title: Pathologic prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt-Jakob disease.
abstract: BACKGROUND
  Involvement of the peripheral nervous system in the pathogenesis of prion diseases is becoming increasingly evident. However, pathologic protease-resistant prion protein deposition in the peripheral nerves of patients with Creutzfeldt-Jakob disease has never been demonstrated, to our knowledge.
  
  
  OBJECTIVE
  To determine whether mutated prion protein accumulation could be shown in the peripheral nervous system of patients with sporadic Creutzfeldt-Jakob disease.
  
  
  METHODS
  Autopsy study.
  
  
  METHODS
  Three patients with sporadic Creutzfeldt-Jakob disease.
  
  
  METHODS
  Study of the brain, spinal cord, and sciatic and superficial peroneal nerves by immunohistochemistry and Western blot analysis.
  
  
  METHODS
  Demonstration of protease-resistant prion protein accumulation.
  
  
  RESULTS
  In all cases, protease-resistant prion protein accumulation was found in the brain and posterior horns of the spinal cord. In 1 case, protease-resistant prion protein deposits were also evidenced in the dorsal root ganglia and the superficial peroneal nerve.
  
  
  CONCLUSIONS
  Protease-resistant prion protein may be found in the peripheral nervous system of some patients with sporadic Creutzfeldt-Jakob disease. However, a larger series is required to assess the incidence of peripheral nervous system involvement and to discuss the diagnostic usefulness of peripheral nerve biopsy in sporadic Creutzfeldt-Jakob disease.
authors.affiliation: Equipe d'Accueil 2966, Victor Segalen University, Bordeaux, France. alexandre.favereaux@neuropath.u-bordeauz2.fr
authors.forename: Alexandre
authors.initials: A
authors.lastname: Favereaux
authors.forename: Isabelle
authors.initials: I
authors.lastname: Quadrio
authors.forename: Claude
authors.initials: C
authors.lastname: Vital
authors.forename: Armand
authors.initials: A
authors.lastname: Perret-Liaudet
authors.forename: Olivier
authors.initials: O
authors.lastname: Anne
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Laplanche
authors.forename: Klaus G
authors.initials: KG
authors.lastname: Petry
authors.forename: Anne
authors.initials: A
authors.lastname: Vital
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism / Q000473:pathology*; D005260:Female; D005727:Ganglia, Spinal / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010543:Peroneal Nerve / Q000378:metabolism / Q000473:pathology*; D018620:PrPSc Proteins / Q000378:metabolism*; D012584:Sciatic Nerve / Q000378:metabolism / Q000473:pathology; D013116:Spinal Cord / Q000378:metabolism / Q000473:pathology
chemical_list: D018620:PrPSc Proteins