NR ADOT
AU Dormont,D.
TI [Human transmissible subacute spongiform encephalopathy]
OT Les encephalopathies subaigues spongiformes transmissibles humaines
QU Bulletin de l'Académie Nationale de Médecine 1994 May; 178(5): 887-903; discussion 904-5
PT journal article; review; review, tutorial
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 are mainly constituted of neuronal vacuolisation, neuronal death, gliosis with hyperastrocytosis; plaques might be evidenced in kuru and GSS. Neither inflammatory syndrome nor demyelination is detectable. No virus like structure could be identified reproducibly. 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 gonadotrophins and growth hormone. Molecular biochemistry of the CNS investigation revealed that a host encoded protein, the prion protein (PrP), accumulates proportionally to the infectious titer: this abnormality is the only detectable hallmark in TSE. Infectious fractions contain no detectable specific nucleic acid, and are mainly constituted of PrP under an isoform which resists to 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 are only constituted of PrPres; others support the hypothesis of a conventional agent with a specific genetic information.
ZR 47
MH Animal; Creutzfeldt-Jakob Syndrome/etiology; English Abstract; Gerstmann-Sträussler-Scheinker Disease/etiology; Human; Kuru/etiology; Prion Diseases/*etiology
AD Centre de Recherches du Service de Sante des Armees, Commissariat a l'Energie Atomique, DSV/DPTE, Fontenay-aux-Roses.
SP französisch
PO Frankreich