Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes Treatment & Management

  • Author: Mohini Gurme, MD; Chief Editor: Selim R Benbadis, MD   more...
 
Updated: Feb 3, 2012
 

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.[7]
    • 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.[8]
    • 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.[9, 10]
    • 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.[11]
    • Other pharmacologic treatment options are directed toward symptomatic relief only (See Medication).[7]
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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.

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Contributor Information and Disclosures
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  Associate Professor of Neurology, Director, 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, and Phi Beta Kappa

Disclosure: e-medicine Honoraria Other

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

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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

Disclosure: Medscape Salary Employment

Neil A Busis, MD  Chief, Division of Neurology, Department of Medicine, Head, Clinical Neurophysiology Laboratory, University of Pittsburgh Medical Center-Shadyside

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

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, Tampa General Hospital, University of South Florida 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, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Honoraria Speaking, consulting

Additional Contributors

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

References

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  2. 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. Jan-Feb 2012;6(1):40-7. [Medline]. [Full Text].

  3. Klein CM, Vernino S, Lennon VA, et al. The spectrum of autoimmune autonomic neuropathies. Ann Neurol. 2003;53:752-8. [Medline].

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  8. Schroeder C, Vernino S, Birkenfeld AL, et al. Plasma exchange for primary autoimmune autonomic failure. N Engl J Med. Oct 13 2005;353(15):1585-90. [Medline].

  9. Heafield MT, Gammage MD, Nightingale S, Williams AC. Idiopathic dysautonomia treated with intravenous gammaglobulin. Lancet. Jan 6 1996;347(8993):28-9. [Medline].

  10. Quan D, Rich MM, Bird SJ. Acute idiopathic dysautonomia: electrophysiology and response to intravenous immunoglobulin. Neurology. Feb 8 2000;54(3):770-1. [Medline].

  11. Gibbons C, Vernino S, Freeman R. Combined immunomodulation therapy in autoimmune autonomic ganglionopathy. Arch Neurol. Feb 2008;65(2):213-217.

  12. Bannister R, Mathias CJ, Polinsky R. Autonomic failure--A comparison between UK and US experience. In: Autonomic Failure: A Textbook of Clinical Disorders of the Autonomic Nervous System. New York, NY: Oxford University Press; 1988:282-8.

  13. Daniel SE. The neuropharmacology and neurochemistry of multiple system atrophy. In: Autonomic Failure: A Textbook of Disorders of the Autonomic Nervous System. New York, NY: Oxford University Press; 1992:564-85.

  14. Hoeldtke RD, Bryner KD, Hoeldtke ME, Hobbs G. Treatment of autonomic neuropathy, postural tachycardia and orthostatic syncope with octreotide LAR. Clin Auton Res. Dec 2007;17(6):334-40. [Medline].

  15. Kaufmann H. Consensus statement on the definition of orthostatic hypotension, pure autonomic failure and multiple system atrophy. Clin Auton Res. Apr 1996;6(2):125-6. [Medline].

  16. Mathias CJ. Disorders of the autonomic nervous system. In: Neurology in Clinical Practice. Boston, Mass: Butterworth-Heinemann; 1996:1953-81.

  17. Matthews MR. Autonomic ganglia in multiple system atrophy and pure autonomic failure. In: Autonomic Failure: A Textbook of Disorders of the Autonomic Nervous System. New York, NY: Oxford University Press; 1992:593-621.

  18. Quinn NP, Wenning G, Marsden CD. The Shy-Drager syndrome. What did Shy and Drager really describe?. Arch Neurol. Jul 1995;52(7):656-7. [Medline].

  19. Smit AA, Vermeulen M, Koelman JH, Wieling W. Unusual recovery from acute panautonomic neuropathy after immunoglobulin therapy. Mayo Clin Proc. Apr 1997;72(4):333-5. [Medline].

  20. Suarez GA, Fealey RD, Camilleri M, Low PA. Idiopathic autonomic neuropathy: clinical, neurophysiologic, and follow-up studies on 27 patients. Neurology. Sep 1994;44(9):1675-82. [Medline].

  21. van Ingelghem E, van Zandijcke M, Lammens M. Pure autonomic failure: a new case with clinical, biochemical, and necropsy data. J Neurol Neurosurg Psychiatry. Jun 1994;57(6):745-7. [Medline].

  22. Vernino S, Freeman R. Peripheral Autonomic Neuropathies. Continuum Lifelong Learning Neurol. Dec 2007;13(6):89-110.

 
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