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'''Dysautonomia''' describes several different medical disorders of the [[autonomic nervous system]] that cause disturbances in all or some autonomic functions.<ref name="mw">{{Cite web | website=Merrian-Webster Medical Dictionary|access-date=2021-02-20 | title = Definition of DYSAUTONOMIA| url = https://www.merriam-webster.com/dictionary/dysautonomia}}</ref> Dysautonomia can be caused by other disease (e.g., diabetes), or by injury or poisoning/toxins, by genetics, or may not have a known cause.<ref name="mw"/><ref name="what-is"/> People with dysautonomia can have problems regulating autonomic functions, including heart rate, [[blood pressure]], [[body temperature]], and digestion. Thus, dysautonomia can present with various symptoms, such as lightheadedness, [[fainting]], unstable blood pressure, and [[orthostatic intolerance]].<ref name="what-is">{{Cite web | url = http://www.dysautonomiainternational.org/page.php?ID=34 | title = What is dysautonomia International|website=Dysautonomia International|access-date=2018-12-12}}</ref> == Types of dysautonomia == More than 70 million people have dysautonomia. There are various types of dysautonomia, each presenting with different underlying mechanisms as well as some overlapping mechanisms. Forms of dysautonomia include: * '''[[Postural orthostatic tachycardia syndrome|Postural orthostatic tachycardia syndrome (POTS)]]''', a form of orthostatic intolerance that can cause lightheadedness, fainting, tachycardia, chest pains, exercise intolerance, temperature sensitivity, and more. * '''[[Neurocardiogenic syncope]]''' is the most common form of dysautonomia, with many individuals having a mild case (fainting once or twice in their lifetime). Severe cases can result in fainting several times per day. * '''[[Multiple system atrophy|Multiple system atrophy (MSA)]]''', a fatal and rare form of dysautonomia, affects 350,000 people worldwide. It is a neurodegenerative disorder occurring in adults over the age of 40. MSA progresses rapidly; patients usually become bedridden within 2 years of diagnosis. * '''[[Familial dysautonomia]] (FD)''' is a rare genetic disorder in persons of Ashkenazi Jewish descent. It is a very serious condition, consisting of abnormal development, progressive degeneration of the sensory and autonomic nervous systems, and is inevitably fatal. FD is caused by a point mutation in the ''IKBAP'' gene, resulting in the severe reduction of the IKAP protein, which is involved in many intracellular processes.<ref name="Dragatsis2016">{{Cite journal | last = Dragatsis | first = Ioannis | last2 = Dietrich | first2 = Paula | last3 = Dragatsis | first3 = Ioannis | last4 = Dietrich | first4 = Paula | date = Dec 2016 | title = Familial Dysautonomia: Mechanisms and Models |url =http://www.scielo.br/scielo.php?script=sci_abstract&pid=S1415-47572016000400497&lng=en&nrm=iso&tlng=en|journal=Genetics and Molecular Biology|volume=39|issue=4 | pages = 497–514|doi=10.1590/1678-4685-gmb-2015-0335|issn=1415-4757}}</ref> * '''[[Autonomic dysreflexia]] (AD)''' is a form of dysautonomia associated with spinal cord injuries, affecting the communication of the autonomic nervous system. * '''[[Baroreflex failure]]'''; the baroreflex is the body’s homeostatic mechanism for maintaining blood pressure. If the baroreceptor or its system fails, patients experience inappropriate blood pressure. * '''[[Cerebral salt wasting syndrome]]''' is a condition in response to physical injury or the presence of tumors in the brain, presenting as hyponatremia (excessive sodium secretion from the kidney) and dehydration. * '''[[Diabetic autonomic neuropathy]]''' is a very common form of dysautonomia, affecting 69 million people worldwide (20% of diabetics). It is a serious complication of diabetes, associated with an increased risk of cardiovascular mortality. * '''[[Panayiotopoulos syndrome]]''', also known as autonomic epilepsy is a childhood-specific seizure disorder consisting of seizures with predominantly autonomic symptoms (without convulsing).<ref name="Covanis2006">{{Cite journal | last = Covanis | first = Athanasios | date = 2006-10-01 | title = Panayiotopoulos Syndrome: A Benign Childhood Autonomic Epilepsy Frequently Imitating Encephalitis, Syncope, Migraine, Sleep Disorder, or Gastroenteritis |url =http://pediatrics.aappublications.org/content/118/4/e1237|journal=Pediatrics|language=en|volume=118|issue=4| pages = e1237–e1243|doi=10.1542/peds.2006-0623|issn=1098-4275|pmid=16950946}}</ref> * '''[[Reflex sympathetic dystrophy]]''' is a an extremely painful neurological condition that is sometimes considered a form of dysautonomia. Dysautonomia can occur independently or secondary to other medical conditions, such as diabetes, [[Multiple sclerosis|MS]], rheumatoid arthritis, and [[Systemic lupus erythematosus|lupus]].