《英文msh詞典》Variants,CJD (V-CJD)
[入口詞] Variants,CJD (V-CJD)
[主題詞] Creutzfeldt-Jakob Syndrome
[英文釋義] 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))