About The Medical Condition Inherited Spongiform Encephalopathy, Gerstmann-Straussler
Find the definition of the medical term Inherited Spongiform Encephalopathy, Gerstmann-Straussler. Inherited Spongiform Encephalopathy, Gerstmann-Straussler defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.
Inherited Spongiform Encephalopathy, Gerstmann-Straussler
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What's The Definition Of The Medical Condition Inherited Spongiform Encephalopathy, Gerstmann-Straussler?
An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)
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