| umls-concept:C0022336 | skos:definition | MSH: 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)),MEDLINEPLUS: <p>Creutzfeldt-Jakob disease (CJD) is a rare, <a href='http://www.nlm.nih.gov/medlineplus/degenerativenervediseases.html'>degenerative brain disorder</a>. Symptoms usually start around age 60. Memory problems, behavior changes, vision problems and poor muscle coordination progress quickly to <a href='http://www.nlm.nih.gov/medlineplus/dementia.html'>dementia</a>, coma and | lld:umls |
