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进行性核上性麻痹之前的长期步态冻结:一项临床病理研究。

Long lasting pure freezing of gait preceding progressive supranuclear palsy: a clinicopathological study.

作者信息

Compta Yaroslau, Valldeoriola Francesc, Tolosa Eduardo, Rey María Jesús, Martí María José, Valls-Solé Josep

机构信息

Servei de Neurologia, Institut Clínic de Neurociències, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic de Barcelona, Barcelona, Catalonia, Spain.

出版信息

Mov Disord. 2007 Oct 15;22(13):1954-8. doi: 10.1002/mds.21612.

Abstract

Primary progressive freezing of gait (PPFG) is the term used to designate an uncommon condition featuring freezing of gait with frequent falls, without bradykinesia, rigidity or tremor, and unresponsive to levodopa. There are very few pathological reports of patients with PPFG in the literature. We report on 2 patients (one with pathological confirmation) diagnosed initially as PPFG and evolving into clinically defined progressive supranuclear palsy (PSP) more than 10 years after onset of symptoms. These 2 cases suggest that PPFG can represent the initial manifestation of a neurodegenerative disease, such as PSP, rather than a differentiated nosological entity.

摘要

原发性进行性步态冻结(PPFG)是用于指一种罕见病症的术语,其特征为步态冻结并频繁跌倒,无运动迟缓、僵硬或震颤,且对左旋多巴无反应。文献中关于PPFG患者的病理学报告非常少。我们报告了2例患者(其中1例有病理证实),最初诊断为PPFG,在症状出现10多年后发展为临床定义的进行性核上性麻痹(PSP)。这2例病例表明,PPFG可能代表一种神经退行性疾病(如PSP)的初始表现,而非一个独立的病种。

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