Prion diseases

Pritchard, Caroline; Hirsch, Nicholas

doi:10.1016/j.mpaic.2007.08.017

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Volume 9, Issue 5 , Pages 207-209, May 2008

Prion diseases

Caroline Pritchard, FRCA, is a specialist registrar on the North Central Thames rotation. She qualified from the University of Cambridge and Charing Cross and Westminster medical school. She has a specialist interest in neuroanaesthesia

Nicholas Hirsch, FRCA, FRCP, is Consultant Neuroanaesthetist at the National Hospital for Neurology and Neurosurgery, Queen Square, London, and Honorary Lecturer at the Institute of Neurology. He qualified from Guy's Hospital in 1978 and trained in anaesthesia in London and at Yale University. His research interests involve neuroanaesthesia and neurocritical care medicine

Abstract

The prion diseases are a group of invariably fatal neurodegenerative conditions caused by a structurally abnormal form of the naturally occurring cellular prion protein. Although rare, recent interest has been intense because of the appearance of a new prion disease – variant Creutzfeldt–Jakob disease caused by ingestion of meat from BSE-infected cattle. The disease is transmissible and therefore has implications for the anaesthetist, including the use of airway devices, fibre-optic equipment and blood transfusion.

Keywords: airway devices, blood transfusion, prion disease, variant Creutzfeldt–Jakob disease