NR AFFO
AU Hauw,J.J.; Privat,N.; Sazdovitch,V.; Seilhean,D.
TI [Biopathology of transmissible subacute spongiform encephalopathies]
OT Biopathologie des encephalopathies spongiformes subaigues transmissibles
QU Revue du Praticien 1999 May 1; 49(9): 942-7
PT journal article; review; review, tutorial
AB Three lesions can be seen in the central nervous system: vacuolation ("spongiform state"), hypertrophy and proliferation of astrocytes ("astrogliosis"), and neuronal loss. These are poorly specific changes. In contrast, amyloid plaques and other deposits of PrPres are very specific lesions. The recent widening of the clinico-pathological spectrum of PrPres-induced disorders has shown that none of these lesions was constantly present. The mechanisms of PrPres occurrence and development, neuronal death, involvement of the central nervous system after peripheral inoculation are still hypothetical. B lymphocytes, monocyte-macrophages, peripheral nerves, central synapses are important. New data will be provided by experimental models (transgenic mice, transplantations), and by a full study of all patients affected by prion diseases, which necessarily involves autopsy.
ZR 30
MH Animal; Astrocytes/pathology; Autopsy; B-Lymphocytes; Brain/*pathology; Cell Death; Creutzfeldt-Jakob Syndrome/*physiopathology/*transmission; Disease Models, Animal; English Abstract; Human; Hypertrophy; Macrophages; Mice; Mice, Transgenic; Monocytes/immunology; Neurons/pathology; Prions/*adverse effects; Senile Plaques
AD Laboratoire de neuropathologie Raymond Escourolle Groupe hospitalier La Pitie-La Salpetriere, Universite Pierre-et-Marie-Curie, Paris VI INSERM U 360, Association Claude-Bernard.
SP französisch
PO Frankreich