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Autoimmune autonomic ganglionopathy: treatment by plasma exchanges and rituximab

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Abstract

This case report describes successful treatment of autoimmune autonomic ganglionopathy (AAG) in a 74-year-old woman by total plasma exchanges (PLEX) and rituximab. Two series of PLEX temporarily, but dramatically improved orthostatic intolerance and hypotension and baroreflex function, in a manner inversely related to ganglionic neuronal nicotinic receptor antibody titer. After rituximab treatment, the antibody titer was decreased modestly but persistently, and the patient had symptomatic and clinical laboratory evidence of continued benefit for at least 10 months, supporting the autoimmune pathogenesis of AAG.

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References

  1. Goldstein DS, Holmes C, Dendi R, Li ST, Brentzel S, Vernino S (2002) Pandysautonomia associated with impaired ganglionic neurotransmission and circulating antibody to the neuronal nicotinic receptor. Clin Auton Res 12:281–285

    Article  PubMed  Google Scholar 

  2. Schroeder C, Vernino S, Birkenfeld AL, Tank J, Heusser K, Lipp A, Benter T, Lindschau C, Kettritz R, Luft FC, Jordan J (2005) Plasma exchange for primary autoimmune autonomic failure. N Engl J Med 353:1585–1590

    Article  PubMed  CAS  Google Scholar 

  3. Stubgen JP (2008) B cell-targeted therapy with rituximab and autoimmune neuromuscular disorders. J Neuroimmunol 204(1–2):1–12

    Article  PubMed  CAS  Google Scholar 

  4. Vernino S, Lindstrom J, Hopkins S, Wang Z, Low PA (2008) Characterization of ganglionic acetylcholine receptor autoantibodies. J Neuroimmunol 197:63–69

    Article  PubMed  CAS  Google Scholar 

  5. Vernino S, Low PA, Fealey RD, Stewart JD, Farrugia G, Lennon VA (2000) Autoantibodies to ganglionic acetylcholine receptors in autoimmune autonomic neuropathies. N Engl J Med 343:847–855

    Article  PubMed  CAS  Google Scholar 

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Acknowledgments

The authors acknowledge Dr. Kaho Wong, MD who administered rituximab to the patient. The authors thank clinical support personnel of the Clinical Neurocardiology Section, including Sandra Pechnik, RN, LaToya Sewell, CRNP, and Tereza Jenkins. The study was supported by the intramural research program of the NINDS.

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Authors and Affiliations

  1. Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke, Bethesda, MD, 20892-1620, USA

    Richard Imrich, Basil A. Eldadah, Courtney Holmes & David S. Goldstein

  2. Department of Neurology, University of Texas Southwestern Medical Center, Dallas, TX, 75390-9036, USA

    Steven Vernino

  3. Center for Molecular Medicine, Institute of Experimental Endocrinology, Vlarska 3-7, 83101, Bratislava, Slovakia

    Richard Imrich

Authors
  1. Richard Imrich
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  2. Steven Vernino
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  3. Basil A. Eldadah
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  4. Courtney Holmes
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  5. David S. Goldstein
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Correspondence to Richard Imrich.

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Imrich, R., Vernino, S., Eldadah, B.A. et al. Autoimmune autonomic ganglionopathy: treatment by plasma exchanges and rituximab. Clin Auton Res 19, 259–262 (2009). https://doi.org/10.1007/s10286-009-0012-7

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  • DOI: https://doi.org/10.1007/s10286-009-0012-7

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