Medicine:Dysautonomia
Dysautonomia, autonomic failure, or autonomic dysfunction is a condition in which the autonomic nervous system (ANS) does not work properly. This condition 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.[1] A number of conditions can feature dysautonomia, such as Parkinson's disease, Long COVID, multiple system atrophy, dementia with Lewy bodies,[2] 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.[7]
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.[8]
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.[7]
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:
- Anhydrosis or hyperhidrosis[9]
- Blurry or double vision[9]
- Bowel incontinence[9]
- Brain fog[9]
- Constipation[10]
- Dizziness[10]
- Difficulty swallowing[11]
- Exercise intolerance[9]
- Low blood pressure[10]
- Orthostatic hypotension[9][7]
- Sleep apnea[10]
- Syncope[10]
- Tachycardia[1]
- Tunnel vision[10]
- Urinary incontinence or urinary retention[9]
Causes
Dysautonomia may be due to inherited or degenerative neurologic diseases (primary dysautonomia)[1] or injury of the autonomic nervous system from an acquired disorder (secondary dysautonomia).[9][12] Its most common causes include:
- Alcoholism[11][13]
- Amyloidosis[10] * Autoimmune disease, such as Sjögren's disease[14][15][16][17] or systemic lupus erythematosus (lupus), and autoimmune autonomic ganglionopathy * Craniocervical instability[11]
- Diabetes[11]
- Eaton-Lambert syndrome
- Ehlers-Danlos syndrome[18]
- Guillain-Barré syndrome[11][19][20]
- HIV and AIDS[11]
- Long COVID[21][22]
- Multiple sclerosis, meningitis-retention syndrome[11]
- Paraneoplastic syndrome[23]
- Spinal cord injury[11] or traumatic brain injury[24]
- Synucleinopathy, a group of neurodegenerative diseases including pure autonomic failure, Parkinson's disease, dementia with Lewy bodies and multiple system atrophy[2]
- Surgery or injury involving the nerves[11]
- Toxicity (vincristine)[25]
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.[26]
In addition to sometimes being a symptom of dysautonomia, anxiety can sometimes physically manifest symptoms resembling autonomic dysfunction.[27][28][29] 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.[30] 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.[31]
Mitochondrial cytopathies can have autonomic dysfunction manifesting as orthostatic intolerance, sleep-related hypoventilation, and arrhythmias.[6][32][33]
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, for example, slows down the heart rate and aids digestion. Symptoms typically arise from abnormal responses of either the sympathetic or parasympathetic systems based on situation or environment.[1][34][24]
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).[35]
Additional tests and examinations to diagnose dysautonomia include:
Tests to elucidate the cause of dysautonomia can include:
- Evaluation for acute (intermittent) porphyria[9]
- Evaluation of brain and spinal magnetic resonance imaging for myelopathy, stroke and multiple system atrophy
- Evaluation of cerebrospinal fluid by lumbar puncture[9] for infectious/ inflammatory diseases
- Evaluation of MIBG myocardial scintigraphy and DaT scan for Parkinson's disease, dementia with Lewy bodies and pure autonomic failure
- Evaluation of nerve conduction study for autonomic neuropathy
Vegetative-vascular dystonia and Da Costa's syndrome
 | It has been suggested that this section be split out into another page titled Vegetative-vascular dystonia. (Discuss) (February 2026) |
Particularly in the Soviet and post-Soviet medical literature,[36][37] a subtype of dysautonomia that particularly affects the vascular system has been called Vegetative-vascular dystonia.[38] The term "vegetative" reflects an older name for the autonomic nervous system: the vegetative nervous system. While rooted in earlier concepts of neurasthenia, the diagnosis was crystallized by G.F. Lang’s 'neurogenic theory' to describe a functional precursor to essential hypertension:[39] a stress-induced autonomic dysregulation that Soviet physicians observed en masse amidst the devastation of the war of annihilation on the Eastern Front.[36]
Despite official recognition by the medical institutions in the USSR and some other Warsaw-pact countries (and their successor countries), it has also been described as a form of culture-bound syndrome.[36]
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.[40]
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.[41]
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.[42]
Transvascular autonomic modulation, a procedure similar to balloon angioplasty, is not approved in the United States to treat autonomic dysfunction.[43]
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.[1] Autonomic dysfunction symptoms such as orthostatic hypotension, gastroparesis, and gustatory sweating are more frequently identified in mortalities.[44]
See also
- Autonomic neuropathy
- Dopamine beta hydroxylase deficiency
- Familial dysautonomia
- List of investigational orthostatic intolerance drugs
- Orthostatic intolerance
- Postural orthostatic tachycardia syndrome
- Reflex syncope
References
- ↑ 1.0 1.1 1.2 1.3 1.4 "Dysautonomia". NINDS. http://www.ninds.nih.gov/disorders/dysautonomia/dysautonomia.htm.
