Suzuki, Shigeaki



School of Medicine, Department of Internal Medicine (Neurology) Department of Neurology (Shinanomachi)


Associate Professor

Related Websites

Contact Address

Shinanomachi, Shinjuku-ku

Telephone No.


Fax No.


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Profile Summary 【 Display / hide

  • Non-motor symptoms in myasthenia gravis

    Muscle and autoimmunity

Career 【 Display / hide

  • 1993.04


  • 1995.05

    Kawasaki Municipal Hosipital

  • 1996.06


  • 1997.05

    Keio University School of Medicine, Department of Neurology

  • 2003.04

    New York Medical College

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Academic Background 【 Display / hide

  • 1993.03



Academic Degrees 【 Display / hide

  • 医学博士, 慶應義塾大学, 2001.10


Research Areas 【 Display / hide

  • Life Science / Neurology (Autoimmunity)

Research Keywords 【 Display / hide

  • Autoantibodies

Research Themes 【 Display / hide

  • Immune-mediated adverse events, 


  • Inflammatory myopathies, 


  • Myasthenia gravis, 



Papers 【 Display / hide

  • Cytometric cell-based assays for anti-striational antibodies in myasthenia gravis with myositis and/or myocarditis

    Kufukihara K., Watanabe Y., Inagaki T., Takamatsu K., Nakane S., Nakahara J., Ando Y., Suzuki S.

    Scientific Reports (Scientific Reports)  9 ( 1 )  2019.12

     View Summary

    © 2019, The Author(s). The purposes of the present study were to identify anti-striational antibodies in myasthenia gravis (MG) patients with myositis and/or myocarditis using a combination of cell-based assays and flow cytometry (cytometric cell-based assays) and to describe the main clinical implications. Among 2,609 stored samples collected from all over Japan between 2003 and 2016, we had serum samples from 30 MG patients with myositis and/or myocarditis. Cytometric cell-based assays with titin, ryanodine receptor, and voltage-gated Kv1.4 were performed. Autoantibodies were determined by differences in phycoerythin fluorescence between the 293F cells and titin-transfected cells. MG patients with myositis and/or myocarditis as well as late-onset and thymoma-associated MG had anti-titin, anti-ryanodine receptor, and anti-Kv1.4 antibodies. In contrast, patients with early-onset MG, those with other myopathies and healthy controls did not have anti-titin or anti-Kv1.4 antibodies with some exceptions, but they possessed anti-ryanodine receptor antibodies. Thirty MG patients with myositis and/or myocarditis showed a severe generalized form, and 21 of them had thymoma. Anti-titin and anti-Kv1.4 antibodies were found in 28 (93%) and 15 (50%) patients, respectively, and all patients had at least one of these antibodies. Cytometric cell-based assays thus demonstrated that anti-striational antibodies are biomarkers of MG with myositis and/or myocarditis.

  • Diagnostic potential of sarcoplasmic myxovirus resistance protein A expression in subsets of dermatomyositis

    Uruha A., Allenbach Y., Charuel J., Musset L., Aussy A., Boyer O., Mariampillai K., Landon-Cardinal O., Rasmussen C., Bolko L., Maisonobe T., Leonard-Louis S., Suzuki S., Nishino I., Stenzel W., Benveniste O.

    Neuropathology and Applied Neurobiology (Neuropathology and Applied Neurobiology)  45 ( 5 ) 513 - 522 2019.08

    ISSN  03051846

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    © 2018 British Neuropathological Society Aims: To elucidate the diagnostic value of sarcoplasmic expression of myxovirus resistance protein A (MxA) for dermatomyositis (DM) specifically analysing different DM subforms, and to test the superiority of MxA to other markers. Methods: Immunohistochemistry for MxA and retinoic acid-inducible gene I (RIG-I) was performed on skeletal muscle samples and compared with the item presence of perifascicular atrophy (PFA) in 57 DM patients with anti-Mi-2 (n = 6), -transcription intermediary factor 1 gamma (n = 10), -nuclear matrix protein 2 (n = 13), -melanoma differentiation-associated gene 5 (MDA5) (n = 10) or -small ubiquitin-like modifier activating enzyme (n = 1) autoantibodies and with no detectable autoantibody (n = 17). Among the patients, nine suffered from cancer and 22 were juvenile-onset type. Disease controls included antisynthetase syndrome (ASS)-associated myositis (n = 30), immune-mediated necrotizing myopathy (n = 9) and inclusion body myositis (n = 5). Results: Sarcoplasmic MxA expression featured 77% sensitivity and 100% specificity for overall DM patients, while RIG-I staining and PFA reached respectively 14% and 59% sensitivity and 100% and 86% specificity. In any subset of DM, sarcoplasmic MxA expression showed higher sensitivity than RIG-I and PFA. Some anti-MDA5 antibody-positive DM samples distinctively showed a scattered staining pattern of MxA. No ASS samples had sarcoplasmic MxA expression even though six patients had DM skin rash. Conclusions: Sarcoplasmic MxA expression is more sensitive than PFA and RIG-I expression for a pathological diagnosis of DM, regardless of the autoantibody-related subgroup. In light of its high sensitivity and specificity, it may be considered a pathological hallmark of DM per se. Also, lack of MxA expression in ASS supports the idea that ASS is a distinct entity from DM.

