The neuropathology of progressive supranuclear palsy
The macroscopical, histological, ultrastructural and immuno-cytochemical features of progressive supranuclear palsy (PSP) are reviewed. Recent investigations have revealed important differences in the distribution, ultrastructure and immunocytochemical pr
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Summary. 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. Progressive supranuclear palsy (PSP) has a short, but interesting history. PSP was defined as a clinical and neuropathological entity in 1964. Steele, Richardson and Olszewski whose names later served to be the eponymous alternative for the designation of the disease, reported nine cases of a progressive brain disease characterised by supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria and dystonic rigidity of the neck and upper trunk. Other, less constant cerebellar and pyramidal signs, and a relatively mild dementia also occurred. All their patients were male and the disease usually started in the sixth decade leading, through a progressive course, to death within five-to-seven years. The neuropathological picture was rather similar in the seven patients who had died. There was evidence of neuronal loss, gliosis, neurofibrillary tangles, granulovacuolar degeneration and demyelination in various regions of the basal ganglia, brainstem and cerebellum. The distribution of the histological abnormalities showed a E. Tolosa et al. (eds.), Progressive Supranuclear Palsy: Diagnosis, Pathology, and Therapy © Springer-Verlag / Wien 1994
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P. L. Lantos
consistent pattern: the globus pallidus, subthalamic nucleus, red nucleus, substantia nigra, superior colliculi, cuneiform and subcuneiform nuclei, periaqueductal grey matter, pontine tegmentum and the dentate nucleus were most severely affected. An inflammatory response was extremely rare. The authors drew attention to the striking histopathological similarities between PSP, postencephalitic parkinsonism and the parkinsonism-dementia complex of Guam, but emphasised the different distribution of
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