Genetic Creutzfeldt-Jakob disease mimicking chronic inflammatory demyelinating polyneuropathyGenetic Creutzfeldt-Jakob disease mimicking chronic inflammatory demyelinating polyneuropathy
Van Broeckhoven, Christine
Faculty of Pharmaceutical, Biomedical and Veterinary Sciences . Biomedical Sciences
VIB DMG - Neurodegenerative Brain Diseases Group
Neurology Neuroimmunology & Neuroinflammation
2(2015):6, p. 1-2
University of Antwerp
Creutzfeldt-Jakob disease (CJD) is a relentlessly progressive neurodegenerative disorder, belonging to the transmissible spongiform encephalopathies. It is characterized by the deposition of the abnormal isoform of the prion protein (PrPsc), causing spongiform neurodegeneration. The disease is classically known to mainly affect the gray matter of the CNS. However, involvement of the peripheral nervous system (PNS) has been described.1,3 Herein, we describe a patient with genetic CJD, in whom the disease presentation was a demyelinating neuropathy reminiscent of chronic inflammatory demyelinating polyneuropathy (CIDP).