Definition: 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))
Other names Spongiform Encephalopathy, Subacute; New Variant Creutzfeldt-Jakob Disease; Variant Creutzfeldt Jakob Disease; Syndrome, Jakob-Creutzfeldt; Syndrome, Creutzfeldt-Jakob; Subacute Spongiform Encephalopathy; Subacute Spongiform Encephalopathies; Spongiform Encephalopathies, Subacute; New Variant Creutzfeldt Jakob Disease; Jakob Creutzfeldt Syndrome; Jakob Creutzfeldt Disease; Jacob Disease, Creutzfeldt; Familial Creutzfeldt-Jakob Diseases; Familial Creutzfeldt Jakob Disease; Encephalopathy, Subacute Spongiform; Encephalopathies, Subacute Spongiform; Disease, Jakob-Creutzfeldt; Disease, Familial Creutzfeldt-Jakob; Disease, Creutzfeldt-Jakob; Disease, Creutzfeldt Jacob
