Autoantibodies Blocking M3 Muscarinic Receptors Cause Postganglionic Cholinergic Dysautonomia
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A 10‐year‐old girl presented with ileus, urinary retention, dry mouth, lack of tears, fixed dilated pupils, and diffuse anhidrosis 7 days after a febrile illness. We hypothesized that her syndrome was due to autoimmunity against muscarinic acetylcholine receptors, blocking their activation. Using an indirect enzyme‐linked immunosorbent assay for all 5 muscarinic receptors (M 1–M 5), we identified in the patient's serum antibodies that selectively bound to M 3 receptors. In vitro functional studies confirmed that these autoantibodies selectively blocked M 3 receptor activation. Thus, autoantibodies against M 3 acetylcholine receptors cause acute postganglionic cholinergic dysautonomia. ANN NEUROL 2020;88:1237–1243
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Autoantibodies to muscarinic M3 receptors have been reported in selective dysautonomia.
“Autoantibodies to muscarinic M3 receptors have been reported in selective dysautonomia (Palma et al., 2020), and cytomegalovirus infection of the myenteric plexus can produce chronic intestinal pseudo-obstruction (Sonsino et al., 1984).”
From Autonomic nervous system and gastrointestinal manifestations with an emphasis on gastric dysfunction (2026) · cites this paper as Palma et al., 2020
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- Authors
- Palma, J., Gupta, A., Sierra, S., Gomes, I., Balgobin, B., Norcliffe‐Kaufmann, L., Devi, L. A., & Kaufmann, H.
- Affiliations
- NYU Langone Health (US)
- Icahn School of Medicine at Mount Sinai (US)
- Corresponding author
- Kaufmann, Horacio
- ORCID
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 2020-09-10
- Volume
- 88
- Issue
- 6
- Pages
- 1237-1243
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- PMC
- PMC7674213
- OpenAlex
- W3081333330
- Cited by
- 13
- References
- 20
- Citations by year
- 2026: 1 · 2025: 3 · 2024: 2 · 2023: 1 · 2022: 1 · 2021: 5
- FWCI
- 0.76
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Annals of Neurology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- Dysautonomia Foundation (N/A)
- National Institute of Neurological Disorders and Stroke (R01‐NS026880, U54‐NS065736)
- National Institute on Drug Abuse (R37‐DA008863)
- Topics
- Health Sciences › Medicine › Neurology › Autoimmune Neurological Disorders and Treatments
- Health Sciences › Medicine › Neurology › Myasthenia Gravis and Thymoma
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Keywords
- M3 muscarinic receptor, autoantibodies, ileus, urinary retention, anhidrosis, xerostomia, febrile illness, pediatric patients
- MeSH terms
- Autoantibodies, Child, Female, Humans, Receptor, Muscarinic M3, Primary Dysautonomias
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