BRAIN and NERVE－神経研究の進歩63巻7号

2011年07月発行

増大特集 神経筋接合部―基礎から臨床まで

Lambert-Eaton筋無力症候群

著者： 本村政勝¹ 福田卓¹

所属機関： ¹長崎大学大学院医歯薬学総合研究科（第一内科・神経内科）

ページ範囲：P.745 - P.754

文献概要

はじめに

　Lambert-Eaton筋無力症候群（Lambert-Eaton myasthenic syndrome：LEMS）は，50～60％に肺小細胞癌（small cell lung cancer：SCLC）を合併し，SCLCの治療によりLEMS自体も寛解する代表的な傍腫瘍性症候群である^1，2）。一方，その80～90％にP/Q型電位依存性カルシウムチャネル自己抗体（P/Q-type voltage-gated calcium channel antibodies：抗P/Q型VGCC抗体）が検出される神経筋接合部かつ自律神経疾患でもあり^3），血漿交換やステロイド治療に反応する。悪性疾患以外でも，1型糖尿病，自己免疫性甲状腺炎，関節リウマチといった自己免疫疾患の合併例や家族集積性が報告されている^4）。また，約10％に小脳失調がみられ^5，6），その発症機序が傍腫瘍性症候群と推測されている。本稿では，LEMSの最近の知見に基づき，LEMS全般を解説する。

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