Pathologic prion protein spreading in the peripheral nervous system of a patient with sporadic Creutzfeldt-Jakob disease.
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.


METHODS
Autopsy study.


METHODS
Three patients with sporadic Creutzfeldt-Jakob disease.


METHODS
Study of the brain, spinal cord, and sciatic and superficial peroneal nerves by immunohistochemistry and Western blot analysis.


METHODS
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.
Favereaux
Alexandre
A
Equipe d'Accueil 2966, Victor Segalen University, Bordeaux, France. alexandre.favereaux@neuropath.u-bordeauz2.fr
Quadrio
Isabelle
I
Vital
Claude
C
Perret-Liaudet
Armand
A
Anne
Olivier
O
Laplanche
Jean-Louis
JL
Petry
Klaus G
KG
Vital
Anne
A
D000368:Aged; D000369:Aged, 80 and over; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism / Q000473:pathology*; D005260:Female; D005727:Ganglia, Spinal / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D010543:Peroneal Nerve / Q000378:metabolism / Q000473:pathology*; D018620:PrPSc Proteins / Q000378:metabolism*; D012584:Sciatic Nerve / Q000378:metabolism / Q000473:pathology; D013116:Spinal Cord / Q000378:metabolism / Q000473:pathology
D018620:PrPSc Proteins