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[进行性核上性麻痹]

[Progressive supranuclear palsy].

作者信息

Fedorova N V, Bril E V, Kulua T K, Mikhaylova A D

机构信息

Russian Medical Academy of Continuous Professional Education, Moscow, Russia.

Russian State Research Center - Burnasyan Federal Medical Biophysical Center, Moscow, Russia.

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2021;121(5):111-119. doi: 10.17116/jnevro2021121051111.

Abstract

Progressive supranuclear palsy (PSP) is a heterogeneous progressive neurodegenerative disease characterized by onset after 50 years old, Parkinson's syndrome, early development of postural instability, absence or transient reaction to levodopa drugs, neuropsychological disorders, dysphagia and dysarthria and eye movement disorders. The review provides an analysis of modern data on etiology, clinical presentation, differential diagnosis of the disease. The morphological picture and neuroimaging features, as well as modern ideas about treatment, are described. A great clinical polymorphism of the disease, as well as its similarity to other neurodegenerative diseases, manifested by Parkinson's syndrome, complicates the diagnosis of PSP. Establishing an accurate diagnosis makes it possible to determine the prognosis and further tactics of patient management.

摘要

进行性核上性麻痹(PSP)是一种异质性进行性神经退行性疾病,其特征为50岁以后起病、帕金森综合征、姿势不稳早期出现、对左旋多巴药物无反应或仅有短暂反应、神经心理障碍、吞咽困难、构音障碍以及眼球运动障碍。本文综述对该疾病的病因、临床表现、鉴别诊断的现代数据进行了分析。描述了其形态学表现和神经影像学特征,以及关于治疗的现代观点。该疾病具有高度的临床多态性,且与其他以帕金森综合征为表现的神经退行性疾病相似,这使得PSP的诊断变得复杂。准确的诊断有助于确定患者的预后和进一步的治疗策略。

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