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Cited by 25Hereditary Neurological DisordersGastrointestinal motility and disordersCongenital gastrointestinal and neural anomalies

Decreased density of ganglia and neurons in the myenteric plexus of familial dysautonomia patients

Bar-Shai, Amir, Maayan, Channa, Vromen, Amos, Udassin, Raphael, Nissan, Aviram, Freund, Herbert R, Hanani, Menachem·2004·Journal of the Neurological Sciences
DOI 10.1016/j.jns.2004.02.017
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Abstract

Background Familial dysautonomia (FD) is a hereditary disease of the autonomic and sensory nervous system. A prominent manifestation of FD is gastrointestinal dyscoordination, which contributes to the morbidity and mortality in FD. Aim As the myenteric plexus is an essential factor in gastrointestinal motility control, we compared its morphology in appendices of FD patients and controls. Methods Appendices from FD patients (N=19) were obtained during surgery of fundoplication and gastrostomy; normal appendices (N=17) were obtained from patients suspected to suffer from acute appendicitis, in whom, however, the appendix was found to be normal. Specimens were stained histochemically for NADPH diaphorase (NADPH-d) and in a blinded manner examined under a light microscope for seven morphologic parameters: ganglionic density, neuronal density, ganglionic area, number of stained neurons per ganglion, nerve bundle width, ratio between nervous tissue area and total area, and neuronal area. Results Ganglionic density was 10.13 per mm(2) in controls versus 5.01 per mm(2) in FD (p Conclusion Densities of myenteric ganglia and neurons of FD patients were significantly lower than in controls. This deficiency may contribute to the pathogenesis of FD gastroenteropathy.

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  • Structural and functional abnormalities in familial dysautonomia include reduced neuronal density in the myenteric plexus and severe esophageal dysmotility with dilatation and fluid stasis in some patients.

    “Structural and functional abnormalities include reduced neuronal density in the myenteric plexus (Bar-Shai et al., 2004) and severe esophageal dysmotility with dilatation and fluid stasis in some patients (Palma et al., 2018).”

    From Autonomic nervous system and gastrointestinal manifestations with an emphasis on gastric dysfunction (2026) · cites this paper as Bar-Shai et al., 2004

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Authors
Bar-Shai, A., Maayan, C., Vromen, A., Udassin, R., Nissan, A., Freund, H. R., & Hanani, M.
Affiliations
Hadassah Medical Center (IL)
ORCID
Venue
Journal of the Neurological Sciences
Publisher
Elsevier BV
Published
2004-05
Volume
220
Issue
1-2
Pages
89-94
ISSN
0022-510X (ISSN-L)
DOI
10.1016/j.jns.2004.02.017
OpenAlex
W1973328591
Cited by
25
References
39
Citations by year
2026: 2 · 2024: 3 · 2023: 3 · 2022: 1 · 2021: 2 · 2018: 1 · 2017: 1 · 2016: 3 · 2015: 1 · 2012: 2
FWCI
0.35
Type
journal article
Language
en
Open access
closed
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • Journal of the Neurological Sciences (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • Hebrew University of Jerusalem
  • Israel Science Foundation
Topics
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
  • Health Sciences › Medicine › Gastroenterology › Gastrointestinal motility and disorders
  • Health Sciences › Medicine › Surgery › Congenital gastrointestinal and neural anomalies
Keywords
familial dysautonomia, myenteric plexus, neuronal density, gastrointestinal motility disorders, NADPH-diaphorase, appendix, autonomic nervous system, neurodegeneration
MeSH terms
Adolescent, Adult, Appendix, Cell Count, Child, Child, Preschool, Dysautonomia, Familial, Female, Ganglia, Histocytochemistry, Humans, Infant, Male, Myenteric Plexus, NADPH Dehydrogenase, Neurons

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