Important Note: Classic CJD is not related to "mad cow" disease. Classic CJD also is distinct from "variant CJD", another prion disease that is related to BSE.
For information about these diseases, see:
Occurrence and TransmissionClassic CJD has been recognized since the early 1920s. The most common form of classic CJD is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions. This sporadic disease occurs worldwide, including the United States, at a rate of approximately one case per 1 million population per year, although rates of up to two cases per million are not unusual. The risk of CJD increases with age, and in persons aged over 50 years of age, the annual rate is approximately 3.4 cases per million. In the most recent five year period, the United States has reported between 279 and 352 cases a year.
Whereas the majority of cases of CJD (about 85%) occur as sporadic disease, a smaller proportion of patients (5-15%) develop CJD because of inherited mutations of the prion protein gene. These inherited forms include Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia.
Clinical and Pathologic Characteristics of Classic CJDClassic CJD characteristics, as compared to variant CJD, are presented in the table below.
|Date: November 15, 2012 |
Content source: Centers for Disease Control and Prevention
National Center for Emerging and Zoonotic Infectious Diseases (NCEZID)
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