Electron microsocopy of brain amyloid plaques from a patient with new variant Creutzfeldt-Jakob disease |
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Authors: | J -G Fournier N Kopp N Streichenberger F Escaig-Haye J Langeveld P Brown |
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Institution: | Unité 523 INSERM, Institut de Myologie, H?pital Pitié-Salpêtrière, 47 boulevard de l’H?pital, 75651 Paris Cedex 13, France e-mail: fournier@dsvidf.cea.fr, Tel.: +33-1-46549896, Fax: +33-1-46547726, FR Laboratoire de Neuropathologie, H?pital Neurologique, BP Lyon Montchet, 69394 Lyon Cedex 03, France, FR Institute for Animal Science and Health (ID-DLO), Hedelhertweg 15, 8200 Lelystad, The Netherlands, NL National Institute of Neurological Disorders and Stroke, Laboratory of Central nervous System Studies, NIH, Bethesda, MD 20892, USA, US
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Abstract: | Cerebral cortex biopsy from a patient with new variant Creutzfeldt-Jakob disease (nvCJD) has been examined at the electron
microscope level. Spongiform changes corresponded mostly to distended neurites scattered in the neuropil or surrounding amyloid
plaques. These latter exhibited heterogeneous submicroscopic morphology including variable amount of loosely interwoven amyloid
fibrils admixed in a cellular-rich environment constituted essentially by abnormal neuronal processes. By immunoelectron microscopy,
fibrils and some membrane structures reacted with anti-prion protein (PrP) antibodies. One striking aspect was the presence
of many small dystrophic neurites without paired helical filaments. Moreover, amyloid fibrils showed unexpected intimate association
with abnormal membranes, suggesting a relationship between PrP fibrillogenesis and membrane alteration. These ultrastructural
findings provide an additional criterion to distinguish nvCJD- from sporadic CJD-type plaques and reinforce the hypothesis
that nvCJD brain is infected by a distinctive strain of the transmissible agent encephalopathy.
Received: 7 June 1999 / Accepted: 21 September 1999 |
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Keywords: | Biopsy New variant Creutzfeldt-Jakob ?disease Immunoelectron microscopy Amyloid ?plaques Dystrophic neurites |
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