Medical Care

The treatment of autoimmune autonomic neuropathy (AAN) is based on anecdotal evidence. No data from large, controlled trials are available owing to the rarity of the disorder. The treatment of chronic pure autonomic failure syndromes is symptomatic only. Postural orthostatic tachycardia syndrome can be treated by using low doses of beta-blockers as patients are normally sensitive to their adverse effects.

Nonpharmacologic measures are useful for all patients with autonomic dysfunction.^[5]

Discontinue antihypertensive medications and other medications known to lower blood pressure, if feasible.
Increase fluid and salt intake.
Equipment aids may be helpful. These include tight support stockings, abdominal binders, or antigravity suits for symptomatic hypotension and bladder catheterization for urinary retention.
Dietary fiber and enemas may help improve bowel motility and decrease straining during defecation.
Patients with decreased sweating should limit their physical activity, particularly in hot weather. Sponging with water during activity may help prevent overheating.
Large meals may exacerbate hypotension and should be avoided.
Perform positional changes, such as standing up, slowly and gradually.
Elevate the head of the bed and avoid prolonged recumbency.

Immunomodulatory therapy has been used successfully to shorten the duration of symptoms and improve overall prognosis in acute and chronic pandysautonomia.^[11]

Cases in which clinical improvement began within a few days of intravenous immunoglobulin (IVIg) administration (2 g/kg body weight over 2-5 d), along with normalization of autonomic test parameters, have been reported.^{[12, 13]}
Presumably, IVIg has an immunomodulatory action, but the exact mechanism of its effect in this disorder is unclear.
In 1 series, 2 patients with acute pandysautonomia were treated with prednisone 60 mg/d for several months and reported subjective improvement. No quantitative follow-up data were obtained.
In a series of 3 patients with autoimmune autonomic ganglionopathy, patients treated with prednisone, mycophenolate mofetil, and plasmapheresis reported improvement when plasmapheresis or IVIg alone was not effective.^[14]
Other pharmacologic treatment options are directed toward symptomatic relief only (See Medication).^[5]

Activity

Symptoms limit activity. Precautions for falling should be taken in patients who have orthostatic hypotension. In those with decreased sweating, vigorous exercise should be limited, and patients should be advised to have spray bottles of water or wet sponges available during hot weather or during physical activity.

