Laboratory diagnosis of variant Creutzfeldt-Jakob disease

James Ironside, Mark Head, Jeanne E Bell, L McCardle, Bob Will

Research output: Contribution to journalLiterature reviewpeer-review

Abstract

The neuropathological and biochemical features of 33 cases of variant Creutzfeldt-Jakob disease (vCJD) diagnosed up to the end of 1998 are analysed in relation to the 646 cases of suspected CJD referred to the CJD Surveillance Unit laboratory from 1990 to 1998. Morphological studies of the central nervous system, lymphoid tissues and other organs were accompanied by immunocytochemistry; Western blot analysis of PrPRES was performed on frozen brain tissue. The findings were analysed in relation to clinical and genetic data. The pathology of vCJD showed morphological and immunocytochemical characteristics distinct from other cases of CJD. PrP accumulation was widespread in lymphoid tissues in vCJD, but was not identified in other non-neural tissues. PrPRES accumulation in vCJD brain tissue showed a uniform glycotype pattern distinct from sporadic CJD. All analysed cases of vCJD were methionine homozygotes at codon 129 of the PrP gene. No evidence currently exists to suggest that cases of CJD diagnosed in individuals who are MV or VV at codon 129 of the PrP gene represent 'human bovine spongiform encaphalopathy (BSE)'. Continued surveillance is required to further investigate this possibility, with the need to investigate autopsy tissues from suspected cases by histological and biochemical techniques.

Original languageEnglish
Pages (from-to)1-9
Number of pages9
JournalHistopathology
Volume37
Issue number1
DOIs
Publication statusPublished - Jul 2000

Fingerprint Dive into the research topics of 'Laboratory diagnosis of variant Creutzfeldt-Jakob disease'. Together they form a unique fingerprint.

Cite this