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

自己免疫性自律神経節障害・急性自律性感覚性ニューロパチー

著者： 中根俊成¹

所属機関： ¹富山大学脳神経内科

ページ範囲：P.562 - P.568

文献概要

自己免疫性自律神経節障害（autoimmune autonomic ganglionopathy：AAG）と急性自律性感覚性ニューロパチー（acute autonomic and sensory neuropathy：AASN）は免疫機序を基盤に発症する自律神経節あるいは後根神経節の障害を病態とする疾患である。AAG患者血清からはニコチン性自律神経節アセチルコリン受容体（ganglionic acetylcholine receptor：gAChR）に対する自己抗体が検出され，AAGの病原性自己抗体とされている。gAChR抗体はAASN患者血清では認めないとされる。AAGとAASNは現段階では臨床症状や各検査の所見からスペクトラム概念ではないと考えられる。しかし，自律神経外症状である脳症状（精神症状，性格変化を含む），内分泌障害は両者に共通して見られなんらかの病態基盤が共通する可能性を残している。

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