Autoimmune autonomic failure

Srikanth Muppidi, Steven Vernino

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

Autoimmune autonomic ganglionopathy is a syndrome of panautonomic failure caused by antibodies to ganglionic acetylcholine receptors. The clinical syndrome is characterized by significant postural hypotension, diffuse cholinergic and adrenergic impairment, gastrointestinal dysmotility, urinary retention, and pupillary dysfunction. While acute to subacute onset of disease is commonly seen, chronic, slowly progressive variants have been described. Serological testing for ganglionic acetylcholine receptor antibodies helps confirm the diagnosis. These antibodies cause a similar phenotype of autonomic failure in animal models indicating that an antibody-mediated functional impairment of ganglionic transmission is the underlying etiology. Decrease in antibody levels correlates with clinical improvement. Patients may respond to immunomodulatory therapies such as prednisone, intravenous immunoglobulin, plasma exchange, and oral immunosuppressants. A combination treatment is often required as well as symptomatic therapy.

Original languageEnglish (US)
Pages (from-to)321-327
Number of pages7
JournalHandbook of Clinical Neurology
Volume117
DOIs
StatePublished - 2013

Keywords

  • Autonomic failure
  • Autonomic function testing
  • Dysautonomia
  • Ganglionic acetylcholine receptor antibody
  • Immunotherapy

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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