態が考えられ、治療経過中に群発頭痛を呈した一例
    濱田 征宏; 福本 雄太; 河合 滋; 西郷 和真; 桑原 基; 鈴木 秀和; 寒川 真; 楠 進
    日本頭痛学会誌 42 2 133 - 133 (一社)日本頭痛学会 2015年11月
  • RCVS、片麻痺性片頭痛の両者の病態が考えられ、治療経過中に群発頭痛を呈した一例
    濱田 征宏; 福本 雄太; 河合 滋; 西郷 和真; 桑原 基; 鈴木 秀和; 寒川 真; 楠 進
    日本頭痛学会誌 42 2 133 - 133 (一社)日本頭痛学会 2015年11月
  • Samukawa M; Kusunoki S
    Nihon rinsho. Japanese journal of clinical medicine 73 Suppl 7 367 - 373 2015年09月 [査読有り]
  • Kazumasa Saigoh; Jun Mitsui; Makito Hirano; Mitsuaki Shioyama; Makoto Samukawa; Yaeko Ichikawa; Jun Goto; Shoji Tsuji; Susumu Kusunoki
    PARKINSONISM & RELATED DISORDERS 21 3 332 - 334 2015年03月 [査読有り]
  • Yukihiro Hamada; Makito Hirano; Motoi Kuwahara; Makoto Samukawa; Kazuo Takada; Jyoji Morise; Keiko Yabuno; Shogo Oka; Susumu Kusunoki
    NEUROSCIENCE RESEARCH 91 63 - 68 2015年02月 [査読有り]
     
    Anti-myelin-associated-glycoprotein (MAG) neuropathy is an intractable autoimmune polyneuropathy. The antigenic region of MAG is the human natural killer-1 (HNK-1) carbohydrate. We and others previously suggested that the extension of antibody reactivities to HNK-1-bearing proteins other than MAG was associated with treatment resistance, without statistical analyses. In this study, we established an ELISA method with recombinant proteins to test binding specificities of currently available monoclonal antibodies to MAG and another HNK-1-bearing protein, phosphacan. Using this system, we found the distinct binding specificities of anti-MAG antibody in 19 patients with anti-MAG neuropathy. Their clinical relevance was then determined retrospectively with the adjusted 10-points INCAT disability score (0 = normal and 10 = highly disable). The results showed that strong reactivities of anti-MAG antibodies to phosphacan were significantly associated with treatment resistance or progressive clinical courses, indicating a possible clinical relevance of the binding specificities. (C) 2014 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.
  • Kogawa S; Nakajima A; Kobashi S; Samukawa M; Kusunoki S
    Rinsho shinkeigaku = Clinical neurology 55 3 171 - 173 (一社)日本神経学会 2015年 [査読有り]
     
    症例は67歳男性。某日、両下肢違和感出現。3日後に道で転倒し当院救急入院。入院時、脳神経系異常なし。四肢筋力保たれるも両下肢深部覚障害のため起立困難。深部腱反射減弱、表在覚障害なし。腰椎MRI異常なし。神経伝導検査は運動神経異常なく、上下肢で著明な感覚神経振幅低下あり。Gal-C抗体陽性であり、純粋感覚型Guillain-Barre症候群(GBS)と診断。免疫グロブリン大量静注療法をおこなうも深部覚障害残存し入院53日目に転院。Gal-C抗体のみ陽性の純粋感覚型GBS症例の報告はなく貴重な症例と考えられた。(著者抄録)
  • Mogamulizumab投与に関連した抗ガングリオシド抗体陽性脳幹脳炎(Brainstem Encephalitis with anti-ganglioside antibody associated with mogamulizumab treatment)
    Ureshino Hiroshi; Shindo Takero; Miyahara Masaharu; Nishikawa Hiroyoshi; Sakaguchi Shimon; Samukawa Makoto; Kusunoki Susumu; Kimura Shinya
    臨床血液 55 9 1489 - 1489 2014年09月
  • Nobutsune Ishikawa; Yoshiyuki Kobayashi; Yuji Fujii; Makoto Samukawa; Susumu Kusunoki; Masao Kobayashi
    PEDIATRIC NEUROLOGY 51 3 441 - 443 2014年09月 [査読有り]
     
