Pathologic Prion Protein Spreading in the Peripheral Nervous System of a Patient With Sporadic Creutzfeldt-Jakob Disease

Favereaux, Alexandre; Quadrio, Isabelle; Vital, C; Perret‐Liaudet, Armand; Anne, Olivier; Laplanche, Jean‐Louis; Petry, Klaus G.; Vital, Anne

doi:10.1001/archneur.61.5.747

Cited by 17 publications

(15 citation statements)

References 19 publications

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“…This protocol allowed us to detect PrP res in small amounts of tonsil tissue for v-CJD patients. For peroneal nerve analysis in s-CJD as already reported, 27,32 it could also be an alternative to UC as only 20% of sensitivity was found. Streptomycin also seems to be a valid alternative protocol to NaPTa when small amounts of tissue are available.…”

Section: Discussionmentioning

confidence: 68%

“…For post-mortem brain diagnosis, a decrease in the amount of tissue is not a critical step. But for ante-mortem biopsies of tonsils and other tissues as reported for olfactory mucosa and in our previous study on peripheral nerves, 27,30 processing low amounts without a good recovery of PrP sc would be a limitation for certain methods. In the case of tonsil examination in clinically suspected v-CJD cases, our results obtained with streptomycin permitted to clearly detect characteristic PrP res pattern in this lymphoid tissue.…”

Section: Discussionmentioning

confidence: 76%

“…In our laboratory, this protocol is currently performed since 1998 with 100 mg of prefrontal cortex as previously described 27 with slight modifications; prior to ultracentrifugation in Sarkosyl, homogenates were digested by proteinase K (25 mg/ml) for 1 h at 371C before stopping digestion by PMSF 1 mM.…”

Section: Ultracentrifugation Protocolmentioning

confidence: 99%

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Rapid diagnosis of human prion disease using streptomycin with tonsil and brain tissues

Quadrio

Ugnon-Café

Dupin

et al. 2009

Laboratory Investigation

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The use of streptomycin in the pathological prion protein (PrP sc ) detection procedures represents a new and attractive way for diagnostic purpose. With this agent, western blot readily detected PrP sc in 263K scrapie hamster and C57Bl/6 wild-type mice challenged with C506M3 scrapie strain. Our aim was to evaluate this new diagnosis procedure in the field of human transmissible spongiform encephalopathies (TSEs). First, we had confirmed the ability of streptomycin to precipitate PrP res from human brain of Creutzfeldt-Jakob disease (CJD) patient. Second, we compared the detection of PrP res with streptomycin against three other protocols using other precipitations. Then we assessed PrP res detection with streptomycin in 98 brain tissue samples from various aetiologies of human TSEs and 52 brain samples from other dementia. Finally, we applied this protocol for tonsils examination of five patients suspected of variant CJD (v-CJD). Sensitivity and specificity obtained with the streptomycin protocol were both 100% on brain tissue. For tonsil tissues, PrP res was clearly identified in the two post-mortem confirmed v-CJD cases, whereas no characteristic three-band pattern was seen in the three confirmed non-v-CJD samples. In this study, streptomycin demonstrated its efficiency to detect PrP res both in the central nervous system and in the lymphoid tissue without practical difficulty and with rapid preparation. Because of its ability to act as a good agent for PrP sc examination in different tissues, recovery of PrP sc in biological fluids using streptomycin should open further perspectives of applications in CJD diagnostics. Streptomycin effects in vivo might thus also be questioned.

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Section: Discussionmentioning

confidence: 68%

Section: Discussionmentioning

confidence: 76%

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Rapid diagnosis of human prion disease using streptomycin with tonsil and brain tissues

Quadrio

Ugnon-Café

Dupin

et al. 2009

Laboratory Investigation

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“…However, the application of techniques with improved sensitivity allowed the demonstration of PrP Sc deposits also in peripheral tissues,2–4 including autonomic and dorsal root ganglia,5 6 and trigeminal7 and peripheral nerves 8. Despite this progress, studies specifically addressing the prevalence and type of peripheral nervous system (PNS) signs in sCJD are lacking, and neuropathy only anecdotally reported 9–12…”

Section: Introductionmentioning

confidence: 99%

Prion-related peripheral neuropathy in sporadic Creutzfeldt-Jakob disease

Baiardi

Redaelli

Ripellino

et al. 2018

J Neurol Neurosurg Psychiatry

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ObjectiveTo assess whether the involvement of the peripheral nervous system (PNS) belongs to the phenotypic spectrum of sporadic Creutzfeldt-Jakob disease (sCJD).MethodsWe examined medical records of 117 sCJDVV2 (ataxic type), 65 sCJDMV2K (kuru-plaque type) and 121 sCJDMM(V)1 (myoclonic type) subjects for clinical symptoms, objective signs and neurophysiological data. We reviewed two diagnostic nerve biopsies and looked for abnormal prion protein (PrPSc) by western blotting and real-time quaking-induced conversion (RT-QuIC) in postmortem PNS samples from 14 subjects.ResultsSeventy-five (41.2%) VV2-MV2K patients, but only 11 (9.1%) MM(V)1, had symptoms or signs suggestive of PNS involvement occurring at onset in 18 cases (17 VV2-MV2K, 9.3%; and 1 MM(V)1, 0.8%) and isolated in 6. Nerve biopsy showed a mixed predominantly axonal and demyelinating neuropathy in two sCJDMV2K. Electromyography showed signs of neuropathy in half of the examined VV2-MV2K patients. Prion RT-QuIC was positive in all CJD PNS samples, whereas western blotting detected PrPSc in the sciatic nerve in one VV2 and one MV2K.ConclusionsPeripheral neuropathy, likely related to PrPSc deposition, belongs to the phenotypic spectrum of sCJDMV2K and VV2 and may mark the clinical onset. The significantly lower prevalence of PNS involvement in typical sCJDMM(V)1 suggests that the PNS tropism of sCJD prions is strain dependent.

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“…An extensive accumulation of PrPsc in the dorsal root ganglia and autonomic ganglia has been reported and in all subgroups of spongiform encephalopathy [3,6]. In non-iatrogenic CJD, some immunohistochemical studies have revealed PrP deposits in the PNS of patients presenting no clinical peripheral signs [2,5]. Antoine et al performed a pathological analysis of the PNS in a sporadic CJD patient presenting with demyelinating polyneuropathy, reporting aspects of inflammatory demyelination and remyelination in the nerve biopsy specimen and in the proximal nerve trunks Fig.…”

Section: Discussionmentioning

confidence: 99%

Severe and rapidly evolving peripheral neuropathy revealing sporadic Creutzfeldt-Jakob disease

et al. 2009

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Pathologic Prion Protein Spreading in the Peripheral Nervous System of a Patient With Sporadic Creutzfeldt-Jakob Disease

Cited by 17 publications

References 19 publications

Rapid diagnosis of human prion disease using streptomycin with tonsil and brain tissues

Rapid diagnosis of human prion disease using streptomycin with tonsil and brain tissues

Prion-related peripheral neuropathy in sporadic Creutzfeldt-Jakob disease

Severe and rapidly evolving peripheral neuropathy revealing sporadic Creutzfeldt-Jakob disease

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