Dysautonomia Explained

Dysautonomia
Synonyms:Autonomic failure, Autonomic dysfunction
Field:Neurology
Symptoms:Anhidrosis or hyperhidrosis, blurry vision, tunnel vision, orthostatic hypotension, constipation, diarrhea, dysphagia, bowel incontinence, urinary retention or urinary incontinence, dizziness, brain fog, exercise intolerance, tachycardia, vertigo, weakness and pruritus.
Causes:Inadequacy of sympathetic, or parasympathetic, components of autonomic nervous system
Risks:Alcoholism and Diabetes[1]
Diagnosis:Ambulatory Blood pressure, as well as EKG monitoring
Treatment:Symptomatic and supportive[2]

Dysautonomia, autonomic failure, or autonomic dysfunction is a condition in which the autonomic nervous system (ANS) does not work properly. This may affect the functioning of the heart, bladder, intestines, sweat glands, pupils, and blood vessels. Dysautonomia has many causes, not all of which may be classified as neuropathic. A number of conditions can feature dysautonomia, such as Parkinson's disease, multiple system atrophy, dementia with Lewy bodies, Ehlers–Danlos syndromes,[3] autoimmune autonomic ganglionopathy and autonomic neuropathy,[4] HIV/AIDS,[5] mitochondrial cytopathy,[6] pure autonomic failure, autism, and postural orthostatic tachycardia syndrome.

Diagnosis is made by functional testing of the ANS, focusing on the affected organ system. Investigations may be performed to identify underlying disease processes that may have led to the development of symptoms or autonomic neuropathy. Symptomatic treatment is available for many symptoms associated with dysautonomia, and some disease processes can be directly treated. Depending on the severity of the dysfunction, dysautonomia can range from being nearly symptomless and transient to disabling and/or life-threatening.[7]

Signs and symptoms

Dysautonomia, a complex set of conditions characterized by autonomic nervous system (ANS) dysfunction, manifests clinically with a diverse array of symptoms, of which postural orthostatic tachycardia syndrome (POTS) stands out as the most common.[8]

The symptoms of dysautonomia, which are numerous and vary widely for each person, are due to inefficient or unbalanced efferent signals sent via both systems. Symptoms in people with dysautonomia include:

Causes

Dysautonomia may be due to inherited or degenerative neurologic diseases (primary dysautonomia) or injury of the autonomic nervous system from an acquired disorder (secondary dysautonomia).[9] [10] Its most common causes include:In the sympathetic nervous system (SNS), predominant dysautonomia is common along with fibromyalgia, chronic fatigue syndrome, irritable bowel syndrome, and interstitial cystitis, raising the possibility that such dysautonomia could be their common clustering underlying pathogenesis.[11]

In addition to sometimes being a symptom of dysautonomia, anxiety can sometimes physically manifest symptoms resembling autonomic dysfunction.[12] [13] [14] A thorough investigation ruling out physiological causes is crucial, but in cases where relevant tests are performed and no causes are found or symptoms do not match any known disorders, a primary anxiety disorder is possible but should not be presumed.[15] For such patients, the anxiety sensitivity index may have better predictivity for anxiety disorders, while the Beck Anxiety Inventory may misleadingly suggest anxiety for patients with dysautonomia.[16]

Mitochondrial cytopathies can have autonomic dysfunction manifesting as orthostatic intolerance, sleep-related hypoventilation and arrhythmias.[17] [18]

Mechanism

The autonomic nervous system is a component of the peripheral nervous system and comprises two branches: the sympathetic nervous system (SNS) and the parasympathetic nervous system (PSNS). The SNS controls the more active responses, such as increasing heart rate and blood pressure. The PSNS slows down the heart rate and aids digestion, for example. Symptoms typically arise from abnormal responses of either the sympathetic or parasympathetic systems based on situation or environment.[19]

Diagnosis

Diagnosis of dysautonomia depends on the overall function of three autonomic functions—cardiovagal, adrenergic, and sudomotor. A diagnosis should at a minimum include measurements of blood pressure and heart rate while lying flat and after at least three minutes of standing. The best way to make a diagnosis includes a range of testing, notably an autonomic reflex screen, tilt table test, and testing of the sudomotor response (ESC, QSART or thermoregulatory sweat test).

Additional tests and examinations to diagnose dysautonomia include:Tests to elucidate the cause of dysautonomia can include:

Vegetative-vascular dystonia

Particularly in the Russian literature,[20] a subtype of dysautonomia that particularly affects the vascular system has been called vegetative-vascular dystonia.[21] The term "vegetative" reflects an older name for the autonomic nervous system: the vegetative nervous system.

