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Alexandre Favereaux, PhD; Isabelle Quadrio, MD; Claude Vital, MD; Armand Perret-Liaudet, MD; Olivier Anne, MD; Jean-Louis Laplanche, MD; Klaus G. Petry, PhD; Anne Vital, MD, PhD

Arch Neurol. 2004;61:747-750.

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.

From Equipe d'Accueil 2966 (Drs Favereaux, C. Vital, Anne, Petry, and A. Vital) and Departments of Neuropathology (Drs C. Vital and A. Vital) and Neurology (Dr Anne), Victor Segalen University, Bordeaux, France; Centre de Diagnostic, de Traitement et de Prévention des Maladies à Prions, Neurology and Neurosurgery Hospital, Lyon, France (Drs Quadrio and Perret-Liaudet); and Biochemistry Department, Centre de Recherche Claude Bernard, Lariboisière Hospital, Paris, France (Dr Laplanche).

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