dysautonomia

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.{{Cite journal |vauthors=Peltier AC |date=June 2024 |title=Autonomic Dysfunction from Diagnosis to Treatment |journal=Prim Care |volume=51 |issue=2 |pages=359–373 |doi=10.1016/j.pop.2024.02.006 |pmid=38692780}}

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.{{Medical citation needed|date=February 2021}} Symptoms in people with dysautonomia include:

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• Anhydrosis or hyperhidrosis
• Blurry or double vision
• Bowel incontinence
• Brain fog
• Constipation
• Dizziness
• Difficulty swallowing
• Exercise intolerance
• Low blood pressure
• Orthostatic hypotension{{Cite web |title=Autonomic Neuropathy Clinical Presentation: History, Physical, Causes |url=http://emedicine.medscape.com/article/1173756-clinical |access-date=2016-02-21 |website=emedicine.medscape.com}}
• Syncope
• Tachycardia
• Tunnel vision
• Urinary incontinence or urinary retention
• Sleep apnea

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File:Vincristine3DanBS.gif

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).{{Cite journal |vauthors=Kirk KA, Shoykhet M, Jeong JH, Tyler-Kabara EC, Henderson MJ, Bell MJ, Fink EL |date=August 2012 |title=Dysautonomia after pediatric brain injury |journal=Developmental Medicine and Child Neurology |volume=54 |issue=8 |pages=759–64 |doi=10.1111/j.1469-8749.2012.04322.x |pmc=3393822 |pmid=22712762}} Its most common causes include:

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• Alcoholism{{Cite journal |last=Tateno |first=F |last2=Sakakibara |first2=R |last3=Aiba |first3=Y |last4=Ogata |first4=T |date=2020 |title=Alcoholism mimicking multiple system atrophy: a case report |journal=Clin Auton Res |volume=30 |issue=6 |pages=581–584 |doi=10.1007/s10286-020-00708-y |pmid=32607716 |s2cid=220260700}}
• Amyloidosis{{better source needed|date=February 2021}}{{better source needed|date=February 2021}}{{Cite web |title=Autonomic Neuropathy. Information about AN. Patient {{!}} Patient |url=http://patient.info/doctor/autonomic-neuropathy |access-date=2016-02-21 |website=Patient info}}
• Autoimmune disease, such as Sjögren's syndrome{{Cite journal |last=Davies |first=Kristen |last2=Ng |first2=Wan-Fai |date=2021 |title=Autonomic Nervous System Dysfunction in Primary Sjögren's Syndrome |journal=Frontiers in Immunology |volume=12 |doi=10.3389/fimmu.2021.702505 |pmc=8350514 |pmid=34381453 |doi-access=free}}{{Cite journal |last=Imrich |first=R. |last2=Alevizos |first2=I. |last3=Bebris |first3=L. |last4=Goldstein |first4=D. S. |last5=Holmes |first5=C. S. |last6=Illei |first6=G. G. |last7=Nikolov |first7=N. P. |date=2015 |title=Predominant Glandular Cholinergic Dysautonomia in Patients with Primary Sjögren's Syndrome |journal=Arthritis & Rheumatology |volume=67 |issue=5 |pages=1345–1352 |doi=10.1002/art.39044 |pmc=4414824 |pmid=25622919}}{{Cite web |title=Dysautonomia: Malfunctions in Your Body's Automatic Functions |url=https://my.clevelandclinic.org/health/diseases/6004-dysautonomia}}{{Cite web |date=26 October 2023 |title=Dysautonomia in Sjögren's |url=https://sjogrens.org/blog/2023/dysautonomia-in-sjogrens}} or systemic lupus erythematosus (lupus), and autoimmune autonomic ganglionopathy{{citation needed|date=September 2016}}
• Craniocervical instability
• Diabetes
• Eaton-Lambert syndrome{{Medical citation needed|date=February 2021}}
• Ehlers-Danlos syndrome{{Cite journal |vauthors=De Wandele I, Rombaut L, Leybaert L, Van de Borne P, De Backer T, Malfait F, De Paepe A, Calders P |date=August 2014 |title=Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome |url=https://biblio.ugent.be/publication/4224752/file/7204040 |journal=Seminars in Arthritis and Rheumatism |volume=44 |issue=1 |pages=93–100 |doi=10.1016/j.semarthrit.2013.12.006 |pmid=24507822}}
• Guillain-Barré syndrome{{Cite journal |vauthors=Zaeem Z, Siddiqi ZA, Douglas W, Zochodne DW |date=2019 |title=Autonomic involvement in Guillain-Barré syndrome: an update |journal=Clin Auton Res |volume=29 |issue=3 |pages=289–299 |doi=10.1007/s10286-018-0542-y |pmid=30019292 |s2cid=49868730}}{{Cite journal |vauthors=Sakakibara R, Uchiyama T, Kuwabara S, Mori M, Ito T, Yamamoto T, Awa Y, Yamaguchi C, Yuki N, Vernino S, Kishi M, Shirai K |date=2009 |title=Prevalence and mechanism of bladder dysfunction in Guillain-Barre Syndrome |journal=Neurourol Urodyn |volume=28 |issue=5 |pages=432–437 |doi=10.1002/nau.20663 |pmid=19260087 |s2cid=25617551}}
• HIV and AIDS{{MedlinePlusEncyclopedia|000776|Autonomic neuropathy}}
• Long COVID{{Cite journal |last=Paliwal |first=V. K. |last2=Garg |first2=R. K. |last3=Gupta |first3=A. |last4=Tejan |first4=N. |year=2020 |title=Neuromuscular presentations in patients with COVID-19 |journal=Neurological Sciences |volume=41 |issue=11 |pages=3039–3056 |doi=10.1007/s10072-020-04708-8 |pmc=7491599 |pmid=32935156}}{{Cite journal |vauthors=Astin R, Banerjee A, Hall CN |date=2023 |title=Long COVID: mechanisms, risk factors and recovery |journal=Experimental Physiology |volume=108 |issue=1 |pages=12–27 |doi=10.1113/EP090802 |pmc=10103775 |pmid=36412084}}
• Multiple sclerosis, meningitis-retention syndrome
• Paraneoplastic syndrome{{Cite web |title=Paraneoplastic syndromes of the nervous system |url=http://www.mayoclinic.org/diseases-conditions/paraneoplastic-syndromes/basics/definition/con-20028459 |access-date=13 September 2016 |website=Mayo Clinic |ref=mayo-pns}}
• Spinal cord injury or traumatic brain injuryAcob, Lori Mae Yvette. (2021). Autonomic Nervous System Dysfunction – Concussion Alliance. Retrieved 21 September 2021, from https://www.concussionalliance.org/autonomic-nervous-system-dysfunction
• Synucleinopathy, a group of neurodegenerative diseases including pure autonomic failure, Parkinson's disease, dementia with Lewy bodies and multiple system atrophy{{Cite journal |vauthors=Palma JA, Kaufmann H |date=March 2018 |title=Treatment of autonomic dysfunction in Parkinson disease and other synucleinopathies |journal=Mov Disord |type=Review |volume=33 |issue=3 |pages=372–90 |doi=10.1002/mds.27344 |pmc=5844369 |pmid=29508455}}
• Surgery or injury involving the nerves
• Toxicity (vincristine){{Cite journal |vauthors=Aiba Y, Sakakibara R, Tateno F, Shimizu N |date=May 2021 |title=Orthostatic hypotension possibly caused by vincristine |journal=Neurology and Clinical Neuroscience |volume=9 |issue=4 |pages=365–366 |doi=10.1111/ncn3.12517 |s2cid=235628396}}}}

