High levels of disease related prion protein in the ileum in variant Creutzfeldt-Jakob disease
- MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, National Hospital for Neurology and Neurosurgery, London, UK
- Correspondence to:
Professor J Collinge
MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK;
Disease related prion protein (PrPSc) is readily detectable in lymphoreticular tissues in variant Creutzfeldt-Jakob disease (vCJD) but not in other forms of human prion disease.1–5 This distinctive pathogenesis together with the unknown population prevalence of asymptomatic vCJD infection1,5,6 has led to significant concerns that secondary transmission of vCJD prions will occur through a wide range of surgical procedures.1,3,7 Risk assessment for intestinal endoscopy, biopsy, and surgery is currently limited by a lack of knowledge about relative PrPSc levels and prion titres within intestinal tissues in vCJD patients. Because of its high content of lymphoid follicles, terminal ileum is regarded as the intestinal tissue having the highest potential for iatrogenic transmission of vCJD prions.8,9 Here we provide the first report of relative PrPSc concentrations in vCJD terminal ileum.
Tissues were obtained at autopsy with consent from relatives from four patients with neuropathologically confirmed vCJD and two …