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Creutzfeldt-Jakob Syndrome MeSH Descriptor Data 2026
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))
Entry Term(s)
CJD (Creutzfeldt-Jakob Disease)
Creutzfeldt Jacob Disease
Creutzfeldt-Jakob Disease
Creutzfeldt-Jakob Disease, Familial
Creutzfeldt-Jakob Disease, New Variant
Creutzfeldt-Jakob Disease, Variant
Familial Creutzfeldt-Jakob Disease
Jakob-Creutzfeldt Disease
Jakob-Creutzfeldt Syndrome
New Variant Creutzfeldt-Jakob Disease
Spongiform Encephalopathy, Subacute
V-CJD (Variant-Creutzfeldt-Jakob Disease)
Variant Creutzfeldt-Jakob Disease
NLM Classification #
WL 301
Previous Indexing
Central Nervous System Diseases (1966-1968)
Public MeSH Note
1991; see JAKOB-CREUTZFELDT SYNDROME 1981-1990, see CREUTZFELDT-JAKOB DISEASE 1973-1980
History Note
1991; use JAKOB-CREUTZFELDT SYNDROME 1981-1990, use CREUTZFELDT-JAKOB DISEASE 1969-1980
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))