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))

DUI

D007562 MeSH Browser

CUI

M0011818

Previous indexing

Central Nervous System Diseases (1966-1968)

History note

1991; use JAKOB-CREUTZFELDT SYNDROME 1981-1990, use CREUTZFELDT-JAKOB DISEASE 1969-1980

Public note

1991; see JAKOB-CREUTZFELDT SYNDROME 1981-1990, see CREUTZFELDT-JAKOB DISEASE 1973-1980