Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease
Open Access
- 1 March 2005
- journal article
- case report
- Published by BMJ in Journal of Neurology, Neurosurgery & Psychiatry
- Vol. 76 (3) , 325-329
- https://doi.org/10.1136/jnnp.2003.035154
Abstract
Objective: To investigate abnormal prion protein (PrP) deposition in the peripheral nervous system (PNS) in human prion diseases. Methods: Eight patients with prion diseases were examined: three with sporadic Creutzfeldt–Jakob disease (sCJD), two with dural graft associated CJD (dCJD), one with Gerstmann–Sträussler–Scheinker disease (GSS) with a PrP P102L mutation (GSS102), and two with a P105L mutation (GSS105). An atypical case of sCJD with PrP plaques in the brain presented clinically with peripheral neuropathy, and showed demyelination in 12% of the teased fibres of the sural nerve. The PNS was investigated by immunohistochemical and western blotting analyses of PrP. Results: In immunohistochemical studies, granular PrP deposits were detected in some neurones of dorsal root ganglia and a few fibres of peripheral nerves and spinal posterior roots in one sCJD and two dCJD patients, but not in GSS102 or GSS105 patients. The atypical case of sCJD with peripheral neuropathy showed no obvious PrP deposition in the nerves. Western blotting analysis of the PNS from the dCJD patients revealed a small amount of protease K resistant PrP in the dorsal root ganglia and peripheral nerves. Conclusions: Abnormal PrP deposition occurs in the dorsal root ganglia and peripheral nerves in sCJD and dCJD. The PrP deposits in the PNS are not correlated with clinical manifestation of peripheral neuropathy in CJD.Keywords
This publication has 33 references indexed in Scilit:
- Peripheral Tissue Involvement in Sporadic, Iatrogenic, and Variant Creutzfeldt-Jakob DiseaseThe American Journal of Pathology, 2004
- Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt–Jakob DiseaseNew England Journal of Medicine, 2003
- Prion protein accumulation involving the peripheral nervous system in a sporadic case of Creutzfeldt-Jakob diseaseNeuropathology and Applied Neurobiology, 2003
- Creutzfeldt-Jakob disease after Jannetta's operation with cadaveric dura mater graft: initial manifestations related to the grafted siteZeitschrift für Neurologie, 2002
- Atypical form of dural graft associated Creutzfeldt-Jakob disease: report of a postmortem case with review of the literatureJournal of Neurology, Neurosurgery & Psychiatry, 2001
- Improvement of PrPSc-detection in mouse spleen early at the preclinical stage of scrapie with collagenase-completed tissue homogenization and Sarkosyl-NaCl extraction of PrPScArchiv für die gesamte Virusforschung, 1996
- Prion protein accumulation in the spinal cords of patients with sporadic and growth hormone associated Creutzfeldt-Jakob diseaseNeuroscience Letters, 1995
- Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteinsCell, 1994
- Clinical heterogeneity and unusual presentations of Creutzfeldt-Jakob disease in Jewish patients with the PRNP codon 200 mutation.Journal of Neurology, Neurosurgery & Psychiatry, 1993
- Demyelinating peripheral neuropathy in Creutzfeldt–Jakob diseaseMuscle & Nerve, 1992