Demyelinating peripheral neuropathy in Creutzfeldt-Jakob disease.
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.
Department of Neurology, Tel-Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel-Aviv University, Israel.
M Y
MY
Neufeld
J
J
Josiphov
A D
AD
Korczyn
D000200:Action Potentials; D003062:Codon; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000235:genetics; D003711:Demyelinating Diseases / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D004568:Electrodiagnosis; D004576:Electromyography; D006801:Humans; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000503:physiopathology; D009154:Mutation; D009419:Nerve Tissue Proteins / Q000235:genetics; D009431:Neural Conduction; D010523:Peripheral Nervous System Diseases / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D018620:PrPSc Proteins; D011328:Prions / Q000235:genetics
D003062:Codon; D009419:Nerve Tissue Proteins; D018620:PrPSc Proteins; D011328:Prions