Myasthenia gravis with autoimmune autonomic neuropathy

Steven Vernino, William P. Cheshire, Vanda A. Lennon

Research output: Contribution to journalArticle

67 Scopus citations

Abstract

The autoantibodies that impair neuromuscular junction transmission in myasthenia gravis are specific for the nicotinic acetylcholine receptor (AChR) of muscle. Antibodies specific for AChRs in ganglionic neurons are found in a majority of patients with subacute autonomic neuropathy. Dysautonomia is not a recognized feature of myasthenia gravis, but there have been rare reports of myasthenia gravis coexisting with autonomic failure, usually in association with thymoma.Here we report seven patients who had myasthenia gravis with subacute autonomic failure. Their autonomic dysfunction ranged from isolated gastroparesis to severe panautonomic failure. Gastrointestinal dysmotility was a common feature. All had antibodies against muscle AChR, and three (all of whom had thymoma) had antibodies against neuronal ganglionic AChRs. In several patients, gastrointestinal function improved clinically after administration of an acetylcholinesterase inhibitor. These observations support a rare but definite clinical association between myasthenia gravis and autonomic failure and strengthen the concept that subacute autonomic neuropathy is an autoimmune disorder.

Original languageEnglish (US)
Pages (from-to)187-192
Number of pages6
JournalAutonomic Neuroscience: Basic and Clinical
Volume88
Issue number3
DOIs
StatePublished - May 14 2001

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Keywords

  • Antibodies
  • Autonomic neuropathy
  • Lambert-Eaton myasthenic syndrome
  • Neuronal ganglionic nicotinic AChR
  • Thymoma

ASJC Scopus subject areas

  • Endocrine and Autonomic Systems
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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