  • A case of anti-titin antibody positive nivolumab-related necrotizing myopathy with myasthenia gravis

    Isami A., Uchiyama A., Shimaoka Y., Suzuki S., Kawachi I., Fujita N.

    Rinsho shinkeigaku = Clinical neurology (Rinsho shinkeigaku = Clinical neurology)  59 ( 7 ) 431 - 435 2019.07

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    A 53-year-old man suffering from squamous cell lung cancer presented with bilateral ptosis and bulbar palsy a month after initial treatment with the immune checkpoint inhibitor nivolumab. The symptoms showed worsening from midday, suggesting myasthenia gravis (MG), although anti-AChR antibody was negative. Although no muscle weakness was detected, the CK level was elevated to 5,255 IU/l, and MRI of the thigh revealed inflammation of the bilateral rectus femoris muscle. A muscle biopsy showed signs of necrotizing myopathy with expression of sarcolemmal HLA class I and accumulation of macrophages, CD4, CD8, and CD20-positive lymphocytes. Positivity for anti-titin antibody, one of the anti-striated muscle antibodies, was evident. The patient was diagnosed as having nivolumab-related necrotizing myopathy with myasthenia gravis, an immune-related adverse event (irAE). Treatment with prednisolone rapidly ameliorated the symptoms, and the serum CK level normalized. There have been several reports of nivolumab-related myositis with MG. On the basis of the muscle pathology and antibody data, we were able to clarify that necrotizing myopathy was related to the pathogenesis of this case.

  • Concurrent positive anti-3-hydroxy-3-methylglutaryl-coenzyme a reductase antibody with reducing body myopathy: Possible double trouble

    Tanboon J., Sanmaneechai O., Charuvanij S., Sangruchi T., Galindo-Feria A., Lundberg I., Ohnuki Y., Shiina T., Suzuki S., Nishino I.

    Neuromuscular Disorders (Neuromuscular Disorders)  29 ( 7 ) 543 - 548 2019.07

    ISSN  09608966

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    © 2019 Elsevier B.V. Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myopathy is less common in children but has been associated with more favorable prognosis than adult patients after immunotherapies. We report anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody positivity in a 6-year-old boy with progressive muscle weakness, scoliosis, spinal rigidity, multiple joint contractures, mild left ventricular hypertrophy, and elevated serum creatine kinase. In contrast to most of previously reported pediatric anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase myopathy, he showed little response to immunotherapies. Muscle biopsy contained changes suggestive of myofiber necrosis and regeneration and reducing bodies. The diagnosis of reducing body myopathy was later confirmed by reported c.368A>G (p.His123Arg) mutation in the FHL1 gene. Although the level of association between these two conditions is still inconclusive, this is the first report of concurrent positive anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody with reducing body myopathy emphasizing the possibility of co-occurrence of immune mediated necrotizing myopathy and muscular dystrophy and importance of comprehensive diagnostic investigations in unusual cases.

  • Inflammatory myopathy associated with PD-1 inhibitors

    Seki M., Uruha A., Ohnuki Y., Kamada S., Noda T., Onda A., Ohira M., Isami A., Hiramatsu S., Hibino M., Nakane S., Noda S., Yutani S., Hanazono A., Yaguchi H., Takao M., Shiina T., Katsuno M., Nakahara J., Matsubara S., Nishino I., Suzuki S.

    Journal of Autoimmunity (Journal of Autoimmunity)  100   105 - 113 2019.06

    ISSN  08968411

     View Summary

    © 2019 Elsevier Ltd Objective: To characterize the inflammatory myopathy associated with programmed cell death 1 inhibitors (PD-1 myopathy). Methods: We studied 19 Japanese patients with PD-1 myopathy (13 men and 6 women, mean age 70 years), who were referred to Keio University. As control groups, we used 68 patients with anti-signal recognition particle antibodies, 51 patients with anti-aminoacyl transfer RNA synthetase antibodies and 460 healthy subjects. Results: In regard to muscle-disease severity, 10 patients showed a mild form of disease and 9 patients showed a severe form. Non-small cell lung cancer was the most common underlying cancer. PD-1 inhibitor consisted of 11 nivolumab and 8 pembrolizumab. PD-1 myopathy occurred 29 days on average after the first administration of PD-1 inhibitor. The initial manifestation of muscle weakness was ptosis in 10 patients, 15 patients had ptosis, 13 diplopia, 8 facial muscle weakness, 10 bulbar symptoms, 13 limb weakness, 14 neck weakness, 4 cardiac involvement, 6 respiratory involvement and 16 myalgia. Ocular, facial, cardiac and respiratory involvement and myalgia were more frequently observed than controls. Serum creatine kinase was increased to 5247 IU/L on average. Autoantibodies related to inflammatory myopathy were negative, while anti-striational antibodies were found in 13 (68%)patients. HLA-C*12:02 alleles were more frequently detected than healthy controls. Muscle pathology was characterized by multifocal necrotic myofibers with endomysial inflammation and expression of MHC class I. Immunosuppressive therapy with corticosteroids was generally effective for muscle weakness. Conclusions: Based on our clinical, histological and immunological findings, PD-1 myopathy is a discrete subset of inflammatory myopathy.