Medication

Brooks M. FDA Clears Droxidopa for Neurogenic Orthostatic Hypotension. Medscape Medical News. Available at http://www.medscape.com/viewarticle/820786. Accessed: February 24, 2014.
Klein CM, Vernino S, Lennon VA, et al. The spectrum of autoimmune autonomic neuropathies. Ann Neurol. 2003. 53:752-8. [QxMD MEDLINE Link].
Sandroni P, Vernino S, Klein CM, et al. Idiopathic autonomic neuropathy: comparison of cases seropositive and seronegative for ganglionic acetylcholine receptor antibody. Arch Neurol. 2004 Jan. 61(1):44-8. [QxMD MEDLINE Link].
Gilman S, Low PA, Quinn N, et al. Consensus statement on the diagnosis of multiple system atrophy. J Neurol Sci. 1999 Feb 1. 163(1):94-8. [QxMD MEDLINE Link].
Freeman R. Clinical practice. Neurogenic orthostatic hypotension. N Engl J Med. 2008 Feb 7. 358(6):615-24. [QxMD MEDLINE Link].
Yu X, Stavrakis S, Hill MA, Huang S, Reim S, Li H, et al. Autoantibody activation of beta-adrenergic and muscarinic receptors contributes to an "autoimmune" orthostatic hypotension. J Am Soc Hypertens. 2012 Jan-Feb. 6(1):40-7. [QxMD MEDLINE Link]. [Full Text].
Barclay L. Low B12 Linked to Orthostatic Tachycardia in Adolescents. Medscape Medical News. Dec 23 2013. Available at http://www.medscape.com/viewarticle/818279. Accessed: Jan 7 2014.
Oner T, Guven B, Tavli V, Mese T, Yilmazer MM, Demirpence S. Postural Orthostatic Tachycardia Syndrome (POTS) and Vitamin B12 Deficiency in Adolescents. Pediatrics. 2014 Jan. 133(1):e138-42. [QxMD MEDLINE Link].
Sithinamsuwan P, Orrawanhanothai P, Thithum K, Udommongkol C, Chairangsaris P, Chinvarun Y, et al. Orthostatic hypotension: a non-motor complication assessment in 82 patients with idiopathic Parkinson's disease in Phramongkutklao Hospital. J Med Assoc Thai. 2010 Nov. 93 Suppl 6:S93-9. [QxMD MEDLINE Link].
Sommer C, Lauria G. Skin biopsy in the management of peripheral neuropathy. Lancet Neurol. 2007 Jul. 6(7):632-42. [QxMD MEDLINE Link].
Schroeder C, Vernino S, Birkenfeld AL, et al. Plasma exchange for primary autoimmune autonomic failure. N Engl J Med. 2005 Oct 13. 353(15):1585-90. [QxMD MEDLINE Link].
Heafield MT, Gammage MD, Nightingale S, Williams AC. Idiopathic dysautonomia treated with intravenous gammaglobulin. Lancet. 1996 Jan 6. 347(8993):28-9. [QxMD MEDLINE Link].
Quan D, Rich MM, Bird SJ. Acute idiopathic dysautonomia: electrophysiology and response to intravenous immunoglobulin. Neurology. 2000 Feb 8. 54(3):770-1. [QxMD MEDLINE Link].
Gibbons C, Vernino S, Freeman R. Combined immunomodulation therapy in autoimmune autonomic ganglionopathy. Arch Neurol. Feb 2008. 65(2):213-217.
Bannister R, Mathias CJ, Polinsky R. Autonomic failure--A comparison between UK and US experience. Autonomic Failure: A Textbook of Clinical Disorders of the Autonomic Nervous System. New York, NY: Oxford University Press; 1988. 282-8.
Daniel SE. The neuropharmacology and neurochemistry of multiple system atrophy. Autonomic Failure: A Textbook of Disorders of the Autonomic Nervous System. New York, NY: Oxford University Press; 1992. 564-85.
Hoeldtke RD, Bryner KD, Hoeldtke ME, Hobbs G. Treatment of autonomic neuropathy, postural tachycardia and orthostatic syncope with octreotide LAR. Clin Auton Res. 2007 Dec. 17(6):334-40. [QxMD MEDLINE Link].
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Author

Mohini Gurme, MD Resident Physician, Department of Neurology, University of California, Davis, School of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Dianna Quan, MD Professor of Neurology, Director of Electromyography Laboratory, University of Colorado School of Medicine

Dianna Quan, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Neurological Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Association of Neuromuscular and Electrodiagnostic Medicine Received research grant from: Alnylam; Pfizer; Ionis; Cytokinetics; Momenta; Viela Bio; .

Bjorn E Oskarsson, MD Assistant Professor, Department of Neurology, University of California, Davis, School of Medicine

Bjorn E Oskarsson, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Flex Pharma Serve(d) as a speaker or a member of a speakers bureau for: Grifols Received research grant from: Neuraltus, Glaxo, Eisai, Cytokinetics, Genentech,.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Neil A Busis, MD Chief of Neurology and Director of Neurodiagnostic Laboratory, UPMC Shadyside; Clinical Professor of Neurology and Director of Community Neurology, Department of Neurology, University of Pittsburgh Physicians

Neil A Busis, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: American Academy of Neurology Serve(d) as a speaker or a member of a speakers bureau for: American Academy of Neurology Received income in an amount equal to or greater than $250 from: American Academy of Neurology.

Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani College of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bioserenity, Ceribell, Eisai, Jazz, LivaNova, Neurelis, Neuropace, SK life science, Sunovion, Takeda, UCB Serve(d) as a speaker or a member of a speakers bureau for: Aquestive, Bioserenity, Eisai, Jazz, LivaNova, Neurelis, SK life science, Stratus, Sunovion, UCB Received research grant from: Cerevel Therapeutics, Ovid Therapeutics, Neuropace, Greenwich Jazz, SK Life Science, Xenon Pharmaceuticals, UCB, Marinus, Neuroelectrics Corporation, Longboard, Janssen, Equilibre Biopharmaceuticals.

Additional Contributors

Christopher Luzzio, MD Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison School of Medicine and Public Health

Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Jeffrey Tam Sing, MD to the development and writing of this article.

Sections Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes
Overview
Presentation
DDx
Workup
Treatment
Medication
Follow-up
Questions & Answers
References

Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes Treatment & Management

Medical Care

Activity

References

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