Stamelou Maria, Höglinger Günter
Department of Neurology, Philipps University, Marburg, Germany.
Movement Disorders Department, HYGEIA Hospital, Athens, Greece.
CNS Drugs. 2016 Jul;30(7):629-36. doi: 10.1007/s40263-016-0347-2.
Progressive supranuclear palsy (PSP) is an atypical parkinsonian condition characterized by a symmetric akinetic-rigid syndrome, early falls, supranuclear gaze palsy, and a frontotemporal behavioral syndrome. The typical phenotype is termed Richardson's syndrome, but numerous other phenotypes have been described. The pathophysiology of PSP is not fully understood, but dysfunction of the tau protein seems to play a central role. Despite exciting new knowledge on the pathophysiology of PSP, there is still no highly effective symptomatic or disease-modifying treatment. We review the evidence on pharmacotherapy and experimental therapies in PSP and provide levels of recommendation for the off-label use of commonly used drugs in this disorder.
进行性核上性麻痹(PSP)是一种非典型帕金森综合征,其特征为对称性运动不能-强直综合征、早期跌倒、核上性凝视麻痹以及额颞叶行为综合征。典型表型被称为理查森综合征,但也描述了许多其他表型。PSP的病理生理学尚未完全了解,但tau蛋白功能障碍似乎起着核心作用。尽管在PSP病理生理学方面有了令人兴奋的新知识,但仍然没有高效的对症治疗或疾病修饰治疗方法。我们回顾了PSP药物治疗和实验性治疗的证据,并针对该疾病中常用药物的超说明书使用提供推荐等级。