Progressive subcortical gliosis
نویسنده
چکیده
The author explains the clinical presentation, etiology, differential diagnosis, and diagnostic workup of progressive subcortical gliosis, a chromosome-17-linked dementia with unique pathologic features. Microscopically, the major pathologic change is a marked fibrillary astrocytosis, particularly in the area of the short cortical association tracts at the junction of cortical lamina VI and the subcortical white matter and in the subpial cerebral cortex. Overall, the survival of patients with progressive subcortical gliosis averages about 10 years and is similar to that of other types of frontotemporal dementia.
منابع مشابه
A case of progressive subcortical gliosis presenting clinically as Steele-Richardson-Olszewski syndrome.
A patient presenting with the characteristic clinical features of Steele-Richardson-Olszewski syndrome is described, in whom neuropathological examination revealed atypical features, including extensive cortical and subcortical gliosis. The clinical and pathological features are discussed with particular reference to Creutzfeldt-Jacob disease and it is proposed that the case should be classifie...
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1 Neumann MA, Cohn R. Progressive subcortical gliosis, a rare form of presenile dementia. Brain 1967;90:405-18. 2 Tomlinson BE. Ageing and the dementias. In: Adams JH, Duchen LW, eds. Greenfield's neuropathology, 5th ed, London: Edward Arnold, 1992:1284-410. 3 Will RG, Lees AJ, Gibb W, Barnard RO. A case of progressive subcortical gliosis presenting clinically as Steele-RichardsonOlszewski synd...
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