gms | German Medical Science

28. Wissenschaftlicher Kongress der Deutschen Hochdruckliga

24. bis 27.11.2004, Hannover

Endogenous Ganglionic Blockade through Autoantibodies against the Nicotinic Acetylcholine Receptor

Endogene Ganglienblockade durch Autoantikörper gegen den nikotinergen Acetylcholinrezeptor

Meeting Abstract (Hypertonie 2004)

  • A. Birkenfeld - Franz-Volhard Clinical Research Center, Helios Klinikum (Berlin, D)
  • K. Heusser - Franz-Volhard Clinical Research Center, Helios Klinikum (Berlin, D)
  • R. Kettritz - Franz-Volhard Clinical Research Center, Helios Klinikum (Berlin, D)
  • F. Luft - Franz-Volhard Clinical Research Center, Helios Klinikum
  • S. Vernino - Department of Neurology, Mayo Clinic (Rochester, USA)
  • presenting/speaker J. Jordan - Department of Neurology, Mayo Clinic (Rochester, USA)

Hypertonie 2004. 28. Wissenschaftlicher Kongress der Deutschen Hochdruckliga. Hannover, 24.-27.11.2004. Düsseldorf, Köln: German Medical Science; 2005. Doc04hochP94

The electronic version of this article is the complete one and can be found online at: http://www.egms.de/en/meetings/hoch2004/04hoch094.shtml

Published: August 10, 2005

© 2005 Birkenfeld et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

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Introduction: Ganglionic nicotinic acetylcholine receptor (AChR) antibodies have been identified in some patients with subacute autonomic failure. However, a causal relationship between ganglionic AChR antibodies and autonomic failure has not been convincingly demonstrated in humans.

Methods: We identified a 43 year-old men a >20 years history of progressive autonomic failure. His symptoms included profound orthostatic hypotension, anhidrosis, decreased saliva production, and constipation. The patient tested positive for AChR antibodies. He underwent large volume plasma exchanges. Autonomic cardiovascular testing and baroreflex testing with phenylephrine and nitroprusside were conducted before and one week after the plasma exchange. During this testing, arterial blood pressure was monitored invasively.

Results: AChR concentration was 0.30 nmol/L before and 0.06 nmol/L after four plasma exchanges. Before the plasma exchange, blood pressure decreased 78/46 mm Hg after one minute of standing leading to presyncope. After the plasma exchange, orthostatic hypotension was markedly improved (-21/-11mmHg after one minute). The patient stood >15 minutes without symptoms. Antibody concentrations were correlated with orthostatic hypotension (r2=.36, p=.006 for systolic and r2=.45, p=.001 for diastolic blood pressure). Norepinephrine concentrations increased from 0.2nmol/L before to 0.8nmol/L after treatment. Blood pressure responsiveness to i.v. phenylephrine and nitroprusside was markedly attenuated after plasma exchange, consistent with improved baroreflex buffering. The baroreflex slope increased from 0 msec/mmHg before to 5 msec/mmHg after treatment. The patient noted a major improvement in sweating, saliva and tear production, bladder emptying, and gastrointestinal symptoms.

Conclusion: Ganglionic AChR antibodies can cause severe autonomic dysfunction in humans. Removal of antibodies by plasma exchange may lead to major clinical improvements in these patients.