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.{{Cite journal |vauthors=Martínez-Martínez LA, Mora T, Vargas A, Fuentes-Iniestra M, Martínez-Lavín M |date=April 2014 |title=Sympathetic nervous system dysfunction in fibromyalgia, chronic fatigue syndrome, irritable bowel syndrome, and interstitial cystitis: a review of case-control studies |url=https://zenodo.org/record/894676 |journal=Journal of Clinical Rheumatology |volume=20 |issue=3 |pages=146–50 |doi=10.1097/RHU.0000000000000089 |pmid=24662556 |s2cid=23799955}}

In addition to sometimes being a symptom of dysautonomia, anxiety can sometimes physically manifest symptoms resembling autonomic dysfunction.{{Cite journal |vauthors=Soliman K, Sturman S, Sarkar PK, Michael A |year=2010 |title=Postural orthostatic tachycardia syndrome (POTS): a diagnostic dilemma. |url=http://www.medscape.com/viewarticle/717904_5 |journal=British Journal of Cardiology |volume=17 |issue=1 |pages=36–9}}{{Cite book |last=Ackerman |first=Kurt |url=https://books.google.com/books?id=C6TKBwAAQBAJ&pg=PA60 |title=Psychosomatic Medicine |last2=DiMartini |first2=Andrea F. |date=2015 |publisher=Oxford University Press, Incorporated |isbn=978-0-19-932931-1 |language=en}}{{Cite book |last=Carr |first=Alan |url=https://books.google.com/books?id=liXeCwAAQBAJ&pg=PA440 |title=The Handbook of Adult Clinical Psychology: An Evidence Based Practice Approach |last2=McNulty |first2=Muireann |date=2016-03-31 |publisher=Routledge |isbn=978-1-317-57614-3 |language=en}} 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.{{Cite book |last=Tasman |first=Allan |url=https://books.google.com/books?id=YEkoBgAAQBAJ&pg=PA585 |title=Psychiatry, 2 Volume Set |last2=Kay |first2=Jerald |last3=First |first3=Michael B. |last4=Lieberman |first4=Jeffrey A. |last5=Riba |first5=Michelle |date=2015-03-30 |publisher=John Wiley & Sons |isbn=978-1-118-84547-9 |language=en}} 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.{{Cite journal |vauthors=Raj V, Haman KL, Raj SR, Byrne D, Blakely RD, Biaggioni I, Robertson D, Shelton RC |date=March 2009 |title=Psychiatric profile and attention deficits in postural tachycardia syndrome |journal=Journal of Neurology, Neurosurgery, and Psychiatry |volume=80 |issue=3 |pages=339–44 |doi=10.1136/jnnp.2008.144360 |pmc=2758320 |pmid=18977825}}

