BRAIN and NERVE－神経研究の進歩76巻5号

2024年05月発行

増大特集 末梢神経・筋肉の自己免疫性・炎症性疾患ハンドブック

第2章　疾患各論

MAGニューロパチー

著者： 桑原基¹

所属機関： ¹近畿大学医学部脳神経内科学教室

ページ範囲：P.540 - P.546

文献概要

MAGニューロパチーはMGUSなどのIgM型M蛋白血症を背景に発症する脱髄性ニューロパチーである。緩徐進行性の感覚障害または感覚運動障害と運動失調を呈する。診断はM蛋白とMAG抗体の検出によってなされるが，神経伝導検査では遠位潜時が延長し，腓腹神経病理では電子顕微鏡でwidely spaced myelinが見られる。一般的に免疫療法への反応性は不良であるが，リツキシマブが約半数で有効であり，今後の治療開発が期待される。

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