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Previous Volume 343:847-855 September 21, 2000 Number 12 Next

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ABSTRACT

Background Idiopathic autonomic neuropathy is a severe, subacute disorder with a presumed autoimmune basis. It is indistinguishable from the subacute autonomic neuropathy that may accompany lung cancer or other tumors. Autoantibodies specific for nicotinic acetylcholine receptors in the autonomic ganglia are potentially pathogenic and may serve as serologic markers of various forms of autoimmune autonomic neuropathy.

Methods We tested serum from 157 patients with a variety of types of dysautonomia. Immunoprecipitation assays with iodine-125–labeled epibatidine and solubilized human neuroblastoma acetylcholine receptors were used to detect autoantibodies that bound to or blocked ganglionic receptors.

Results Ganglionic-receptor–binding antibodies were found in 19 of 46 patients with idiopathic or paraneoplastic autonomic neuropathy (41 percent), in 6 of 67 patients with postural tachycardia syndrome, idiopathic gastrointestinal dysmotility, or diabetic autonomic neuropathy (9 percent), and in none of 44 patients with other autonomic disorders. High levels of the binding antibodies correlated with more severe autonomic dysfunction (including the presence of tonic pupils). Levels of these antibodies decreased in patients who had clinical improvement. All seven patients with ganglionic-receptor–blocking antibodies had ganglionic-receptor–binding antibodies and had idiopathic or paraneoplastic autonomic neuropathy.

Conclusions Seropositivity for antibodies that bind to or block ganglionic acetylcholine receptors identifies patients with various forms of autoimmune autonomic neuropathy and distinguishes these disorders from other types of dysautonomia. The positive correlation between high levels of ganglionic-receptor antibodies and the severity of autonomic dysfunction suggests that the antibodies have a pathogenic role in these types of neuropathy.

Source Information

From the Departments of Neurology (S.V., P.A.L., R.D.F., V.A.L.), Immunology (V.A.L.), and Laboratory Medicine and Pathology (V.A.L.) and the Division of Gastroenterology and Hepatology (G.F.), Mayo Clinic, Rochester, Minn.; and the Department of Neurology and Neurosurgery, Montreal Neurological Hospital and Institute, McGill University, Montreal (J.D.S.).

Address reprint requests to Dr. Vernino at the Department of Neurology, Mayo Clinic, 200 First St., S.W., Rochester, MN 55905, or at verns{at}mayo.edu.

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