Autoantibodies Blocking M<sub>3</sub> Muscarinic Receptors Cause Postganglionic Cholinergic Dysautonomia.

Palma, Jose-Alberto; Gupta, Achla; Sierra, Salvador; Gomes, Ivone; Balgobin, Bhumika; Norcliffe-Kaufmann, Lucy; Devi, Lakshmi A; Kaufmann, Horacio · Ann Neurol · 2020

case_report · Level V

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Abstract

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<sub>1</sub> -M<sub>5</sub> ), we identified in the patient's serum antibodies that selectively bound to M<sub>3</sub> receptors. In vitro functional studies confirmed that these autoantibodies selectively blocked M<sub>3</sub> receptor activation. Thus, autoantibodies against M<sub>3</sub> acetylcholine receptors cause acute postganglionic cholinergic dysautonomia. ANN NEUROL 2020;88:1237-1243.

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