Accumulation of prion protein in the vagus nerve in creutzfeldt–jakob disease

Kresl, Philip; Rahimi, Jasmin; Gelpí, Ellen; Aldecoa, Ibán; Ricken, Gerda; Danics, Krisztina; Keller, Éva; Kovács, Gábor G.

doi:10.1002/ana.25451

Cited by 13 publications

(7 citation statements)

References 25 publications

(81 reference statements)

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“…There are some previous reports suggesting that the vagus nerve from the gastrointestinal tract, parasympathetic and sympathetic nerves, and lymphoreticular system play a central role in the pathogenesis of transmission in prion disease. [ 14 – 19 ] Kresl et al [ 20 ] reported that 5.7% of sporadic and genetic CJD cases showed prion protein immunoreactivity in the peripheral segment of the intracranial portion of the vagus nerve. Kresl et al [ 20 ] also pointed out that prion protein in CJD may have similarities with alpha-synuclein deposits in PD, where the olfactory and gastrointestinal tract have been discussed as potential routes of alpha-synuclein spreading to the brain.…”

Section: Discussionmentioning

confidence: 99%

“…[ 14 – 19 ] Kresl et al [ 20 ] reported that 5.7% of sporadic and genetic CJD cases showed prion protein immunoreactivity in the peripheral segment of the intracranial portion of the vagus nerve. Kresl et al [ 20 ] also pointed out that prion protein in CJD may have similarities with alpha-synuclein deposits in PD, where the olfactory and gastrointestinal tract have been discussed as potential routes of alpha-synuclein spreading to the brain. Glatzel et al [ 21 ] reported that the sympathetic nervous system was essentially involved in the process of neuroinvasion via peripheral nerves in prion disease and showed that mice with a depressed sympathetic nervous system showed a delayed development of scrapie after peripheral prion inoculation.…”

Section: Discussionmentioning

confidence: 99%

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V180I genetic Creutzfeldt-Jakob disease with cardiac sympathetic nerve denervation masquerading as Parkinson's disease

et al. 2021

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Rationale: Creutzfeldt-Jakob disease (CJD) with a point mutation of valine to isoleucine at codon 180 of the prion protein gene (V180I) is the most frequent form of genetic CJD in Japan. However, peripheral nerve involvement, especially cardiac sympathetic denervation, has not been investigated in cases with V180I genetic CJD. We herein report a genetically confirmed case of V180I genetic CJD presenting with parkinsonism and cardiac sympathetic nerve denervation. Patient concerns: The patient was a 79-year-old Japanese woman who presented with subacute progressive gait disturbance and cognitive impairment. Clinical diagnosis of Parkinson's disease (PD) with mild cognitive impairment was initially suspected based on parkinsonism, such as bradykinesia, rigidity and tremor, and reduced accumulation of cardiac meta-iodobenzylguanidine (MIBG) scintigraphy. Interventions: Based on parkinsonism and impaired cardiac MIBG findings, levodopa/decarboxylase inhibitor was administered up to 300 mg/day; however, her symptoms were not improved. Outcomes: Her motor and cognitive function progressively deteriorated. Diagnosis: Although the patient had no family history of CJD, genetic CJD was diagnosed according to extensive hyperintensities in the bilateral cortices on diffusion-weighted magnetic resonance images, positive tau protein and 14-3-3 protein in the cerebrospinal fluid and a V180I mutation with methionine homozygosity at codon 129 by prion protein gene analysis. Lessons: We should be aware that reduced uptake of cardiac MIBG scintigraphy in patients presenting with parkinsonism cannot confirm a diagnosis of PD. CJD should be considered when patients show a rapid progressive clinical course with atypical manifestations of PD.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

V180I genetic Creutzfeldt-Jakob disease with cardiac sympathetic nerve denervation masquerading as Parkinson's disease

et al. 2021

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“…Studies have now identified PrP Sc in retinal and optic nerve tissue 196 , 197 , although at concentrations lower than those found in the brain. PrP Sc has also been detected in intracranial portions of the vagus nerve, but extracranial portions have not been tested 198 . A recent study 194 demonstrated detectable PrP Sc in a range of peripheral tissues including skin and femoral nerve.…”

Section: Ongoing Public Health Concernsmentioning

confidence: 99%

The importance of ongoing international surveillance for Creutzfeldt–Jakob disease

et al. 2021

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Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. Emerging evidence from peripheral tissue distribution studies employing high-sensitivity assays suggests that all forms of human prion disease carry a theoretical risk of iatrogenic transmission. Finally, emerging diseases, such as chronic wasting disease and camel prion disease, pose further risks to public health. In this Review, we provide an up-to-date overview of the transmission of prion diseases in human populations and argue that CJD surveillance remains vital both from a public health perspective and to support essential research into disease pathophysiology, enhanced diagnostic tests and much-needed treatments.

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“…In these cases, transmissible prions are likely to travel from enteric neurons to vagal neurons that innervate the gut and the brain (Mcbride and Beekes, 1999;Sigurdson et al, 2001). Intriguingly, a number of sporadic and genetic cases also exhibit prion deposition in the vagus nerve (Kresl et al, 2019), raising the possibility that the ENS plays a role in nontransmissible as well as transmissible prion disease. Propagation of prions requires expression of normally folded protein PrP c by the host, which misfolds upon coming into contact with the infectious isoform PrP sc .…”

Section: Roles For Enteric Glia In Neurological Diseasementioning

confidence: 99%

Enteric glia in homeostasis and disease: From fundamental biology to human pathology

Rosenberg

Rao

2021

iScience

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Glia, the non-neuronal cells of the nervous system, were long considered secondary cells only necessary for supporting the functions of their more important neuronal neighbors. Work by many groups over the past two decades has completely overturned this notion, revealing the myriad and vital functions of glia in nervous system development, plasticity, and health. The largest population of glia outside the brain is in the enteric nervous system, a division of the autonomic nervous system that constitutes a key node of the gut-brain axis. Here, we review the latest in the understanding of these enteric glia in mammals with a focus on their putative roles in human health and disease.From their first description by Russian anatomist Alexandre Dogiel in 1899 (Dogiel, 1899), glia in the gut were considered to be analogous to Schwann cells, the dominant population of glia in the extraenteric peripheral nervous system (PNS). Many Schwann cells myelinate nerve fibers and, like enteric glia, are neural crest-derived. Detailed studies of ENS ultrastructure by electron microscopy (EM), however, showed that while glia in both rodent and human intestines partially ensheathed axons, no evidence of myelin-like

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Accumulation of prion protein in the vagus nerve in creutzfeldt–jakob disease

Cited by 13 publications

References 25 publications

V180I genetic Creutzfeldt-Jakob disease with cardiac sympathetic nerve denervation masquerading as Parkinson's disease

V180I genetic Creutzfeldt-Jakob disease with cardiac sympathetic nerve denervation masquerading as Parkinson's disease

The importance of ongoing international surveillance for Creutzfeldt–Jakob disease

Enteric glia in homeostasis and disease: From fundamental biology to human pathology

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