Division of Clinical Neurobiology, Department of Neurology, University of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.
Rev Neurol (Paris). 2010 Oct;166(10):829-33. doi: 10.1016/j.neurol.2010.07.004. Epub 2010 Sep 1.
The atypical parkinsonian disorders (APD) embrace a heterogeneous group of movement disorders all characterized by prominent parkinsonism, accompanied by specific additional features such as cerebellar ataxia, early autonomic dysfunction, early dementia, pyramidal tract signs, myoclonus, supranuclear gaze palsy, apraxia which are atypical for idiopathic Parkinson's disease (PD). Beside these features, rapid disease progression and poor or absent response to L-Dopa therapy both raise the suspicion of an APD. Currently, multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD) and dementia with Lewy bodies (DLB) are referred to as APD. Clinical diagnosis can be difficult in early stages and although the predictive value of the widely established, diagnostic criteria is high at first neurological evaluation sensitivity tends to be poor and may be less than 30%. In this review, we will discuss diagnostic issues in MSA and PSP.
不典型帕金森病（APD）包括一组异质性运动障碍，其特征均为明显的帕金森病，伴有特定的附加特征，如小脑共济失调、早期自主神经功能障碍、早期痴呆、锥体束征、肌阵挛、核上性眼球运动麻痹、运动不能，这些均为特发性帕金森病（PD）所不典型。除了这些特征外，疾病快速进展和对 L-Dopa 治疗的不良或无反应均提示为 APD。目前，多系统萎缩（MSA）、进行性核上性麻痹（PSP）、皮质基底节变性（CBD）和路易体痴呆（DLB）被归类为 APD。在早期阶段，临床诊断可能较为困难，尽管广泛建立的诊断标准在首次神经学评估时具有较高的预测价值，但敏感性往往较差，可能低于 30%。在这篇综述中，我们将讨论 MSA 和 PSP 的诊断问题。
