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DeCS
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Descriptor English:
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Creutzfeldt-Jakob Syndrome
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Descriptor Spanish:
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Síndrome de Creutzfeldt-Jakob
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Descriptor Portuguese:
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Síndrome de Creutzfeldt-Jakob
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Synonyms English:
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New Variant Creutzfeldt-Jakob Disease
Spongiform Encephalopathy, Subacute
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Tree Number:
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C10.228.140.380.165
C10.228.228.800.230
F03.087.400.300
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Definition English:
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A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339(27)) |
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History Note English:
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1991; use JAKOB-CREUTZFELDT SYNDROME 1981-1990, use CREUTZFELDT-JAKOB DISEASE 1969-1980
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Allowable Qualifiers English:
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Record Number:
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29254
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Unique Identifier:
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D007562
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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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