Prion diseases sporadic etiology

Human prion disease most commonly presents with a sporadic etiology 793... 

Human prion diseases encompass the three etiological types of prion disease inherited, sporadic and acquired forms. 

The sites of prion agent replication and spread are largely cUctated by v hether the etiology of prion disease is sporadic, familial, or acquired. For example, in sCJD and fCJD, disease initiates in, and is largely confined to, the nervous system. In... 

Creutzfeldt-Jakob disease (CJD) the most common prion disease of humans and can have a sporadic, familial or acquired etiology. 

Recently a serious public health problem has arisen by showing that a prion disease in cattle can cross species barriers and infect humans. This occurred when cattle were fed meal made from sheep infected with scrapie. The cattle developed BSE (commonly called mad cow disease ). Subsequently, when people consumed prion-contaminated beef, a small number, primarily in Great Britain, developed a variant of CJD (vCJD) approximately five years afterward. The variant form of CJD is a unique form of prion disease occurring in a much younger population than would be expected from inherited or sporadic CJD. Both BSE and vCJD share many similar pathologic characteristics suggesting an etiologic link between human vCJD and cattle BSE. 

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