A similar form of this disorder has been historically noticed in various wars, including the Crimean War and American Civil War, and among British troops who colonized India. This disorder was called "irritable heart syndrome" (Da Costa's syndrome) in 1871 by American physician Jacob DaCosta.[22]

Management

Treatment of dysautonomia can be difficult; since it is made up of many different symptoms, a combination of drug therapies is often required to manage individual symptomatic complaints. In the case of autoimmune neuropathy, treatment with immunomodulatory therapies is done. If diabetes mellitus is the cause, control of blood glucose is important.[9] Treatment can include proton-pump inhibitors and H2 receptor antagonists used for digestive symptoms such as acid reflux.[23]

To treat genitourinary autonomic neuropathy, medications may include sildenafil (a guanine monophosphate type-5 phosphodiesterase inhibitor). To treat hyperhidrosis, anticholinergic agents such as trihexyphenidyl or scopolamine can be used. Intracutaneous injection of botulinum toxin type A can also be used in some cases.[24]

Balloon angioplasty, a procedure called transvascular autonomic modulation, is specifically not approved in the United States to treat autonomic dysfunction.[25]

In contrast to orthostatic hypotension (OH) in which neurodegenerative diseases might underlie, postural orthostatic tachycardia syndrome (POTS) in which psychiatric diseases might underlie responds to psychiatric intervention/ medication, or shows spontaneous remission. [26] [27]

Prognosis

The prognosis of dysautonomia depends on several factors; people with chronic, progressive, generalized dysautonomia in the setting of central nervous system degeneration such as Parkinson's disease or multiple system atrophy generally have poorer long-term prognoses. Dysautonomia can be fatal due to pneumonia, acute respiratory failure, or sudden cardiopulmonary arrest.[28] Autonomic dysfunction symptoms such as orthostatic hypotension, gastroparesis, and gustatory sweating are more frequently identified in mortalities.[29]

