| Descriptor English: | Gerstmann-Straussler-Scheinker Disease | ||||||
| Descriptor Spanish: |
Enfermedad de Gerstmann-Straussler-Scheinker
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| Descriptor Portuguese: | Doença de Gerstmann-Straussler-Scheinker | ||||||
| Descriptor French: | Syndrome de Gerstmann-Sträussler-Scheinker | ||||||
| Entry term(s): |
Disease, Gerstmann-Straussler Diseases, Gerstmann-Straussler Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type Gerstmann Straussler Disease Gerstmann Straussler Inherited Spongiform Encephalopathy Gerstmann Straussler Scheinker Disease Gerstmann Straussler Scheinker Syndrome Gerstmann Straussler Syndrome Gerstmann-Straussler Disease Gerstmann-Straussler Diseases Gerstmann-Straussler Inherited Spongiform Encephalopathy Gerstmann-Straussler Syndrome Gerstmann-Straussler-Scheinker Syndrome Inherited Spongiform Encephalopathy, Gerstmann Straussler Inherited Spongiform Encephalopathy, Gerstmann-Straussler |
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| Tree number(s): |
C01.207.800.350 C10.228.228.800.350 C10.574.500.425 C10.574.843.400 C16.320.400.350 |
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| RDF Unique Identifier: | https://id.nlm.nih.gov/mesh/D016098 | ||||||
| Scope 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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| Annotation: | a prion dis: do not confuse with GERSTMANN SYNDROME, a type of agnosia |
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| Allowable Qualifiers: |
BL blood CF cerebrospinal fluid CI chemically induced CL classification CN congenital CO complications DG diagnostic imaging DH diet therapy DI diagnosis DT drug therapy EC economics EH ethnology EM embryology EN enzymology EP epidemiology ET etiology GE genetics HI history IM immunology ME metabolism MI microbiology MO mortality NU nursing PA pathology PC prevention & control PP physiopathology PS parasitology PX psychology RH rehabilitation RT radiotherapy SU surgery TH therapy TM transmission UR urine VE veterinary VI virology |
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| Previous Indexing: |
Slow Virus Diseases (1988-1990) |
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| Public MeSH Note: | 2000; see GERSTMANN-STRAUSSLER SYNDROME 1991-1999; see SLOW VIRUS DISEASES 1988-1990; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE was GERSTMANN-STRAUSSLER-SCHEINDER DISEASE see GERSTMANN-STRAUSSLER SYNDROME 1991 |
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| History Note: | 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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| Entry Version: | GERSTMANN STRAUSSLER SCHEINKER DIS |
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| DeCS ID: | 28967 | ||||||
| Unique ID: | D016098 | ||||||
| Documents indexed in the Virtual Health Library (VHL): | Click here to access the VHL documents | ||||||
| Date Established: | 1991/01/01 | ||||||
| Date of Entry: | 1990/06/06 | ||||||
| Revision Date: | 2015/06/22 |
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DISEASES
Infections [C01]Infections
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Gerstmann-Straussler-Scheinker Disease
- Preferred
| Concept UI |
M0024593 |
| Scope 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) |
| Preferred term | Gerstmann-Straussler-Scheinker Disease |
| Entry term(s) |
Disease, Gerstmann-Straussler Diseases, Gerstmann-Straussler Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type Gerstmann Straussler Disease Gerstmann Straussler Inherited Spongiform Encephalopathy Gerstmann Straussler Scheinker Disease Gerstmann Straussler Scheinker Syndrome Gerstmann Straussler Syndrome Gerstmann-Straussler Disease Gerstmann-Straussler Diseases Gerstmann-Straussler Inherited Spongiform Encephalopathy Gerstmann-Straussler Syndrome Gerstmann-Straussler-Scheinker Syndrome Inherited Spongiform Encephalopathy, Gerstmann Straussler Inherited Spongiform Encephalopathy, Gerstmann-Straussler |
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