Creutzfeldt–Jakob Disease

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Abstract
Creutzfeldt–Jakob disease is an unusual neurologic disorder, with a prevalence of approximately 1 case per million population, which is about 1/10,000 that of Alzheimer's disease. Creutzfeldt–Jakob disease has captured widespread attention, in part because of the recent epidemic of bovine spongiform encephalopathy (“mad cow disease”) and the appearance of 139 cases of “new-variant” Creutzfeldt–Jakob disease, including two in North America. The disorder is due to neuronal degeneration resulting from the accumulation of a pathologic isoform (PrPCJD) of the prion protein (PrPC), a normal cellular protein. Eighty-five percent of cases of Creutzfeldt–Jakob disease are sporadic, with familial and iatrogenic . . .

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