Pathologic prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt-Jakob disease.
Arch Neurol. 2004-05-01; 61(5): 747
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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
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
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.