Center of Neurology, Department of Neurodegeneration, Hertie Institute for Clinical Brain Research, University of Tuebingen, Germany.
Acta Neurol Scand. 2013 May;127(5):362-70. doi: 10.1111/ane.12067. Epub 2013 Feb 13.
Progressive supranuclear palsy (PSP) is the most common atypical parkinsonian syndrome comprising two main clinical subtypes: Richardson's syndrome (RS), characterized by prominent postural instability, supranuclear vertical gaze palsy and frontal dysfunction; and PSP-parkinsonism (PSP-P) which is characterized by an asymmetric onset, tremor and moderate initial therapeutic response to levodopa. The early clinical features of PSP-P are often difficult to discern from idiopathic Parkinson's disease (PD), and other atypical parkinsonian disorders, including multiple system atrophy (MSA) and corticobasal syndrome (CBS). In addition, rare PSP subtypes may be overlooked or misdiagnosed if there are atypical features present. The differentiation between atypical parkinsonian disorders and PD is important because the prognoses are different, and there are different responses to therapy. Structural and functional imaging, although currently of limited diagnostic value for individual use in early disease, may contribute valuable information in the differential diagnosis of PSP. A growing body of evidence shows the importance of CSF biomarkers in distinguishing between atypical parkinsonian disorders particularly early in their course when disease-modifying therapies are becoming available. However, specific diagnostic CSF biomarkers have yet to be identified. In the absence of reliable disease-specific markers, we provide an update of the recent literature on the assessment of clinical symptoms, pathology, neuroimaging and biofluid markers that might help to distinguish between these overlapping conditions early in the course of the disease.
进行性核上性麻痹（PSP）是最常见的非典型帕金森综合征，包括两种主要的临床亚型：理查森综合征（RS），其特征是明显的姿势不稳、核上垂直注视麻痹和额叶功能障碍；和 PSP 帕金森病（PSP-P），其特征是不对称发病、震颤和初始对左旋多巴的中度治疗反应。PSP-P 的早期临床特征常常难以与特发性帕金森病（PD）和其他非典型帕金森病障碍区分，包括多系统萎缩（MSA）和皮质基底节综合征（CBS）。此外，如果存在非典型特征，罕见的 PSP 亚型可能会被忽视或误诊。区分非典型帕金森病障碍和 PD 很重要，因为预后不同，治疗反应也不同。结构和功能影像学虽然目前对早期疾病的个体使用具有有限的诊断价值，但可能有助于 PSP 的鉴别诊断。越来越多的证据表明 CSF 生物标志物在区分非典型帕金森病障碍中的重要性，尤其是在疾病修饰治疗可用的早期阶段。然而，尚未确定特定的诊断性 CSF 生物标志物。在缺乏可靠的疾病特异性标志物的情况下，我们更新了最近关于评估临床症状、病理学、神经影像学和生物流体标志物的文献，这些标志物可能有助于在疾病早期区分这些重叠的疾病。
