NR AISB
AU Nailon,W.H.; Ironside,J.W.
TI Variant Creutzfeldt-Jakob disease: immunocytochemical studies and image analysis.
QU Microscopy Research and Technique 2000 Jul 1; 50(1): 2-9
PT journal article; review; review, tutorial
AB Variant Creutzfeldt-Jakob disease (vCJD) is a recently identified human prion disease that appears to arise from exposure to the bovine spongiform encephalopathy agent. The clinical features and neuropathology of vCJD are distinctive, particularly the patterns of PrPsc accumulation in the brain. PrP immunocytochemistry has also demonstrated the accumulation of PrPsc in tissues outside the central nervous system, including sensory ganglia and lymphoid tissues. These observations have allowed the use of tonsillar biopsy as an investigation to aid the diagnosis of vCJD, since accumulation of PrPsc in lymphoid tissues does not occur in other forms of human prion disease. The patterns of PrPsc accumulation in vCJD can be studied by image analysis techniques, using both quantitative and qualitative approaches. Preliminary results of textural analysis are presented, which indicate that this approach can be used to discriminate and study the unique features of PrPsc accumulation in the brain in vCJD. This technique has major potential as a research tool in human prion diseases, particularly for the characterisation of disease phenotype in large series of cases.
ZR 49
MH Animal; Basal Ganglia/metabolism; Cerebellar Cortex/metabolism; Cluster Analysis; Creutzfeldt-Jakob Syndrome/*metabolism/pathology; Diagnosis, Differential; Endopeptidase K; Human; Image Processing, Computer-Assisted; Immunohistochemistry/*methods; Lymphoid Tissue/metabolism; PrPsc Proteins/analysis/metabolism; Prions/analysis/metabolism; Support, Non-U.S. Gov't
AD CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom. william.nailon@ed.ac.uk
SP englisch
PO USA