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Gerstmannova-Sträusslerova-Scheinkerova choroba
Record number d016098 Date 06.06.2025 Type M Topical term Gerstmannova-Sträusslerova-Scheinkerova choroba Other term English (Pseudonym) Gerstmann-Straussler Syndrome
Slovak (Pseudonym) encefalopatia cerebelárna spongiformná chronická
Slovak (Pseudonym) Gerstmannov-Sträusslerov syndróm
UDC C01.207.800.350C10.228.228.800.350C10.574.500.425C10.574.843.400C16.320.400.350 Note 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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