Demyelinating peripheral neuropathy in Creutzfeldt-Jakob disease

Muscle Nerve. 1992 Nov;15(11):1234-9. doi: 10.1002/mus.880151103.

Abstract

We describe 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.

Publication types

  • Case Reports

MeSH terms

  • Action Potentials
  • Codon
  • Creutzfeldt-Jakob Syndrome / complications*
  • Creutzfeldt-Jakob Syndrome / genetics
  • Demyelinating Diseases / complications*
  • Demyelinating Diseases / diagnosis
  • Demyelinating Diseases / genetics
  • Electrodiagnosis
  • Electromyography
  • Humans
  • Male
  • Middle Aged
  • Muscles / physiopathology
  • Mutation
  • Nerve Tissue Proteins / genetics
  • Neural Conduction
  • Peripheral Nervous System Diseases / complications*
  • Peripheral Nervous System Diseases / diagnosis
  • Peripheral Nervous System Diseases / genetics
  • PrPSc Proteins
  • Prions / genetics

Substances

  • Codon
  • Nerve Tissue Proteins
  • PrPSc Proteins
  • Prions