BRAIN and NERVE－神経研究の進歩76巻8号

2024年08月発行

総説

スティッフパーソン症候群—初の全国疫学調査結果を踏まえた現状と課題

著者： 松井尚子¹ 田中惠子²³ 和泉唯信¹

所属機関： ¹徳島大学大学院医歯薬学研究部臨床神経科学分野（脳神経内科） ²新潟大学脳研究所モデル動物開発分野 ³福島県立医科大学多発性硬化症治療学講座

ページ範囲：P.961 - P.967

文献概要

スティッフパーソン症候群は体幹を主部位として，間欠的に筋硬直や筋攣縮が発生し，さらには全身へと症状が進行する稀な自己免疫疾患である。2018年に実施された全国調査において，罹患率は人口10万人あたり0.2人と推定された。病因としてグルタミン酸脱炭酸酵素（glutamate decarboxylase：GAD）抗体が最も多く，グリシン受容体（glycine receptor：GlyR）抗体がそれに次いだ。治療への反応は欧米の報告と比べると比較的良好であったが，治療への反応の乏しい症例も存在することから，早期診断と治療アルゴリズムの確立に向けた整備が重要である。

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