多系統萎縮症の病状説明における困難さ

Challenges in Delivering the Diagnosis of Multiple System Atrophy

多系統萎縮症（multiple system atrophy：MSA）は，進行性の神経変性疾患であり，突然死のリスクを有することや，運動症状以外に自律神経障害や高次脳機能障害による症状など多彩な症状を呈し得るという特徴がある。MSAの告知に関する大規模な調査研究は乏しく，MSA告知指針の基盤となるようなエビデンスが求められている。われわれの行った医師対象の大規模オンライン質問紙調査研究の結果を含め，MSAの病状説明における困難さについて概説する。

Abstract

Multiple system atrophy (MSA) is a progressive, incurable neurodegenerative disease characterized by the risk of sudden death and various symptoms, including autonomic and cognitive dysfunction, as well as motor symptoms such as cerebellar ataxia and parkinsonism. These clinical features make the diagnosis of MSA challenging for neurologists. Our questionnaire survey on the delivery of an MSA diagnosis revealed that 92.3% of the participating neurologists found it difficult to deliver the diagnosis. However, 82.8% perceived explaining the risk of sudden death to be challenging. Factors independently associated with difficulties in delivering a diagnosis included perceived challenges in the differential diagnosis of MSA, conveying information about the risk of sudden death, and explaining the importance of the family's decision-making process in life-prolonging treatment. Further research is required to develop guidelines for delivering the diagnosis of MSA.