Mitochondrial cytopathies can have autonomic dysfunction manifesting as orthostatic intolerance, sleep-related hypoventilation, and arrhythmias.{{Cite journal |last=Emanuel |first=Hina |last2=Ahlstrom |first2=Katie |last3=Mitchell |first3=Sarah |last4=McBeth |first4=Katrina |last5=Yadav |first5=Aravind |last6=Oria |first6=Carlos Flores |last7=Da Costa |first7=Candice |last8=Stark |first8=James M. |last9=Mosquera |first9=Ricardo A. |last10=Jon |first10=Cindy |date=2021-04-01 |title=Cardiac arrhythmias associated with volume-assured pressure support mode in a patient with autonomic dysfunction and mitochondrial disease |journal=Journal of Clinical Sleep Medicine |volume=17 |issue=4 |pages=853–857 |doi=10.5664/jcsm.9024 |issn=1550-9397 |pmc=8020692 |pmid=33231166}}{{Cite journal |last=Parikh |first=Sumit |last2=Gupta |first2=Ajay |date=March 2013 |title=Autonomic dysfunction in epilepsy and mitochondrial diseases |url=https://pubmed.ncbi.nlm.nih.gov/23465772/ |journal=Seminars in Pediatric Neurology |volume=20 |issue=1 |pages=31–34 |doi=10.1016/j.spen.2013.01.003 |issn=1558-0776 |pmid=23465772}}

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.{{Cite web |title=Autonomic Nervous System — National Library of Medicine |url=https://www.ncbi.nlm.nih.gov/pubmedhealth/PMHT0025455/ |access-date=2016-02-21 |website=PubMed Health |publisher=National Center for Biotechnology Information}}

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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:

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• Ambulatory blood pressure and EKG monitoring{{better source needed|date=February 2021}}
• Cold pressor test
• Deep breathing
• Electrochemical skin conductance{{citation needed|date=October 2021}}
• Hyperventilation test
• Nerve biopsy for small fiber neuropathy
• Quantitative sudomotor axon reflex test (QSART)
• Testing for orthostatic intolerance
• Thermoregulatory sweat test
• Tilt table test{{Cite journal |vauthors=Mustafa HI, Fessel JP, Barwise J, Shannon JR, Raj SR, Diedrich A, Biaggioni I, Robertson D |date=January 2012 |title=Dysautonomia: perioperative implications |journal=Anesthesiology |volume=116 |issue=1 |pages=205–15 |doi=10.1097/ALN.0b013e31823db712 |pmc=3296831 |pmid=22143168}}
• Valsalva maneuver

}}

Tests to elucidate the cause of dysautonomia can include:

• Evaluation for acute (intermittent) porphyria
• Evaluation of cerebrospinal fluid by lumbar puncture for infectious/ inflammatory diseases
• Evaluation of nerve conduction study for autonomic neuropathy
• Evaluation of brain and spinal magnetic resonance imaging for myelopathy, stroke and multiple system atrophy
• Evaluation of MIBG myocardial scintigraphy and DaT scan for Parkinson's disease, dementia with Lewy bodies and pure autonomic failure

Particularly in the Russian literature,{{Cite journal |last=Loganovsky |first=Konstantin |year=1999 |title=Vegetative-Vascular Dystonia and Osteoalgetic Syndrome or Chronic Fatigue Syndrome as a Characteristic After-Effect of Radioecological Disaster |journal=Journal of Chronic Fatigue Syndrome |volume=7 |issue=3 |pages=3–16 |doi=10.1300/J092v07n03_02}} a subtype of dysautonomia that particularly affects the vascular system has been called vegetative-vascular dystonia.{{Cite journal |vauthors=Ivanova ES, Mukharliamov FI, Razumov AN, Uianaeva AI |year=2008 |title=[State-of-the-art corrective and diagnostic technologies in medical rehabilitation of patients with vegetative vascular dystonia] |journal=Voprosy Kurortologii, Fizioterapii, I Lechebnoi Fizicheskoi Kultury |issue=1 |pages=4–7 |pmid=18376477}} The term "vegetative" reflects an older name for the autonomic nervous system: the vegetative nervous system.{{citation needed|date=July 2017}}

