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多系統萎縮症(multiple system atrophy:MSA)の診断に関する第2回合意声明は,MSAの概念を統一するとともに,臨床研究や創薬研究を大きく前に進めてきた。しかし,その後の研究の進歩により,さまざまな課題が明らかとなった。ここでは,第2回合意声明の有していた問題点について,診断感度,起立性低血圧の取扱い,診断カテゴリー,除外基準(高齢発症,家族歴,認知症),進行性核上性麻痺との鑑別,画像所見の問題を中心に整理したい。
Abstract
The Second Consensus Statement on the Diagnosis of Multiple System Atrophy (MSA), issued in 2008, has unified the concept of MSA and significantly advanced clinical and drug discovery research. However, subsequent developments in research have revealed several critical findings that would affect the diagnostic sensitivity, specificity, and positive predictive value of the consensus statement. In this review, we discuss the limitations of diagnostic sensitivity for early diagnosis; positioning of orthostatic hypotension; diagnostic categories; exclusion criteria such as elderly onset, family history, and dementia; differentiation from progressive supranuclear palsy; and imaging findings of the Second Consensus Statement.
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