Lantos P L
Department of Neuropathology, Institute of Psychiatry, London, United Kingdom.
J Neural Transm Suppl. 1994;42:137-52. doi: 10.1007/978-3-7091-6641-3_11.
The macroscopical, histological, ultrastructural and immunocytochemical features of progressive supranuclear palsy (PSP) are reviewed. Recent investigations have revealed important differences in the distribution, ultrastructure and immunocytochemical profile of neurofibrillary tangles in PSP and in Alzheimer's disease. Cortical involvement, as demonstrated by the presence of tangles and neuropil threads has extended the neuropathological spectrum of PSP. Quantitative assessments of neuronal populations show neuronal loss, not only in various nuclei of the brainstem, diencephalon and cerebellum, but also in other areas, including the nucleus basalis of Meynert, substantia nigra and neostriatum. A new classification, based on neuropathological criteria, is suggested in order to take into consideration the phenotypic heterogeneity of PSP. This new classification distinguishes three types: typical, atypical and combined cases. Typical (Type 1) cases conform to the original definition of PSP. Type 2, atypical cases are variants of the histological changes characteristic of PSP: either the severity or the distribution of abnormalities, or both of these deviate from the typical pattern. Cases with combined pathology belong to type 3 group: in these the typical pathology of PSP is accompanied by lesions characteristic of another neurodegenerative or vascular disease.
本文综述了进行性核上性麻痹（PSP）的宏观、组织学、超微结构和免疫细胞化学特征。最近的研究揭示了PSP和阿尔茨海默病中神经原纤维缠结在分布、超微结构和免疫细胞化学特征方面的重要差异。缠结和神经毡丝的存在表明皮质受累，这扩展了PSP的神经病理学范围。对神经元群体的定量评估显示，不仅脑干、间脑和小脑的各个核团存在神经元丢失，其他区域也存在，包括Meynert基底核、黑质和新纹状体。为了考虑PSP的表型异质性，建议基于神经病理学标准进行新的分类。这种新分类区分三种类型：典型型、非典型型和复合型病例。典型（1型）病例符合PSP的原始定义。2型非典型病例是PSP特征性组织学变化的变体：异常的严重程度或分布，或两者均偏离典型模式。具有合并病理学的病例属于3型组：在这些病例中，PSP的典型病理学伴有另一种神经退行性或血管性疾病的特征性病变。
