Myasthenia gravis with autoimmune autonomic neuropathy.
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.
Vernino
S
S
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Cheshire
W P
WP
Lennon
V A
VA
D000328:Adult; D000368:Aged; D000906:Antibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000150:complications* / Q000276:immunology; D001342:Autonomic Nervous System Diseases / Q000150:complications* / Q000276:immunology; D002800:Cholinesterase Inhibitors / Q000627:therapeutic use; D005260:Female; D005767:Gastrointestinal Diseases / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000150:complications*; D010257:Paraneoplastic Syndromes / Q000150:complications; D011729:Pyridostigmine Bromide / Q000627:therapeutic use; D011950:Receptors, Cholinergic / Q000276:immunology; D013945:Thymoma / Q000209:etiology / Q000276:immunology; D013953:Thymus Neoplasms / Q000209:etiology / Q000276:immunology
D000906:Antibodies; D002800:Cholinesterase Inhibitors; D011950:Receptors, Cholinergic; D011729:Pyridostigmine Bromide