NR APTK
AU Dormont,D.
TI Human transmissible subacute spongiform encephalopathies
QU Medecine et Maladies Infectieuses 1996; 26(NBIS): 243-50
PT Article
AB Human transmissible spongiform encephalopathies (TSE) are rare chronic subacute degenerative diseases of the central nervous system (CNS) which include Creutzfeldt-Jakob disease (CJD), Kuru, Gerstmann-Sträussler-Scheinker syndrome (GSS) and Fatal Familial Insomnia (FFI). CJD can be either inherited or sporadic. All these diseases are always fatal. Neuropathological features mainly comprise neuronal vacuolization, neuronal death and gliosis with hyperastrocytosis; plaques may be evidenced in Kuru and GSS. Neither inflammatory syndrome nor demyelinization is detectable. No virus-like structure has been identified and subsequently reproduced. Human TSE are transmissible to non- human primates and rodents. Iatrogenic CJD have been described after tissue grafting (cornea, dura mater), neurosurgery, electrophysiology investigation and treatment with pituitary-derived gonadotrophines and growth hormone. Molecular biochemistry investigation of the CNS has revealed that a host encoded protein, the prion protein (PrP), accumulates in proportion to the infectious titer. This abnormality is the only hallmark of TSE. Infectious fractions contain no detectable specific nucleic acid and mainly comprise PrP in an isoform which resists proteinase K digestion (PrPres). The PrP gene (PRNP) is located on chromosome 20 in humans. Several mutations of this gene have been described in all inherited TSE (CJD, GSS and IFF). No treatment is available today. Agents inducing TSE (TSA) are not known. Several authors claim that TSA only consist of PrP- res; others support the hypothesis of a conventional agent with specific genetic information.
ZR 47
SP französisch