Variant Creutzfeldt-Jakob disease: immunocytochemical studies and image analysis.
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 PrP(sc) accumulation in the brain. PrP immunocytochemistry has also demonstrated the accumulation of PrP(sc) 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 PrP(sc) in lymphoid tissues does not occur in other forms of human prion disease. The patterns of PrP(sc) 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 PrP(sc) 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.
Nailon
W H
WH
CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, United Kingdom. william.nailon@ed.ac.uk
Ironside
J W
JW
D000818:Animals; D001479:Basal Ganglia / Q000378:metabolism; D002525:Cerebellar Cortex / Q000378:metabolism; D016000:Cluster Analysis; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism* / Q000473:pathology; D003937:Diagnosis, Differential; D019286:Endopeptidase K; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007150:Immunohistochemistry / Q000379:methods*; D008221:Lymphoid Tissue / Q000378:metabolism; D018620:PrPSc Proteins / Q000032:analysis / Q000378:metabolism; D011328:Prions / Q000032:analysis / Q000378:metabolism
D018620:PrPSc Proteins; D011328:Prions; D019286:Endopeptidase K