Clinical Autonomic Research

, Volume 28, Issue 1, pp 115–123 | Cite as

Seronegative autoimmune autonomic neuropathy: a distinct clinical entity

  • Elisabeth P. Golden
  • Meredith A. Bryarly
  • Steven Vernino
Research Article

Abstract

Purpose

Autoimmune autonomic ganglionopathy (AAG) is associated with ganglionic acetylcholine receptor (gAChR) antibodies. We describe a similar but distinct series of patients with autoimmune autonomic failure lacking this antibody.

Methods

Retrospective chart review.

Results

Six patients presented with subacute autonomic failure, seronegative for gAChR antibodies. Orthostatic hypotension and gastrointestinal complaints were common. Autonomic testing revealed predominant sympathetic failure and no premature pupillary redilation. All patients had sensory symptoms and/or pain, which was severe in three. Immunotherapy with plasma exchange, intravenous immunoglobulin, and rituximab was ineffective. Three patients responded to intravenous steroids.

Conclusion

In these cases of autoimmune autonomic failure, key differences from seropositive AAG emerge. Testing showed prominent sympathetic (rather than cholinergic) failure, specific pupillary findings of AAG were absent, and sensory symptoms were prominent. AAG responds to antibody-targeted immunotherapy, while these patients responded best to steroids. This seronegative autoimmune autonomic neuropathy is a distinct clinical entity requiring a different treatment approach from AAG.

Keywords

Autoimmune autonomic ganglionopathy Orthostatic hypotension Autonomic neuropathy Autonomic testing Immunotherapy 

Notes

Acknowledgements

We would like to thank Samarpita Sengupta, PhD, for assistance with preparation of the manuscript.

Compliance with ethical standards

Conflict of interest

Drs. Golden and Bryarly have no conflict of interest to report. Dr. Vernino has served as a consultant for Athena Diagnostics and Lundbeck and on the speaker bureau for Lundbeck.

Ethical approval

This study was approved by the UT Southwestern Institutional Review Board and was therefore performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. Subjects gave their informed consent prior to their inclusion in the study.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2017

Authors and Affiliations

  1. 1.Department of Neurology and NeurotherapeuticsUT Southwestern Medical CenterDallasUSA

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