Archives of neurology · 2004
Pathologic prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt-Jakob disease.
СтатьяНазвание и аннотация: Перевод в очереди
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Favereaux Alexandre, Quadrio Isabelle, Vital Claude, Perret-Liaudet Armand, Anne Olivier, Laplanche Jean-Louis, Petry Klaus G, Vital Anne
Аннотация
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.
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.