NR AQJH
AU Favereaux,A.; Quadrio,I.; Vital,C.; Perret-Liaudet,A.; Anne,O.; Laplanche,J.L.; Petry,K.G.; Vital,A.
TI Pathologic prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt-Jakob disease
QU Archives of Neurology 2004 May; 61(5): 747-50
PT journal article
AB 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. DESIGN: Autopsy study. Patients Three patients with sporadic Creutzfeldt-Jakob disease. INTERVENTIONS: Study of the brain, spinal cord, and sciatic and superficial peroneal nerves by immunohistochemistry and Western blot analysis. Main Outcome Measure 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.
MH Aged; Aged, 80 and over; Blotting, Western; Brain/metabolism/pathology; Creutzfeldt-Jakob Syndrome/metabolism/*pathology; Female; Ganglia, Spinal/metabolism/*pathology; Human; Immunohistochemistry; Male; Peroneal Nerve/metabolism/*pathology; PrPsc Proteins/*metabolism; Sciatic Nerve/metabolism/pathology; Spinal Cord/metabolism/pathology
AD Equipe d'Accueil 2966, Victor Segalen University, Bordeaux, France. alexandre.favereaux@neuropath.u-bordeauz2.fr
SP englisch
PO USA