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DeCS
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Descriptor English:
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Gerstmann-Straussler-Scheinker Disease
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Descriptor Spanish:
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Enfermedad de Gerstmann-Straussler-Scheinker
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Descriptor Portuguese:
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Doença de Gerstmann-Straussler-Scheinker
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Synonyms English:
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Gerstmann-Straussler Syndrome
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Tree Number:
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C10.228.228.800.350
C10.574.500.425
C10.574.843.400
C16.320.400.350
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Definition English:
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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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Indexing Annotation English:
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a prion dis: do not confuse with GERSTMANN SYNDROME, a type of agnosia
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History Note English:
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2000(1991); use SLOW VIRUS DISEASES 1988-1990; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE was GERSTMANN-STRAUSSLER-SCHEINDER DISEASE see GERSTMANN-STRAUSSLER SYNDROME 1991
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Allowable Qualifiers English:
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Record Number:
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28967
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Unique Identifier:
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D016098
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Occurrence in VHL:
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Similar:
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DeCS CID-10 SciELO LILACS LIS
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