    INTRODUCTION: Postinfectious peripheral neuropathy can be associated with various viral or bacterial infections. Group A beta-hemolytic Streptococcus infection can lead to neurological disorders, which involve predominantly the central nervous system, whereas peripheral neuropathy during childhood is rare. PATIENT DESCRIPTION: We describe a 12-year-old boy who presented with peripheral polyneuropathy associated with Group A beta-hemolytic Streptococcus infection. Anti-GM1 IgM was significantly elevated in his serum during the acute phase, which suggested that it was related with the pathophysiology in this patient. CONCLUSION: Group A beta-hemolytic Streptococcus infection may cause peripheral neuropathy via the autoimmune system and glycolipids.
  • 寒川 真
    Journal of the Neurological Sciences 337 1-2 55 - 60 2014年02月 [査読有り]
     
    Introduction: Guillain-Barre syndrome (GBS) has often been associated with antibodies to glycolipids, such as galactocerebroside (Gal-C), a component of myelin. Whether patients who have GBS with anti-Gal-C antibody (Gal-C-GBS) more often have demyelinating neuropathy or axonal neuropathy remains controversial. Their clinical features have also been unestablished. Methods: We enrolled 47 patients with Gal-C-GBS. Their clinical and electrophysiological data were retrospectively reviewed and compared to 119 patients with CBS without anti-Gal-C antibody (non-Gal-C-GBS). Results: Demyelinating polyneuropathy occurred 4 times more frequently than axonal polyneuropathy in patients with Gal-C-GBS, but without statistical significance compared to patients with non-Gal-C-GBS (2.2:1). Patients with Gal-C-GBS had more frequent sensory deficits, autonomic involvements, and antecedent Mycoplasma pneumoniae (MP) infection than patients with non-Gal-C-GBS. Conclusions: This is the largest study clarifying the clinical and electrophysiological findings that more frequent sensory deficits, autonomic involvements, and antecedent MP infection are associated with Gal-C-GBS. (C) 2013 Elsevier B.V. All rights reserved.
  • IgMパラプロテイン血症を伴うニューロパチーにおけるHNK-1抗体活性と臨床特徴の関連
    濱田 征宏; 寒川 真; 桑原 基; 藪野 景子; 森瀬 譲二; 高田 和男; 宮本 勝一; 岡 昌吾; 楠 進
    臨床神経学 53 12 1507 - 1507 (一社)日本神経学会 2013年12月
  • Samukawa Makoto; Ichihara Gaku; Ueda Masami; Mitsui Yoshiyuki; Oka Nobuyuki; Kusunoki Susumu
    ANNALS OF NEUROLOGY 74 S4  2013年10月 [査読有り]
  • 寒川真; 塩山実章; 鈴木秀和; 上田昌美; 岡伸幸; 市原学; 三井良之; 楠進
    産業医学ジャーナル 36 4 16 - 21 産業医学振興財団 2013年07月 
    症例は43歳男性で、2年前より金属部品洗浄業務に従事していた。右下肢痛に続く左下肢痛、ふらつきの出現で紹介受診し、四肢の異常感覚、疼痛、運動失調を認め、両下肢の温痛覚低下、振動覚消失が認められた。胸部X線・胸腹骨盤造影CT・頸髄MRIで異常所見なく、末梢神経伝導検査で運動神経・感覚神経とも下肢優位に遠位潜時延長、振幅低下、伝導速度低下を認め、精査加療目的に入院した。腓腹神経の生検でときほぐし線維法にて約30%に軸索障害を示唆するmyelin ovoidを認め、エポン包埋標本では大径有髄線維の減少とその10~20%に軸索腫大や消失、myelin ovoidが認められた。詳細な問診で金属紛洗浄剤中に1-Bromopropane(1-BP)の含有が判明し、初診時の残血清検査で血清臭素値の高値を認め、1-BP暴露による中毒性神経障害と判断し、退院の上外来での経過観察とした。徐々に症状は改善し、最終暴露後7ヵ月時点で独歩外出、自転車走行も可能となった。
  • Motoi Kuwahara; Hidekazu Suzuki; Makoto Samukawa; Yukihiro Hamada; Kazuo Takada; Susumu Kusunoki
    JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY 84 5 573 - 575 2013年05月 [査読有り]
     