- ↑ 2.0 2.1 "Treatment of autonomic dysfunction in Parkinson disease and other synucleinopathies". Mov Disord 33 (3): 372–90. March 2018. doi:10.1002/mds.27344. PMID 29508455.
- ↑ "Neurological manifestations of Ehlers-Danlos syndrome(s): A review". Iranian Journal of Neurology 13 (4): 190–208. October 2014. PMID 25632331.
- ↑ "Ganglionic Acetylcholine Receptor Antibodies and Autonomic Dysfunction in Autoimmune Rheumatic Diseases". Int J Mol Sci 21 (4): 1332. February 2020. doi:10.3390/ijms21041332. PMID 32079137.
- ↑ "A meta-analysis of HIV and heart rate variability in the era of antiretroviral therapy". Clin Auton Res 26 (4): 287–94. August 2016. doi:10.1007/s10286-016-0366-6. PMID 27395409.
- ↑ 6.0 6.1 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. doi:10.1002/clc.20805. ISSN 1932-8737. PMID 20960537.
- ↑ 7.0 7.1 7.2 "Autonomic Dysfunction from Diagnosis to Treatment". Prim Care 51 (2): 359–373. June 2024. doi:10.1016/j.pop.2024.02.006. PMID 38692780.
- ↑ "Efficacy of immunotherapy in seropositive and seronegative putative autoimmune autonomic ganglionopathy". Neurology 72 (23): 2002–8. June 2009. doi:10.1212/WNL.0b013e3181a92b52. PMID 19506222.
- ↑ 9.00 9.01 9.02 9.03 9.04 9.05 9.06 9.07 9.08 9.09 9.10 9.11 "Autonomic Neuropathy Clinical Presentation: History, Physical, Causes". http://emedicine.medscape.com/article/1173756-clinical.
- ↑ 10.0 10.1 10.2 10.3 10.4 10.5 10.6 "Autonomic Neuropathy. Information about AN. Patient | Patient". http://patient.info/doctor/autonomic-neuropathy.
- ↑ 11.0 11.1 11.2 11.3 11.4 11.5 11.6 11.7 11.8 MedlinePlus Encyclopedia Autonomic neuropathy
- ↑ "Dysautonomia after pediatric brain injury". Developmental Medicine and Child Neurology 54 (8): 759–64. August 2012. doi:10.1111/j.1469-8749.2012.04322.x. PMID 22712762.
- ↑ Tateno, F; Sakakibara, R; Aiba, Y; Ogata, T (2020). "Alcoholism mimicking multiple system atrophy: a case report". Clin Auton Res 30 (6): 581–584. doi:10.1007/s10286-020-00708-y. PMID 32607716.
- ↑ Davies, Kristen; Ng, Wan-Fai (2021). "Autonomic Nervous System Dysfunction in Primary Sjögren's Syndrome". Frontiers in Immunology 12. doi:10.3389/fimmu.2021.702505. PMID 34381453.
- ↑ Imrich, R.; Alevizos, I.; Bebris, L.; Goldstein, D. S.; Holmes, C. S.; Illei, G. G.; Nikolov, N. P. (2015). "Predominant Glandular Cholinergic Dysautonomia in Patients with Primary Sjögren's Syndrome". Arthritis & Rheumatology 67 (5): 1345–1352. doi:10.1002/art.39044. PMID 25622919.
