进行性核上性麻痹（斯蒂尔-理查森-奥尔谢夫斯基综合征）的临床诊断及诊断标准。

Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).

作者信息

Tolosa E, Valldeoriola F, Marti M J

机构信息

Neurology Department, Hospital Clinic, Faculty of Medicine, University of Barcelona, Spain.

出版信息

J Neural Transm Suppl. 1994;42:15-31. doi: 10.1007/978-3-7091-6641-3_2.

DOI:10.1007/978-3-7091-6641-3_2

PMID:7964684

Abstract

Progressive supranuclear palsy (PSP) is characterized clinically by supranuclear gaze palsy, neck dystonia, parkinsonism, pseudobulbar palsy, gait imbalance with frequent falls and frontal lobe-type dementia. In the advanced typical case, when supranuclear gaze palsy and other main features are present diagnosis is relatively easy. Diagnostic problems, though, are frequent in the early stages due to the variable clinical presentation and in those atypical cases in which gaze palsy does not develop or that present as a severe dementia disorder or as an isolated akinetic-rigid syndrome. In this review we summarize the clinical features of PSP and emphasize those aspects helpful in the differential diagnosis with Parkinson's disease and other motor and cognitive disorders that can pose difficult diagnostic problems. Clinical diagnostic criteria are also discussed and modifications of those currently in used are proposed.

摘要

进行性核上性麻痹（PSP）的临床特征为核上性凝视麻痹、颈部肌张力障碍、帕金森综合征、假性延髓麻痹、步态失衡伴频繁跌倒以及额叶型痴呆。在典型的晚期病例中，当存在核上性凝视麻痹和其他主要特征时，诊断相对容易。然而，由于临床表现多样，在早期阶段以及那些不出现凝视麻痹、表现为严重痴呆症或孤立的运动不能-强直综合征的非典型病例中，诊断问题很常见。在本综述中，我们总结了PSP的临床特征，并强调了有助于与帕金森病以及其他可能带来诊断难题的运动和认知障碍进行鉴别诊断的那些方面。还讨论了临床诊断标准，并提出了对目前使用的标准的修改建议。