In most countries where the occurrence of CJD has been assessed, including in the United States, the crude incidence rate is approximately one case per 1 million population per year, although rates of up to two cases per million are not extremely unusual. The risk of CJD increases with age, and in persons older than 50 years of age, the annual rate is approximately 3.4 cases per million. The median age of death from CJD is 68 years. In recent years, the United States has reported fewer than 300 cases of CJD a year.3

The overall annual rates of CJD have been relatively stable since 1979 (see Figure 1). As illustrated in Figure 2, more than 97 percent of CJD deaths occur in adults older than 44 years of age; no deaths occurred in patients younger than 21 years of age.

Although sCJD is much more common in older individuals, one of the subtypes (V/V 1) tends to affect younger individuals, with one case diagnosed in a 24-year-old.7

(Figure 1)

Source: Centers for Disease Control and Prevention CJD Homepage: http://www.cdc.gov/ncidod/dvrd/cjd/

 

(Figure 2)

Source: CDC Division of Viral and Rickettsial Diseases, “Epidemiology and Surveillance of Creutzfeldt-Jakob Disease in the United States”

 

The National Prion Disease Pathology Surveillance Center (NPDPSC) was officially established in 1997 at the Division of Neuropathology of Case Western Reserve University. Among NPDPSC’s purposes are to acquire tissue samples and clinical information from as many clinically diagnosed and suspected cases of human prion disease occurring in the United States as possible in order to help: 1) monitor the occurrence of vCJD and the possible emergence of some other new human prion disease in the United States; 2) provide U.S. physicians with free-of-charge, state-of-the-art, neuropathological prion disease diagnostic services; and 3) determine the relative frequency and types of human prion diseases occurring in this country.8

Figure 3 (below) breaks down the cases examined by NPDPSC through June 2007 into the type of prion disease identified.

(Figure 3)

Source: National Prion Disease Pathology Surveillance Center


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