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Gerstmannova-Sträusslerova-Scheinkerova choroba

Č. záznamu	d016098
Dátum	06.06.2025
Typ	M - MESH
Tematický termín	Gerstmannova-Sträusslerova-Scheinkerova choroba
Iný termín	Angličtina (Pseudonym) Gerstmann-Straussler Syndrome Slovenčina (Pseudonym) encefalopatia cerebelárna spongiformná chronická Slovenčina (Pseudonym) Gerstmannov-Sträusslerov syndróm
MDT	C01.207.800.350C10.228.228.800.350C10.574.500.425C10.574.843.400C16.320.400.350
Poznámka	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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