BRAIN and NERVE－神経研究の進歩65巻4号

2013年04月発行

増大特集 Antibody Update

抗VGCC抗体とランバート・イートン筋無力症候群

著者： 酒井和香¹ 中根俊成¹² 松尾秀徳¹

所属機関： ¹国立病院機構長崎川棚医療センター神経内科 ²国立病院機構長崎川棚医療センター臨床研究部

ページ範囲：P.441 - P.448

文献概要

はじめに

　1970年代にアセチルコリン受容体（AChR）に対する自己抗体が発見されて以来，多くの神経筋疾患で自己抗体が関与することが明らかになった。その後，リガンド蛋白や電位依存性イオンチャネルに対する自己抗体が発見され，神経筋接合部疾患との関わりが明らかになってきた。

　神経終末（シナプス前部）に存在するP/Q型電位依存性カルシウムチャネル（voltage-gated calcium channel：VGCC）に対する自己抗体（抗P/Q型VGCC抗体）が発見され，ランバート・イートン筋無力症候群（Lambert-Eaton myasthenic syndrome：LEMS）との関わりが明らかになった。LEMSは神経筋接合部疾患の1つであり，神経終末からのアセチルコリン（ACh）の遊離が阻害され，筋脱力や自律神経障害をきたす。また，傍腫瘍性症候群の1つでもあり，50～60％の症例で肺小細胞癌を合併する。さらに，約10％の症例で小脳性運動失調を認め，病態に関連していると考えられている。本稿では抗VGCC抗体が病態に深く関わるLEMSについて臨床像や治療法を中心に解説する。

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