Can I read Human Prion Diseases: Cause, Clinical and Diagnostic Aspects on EtoBox?

Human Prion Diseases: Cause, Clinical and Diagnostic Aspects by R. Knight; S. Collins is a book available to read on EtoBox.

What is Human Prion Diseases: Cause, Clinical and Diagnostic Aspects about?

There are four predominant human prion disease phenotypes: Creutzfeldt-Jakob disease (CJD); Gerstmann-Stra ̈ussler-Scheinker syndrome (GSS); fatal familial insomnia (FFI), and kuru. The commonest is CJD, varying in causation from genetic through acquired to unknown, with different epidemiological and clinical characteristics (table 1). However, all prionoses are progressive, invariably fatal neurodegenerative diseases, with broadly similar neuropathological features, most specifically in terms of PrP Sc deposition, as well as their transmissibility to a range of laboratory animal hosts. Our understanding of these diseases, especially that which has arisen from molecular biology, suggests a need for nosological reconsideration. CJD has become a generic diagnostic label for syndromes with different aetiologies (idiopathic, acquired and inherited) and with rather different clinico-pathological phenotypes (as is the case, for example, with sporadic and variant CJD). GSS, originally delineated as an autosomal dominantly inherited cerebellar ataxia with a characteristic pathology, is now something of an umbrella term, covering a group of illnesses with differing clinico-pathological fea

Author

R. Knight; S. Collins

Publisher

KARGER

Published

2000

Language

EN