    Background LM1 is the predominant glycolipid in human peripheral nerve myelin and antibodies to LM1 and LM1-containing ganglioside complexes are detected in some patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The clinical features of patients with such antibodies have not yet been investigated. Methods Serum antibodies to LM1, a mixture of GM1 and LM1 (GM1/LM1), and that of GD1b and LM1 (GD1b/LM1) were examined in 75 consecutive patients with CIDP. The clinical features of the CIDP patients with such antibodies in the present series and those in the previous reports were investigated and compared with those of antibody-negative patients. Results Of the 75 patients with CIDP, two had antibodies to LM1, three had anti-GM1/LM1 complex antibody, one had anti-GD1b/LM1 complex antibody and two had antibodies to both the GM1/LM1 and GD1b/LM1 complexes. Patients with the LM1-associated antibodies did not have cranial nerve deficits (p<0.05) and exhibited ataxia more frequently than the antibody-negative patients (p<0.01). Conclusion In humans, LM1 is contained more in the dorsal root than in the cranial nerves. The clinical features of CIDP patients with antibodies to LM1 and LM1-containing complexes may be associated with the distribution of the LM1 antigen. LM1-associated antibodies are possible markers for a subclass of CIDP.
  • 鈴木秀和; 桑原基; 山藤聖子; 寒川真; 濱田征宏; 楠進
    神経治療学 30 2 173 - 179 (一社)日本神経治療学会 2013年03月 
    慢性炎症性脱髄性多発ニューロパチー(chronic inflammatory demyelinating polyradiculoneuropathy:CIDP)は、難治性の末梢神経に対する自己免疫性疾患であるが、その病態は未解明の部分が多い。本研究では、2010年のEuropean Federation of Neurological Societies/Peripheral Nerve Society(EFNS/PNS)のCIDP診断基準に基づいてprobable以上であった106例を解析した。抗糖脂質抗体の測定と、ファーストラインの治療である免疫グロブリン大量療法(intravenous immunoglobulins:IVIG)、副腎皮質ステロイド療法の反応性を中心にアンケート調査に基づいて、臨床的解析を行った。抗糖脂質抗体陽性率は8.4%であった。IVIGの有効率は86%、副腎皮質ステロイド療法の有効率は69%であった。髄液中総蛋白が高値(>100mg/dl)であった症例はIVIGもしくは副腎皮質ステロイド療法にて反応性が得られていた。また、少数例での検討であったが、抗糖脂質抗体陽性例は感覚障害が優位である例が多かった。(著者抄録)
  • Hikaru Sakamoto; Makito Hirano; Makoto Samukawa; Shuichi Ueno; Shunji Maekura; Harutoshi Fujimura; Motoi Kuwahara; Yukihiro Hamada; Chiharu Isono; Keiko Tanaka; Susumu Kusunoki; Yusaku Nakamura
    European Neurology 69 1 21 - 26 2013年02月 [査読有り]
     
    Anti-N-methyl-d-aspartate receptor (anti-NMDAR) antibody-associated encephalitis is an immunologic disease characterized by a female preponderance. Males are infrequently affected. The clinical symptoms of affected boys as well as girls have been summarized, and they have some clinical features distinct from those of adults. However, the characteristics of men have been described in only a few reports. We describe in detail four men with anti-NMDAR encephalitis who presented with several clinical features that complicated disease management and recovery, including venous thrombosis, bilateral hippocampal involvement, hypersexuality, and joint contracture. We also report the first detailed clinical information about a male patient who died of this disease. In addition, we summarize the clinical characteristics of five patients previously reported by others. © 2012 S. Karger AG, Basel.
  • Makoto Samukawa; Makito Hirano; Jun Tsugawa; Hikaru Sakamoto; Emi Tabata; Kazuo Takada; Motoi Kuwahara; Seiko Suzuki; Mari Kitada; Tatsuo Yamada; Hideo Hara; Yoshio Tsuboi; Yusaku Nakamura; Susumu Kusunoki
    NEUROSCIENCE RESEARCH 74 3-4 284 - 289 2012年12月 [査読有り]
     
