BRAIN and NERVE－神経研究の進歩59巻10号

2007年10月発行

増大特集 ALS―研究と診療の進歩

ALSの病型―臨床と病理

著者： 佐々木彰一¹

所属機関： ¹東京女子医科大学神経内科

ページ範囲：P.1013 - P.1021

文献概要

はじめに

　従来，孤発性筋萎縮性側索硬化症（amyotrophic lateral sclerosis：ALS）は，下位運動ニューロン障害のみを示す脊髄性進行性筋萎縮症，球麻痺症状のみを示す進行性球麻痺，上位運動ニューロン障害のみを示す原発性側索硬化症，およびそれらのすべてがみられる古典型ALSに分類されることが多かった。いずれの亜型で発病しても数年後にはすべての症状が出そろい，古典型ALSになることが多いとされてきた。しかし，近年，病変が運動系を超えて感覚系を含めた広範な部位に及ぶ多系統病変型，dementiaを伴うALS，両上肢近位部および肩甲帯に筋萎縮が限局するflail arm syndromeなど，種々の非典型的なALSが報告されるようになり，ALSはheterogeneousな疾患単位から成ることから，ALS syndromeと呼称されることもある。今回は，古典型ALSおよびそれ以外の，種々の亜型の臨床および病理について，特徴を概説したい。

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