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BRAIN and NERVE Volume 65, Issue 4 （April 2013）

BRAIN and NERVE 65巻4号（2013年4月発行）

Japanese English

総説

重症筋無力症の非運動症状―胸腺腫に伴う免疫異常を中心に Non-motor Symptoms in Myasthenia Gravis: Attributed to T-cell Clones from Thymoma 鈴木重明 ¹ Shigeaki Suzuki ¹ ¹慶應義塾大学医学部神経内科 ¹Department of Neurology, Keio University School of Medicine キーワード： thymoma , myasthenia gravis , CD8⁺T-cell cytotoxicity , autoantibodies , voltage-gated potassium channels , non-motor symptoms Keyword: thymoma , myasthenia gravis , CD8⁺T-cell cytotoxicity , autoantibodies , voltage-gated potassium channels , non-motor symptoms pp.477-483

発行日 2013年4月1日 Published Date 2013/4/1

DOI https://doi.org/10.11477/mf.1416101479

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参考文献 Reference

はじめに

　自己免疫性重症筋無力症（autoimmune myasthenia gravis：MG）は神経筋接合部におけるアセチルコリン受容体（AChR）あるいは筋特異的チロシンキナーゼ（MuSK）に対する自己抗体が原因となる臓器特異性自己免疫疾患である^1,2)。全身の骨格筋における易疲労性と筋力低下などの運動症状がMGの主要な症状である。

　近年，パーキンソン病をはじめとする神経疾患において運動症状以外にも多彩な非運動症状（non-motor symptoms）が認められることが広く知られている。これまで注目されてこなかったものの，MG患者において非運動症状が出現し，運動症状よりも患者QOL（quality of life）に大きな影響を与える可能性がある。

Abstract

　Autoimmune myasthenia gravis (MG) comprises a broad spectrum of non-motor symptoms. Patients with MG sometimes have psychiatric symptoms, such as depression and anxiety, which impair their quality of life. However, these symptoms are not principally immune-mediated. Moreover, some patients with MG have additional autoimmune disorders. The clinical manifestations of these diseases are also considered non-motor symptoms, even though the autoimmune mechanisms underlying the association between MG and these disorders are not fully understood. In this review, we clarify that the non-motor symptoms of autoimmune disorders are attributed to abnormal T-cell clones from thymomas. We review the clinical characteristics of patients with MG and associated thymomas who exhibit non-motor symptoms based on previously reported cases and our multicenter cooperative study. CD8⁺ T-cell cytotoxicity against hematopoietic precursor cells in the bone marrow and unidentified autoantigens in hair follicles result in the development of pure red cell aplasia, immunodeficiency, and alopecia areata. In contrast, neuromyotonia, limbic encephalitis, myocarditis, and taste disorders are autoantibody-mediated disorders, as is MG. Autoantibodies to several types of voltage-gated potassium channels and related molecules can evoke various neurological and cardiac disorders. Approximately 25% of patients with MG and associated thymomas experience at least one non-motor symptom. Non-motor symptoms affect many target organs and result in a broad disease spectrum, ranging from the impairment of quality of life to lethal conditions.