Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease
Creutz·feldt-Ja·kob disease also Creutz·feld-Ja·kob disease .krȯits-.felt-.yä-(.)kōb- n a rare progressive fatal spongiform encephalopathy now usu. considered to be caused by a prion and marked by the development of porous brain tissue, premature dementia in middle age, and gradual loss of muscular coordination abbr. CJD called also Jakob-Creutzfeldt disease see variant Creutzfeldt-Jakob disease
Creutz·feldt 'kroits-.felt Hans Gerhard (1885-1964), and
Ja·kob 'yä-.kōp Alfons Maria (1884-1931)
German psychiatrists. Creutzfeldt published his description of the disease now known as Creutzfeldt-Jakob disease in 1920. A year later in the same neurological journal Jakob offered his description. Although Creutzfeldt's description is the original one, Jakob's is the better known of the two, so the disease is named in honor of both men.

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a rapidly progressive rare neurological disease, a form of human spongiform encephalopathy in which dementia progresses to death after a period of 3-12 months. There is no effective treatment. The causative agent is currently believed to be an abnormal prion protein that accumulates in the brain and causes widespread destruction of tissue. CJD typically affects middle-aged to elderly people. Some 15% of cases are due to a form of the disease that is inherited as an autosomal dominant trait but most cases are sporadic, susceptibility being genetically determined. A few cases of CJD are acquired: the agent is known to have been transmitted by tissue and organ transplantation and by human growth hormone injections, but the disease may take years to manifest itself. A variant form of acquired CJD (vCJD) affecting younger people has been linked with the consumption of meat products containing tissue from cattle infected with bovine spongiform encephalopathy (BSE).
H. G. Creutzfeldt (1885-1964) and A. M. Jakob (1884-1931), German psychiatrists

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Creutz·feldt-Ja·kob disease (kroitsґfelt yahґkōb) [Hans Gerhard Creutzfeldt, German psychiatrist, 1885–1964; Alfons Maria Jakob, German psychiatrist, 1884–1931] see under disease.

Medical dictionary. 2011.

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