NR AAXH
AU Baker,H.F.; Duchen,L.W.; Jacobs,J.M.; Ridley,R.M.
TI Spongiform encephalopathy transmitted experimentally from Creutzfeldt-Jakob and familial Gerstmann-Sträussler-Scheinker diseases
QU Brain: A Journal of Neurology 1990 Dec; 113(6): 1891-909
PT journal article
AB A comparison was made of the effects of experimental intracerebral inoculation into marmosets of brain homogenates from a case of Creutzfeldt-Jakob disease (CJD) and from a member of the Wo. family with cerebral amyloid and spongiform encephalopathy - the Gerstmann-Sträussler-Scheinker (GSS) syndrome. All the inoculated marmosets developed spongiform encephalopathy (SE) after incubation times of 20-23 months in the CJD group and 25-32 months in the GSS group. Subsequent passage from 1 affected animal in each group resulted in SE developing after 17 months incubation. In every animal inoculated with CJD or GSS material and in the 2 passage experiments the most severely affected region of the brain was the thalamus which in all cases was almost totally occupied by vacuoles. Other grey matter masses were less severely and less consistently affected. Vacuolation was observed in the cerebellar granule cell layer as well as in the molecular layer and the brain stem was finely vacuolated in all cases. There were only minor and inconsistent differences between the disease transmitted from CJD compared with GSS and some differences between the original transmissions and the SE caused by passaged inocula. Severe astrocytic gliosis accompanied the spongiform changes but no amyloid was identified in any of the marmosets with experimentally transmitted disease. The pathogenesis of the spongiform change in the thalamus was studied in a series of marmosets by light and electron microscopy 3-22 months after the intracerebral inoculation of CJD or GSS homogenates and was compared with controls. Dilated irregularly-shaped cisternae and the large complex vacuoles typical of SE, present in abundance after 18 and 22 months incubation, were considered most probably to be derived from cisternae of neuronal smooth endoplasmic reticulum.
IN Gehirnmaterial von einem Creutzfeldt-Jakob-Patienten und einem Gerstmann-Sträussler-Scheinker-Patienten wurde Weißpinseläffchen aus der Gruppe der südamerikanischen Krallenaffen in die Gehirne gespritzt. Alle mit CJD infizierten Affen erkrankten binnen 20-23 Monaten. Alle mit GSS infizierten Affen erkrankten binnen 25-32 Monaten. Ein Affe von jeder Gruppe übertrug seine spongiforme Enzephalopathie wiederum intracerebral auf weitere Weißpinseläffchen in nur 17 Monaten. Bei allen infizierten Tieren war der Thalamus am schwersten betroffen und fast vollständig mit Vakuolen durchsetzt. Aber auch im Kleinhirn und im Hirnstamm der Tiere wurde eine feine Durchlöcherung festgestellt. Immer wurde die Durchlöcherung von einer starken Astrozytose begleitet, aber nie wurde Amyloid nachgewiesen. Die Unterschiede zwischen den mit CJD und den mit GSS infiziertenTieren waren gering und nicht immer erkennbar. Es gab Unterschiede zwischen den primär und den nach der ersten Passage infizierten Affen.
MH Animal; Brain/*pathology/ultrastructure; Callithrix; Case Report; Cerebral Cortex/pathology; Creutzfeldt-Jakob Syndrome/*pathology/transmission; Female; Gerstmann-Sträussler-Scheinker Disease/*pathology/transmission; Human; Male; Microscopy, Electron; Middle Age; Thalamus/pathology; Time Factors; Tissue Extracts/*toxicity
AD Division of Psychiatry, MRC Clinical Research Centre, Harrow, Middlesex, UK
SP englisch
PO England