Transmissible spongiform encephalopathies in humans.
Creutzfeldt-Jakob disease (CJD), the first transmissible spongiform encephalopathy (TSE) to be described in humans, occurs in a sporadic, familial, or iatrogenic form. Other TSEs in humans, shown to be associated with specific prion protein gene mutations, have been reported in different parts of the world. These TSEs compose a heterogeneous group of familial diseases that traditionally have been classified as familial CJD, Gerstmann-Sträussler-Scheinker syndrome, or fatal familial insomnia. In 1996, a newly recognized variant form of CJD among young patients (median age, 28 years) with unusual clinical features and a unique neuropathologic profile was reported in the United Kingdom. In the absence of known CJD risk factors or prion protein gene abnormalities, the UK government concluded that the clustering of these cases may represent transmission to humans of the agent causing bovine spongiform encephalopathy. Additional epidemiologic and recent laboratory data strongly support the UK government's conclusion.
Belay
E D
ED
Division of Viral and Rickettsial Diseases, National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia 30333, USA. ebb8@cdc.gov
D000328:Adult; D000818:Animals; D001921:Brain / Q000473:pathology; D002417:Cattle; D016000:Cluster Analysis; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology / Q000209:etiology / Q000235:genetics / Q000635:transmission; D016643:Encephalopathy, Bovine Spongiform / Q000635:transmission; D014644:Genetic Variation; D016098:Gerstmann-Straussler-Scheinker Disease / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D006801:Humans; D017096:Prion Diseases / Q000145:classification / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D011328:Prions / Q000235:genetics
D011328:Prions