creutzfeldt-jakob syndromemedical dictionary


A rare, usually fatal, transmissible encephalopathy occurring in middle life, in which there is partial degeneration of the pyramidal and extrapyramidal systems accompanied by progressive dementia and sometimes muscle wasting, tremor, athetosis, and spastic dysarthria. A familial form has been shown to exhibit autosomal dominant inheritance. The more common sporadic form is probably caused by mutations that produce abnormal prion proteins (prions). A variant of cjd has been reported in patients younger than 30 years old, probably caused by exposure to beef harboring the bovine spongiform encephalopathy agent.

(12 Dec 1998)