See also

Further reading

Notes and References

  1. Web site: Dysautonomia Autonomic Nervous System Disorders MedlinePlus. NIH. 2 January 2018. en.
  2. Web site: Dysautonomia Information Page National Institute of Neurological Disorders and Stroke. www.ninds.nih.gov. 2 January 2018.
  3. Castori M, Voermans NC . Neurological manifestations of Ehlers-Danlos syndrome(s): A review . Iranian Journal of Neurology . 13 . 4 . 190–208 . October 2014 . 25632331 . 4300794 .
  4. Imamura M, Mukaino A, Takamatsu K, Tsuboi H, Higuchi O, Nakamura H, Abe S, Ando Y, Matsuo H, Nakamura T, Sumida T, Kawakami A, Nakane S . Ganglionic Acetylcholine Receptor Antibodies and Autonomic Dysfunction in Autoimmune Rheumatic Diseases . Int J Mol Sci . 21 . 4 . February 2020 . 1332 . 32079137 . 7073227 . 10.3390/ijms21041332 . Review. free .
  5. McIntosh RC . A meta-analysis of HIV and heart rate variability in the era of antiretroviral therapy . Clin Auton Res . 26 . 4 . 287–94 . August 2016 . 27395409 . 10.1007/s10286-016-0366-6 . 20256879 . Review.
  6. Kanjwal . Khalil . Karabin . Beverly . Kanjwal . Yousuf . Saeed . Bilal . Grubb . Blair P. . October 2010 . Autonomic dysfunction presenting as orthostatic intolerance in patients suffering from mitochondrial cytopathy . Clinical Cardiology . 33 . 10 . 626–629 . 10.1002/clc.20805 . 1932-8737 . 6653231 . 20960537.
  7. Iodice V, Kimpinski K, Vernino S, Sandroni P, Fealey RD, Low PA . Efficacy of immunotherapy in seropositive and seronegative putative autoimmune autonomic ganglionopathy . Neurology . 72 . 23 . 2002–8 . June 2009 . 19506222 . 2837591 . 10.1212/WNL.0b013e3181a92b52 . aagnihtreat .
  8. Peltier AC . Autonomic Dysfunction from Diagnosis to Treatment . Prim Care . 51 . 2 . 359–373 . June 2024 . 38692780 . 10.1016/j.pop.2024.02.006 .
  9. Web site: Autonomic Neuropathy Clinical Presentation: History, Physical, Causes. emedicine.medscape.com. 2016-02-21.
  10. Kirk KA, Shoykhet M, Jeong JH, Tyler-Kabara EC, Henderson MJ, Bell MJ, Fink EL . Dysautonomia after pediatric brain injury . Developmental Medicine and Child Neurology . 54 . 8 . 759–64 . August 2012 . 22712762 . 3393822 . 10.1111/j.1469-8749.2012.04322.x .
  11. Martínez-Martínez LA, Mora T, Vargas A, Fuentes-Iniestra M, Martínez-Lavín M . Sympathetic nervous system dysfunction in fibromyalgia, chronic fatigue syndrome, irritable bowel syndrome, and interstitial cystitis: a review of case-control studies . Journal of Clinical Rheumatology . 20 . 3 . 146–50 . April 2014 . 24662556 . 10.1097/RHU.0000000000000089 . 23799955 .
  12. Soliman K, Sturman S, Sarkar PK, Michael A . Postural orthostatic tachycardia syndrome (POTS): a diagnostic dilemma. . British Journal of Cardiology . 2010 . 17 . 1 . 36–9 .
  13. Book: Psychosomatic Medicine. Ackerman. Kurt. DiMartini. Andrea F. . 2015. Oxford University Press, Incorporated. 978-0-19-932931-1. en.
  14. Book: The Handbook of Adult Clinical Psychology: An Evidence Based Practice Approach. Carr. Alan. McNulty. Muireann . 2016-03-31. Routledge. 978-1-317-57614-3. en.
  15. Book: Psychiatry, 2 Volume Set . Tasman . Allan . Kay . Jerald . First . Michael B. . Lieberman . Jeffrey A. . Riba . Michelle . 2015-03-30. John Wiley & Sons. 978-1-118-84547-9. en.
  16. Raj V, Haman KL, Raj SR, Byrne D, Blakely RD, Biaggioni I, Robertson D, Shelton RC . Psychiatric profile and attention deficits in postural tachycardia syndrome . Journal of Neurology, Neurosurgery, and Psychiatry . 80 . 3 . 339–44 . March 2009 . 18977825 . 2758320 . 10.1136/jnnp.2008.144360 .
  17. Emanuel . Hina . Ahlstrom . Katie . Mitchell . Sarah . McBeth . Katrina . Yadav . Aravind . Oria . Carlos Flores . Da Costa . Candice . Stark . James M. . Mosquera . Ricardo A. . Jon . Cindy . 2021-04-01 . Cardiac arrhythmias associated with volume-assured pressure support mode in a patient with autonomic dysfunction and mitochondrial disease . Journal of Clinical Sleep Medicine . 17 . 4 . 853–857 . 10.5664/jcsm.9024 . 1550-9397 . 8020692 . 33231166.
  18. Parikh . Sumit . Gupta . Ajay . March 2013 . Autonomic dysfunction in epilepsy and mitochondrial diseases . Seminars in Pediatric Neurology . 20 . 1 . 31–34 . 10.1016/j.spen.2013.01.003 . 1558-0776 . 23465772.
  19. Web site: Autonomic Nervous System — National Library of Medicine. PubMed Health. 2016-02-21. National Center for Biotechnology Information.
  20. 10.1300/J092v07n03_02 . Vegetative-Vascular Dystonia and Osteoalgetic Syndrome or Chronic Fatigue Syndrome as a Characteristic After-Effect of Radioecological Disaster . 1999 . Loganovsky . Konstantin . Journal of Chronic Fatigue Syndrome . 7 . 3 . 3–16.
  21. Ivanova ES, Mukharliamov FI, Razumov AN, Uianaeva AI . [State-of-the-art corrective and diagnostic technologies in medical rehabilitation of patients with vegetative vascular dystonia] . Voprosy Kurortologii, Fizioterapii, I Lechebnoi Fizicheskoi Kultury . 1 . 4–7 . 2008 . 18376477 .
  22. Halstead. Megan. 2018-01-01. Postural Orthostatic Tachycardia Syndrome: An Analysis of Cross-Cultural Research, Historical Research, and Patient Narratives of the Diagnostic Experience. Senior Honors Theses & Projects.
  23. Web site: H2 Blockers. Reducing stomach acid with H2 Blockers. Patient. Patient. 2016-02-21. en-GB.
  24. Web site: Diabetic Autonomic Neuropathy.
  25. Web site: Safety Alerts for Human Medical Products — Balloon angioplasty devices to treat autonomic dysfunction: FDA Safety Communication — FDA concern over experimental procedures. fda.gov. 5 December 2020. en.
  26. Tsuchida T, Ishibashi Y, Inoue Y et al. . Treatment of long COVID complicated by postural orthostatic tachycardia syndrome-Case series research . J Gen Fam Med . 25 . 1 . 53–61 . 2023 . 10.1002/jgf2.670 . 38240001 . 10792321 .
  27. Stallkamp Tidd SJ, Nowacki AS, Singh T et al. . Comorbid anxiety is associated with more changes in the Management of Postural Orthostatic Tachycardia Syndrome . Gen Hosp Psychiatry . 87 . 1–6 . 2024 . 10.1016/j.genhosppsych.2024.01.003 . 38224642 . 266997580 .
  28. Web site: Dysautonomia . NINDS . 2012-04-03 . https://web.archive.org/web/20161202234006/http://www.ninds.nih.gov/disorders/dysautonomia/dysautonomia.htm . 2016-12-02 . dead .
  29. Vinik AI, Maser RE, Mitchell BD, Freeman R . Diabetic autonomic neuropathy . Diabetes Care . 26 . 5 . 1553–79 . May 2003 . 12716821 . 10.2337/diacare.26.5.1553 . free .

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