neurologic disorders
Creutzfeldt Jakob Disease (CJD)
Introduction
Creutzfeldt-Jakob disease (CJD) is the most common human prion disease. It occurs worldwide and has several forms and subtypes. CJD symptoms include dementia, myoclonus, and other central nervous system deficits; death usually occurs between 4 months and 2 years after onset, depending on the CJD form and subtype. Treatment is supportive. (See also Overview of Prion Diseases .) Creutzfeldt-Jakob disease has three forms ( General references ): Sporadic (sCJD) Familial Acquired sCJDis the most common type, accounting for about 85% of cases. sCJD typically affects people > 40 years (median, about 65 years). Familial CJDoccurs in about 5 to 15% of cases. Inheritance is almost universally autosomal dominant; age at onset is usually earlier than that in sCJD, and disease duration can be longer on average. probably accounts for < 1% of cases. It has occurred after the ingestion of beef contaminated by prions (in variant CJD [vCJD]). Iatrogenic CJD (iCJD) has been acquired via use of cadaveric corneal or dural transplants, stereotactic intracerebral electrodes, growth hormone, or gonadotropins prepared from human pituitary glands ( General references ). About half of iCJD cases involve changes similar to those of Alzheimer disease, suggesting that in iCJD, a disorder that resembles Alzheimer disease (in addition to CJD) can be acquired iatrogenically ( 