Creutzfeldt-Jakob Syndrome

**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))

**Tree numbers:** - C01.207.800.230 - C10.228.140.380.165 - C10.228.228.800.230 - F03.615.400.300

**Synonyms:** - Jakob-Creutzfeldt Disease - Jakob-Creutzfeldt Syndrome - Creutzfeldt-Jakob Disease - Spongiform Encephalopathy, Subacute - CJD (Creutzfeldt-Jakob Disease) - Creutzfeldt Jacob Disease