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))
Also Known As:
Creutzfeldt-Jakob Disease; Encephalopathy, Subacute Spongiform; Subacute Spongiform Encephalopathy; Syndrome, Creutzfeldt-Jakob; CJD (Creutzfeldt-Jakob Disease); Creutzfeldt Jacob Disease; Creutzfeldt-Jakob Disease, Familial; Creutzfeldt-Jakob Disease, New Variant; Creutzfeldt-Jakob Disease, Variant; Familial Creutzfeldt-Jakob Disease; Jakob-Creutzfeldt Disease; Jakob-Creutzfeldt Syndrome; V-CJD (Variant-Creutzfeldt-Jakob Disease); Variant Creutzfeldt-Jakob Disease; CJD (Creutzfeldt Jakob Disease); Creutzfeldt Jakob Disease; Creutzfeldt Jakob Disease, Familial; Creutzfeldt Jakob Disease, New Variant; Creutzfeldt Jakob Disease, Variant; Creutzfeldt Jakob Syndrome; Creutzfeldt-Jakob Diseases, Familial; Disease, Creutzfeldt Jacob; Disease, Creutzfeldt-Jakob; Disease, Familial Creutzfeldt-Jakob; Disease, Jakob-Creutzfeldt; Encephalopathies, Subacute Spongiform; Familial Creutzfeldt Jakob Disease; Familial Creutzfeldt-Jakob Diseases; Jacob Disease, Creutzfeldt; Jakob Creutzfeldt Disease; Jakob Creutzfeldt Syndrome; New Variant Creutzfeldt Jakob Disease; Spongiform Encephalopathies, Subacute; Subacute Spongiform Encephalopathies; Syndrome, Jakob-Creutzfeldt; V CJD (Variant Creutzfeldt Jakob Disease); Variant Creutzfeldt Jakob Disease; New Variant Creutzfeldt-Jakob Disease; Spongiform Encephalopathy, Subacute