2nd Department of Neurology, Faculty of Medicine, Comenius University, Bratislava, Slovakia; Department of Neurology, Zvolen Hospital, Kuzmányho nábrežie, 28, 960 01 Zvolen, Slovakia.
Department of Neurology, Zvolen Hospital, Kuzmányho nábrežie, 28, 960 01 Zvolen, Slovakia.
Rev Neurol (Paris). 2024 Jan-Feb;180(1-2):12-23. doi: 10.1016/j.neurol.2023.02.070. Epub 2023 Aug 3.
Progressive supranuclear palsy-Richardson syndrome (PSP-RS) is a sporadic atypical parkinsonian syndrome with levodopa-unresponsive axial-predominant parkinsonism, early postural instability, vertical supranuclear gaze palsy, dysarthria, executive dysfunction and behavioural changes. PSP-RS can be mimicked by numbers of other disorders, generally known as PSP mimics, or PSP-like syndromes. Their aetiological spectrum includes neurodegenerative (mostly genetic), vascular, infectious and drug-induced illnesses as well as other causes. Based on the available data, we have tried to create a definition of PSP-RS mimics: a syndrome resembling PSP-RS with at least one of the following red flags: 1) positive family history; 2) onset before 45 years of age; 3) rapid or stepwise progression; 4) acute or subacute onset; 5) atypical symptoms and/or signs; 6) normal or atypical brain MRI; 7) history of HIV or untreated syphilis, aortal surgery or recent therapy with dopamine-blocking agents. We have suggested a short diagnostic algorithm leading to the identification of PSP-RS mimics and the recommended diagnostic work-up. The key point of the diagnostic process is the early identification and treatment of potentially treatable PSP-RS mimics.
进行性核上性麻痹-理查森综合征（PSP-RS）是一种散发的非典型帕金森综合征，具有对左旋多巴无反应的以轴性为主的帕金森病、早发性姿势不稳、垂直核上性眼球运动障碍、构音障碍、执行功能障碍和行为改变。许多其他疾病可模拟 PSP-RS，通常称为 PSP 模拟物或 PSP 样综合征。其病因谱包括神经退行性疾病（主要是遗传）、血管性、感染性和药物诱导性疾病以及其他原因。根据现有数据，我们试图为 PSP-RS 模拟物定义：一种类似于 PSP-RS 的综合征，至少有以下一个红旗标志：1）阳性家族史；2）发病年龄在 45 岁之前；3）快速或阶梯式进展；4）急性或亚急性发病；5）非典型症状和/或体征；6）正常或非典型脑 MRI；7）HIV 或未经治疗的梅毒、主动脉手术或最近使用多巴胺阻断剂的病史。我们提出了一个简短的诊断算法，用于识别 PSP-RS 模拟物和推荐的诊断性检查。诊断过程的关键点是早期识别和治疗潜在可治疗的 PSP-RS 模拟物。
