Medscape is available in 5 Language Editions – Choose your Edition here.


Idiopathic Orthostatic Hypotension and other Autonomic Failure Syndromes Clinical Presentation

  • Author: Mohini Gurme, MD; Chief Editor: Selim R Benbadis, MD  more...
Updated: Jul 13, 2016


Features of autonomic disturbance in any of these conditions may include orthostasis, nausea, constipation, urinary retention or incontinence, nocturia, impotence, heat intolerance, and dry mucous membranes. Less commonly, patients experience periods of apnea or inspiratory stridor. Postural orthostatic tachycardia syndrome (POTS) results in prominent excessive adrenergic symptoms, especially tachycardia.

  • Symptoms of decreased sympathetic function may include the following:
    • Orthostatic hypotension
    • Decreased sweating
    • Ejaculatory dysfunction
    • Ptosis associated with Horner syndrome
  • Symptoms of decreased parasympathetic function may include the following:
    • Constipation
    • Nausea
    • Urinary retention
    • Erectile dysfunction
  • Pure autonomic failure (PAF)
    • PAF is by definition not associated with CNS symptoms. Careful questioning is required to exclude symptoms of CNS dysfunction, such as gait disturbance or spasticity. Patients should also be questioned in detail about sensory loss or neuropathic pain, which may suggest autoimmune autonomic neuropathy (AAN).
    • In older literature, the terms PAF and idiopathic orthostatic hypotension were sometimes used interchangeably. Orthostatic hypotension is the most common complaint in this group of patients.
    • Abnormalities of urination, salivation, sweating, and defecation can occur, though these are less common in PAF than in AAN.
  • Autoimmune autonomic neuropathy
    • Patients with apparent PAF should be questioned carefully regarding dry mouth or dry eyes.
    • Such sicca symptoms may be associated with ganglionic AChR autoantibodies.
    • Mild sensory disturbances may be present and overshadowed by autonomic dysfunction.
  • Multiple system atrophy
    • MSA is a chronic, progressive disorder with mixed features of chronic autonomic dysfunction, parkinsonism, and ataxia.[9]
    • Autonomic dysfunction is a common finding in MSA and in the absence of pathological findings essential to the diagnosis.
    • A subset of patients with PAF may eventually develop MSA, but no clinical or diagnostic markers identify this group at the outset.
    • Depending on their clinical features, patients with MSA may be categorized as parkinsonian (MSAp) or cerebellar (MSAc) variants, depending on the most prominent symptoms and findings on physical examination.
  • Postural orthostatic tachycardia syndrome
    • POTS is a relatively benign disorder that is often self-limiting.
    • Patients may complain of dizziness, blurry vision, weakness, lightheadedness, and fatigue upon standing. Palpitations, tremulousness, and anxiety can also be seen.
    • Other associated symptoms include neurocognitive or sleep disorders, exercise intolerance, hyperpnea, dyspnea, nausea, abdominal pain, and sweating.


See the list below:

  • Pure autonomic failure
    • Cardiovascular manifestations include orthostatic hypotension with an inappropriate lack of compensatory increase in heart rate with standing. Orthostatic hypotension is defined as a decrease of at least 20 mm Hg in systolic blood pressure or at least 10 mm Hg in diastolic blood pressure within 3 minutes of standing.
    • Gastroparesis is common and is associated with nausea or constipation. The abdomen may be distended, and patients may have discomfort on palpation. An acute abdomen is unusual. Diarrhea may also occur, with or without fecal incontinence.
    • Urinary retention is seen frequently and may cause bladder distention. A distended bladder can be detected on examination by percussion or palpation. Bladder emptying may be incomplete with post-void residuals of 100 mL or more.
    • Decreased sweating manifests as heat or exercise intolerance. Patients may have noticeably warm and/or dry skin.
    • The eyes may be affected. Careful ophthalmologic examination may reveal ptosis, anisocoria, Horner syndrome, or tonic pupils
    • Failure of either erection or ejaculation is a common physical manifestation in males. Female sexual dysfunction has not been well studied in these disorders.
  • Autoimmune autonomic neuropathy: The overall physical findings are similar to those observed in PAF. Patients may have additional findings of sensory abnormalities, pain, or loss of deep tendon reflexes.
  • Multiple system atrophy: Autonomic manifestations are similar to those observed in AAN and PAF. However, additional neurologic features may be present.
    • Pyramidal or cerebellar abnormalities including weakness, ataxia, incoordination, and eye-movement abnormalities may precede the autonomic features by as long as 2 years.
    • Patients with the MSA parkinsonian variant have variable parkinsonian findings, including rigidity, bradykinesia, tremor, and truncal instability, that do not respond to levodopa.
    • Patients with the MSA cerebellar variant have evidence of cerebellar dysfunction that manifests as ataxia, dysmetria, dysdiadochokinesia, and incoordination. Eye-movement abnormalities are frequently present.
  • Postural orthostatic tachycardia syndrome: A greater than 30-bpm increase in heart rate on standing, without a clinically significant decrease in blood pressure, is diagnostic.
Contributor Information and Disclosures

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

Disclosure: Nothing to disclose.


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: Nothing to disclose.

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, American Association of Neuromuscular and Electrodiagnostic Medicine

Disclosure: Nothing to disclose.

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 Neurodagnostic 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: 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 Medical Association, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cyberonics; Eisai; Lundbeck; Sunovion; UCB; Upsher-Smith<br/>Serve(d) as a speaker or a member of a speakers bureau for: Cyberonics; Eisai; Glaxo Smith Kline; Lundbeck; Sunovion; UCB<br/>Received research grant from: Cyberonics; Lundbeck; Sepracor; Sunovion; UCB; Upsher-Smith.

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.


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.

  1. Brooks M. FDA Clears Droxidopa for Neurogenic Orthostatic Hypotension. Medscape Medical News. Available at Accessed: February 24, 2014.

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

  3. 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. [Medline].

  4. 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. [Medline].

  5. Freeman R. Clinical practice. Neurogenic orthostatic hypotension. N Engl J Med. 2008 Feb 7. 358(6):615-24. [Medline].

  6. 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. [Medline]. [Full Text].

  7. Barclay L. Low B12 Linked to Orthostatic Tachycardia in Adolescents. Medscape Medical News. Dec 23 2013. Available at Accessed: Jan 7 2014.

  8. 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. [Medline].

  9. 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. [Medline].

  10. Sommer C, Lauria G. Skin biopsy in the management of peripheral neuropathy. Lancet Neurol. 2007 Jul. 6(7):632-42. [Medline].

  11. 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. [Medline].

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

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

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

  15. 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.

  16. 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.

  17. 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. [Medline].

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

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

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

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

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

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

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

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

All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.