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Sudomotor dysfunction in autoimmune autonomic ganglionopathy

From the Department of Neurology (K.K., V.I., P.S., R.D.F., P.A.L.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.V.), UT Southwestern Medical Center, Dallas, TX.

Address correspondence and reprint requests to Dr. Phillip A. Low, Mayo Clinic, 200 First Street SW, Rochester, MN 55905 low{at}mayo.edu

Background: Autoimmune autonomic ganglionopathy is characterized by impairment of multiple autonomic domains of which sudomotor function is among the most common. Many patients with this disorder have difficulties with thermoregulation and anhidrosis. Our objective was to characterize the distribution and severity of sudomotor dysfunction in this disorder.

Methods: Sudomotor function was analyzed in a cohort of 21 patients with ganglionic 3 nicotinic acetylcholine receptor (nAChR) antibody positive autoimmune autonomic ganglionopathy. Standard measurements of sudomotor function were used including the Thermoregulatory Sweat Test and Quantitative Sudomotor Axon Reflex Test.

Results: The clinical presentation in all patients was characterized by widespread sudomotor dysfunction. Sudomotor impairment was predominantly postganglionic in 17 of the 21 patients studied. Higher ganglionic 3 nAChR antibody levels resulted in progressive postganglionic predominant dysfunction (postganglionic, r = 0.637, p = 0.002; mixed ganglionic, r = 0.709, p < 0.001). The pattern of anhidrosis on Thermoregulatory Sweat Testing was consistent with a ganglionopathy in the majority of patients (14 of 21) and a distal pattern in a minority of patients (8 of 21). These patterns of anhidrosis coupled with increasing postganglionic dysfunction in a proximal to distal pattern (foot > distal leg > proximal leg > forearm) indicate lesions at both the ganglia and distal axon of the postganglionic sudomotor sympathetic neuron.

Conclusions: Our data characterize the unique sudomotor dysfunction in autoimmune autonomic ganglionopathy as widespread, predominantly postganglionic, and a result of lesions at both the ganglia and distal axon. This study provides important support to the hypothesis that this disorder represents a ganglionic neuropathy.

Abbreviations: AAG = autoimmune autonomic ganglionopathy; CASS = Composite Autonomic Severity Score; nAChR = nicotinic acetylcholine receptor; QSART = Quantitative Sudomotor Axon Reflex Testing; TST = Thermoregulatory Sweat Test.

Supplemental data at www.neurology.org

Supported in part by the NIH (NS 32352, NS 44233, NS 22352, and NS 43364), Mayo CTSA (UL1 RR24150), and Mayo Funds. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institute of Neurological Disorders and Stroke or the National Institutes of Health.

Disclosure: Author disclosures are provided at the end of the article.

Received April 22, 2009. Accepted in final form July 27, 2009.