Transmissible spongiform encephalopathies in humans

Authors
Citation
Ed. Belay, Transmissible spongiform encephalopathies in humans, ANN R MICRO, 53, 1999, pp. 283-314
Citations number
167
Language
INGLESE
art.tipo
Review
Categorie Soggetti
Microbiology
Journal title
ANNUAL REVIEW OF MICROBIOLOGY
ISSN journal
0066-4227 → ACNP
Volume
53
Year of publication
1999
Pages
283 - 314
Database
ISI
SICI code
0066-4227(1999)53:<283:TSEIH>2.0.ZU;2-C
Abstract
Creutzfeldt-Jakob disease (CJD), the first transmissible spongiform encepha lopathy (TSE) to be described in humans, occurs in a sporadic, familial, or iatrogenic form. Other TSEs in humans, shown to be associated with specifi c prion protein gene mutations, have been reported in different parts of th e world. These TSEs compose a heterogeneous group of familial diseases that traditionally have been classified as familial CJD, Gerstmann-Straussler-S cheinker syndrome, or fatal familial insomnia. In 1996, a newly recognized variant form of CJD among young patients (median age, 28 years) with unusua l clinical features and a unique neuropathologic profile was reported in th e United Kingdom. In the absence of known CJD risk factors or prion protein gene abnormalities, the UK government concluded that the clustering of the se cases may represent transmission to humans of the agent causing bovine s pongiform encephalopathy. Additional epidemiologic and recent laboratory da ta strongly support the UK government's conclusion.