Document Detail

Electron microsocopy of brain amyloid plaques from a patient with new variant Creutzfeldt-Jakob disease.
MedLine Citation:
PMID:  10867797     Owner:  NLM     Status:  MEDLINE    
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.
J G Fournier; N Kopp; N Streichenberger; F Escaig-Haye; J Langeveld; P Brown
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Acta neuropathologica     Volume:  99     ISSN:  0001-6322     ISO Abbreviation:  Acta Neuropathol.     Publication Date:  2000 Jun 
Date Detail:
Created Date:  2000-09-28     Completed Date:  2000-09-28     Revised Date:  2007-11-09    
Medline Journal Info:
Nlm Unique ID:  0412041     Medline TA:  Acta Neuropathol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  637-42     Citation Subset:  IM    
Unité 523 INSERM, Hôpital Pitié-Salpêtrière, Paris, France.
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MeSH Terms
Brain / pathology*,  ultrastructure*
Creutzfeldt-Jakob Syndrome / pathology*
Microscopy, Electron
Senile Plaques / pathology*,  ultrastructure*

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