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Creutzfeldt-Jakob Syndrome
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Definition:
A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ; , visual loss, weakness, muscle atrophy, , 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 ) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of . (From N Engl J Med, 1998 Dec 31;339(27))
Synonym(s):
CJD Variant (V-CJD) /
New Variant Creutzfeldt-Jakob Disease /
Spongiform Encephalopathy, Subacute /
CJD /
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