"Classic CJD [Creutzfeldt-Jakob disease] is a human prion disease. It is a neurodegenerative [brain] disorder with characteristic clinical and diagnostic features. This disease is rapidly progressive and always fatal. Infection with this disease leads to death usually within 1 year of onset of illness.
Classic CJD has been recognized since since the early 1920's. The most common form of classic CJD is believed to occur sporadically, caused by spontaneous transformation [to change the form of] 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 recent years, the United States has reported fewer than 300 cases of CJD a year.
Whereas the majority of cases of CJD (about 85%) occur as sporadic, 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.*"
*Above text and photo/text below: Centers for Disease Control and Prevention, Atlanta, Georgia
"The tissue slide above shows sponge-like lesions in the brain tissue of a classic CJD patient - this lesion is typical of many prion diseases."
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