    Acute disseminated encephalomyelitis causes multifocal demyelination in the central nerve system. Although this disease generally responds well to steroid therapy, it is occasionally steroid-resistant, leading to poor outcomes. Serological markers of prognosis are currently unavailable. We measured anti-glycolipid antibodies in 25 consecutive patients with acute disseminated encephalomyelitis, and found that four patients were positive for anti-galactocerebroside antibodies. All four patients had a poor response to steroids. We summarize clinical information on these four patients and three similar patients reported previously. This is the first report to describe concomitant involvement of the central nerve system and peripheral nervous system in anti-galactocerebroside antibody-associated acute disseminated encephalomyelitis, consistent with the location of galactocerebroside, and to document a dramatic response to repeated intravenous immunoglobulin therapy after unsuccessful steroid treatment in one patient. (C) 2012 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.
  • Makoto Samukawa; Gaku Ichihara; Nobuyuki Oka; Susumu Kusunoki
    Archives of internal medicine 172 16 1257 - 60 2012年09月 [査読有り]
     
    Health hazard alerts to 1-bromopropane, an alternative to ozone layer-damaging organic solvents, have been issued by some countries. Herein, we report a new case of 1-bromopropane-induced neurotoxicity. A 43-year-old male industrial worker developed muscle weakness, pain, numbness, and gait disturbance. Neurological examination indicated sensory ataxic neuropathy associated with mild impairment of upper motor neurons. He had used 1-bromopropane as a cleaning agent for metal parts at his workplace without appropriate protection. The serum bromide level was elevated at the onset of clinical manifestations. Histopathologic examination of sural nerve biopsy showed axonal damage. Under the tentative diagnosis of 1-bromopropane toxicity, he was kept away from exposure to the solvent. This resulted in gradual improvement of symptoms, recovery of motor function, and resolution of sensory deficits. The diagnosis of 1-bromopropane neurotoxicity in this case was based on details of the work environment, the clinical course, and laboratory and pathologic findings. To our knowledge, this is the first report that describes nerve biopsy findings in a human case.
  • 楠 進; 寒川 真; 三井 良之; 鈴木 秀和; 市橋珠里; 長谷川隆典; 北口正孝
    神経治療学 29 4 429 - 434 (一社)日本神経治療学会 2012年07月 
    免疫グロブリン大量静注療法が寛解維持に有効であった抗signal recognition particle(SRP)抗体陽性筋炎の2例を経験した。症例1は33歳女性。X年より筋痛・筋力低下を自覚し当科紹介受診となった。CK上昇と筋生検にて炎症細胞浸潤に乏しい壊死・再生像を観察し、抗SRP抗体陽性で抗SRP抗体陽性筋炎と診断した。ステロイド療法のみでは寛解が得られなかったが、IVIgを追加したところCK低下、筋力の改善が得られた。症例2は56歳女性。Y年頃より歩行困難・筋力低下が出現した。近医にて、著明なCK上昇と筋生検結果より、暫定的に多発筋炎と診断された。ステロイド内服療法にて治療されたが、漸減中による再燃を繰り返したため、Y+6年当科紹介となった。当院で再度施行した筋生検で、炎症細胞浸潤に乏しい壊死・再生像を観察し、抗SRP抗体陽性が判明し抗SRP抗体陽性筋炎と診断した。ステロイド療法のみでは寛解が得られなかったため、IVIgを追加したところ、CK値低下、筋力の改善が得られた。ステロイド漸減中、軽度のCK上昇、筋力低下を認めたため、cyclosporinを併用し寛解が得られている。免疫グロブリン大量静注療法にて寛解維持が得られた抗SRP抗体陽性筋炎の2例について文献的考察とともに報告する。(著者抄録)
  • 痙攣発作と精神症状を呈したMorvan症候群の1例
    楠 進; 寒川 真; 鈴木 秀和; 北田茉里; 塩山 実章; 三井 良之; 中村 雄作
    大阪てんかん研究会雑誌 23 1 7 - 14 大阪てんかん研究会 2012年 
    Morvan症候群は、免疫学的機序により中枢神経障害と末梢神経の過剰興奮を来す稀な疾患である。症例の中には胸腺腫、肺小細胞癌などの腫瘍に随伴することがあり傍腫瘍性神経症候群と考えられている。また、一部の症例ではvoltage-gated potassium channelおよびその関連タンパクに対する自己抗体が陽性となる。免疫療法の反応性は良好とされているが、発症様式や臨床経過は多岐にわたり、診断・治療に難渋する症例も存在する。今回、我々は精神症状にて発症し、亜急性の経過で意識障害、痙攣発作が加わり、当初Creutzfeldt-Jakob diseaseが疑われたMorvan症候群の一例を経験した。症例は63歳男性。X年1月頃から緩徐進行性にうつ症状、食思不振、体重減少が出現した。同年5月に近医入院となった。同年8月中旬から亜急性の経過で意識障害、痙攣が加わり、Creutzfeldt-Jakob disease疑いで8月末に当院へ転院となった。神経学診察では高度な意識障害、筋緊張亢進、および全身性に豊富なmyokymiaを観察した。針筋電図で全身に分布するmyokymic dischargeを確認したためMorvan症候群を疑った。ステロイドパルス療法にて意識障害、筋緊張の劇的な改善、myokymic dischargeの消失が得られたが、うつ症状が残存した。その後、意識障害、myokymic dischargeの再燃時に、血漿交換、免疫グロブリン大量静注療法、ステロイドパルス療法、経口ステロイド投与などの免疫療法を行ったが、うつ症状は治療抵抗性であった。長期間継続するうつ症状の原因として、ステロイド精神病を考え、ステロイド漸減したところ、うつ症状の改善が得られた。積極的に腫瘍検索を行ったが腫瘍は認めず、voltage-gated potassium channelおよびその関連タンパクに対する自己抗体は陰性であった。精神症状、意識障害などの中枢神経障害に加え、fasciculation様の下位ニューロン障害を合併する場合は、重要な鑑別診断として、Morvan症候群を念頭に置く必要があると考えられた。(著者抄録)
  • 上田 昌美; 豊増 麻美; 大洞 佳代子; 福田 寛二; 鈴木 秀和; 寒川 真; 上野 周一; 市橋 珠里; 楠 進
    The Japanese Journal of Rehabilitation Medicine The Japanese Journal of Rehabilitation Medicine 48 12 795 - 795 (公社)日本リハビリテーション医学会 2011年12月
  • Makoto Samukawa; Makito Hirano; Hikaru Sakamoto; Mari Kitada; Susumu Kusunoki; Yusaku Nakamura
    MOVEMENT DISORDERS 26 14 2572 - 2573 2011年12月 [査読有り]
  • H. Suzuki; M. Samukawa; M. Kitada; J. Ichihashi; Y. Mistui; K. Tanaka; S. Kusunoki
    EUROPEAN JOURNAL OF NEUROLOGY 18 11 E145 - E146 2011年11月
  • Samukawa M; Hirano M; Saigoh K; Kawai S; Hamada Y; Takahashi D; Nakamura Y; Kusunoki S
    Cerebellum. Epub ahead of print [査読有り]

