Demyelinating peripheral neuropathy in Creutzfeldt–Jakob disease

Abstract
We desribe 2 patients of Jewish Libyan descent, who presented with a clinical syndrome compatible with Creutzfeldt–Jakob disease and who were found to have a mutation of codon 200 in the prion protein. The patients developed symptoms and signs of peripheral nerve involvement diagnosed by electrodiagnostic and histopathological studies as demyelinating neuropathy. This may be a rare manifestation of Creutzfeldt–Jakob disease. © 1992 John Wiley & Sons, Inc.