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.{{Cite journal |last=Halstead |first=Megan |date=2018-01-01 |title=Postural Orthostatic Tachycardia Syndrome: An Analysis of Cross-Cultural Research, Historical Research, and Patient Narratives of the Diagnostic Experience |url=https://commons.emich.edu/honors/598 |journal=Senior Honors Theses & Projects}}

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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. Treatment can include proton-pump inhibitors and H₂ receptor antagonists used for digestive symptoms such as acid reflux.{{Cite web |title=H2 Blockers. Reducing stomach acid with H2 Blockers. {{!}} Patient |url=http://patient.info/health/h2-blockers |access-date=2016-02-21 |website=Patient |language=en-GB}}

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.{{Cite web |title=Diabetic Autonomic Neuropathy |url=http://www.medscape.com/viewarticle/473205_9}}

Balloon angioplasty, a procedure called transvascular autonomic modulation, is specifically not approved in the United States to treat autonomic dysfunction.{{Cite web |title=Safety Alerts for Human Medical Products — Balloon angioplasty devices to treat autonomic dysfunction: FDA Safety Communication — FDA concern over experimental procedures |url=https://www.fda.gov/medical-devices/safety-communications/fda-concern-over-experimental-procedures-use-balloon-angioplasty-devices-treat-autonomic-dysfunction |access-date=5 December 2020 |website=fda.gov |language=en}}

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.{{Cite web |title=Dysautonomia |url=http://www.ninds.nih.gov/disorders/dysautonomia/dysautonomia.htm |url-status=dead |archive-url=https://web.archive.org/web/20161202234006/http://www.ninds.nih.gov/disorders/dysautonomia/dysautonomia.htm |archive-date=2016-12-02 |access-date=2012-04-03 |publisher=NINDS}} Autonomic dysfunction symptoms such as orthostatic hypotension, gastroparesis, and gustatory sweating are more frequently identified in mortalities.{{Cite journal |vauthors=Vinik AI, Maser RE, Mitchell BD, Freeman R |date=May 2003 |title=Diabetic autonomic neuropathy |journal=Diabetes Care |volume=26 |issue=5 |pages=1553–79 |doi=10.2337/diacare.26.5.1553 |pmid=12716821 |doi-access=free}}

{{Portal|Medicine}}

• Autonomic neuropathy
• Dopamine beta hydroxylase deficiency
• Familial dysautonomia
• Reflex syncope
• Postural orthostatic tachycardia syndrome
• Orthostatic intolerance

{{Reflist}}

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• {{Cite book |last=Brading |first=Alison |title=The autonomic nervous system and its effectors |date=1999 |publisher=Blackwell Science |isbn=978-0-632-02624-1 |location=Oxford}}
• {{Cite book |last=Goldstein |first=David |url=https://neuroscience.nih.gov/publications/PrinciplesofAutonomicMedicine30.pdf |title=Principles of Autonomic Medicine |date=2016 |publisher=National Institute of Neurological Disorders and Stroke, National Institutes of Health |isbn=978-0-8247-0408-7 |edition=free online version |location=Bethesda, Maryland}}
• {{Cite book |last=Jänig |first=Wilfrid |title=Integrative action of the autonomic nervous system : neurobiology of homeostasis |date=2008 |publisher=Cambridge University Press |isbn=978-0-521-06754-6 |edition=Digitally printed version. |location=Cambridge}}
• {{Cite journal |vauthors=Lara A, Damasceno DD, Pires R, Gros R, Gomes ER, Gavioli M, Lima RF, Guimarães D, Lima P, Bueno CR, Vasconcelos A, Roman-Campos D, Menezes CA, Sirvente RA, Salemi VM, Mady C, Caron MG, Ferreira AJ, Brum PC, Resende RR, Cruz JS, Gomez MV, Prado VF, de Almeida AP, Prado MA, Guatimosim S |date=April 2010 |title=Dysautonomia due to reduced cholinergic neurotransmission causes cardiac remodeling and heart failure |journal=Molecular and Cellular Biology |volume=30 |issue=7 |pages=1746–56 |doi=10.1128/MCB.00996-09 |pmc=2838086 |pmid=20123977}}
• {{Cite book |last=Schiffer |first=Randolph B. |url=https://books.google.com/books?id=I2N9COt-4C0C&q=Dysautonomia%2520%2520cause&pg=PA288 |title=Neuropsychiatry |last2=Rao |first2=Stephen M. |last3=Fogel |first3=Barry S. |date=2003-01-01 |publisher=Lippincott Williams & Wilkins |isbn=978-0-7817-2655-9 |language=en}}

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{{Autonomic diseases}}

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Category:Autonomic nervous system

Category:Peripheral nervous system disorders