TY  - JOUR
T1  - PAthologic prion protein spreading in the peripheral nervous system of a patient with sporadic creutzfeldt-jakob disease
AU  - Favereaux A, Quadrio I, Vital C, et al
Y1  - 2004/05/01
N1  - 10.1001/archneur.61.5.747
JO  - Archives of Neurology
SP  - 747
EP  - 750
VL  - 61
IS  - 5
N2  - 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.
SN  - 0003-9942
M3  - doi: 10.1001/archneur.61.5.747
UR  - http://dx.doi.org/10.1001/archneur.61.5.747
ER  -