进行性核上性麻痹的诊疗进展  

Advances in the diagnosis and treatment of progressive supranuclear palsy

作　　者：林正挺 马欣昕[1] 苏闻[1] Lin Zhengting;Ma Xinxin;Su Wen(Department of Neurology,Beijing Hospital,National Center of Gerontology,Institute of Geriatric Medicine,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]北京医院神经内科、国家老年医学中心、中国医学科学院老年医学研究所,北京100730

出　　处：《中华老年医学杂志》2024年第4期523-527,共5页Chinese Journal of Geriatrics

摘　　要：进行性核上性麻痹(PSP)是一种非典型帕金森综合征,其患病率为5/10万~7/10万,以眼球运动障碍(O)、姿势不稳(P)、运动不能(A)和认知障碍(C)为四大核心临床特征。根据四大核心临床特征的不同排列组合,很可能的(probable)或可能的(possible)PSP至少包括7种不同的临床表型。PSP的病理生理学尚不完全清楚,但Tau蛋白的功能障碍似乎起着核心作用。生物标志物的缺乏、早期临床诊断的困难以及对该病确切病理生理学机制的不了解,使目前PSP无显著有效的、足以改变病情进展的治疗手段。本文对PSP的临床诊断标准、生物标志物、治疗方法3个方面的新进展进行综述。Progressive supranuclear palsy(PSP)is considered an atypical form of Parkinsonism,with a prevalence estimated at around 5-7 per 100 o0o individuals.The condition is defined by four primary clinical features:ocular motor dysfunction(O),postural instability(P),akinesia(A),and cognitive dysfunction(C).Different combinations of these core features give rise to at least seven distinct clinical phenotypes classified as'probable'or'possible'.While the exact pathophysiology of PSP remains somewhat uncertain,research suggests that Tau dysfunction plays a significant role.Challenges such as the absence of reliable biomarkers,difficulties in early clinical detection,and limited understanding of the precise pathological mechanisms hinder the development of effective treatments capable of altering the disease's progression.This article provides an overview of recent progress in clinical diagnostic criteria,biomarkers,and treatment strategies for PSP.

关 键 词：核上麻痹 进行性 帕金森病 诊断 治疗 

分 类 号：R741[医药卫生—神经病学与精神病学]