早期より両側声帯運動障害が認められたShy-Drager Syndromeの1例

Shy-Drager Syndrome with Early Onset of Bilateral Vocal Cord Fixation:Report of a Case

　はじめに

　Shy-Drager Syndromeは，1960年にShyとDragerによって報告された^1），起立性低血圧，屎尿失禁，発汗減少，陰萎などの自律神経症状を中心とし，筋固縮，振戦などの体性神経症状が加わった症候群であり（表1），病理学的には線条体，脳幹有色素核（赤核，黒質，青斑核），橋核，小脳皮質，オリーブ核などに変性，萎縮を認めることができる。

　従来，OPCA型の小脳症状，パーキンソニズムに自律神経症状を合併した症候群として考えられてきたが，近年ではmultiple system atrophyの一型であるとする考え方が有力となっており，multiple system atrophy with autonomic failurと記載されることも多くなってきた。

Recently Shy-Drager syndrome has been known as a type of multiple system atrophy. The syn-drome is often accompanied by abnormal mobility of the vocal cords. We report a 42-year-old male who developed disorders of the autonomic nervous system assciated with early onset of respiratory difficulty. Laryngoscopic examination showed bila-teral fixation of the vocal cords at the midline. The patient was diagnosed as having Shy-Drager syndome and the EMG study of the laryngeal muscles revealed that both abductor and adductor muscles showed continuous firing throughout the inspiratory as well as expriatory phase. We con-cluded that there was muscle rigitity in the larynx as often observed in parkinsonism, and the ridigity would be the cause of apparent vocal cord fixa-tion.