- ↑ "Dysautonomia: Malfunctions in Your Body's Automatic Functions". https://my.clevelandclinic.org/health/diseases/6004-dysautonomia.
- ↑ "Dysautonomia in Sjögren's". 26 October 2023. https://sjogrens.org/blog/2023/dysautonomia-in-sjogrens.
- ↑ "Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome". Seminars in Arthritis and Rheumatism 44 (1): 93–100. August 2014. doi:10.1016/j.semarthrit.2013.12.006. PMID 24507822. https://biblio.ugent.be/publication/4224752/file/7204040.
- ↑ "Autonomic involvement in Guillain-Barré syndrome: an update". Clin Auton Res 29 (3): 289–299. 2019. doi:10.1007/s10286-018-0542-y. PMID 30019292.
- ↑ "Prevalence and mechanism of bladder dysfunction in Guillain-Barre Syndrome". Neurourol Urodyn 28 (5): 432–437. 2009. doi:10.1002/nau.20663. PMID 19260087.
- ↑ Paliwal, V. K.; Garg, R. K.; Gupta, A.; Tejan, N. (2020). "Neuromuscular presentations in patients with COVID-19". Neurological Sciences 41 (11): 3039–3056. doi:10.1007/s10072-020-04708-8. PMID 32935156.
- ↑ "Long COVID: mechanisms, risk factors and recovery". Experimental Physiology 108 (1): 12–27. 2023. doi:10.1113/EP090802. PMID 36412084.
- ↑ "Paraneoplastic syndromes of the nervous system". http://www.mayoclinic.org/diseases-conditions/paraneoplastic-syndromes/basics/definition/con-20028459.
- ↑ 24.0 24.1 24.2 24.3 Acob, Lori Mae Yvette. (2021). Autonomic Nervous System Dysfunction – Concussion Alliance. Retrieved 21 September 2021, from https://www.concussionalliance.org/autonomic-nervous-system-dysfunction
- ↑ "Orthostatic hypotension possibly caused by vincristine". Neurology and Clinical Neuroscience 9 (4): 365–366. May 2021. doi:10.1111/ncn3.12517.
- ↑ "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. doi:10.1097/RHU.0000000000000089. PMID 24662556. https://zenodo.org/record/894676.
- ↑ "Postural orthostatic tachycardia syndrome (POTS): a diagnostic dilemma.". British Journal of Cardiology 17 (1): 36–9. 2010. http://www.medscape.com/viewarticle/717904_5.
- ↑ Ackerman, Kurt; DiMartini, Andrea F. (2015) (in en). Psychosomatic Medicine. Oxford University Press, Incorporated. ISBN 978-0-19-932931-1. https://books.google.com/books?id=C6TKBwAAQBAJ&pg=PA60.
- ↑ Carr, Alan; McNulty, Muireann (2016-03-31) (in en). The Handbook of Adult Clinical Psychology: An Evidence Based Practice Approach. Routledge. ISBN 978-1-317-57614-3. https://books.google.com/books?id=liXeCwAAQBAJ&pg=PA440.
- ↑ Tasman, Allan; Kay, Jerald; First, Michael B.; Lieberman, Jeffrey A.; Riba, Michelle (2015-03-30) (in en). Psychiatry, 2 Volume Set. John Wiley & Sons. ISBN 978-1-118-84547-9. https://books.google.com/books?id=YEkoBgAAQBAJ&pg=PA585.
- ↑ "Psychiatric profile and attention deficits in postural tachycardia syndrome". Journal of Neurology, Neurosurgery, and Psychiatry 80 (3): 339–44. March 2009. doi:10.1136/jnnp.2008.144360. PMID 18977825.
- ↑ Emanuel, Hina; Ahlstrom, Katie; Mitchell, Sarah; McBeth, Katrina; Yadav, Aravind; Oria, Carlos Flores; Da Costa, Candice; Stark, James M. et al. (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. doi:10.5664/jcsm.9024. ISSN 1550-9397. PMID 33231166.