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Papers, etc., Registered in KOARA 【 Display / hide

Reviews, Commentaries, etc. 【 Display / hide

Presentations 【 Display / hide

  • 脳虚血・再灌流時のサイクリックAMP応答因子結合蛋白(CREB)リン酸化と細胞保護

    Tanakakoutarou, Suzukishigeaki, Denpoutomohisa, Kosakaiarifumi




  • 実験的脳虚血後のIL-6 cytokine family共有受容体GP130の細胞局在

    Suzukishigeaki, Tanakakoutarou, Nogawashigeru, Denpousomohisa, Kosakaiarifumi, Fukuuchiyasuo



    Oral presentation (general)

  • オリゴデンドロサイトおよびその前駆細胞の脳虚血反応

    Tanaka Koutarou, Nagata Eiichirou, Suzuki Shigeaki, Denpou Tomohisa, Kosakai Arifumi




  • Activation and proleferation of oligodendrocyte progenitor cells after brain ischemia

    Tanaka Kotaro, Nogawa Shigeru, Ito Daisuke, Suzuki Shigeaki, Dembo Tomohisa, Kosakai Arifumi, Fukuuchi Yasuo

    Joint international symposium on molecular mechanism and epochal therapeutics dor ischemic stroke and dementia, 



  • COX-2 expression in brains of patients with familial Alzheimer's disease

    Nogawa Shigeru, Takao Motoki, Suzuki Shigeaki, Tanaka Kortaro, Koto Atsuo, Fukuuchi Yasuo, Hayakawa Isao

    International Symposium on Molecular Mechanism and Epochal Therapeutics for Ischemic Stroke Dementia, 


    Poster presentation

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Research Projects of Competitive Funds, etc. 【 Display / hide

  • 抗横紋筋抗体の病因論的自己抗体としての意義とPD-1ミオパチーの疾患概念の確立


    MEXT,JSPS, Grant-in-Aid for Scientific Research, Grant-in-Aid for Scientific Research (B), Principal investigator

  • 壊死性ミオパチーに関連する自己抗体の病態機序の解明と臨床応用に向けて


    MEXT,JSPS, Grant-in-Aid for Scientific Research, Grant-in-Aid for Scientific Research (C), Principal investigator

  • Clinical and histological features of inflammatory myopathies mediated by autoantibodies


    MEXT,JSPS, Grant-in-Aid for Scientific Research, Grant-in-Aid for Scientific Research (C), Principal investigator

     View Summary

    Immune-mediated necrotizing myopathy (IMNM) is a pathological entity characterized by necrosis in the absence of prominent endomysial lymphocytic infiltration. Autoantibodies against signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with IMNM. The frequencies of anti-SRP and anti-HMGCR antibodies in patients with IMNM were 39% and 25%, respectively. Severe limb muscle weakness, neck weakness, dysphagia, respiratory insufficiency, and muscle atrophy were more frequently observed in patients with anti-SRP antibodies than with anti-HMGCR antibodies. HLA-DRB1 genotyping revealed the association of anti-SRP antibodies with DRB1*08:03 and that of anti-HMGCR antibodies with DRB1*11:01.

  • 横紋筋細胞を用いた免疫沈降法による自己抗体検索の臨床応用に向けて


    文部科学省, 科学研究費(基盤研究C), Research grant, Principal investigator

  • 慢性期脳虚血の病態におけるStat3リン酸化の意義


    文部科学省, 科学研究費(基盤研究C), Research grant, Principal investigator

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Intellectual Property Rights, etc. 【 Display / hide

  • Cytometric cell-based assayによる抗横紋筋抗体の測定

    Date applied:   2018.02 

    Patent, Joint

Awards 【 Display / hide

  • 2015年度日本神経治療学会活動賞


  • 日本神経免疫学会創世賞


  • 日本内科学会奨励賞


  • 慶應医学三四会奨励賞



Courses Taught 【 Display / hide











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Courses Previously Taught 【 Display / hide

  • 神経疾患各論(多発性硬化症・免疫介在性脳炎)

    Keio University