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From the Department of Neurology (V.I., K.K., 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
Objective: To evaluate the efficacy of immunotherapy in the treatment of patients with seropositive and seronegative putative autoimmune autonomic ganglionopathy (AAG) using validated autonomic function tests and instruments.
Background: AAG is an immune-mediated disorder characterized by prominent and selective involvement of autonomic nerve fibers or ganglia. Treatment with IV immunoglobulin (IVIg) or plasma exchange (PE) has been reported to be effective in single case reports.
Methods: We studied six patients, four with seropositive and two with seronegative putative AAG, who underwent autonomic function tests and completed two validated questionnaires, to assess autonomic symptoms before and after immunomodulatory treatment. Patients were treated with standard doses of IVIg, PE, or immunosuppressants in a specific sequential therapy protocol depending on clinical response.
Results: Of the six patients (all women, mean ages 49.3 ± 10.6 years), four patients were ganglionic (
3) AChR autoantibody positive and two were autoantibody negative. All patients showed clinical improvement after treatment. Sudomotor function assessed by quantitative sudomotor axon reflex test and thermoregulatory sweat test improved in four patients after treatment.
Conclusions: Immunomodulatory treatment can be effective in both seropositive and seronegative putative autoimmune autonomic ganglionopathy. Plasma exchange or combined therapy with immunosuppressive agents should be considered in patients who do not benefit from IV immunoglobulin alone.
Abbreviations: AAG = autoimmune autonomic ganglionopathy; AB = ganglionic 3 acetylcholine receptor antibody; AChR = acetylcholine receptor; AE = antecedent event; ASP = autonomic symptom profile; Aza = azathioprine; BP = blood pressure; CASS = Composite Autonomic Severity Score; CCS = COMPASS Change Score; COMPASS = Composite Autonomic Symptom Score; GI = gastrointestinal; HR_db = heart rate response to deep breathing; IVIg = IV immunoglobulin; LGI = lower gastrointestinal tract symptoms; Myc = mycophenolate mofetil; OI = orthostatic intolerance; OH = orthostatic hypotension; NA = not applicable; NCS = nerve conduction studies; PE = plasma exchange; QSART = quantitative sudomotor axon reflex test; TST = thermoregulatory sweat test; UGI = upper gastrointestinal tract symptoms; VR = Valsalva ratio.
Supported by NIH (NS 32352, NS 44233, NS 22352, NS 43364), Mayo CTSA (UL1 RR24150), and Mayo Funds (P.A.L.).
Disclosure: The authors report no disclosures.
Disclaimer: The content of this article 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.
Medications and Medical Devices: A list of medications and medical devices used in this study is provided at the end of the article.
Received August 5, 2008. Accepted in final form March 4, 2009.
This article has been cited by other articles:
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  K. Kimpinski, V. Iodice, P. Sandroni, R. D. Fealey, S. Vernino, and P. A. Low Sudomotor dysfunction in autoimmune autonomic ganglionopathy Neurology, November 3, 2009; 73(18): 1501 - 1506. [Abstract] [Full Text] [PDF]
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