書籍

講演・口頭発表等

MISC

受賞

  • 2021年10月 日本神経摂食嚥下・栄養学会学術集会 第16・17回日本神経摂食嚥下・栄養学会学術集会優秀演題賞
  • 2017年07月 近畿大学医学会 近畿大学医学会賞
     Electrophysiological assessment of Guillain-Barré syndrome with both Gal-C and ganglioside antibodies; tendency for demyelinating type 
    受賞者: 寒川 真
  • 2015年07月 近畿大学医学会 近畿大学医学会賞
     Clinical features in Guillain-Barré syndrome with anti-Gal-C antibody 
    受賞者: 寒川 真
  • 2013年11月 日本神経免疫学会 第25回日本神経免疫学会学術集会ベストポスター賞
     JPN international_society 
    受賞者: 寒川 真
  • 2013年07月 近畿大学医学会 近畿大学医学会賞
     A Case of Severe Neurotoxicity Associated With Exposure to 1-Bromopropane, an Alternative to Ozone-Depleting or Global-Warming Solvents 
    受賞者: 寒川 真

委員歴

  • 2018年08月 - 2022年05月   日本神経学会   重症筋無力症/ランバート・イートン筋無力症候群診療ガイドライン作成委員会研究協力者

その他のリンク

researchmap



Copyright © MEDIA FUSION Co.,Ltd. All rights reserved.