- ↑ Parikh, Sumit; Gupta, Ajay (March 2013). "Autonomic dysfunction in epilepsy and mitochondrial diseases". Seminars in Pediatric Neurology 20 (1): 31–34. doi:10.1016/j.spen.2013.01.003. ISSN 1558-0776. PMID 23465772.
- ↑ "Autonomic Nervous System — National Library of Medicine". National Center for Biotechnology Information. https://www.ncbi.nlm.nih.gov/pubmedhealth/PMHT0025455/.
- ↑ 35.0 35.1 35.2 35.3 35.4 35.5 35.6 35.7 35.8 "Dysautonomia: perioperative implications". Anesthesiology 116 (1): 205–15. January 2012. doi:10.1097/ALN.0b013e31823db712. PMID 22143168.
- ↑ 36.0 36.1 36.2 Beliaeva, Anastasia (2019-04-03). "The case of vegetovascular dystonia: inventing the most common Soviet disease". Canadian Slavonic Papers 61 (2): 146–163. doi:10.1080/00085006.2018.1563452. ISSN 0008-5006. https://doi.org/10.1080/00085006.2018.1563452.
- ↑ Loganovsky, Konstantin (1999). "Vegetative-Vascular Dystonia and Osteoalgetic Syndrome or Chronic Fatigue Syndrome as a Characteristic After-Effect of Radioecological Disaster". Journal of Chronic Fatigue Syndrome 7 (3): 3–16. doi:10.1300/J092v07n03_02.
- ↑ "[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. PMID 18376477.
- ↑ Shlyakhto, E. V.; Tsyrlin, V. A.; Kuzmenko, N. V.; Pliss, M. G. (2021-10-09). "Neurophysiological justification of the G. F. Lang hypothesis about the occurrence of essential hypertension". "Arterial’naya Gipertenziya" ("Arterial Hypertension") 27 (5): 499–508. doi:10.18705/1607-419X-2021-27-5-499-508. ISSN 2411-8524. https://htn.almazovcentre.ru/jour/article/view/2133.
- ↑ 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. https://commons.emich.edu/honors/598.
- ↑ "H2 Blockers. Reducing stomach acid with H2 Blockers. | Patient" (in en-GB). http://patient.info/health/h2-blockers.
- ↑ "Diabetic Autonomic Neuropathy". http://www.medscape.com/viewarticle/473205_9.
- ↑ "Safety Alerts for Human Medical Products — Balloon angioplasty devices to treat autonomic dysfunction: FDA Safety Communication — FDA concern over experimental procedures" (in en). https://www.fda.gov/medical-devices/safety-communications/fda-concern-over-experimental-procedures-use-balloon-angioplasty-devices-treat-autonomic-dysfunction.
- ↑ "Diabetic autonomic neuropathy". Diabetes Care 26 (5): 1553–79. May 2003. doi:10.2337/diacare.26.5.1553. PMID 12716821.
Further reading
- Brading, Alison (1999). The autonomic nervous system and its effectors. Oxford: Blackwell Science. ISBN 978-0-632-02624-1.
- Goldstein, David (2016). Principles of Autonomic Medicine (free online version ed.). Bethesda, Maryland: National Institute of Neurological Disorders and Stroke, National Institutes of Health. ISBN 978-0-8247-0408-7. https://neuroscience.nih.gov/publications/PrinciplesofAutonomicMedicine30.pdf.
- Jänig, Wilfrid (2008). Integrative action of the autonomic nervous system : neurobiology of homeostasis (Digitally printed version. ed.). Cambridge: Cambridge University Press. ISBN 978-0-521-06754-6.
- "Dysautonomia due to reduced cholinergic neurotransmission causes cardiac remodeling and heart failure". Molecular and Cellular Biology 30 (7): 1746–56. April 2010. doi:10.1128/MCB.00996-09. PMID 20123977.
- Schiffer, Randolph B.; Rao, Stephen M.; Fogel, Barry S. (2003-01-01) (in en). Neuropsychiatry. Lippincott Williams & Wilkins. ISBN 978-0-7817-2655-9. https://books.google.com/books?id=I2N9COt-4C0C&q=Dysautonomia%2520%2520cause&pg=PA288.
External links
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