Autonomic dysfunction in the Lambert-Eaton myasthenic syndrome: serologic and clinical correlates.
Autonomic dysfunction is a recognized feature of the Lambert-Eaton myasthenic syndrome (LES). However, the characteristic pattern of dysautonomia has not been clearly documented and its pathophysiologic basis is not known. We therefore abstracted autonomic symptomatology and results of quantitative tests for salivation, and vasomotor, cardiovagal, and sudomotor reflexes from records of 30 LES patients. Dry mouth (77%) and impotence (45% of men) were the most common symptoms. Composite Autonomic Scoring Scale results were abnormal in 93% of patients, and autonomic failure was severe in 20%. The frequency of specific test abnormalities were the following: sudomotor function, 83%; cardiovagal reflexes, 75%; salivation, 44%; and adrenergic function, 37%. Although voltage-gated N-type calcium (Ca2+) channels are implicated in autonomic transmission, the low frequency of serum antibodies to N-type Ca2+ channels found in the patients of this study (31% positive) argues against a pathogenic role in mediating LES-related dysautonomia. In contrast, 93% of the patients were seropositive for P/Q-type Ca2+ channel antibodies. A subset of these antibodies is thought to impair neuromuscular transmission. Autoantibodies of thyrogastric or glutamic acid decarboxylase specificity (markers of predisposition to type 1 diabetes mellitus) were found in 45% of patients, and type 1 antineuronal nuclear antibody (or anti-Hu, a marker of autoimmune neuropathy associated with small-cell lung carcinoma) was found in 3%. No autoantibody correlated with autonomic dysfunction severity. Sensorimotor neuropathy was documented in five patients, and was not significantly associated with autonomic neuropathy. Autonomic failure was most severe in older subjects with cancer (p = 0.02, age by cancer interaction).
O'Suilleabhain
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Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Low
P A
PA
Lennon
V A
VA
D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D001342:Autonomic Nervous System Diseases / Q000276:immunology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D015624:Lambert-Eaton Myasthenic Syndrome / Q000276:immunology* / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction; D009474:Neurons / Q000276:immunology / Q000502:physiology; D012018:Reflex; D012189:Retrospective Studies
D001323:Autoantibodies