<ref name="what-is" /> == Comorbidities/causes of dysautonomia == Comorbidities and causes of dysautonomia include:<ref name="what-is" /> * [[Amyloidosis]] ** A group of rare disorders caused by the accumulation of amyloid protein in various tissues throughout the body. If the amyloid accumulation occurs in the heart or on peripheral nerves, it can cause dysautonomia. * [[Celiac|Celiac disease]] * [[Charcot-Marie-Tooth disease]] * [[Chiari malformation]] * Chronic inflammatory demyelinating polyneuropathy * [[Inflammatory bowel disease]] (such as Crohn’s and ulcerative colitis)<ref name="Lindgren1991">{{Cite journal | last = Lindgren | first = S. | last2 = Lilja | first2 = B. | last3 = Rosén | first3 = I. | last4 = Sundkvist | first4 = G. | date = Apr 1991 | title = Disturbed autonomic nerve function in patients with Crohn's disease | url =https://www.ncbi.nlm.nih.gov/pubmed/2034989|journal=Scandinavian Journal of Gastroenterology|volume=26|issue=4 | pages = 361–366|issn=0036-5521|pmid=2034989}}</ref><ref name="Lindgren1993">{{Cite journal | last = Lindgren | first = S. | last2 = Stewenius | first2 = J. | last3 = Sjölund | first3 = K. | last4 = Lilja | first4 = B. | last5 = Sundkvist | first5 = G. | date = Jul 1993 | title = Autonomic vagal nerve dysfunction in patients with ulcerative colitis |url =https://www.ncbi.nlm.nih.gov/pubmed/8362220|journal=Scandinavian Journal of Gastroenterology|volume=28|issue=7 | pages = 638–642|issn=0036-5521|pmid=8362220}}</ref> * [[Deconditioning]] ** The physical change in the way the body functions due to a decrease in activity. The most common cause of deconditioning is bed rest. Due to debilitating symptoms, patients with various chronic illnesses may experience deconditioning, which can exacerbate symptoms. It is important to take steps to prevent and/or reverse deconditioning. <ref name="Dyckman2012">{{Cite journal | last = Dyckman | first = Damian J. | last2 = Sauder | first2 = Charity L. | last3 = Ray | first3 = Chester A. | date = 2012-01-01 | title = Effects of short-term and prolonged bed rest on the vestibulosympathetic reflex| url = https://www.ncbi.nlm.nih.gov/pubmed/22021328|journal=American Journal of Physiology. Heart and Circulatory Physiology|volume=302|issue=1| pages=H368–374|doi=10.1152/ajpheart.00193.2011|issn=1522-1539|pmc=3334249|pmid=22021328}}</ref> * [[Diabetes]]<ref name="Vinik2013">{{Cite journal | last = Vinik | first = Aaron I | last2 = Erbas | first2 = Tomris | last3 = Casellini | first3 = Carolina M | date = 2013-01-29 | title = Diabetic cardiac autonomic neuropathy, inflammation and cardiovascular disease | url =https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3580884/|journal=Journal of Diabetes Investigation|volume=4|issue=1 | pages = 4–18|doi=10.1111/jdi.12042|issn=2040-1116|pmc=3580884|pmid=23550085}}</ref> * [[Ehlers-Danlos syndrome]]<ref name="Rowe1999">{{Cite journal | date = 1999-10-01 | title = Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome | first = Peter C. | last = Rowe | authorlink = Peter Rowe | first2 = Diana F. | last2 = Barron | first3 = Hugh | last3 = Calkins | first4 = Irene H. | last4 = Maumenee | first5 = Patrick Y. | last5 = Tong | first6 = Michael T. | last6 = Geraghty| url = https://www.sciencedirect.com/science/article/pii/S0022347699701733|journal=The Journal of Pediatrics|language=en|volume=135|issue=4 | pages = 494–499|doi=10.1016/S0022-3476(99)70173-3|issn=0022-3476}}</ref> * [[Mast cell activation syndrome|Mast cell disorders]] * Mitochondrial diseases<ref name="Kanjwal2010">{{Cite journal | last = Kanjwal | first = Khalil | last2 = Karabin | first2 = Beverly | last3 = Kanjwal | first3 = Yousuf | last4 = Saeed | first4 = Bilal | last5 = Grubb | first5 = Blair P. | date = Oct 2010 | title = Autonomic dysfunction presenting as orthostatic intolerance in patients suffering from mitochondrial cytopathy| url = https://www.ncbi.nlm.nih.gov/pubmed/20960537|journal=Clinical Cardiology|volume=33|issue=10 | pages = 626–629|doi=10.1002/clc.20805|issn=1932-8737|pmid=20960537}}</ref> * Paraneoplastic syndrome<ref name="Koike2011">{{Cite journal | last = Koike | first = Haruki | last2 = Tanaka | first2 = Fumiaki | last3 = Sobue | first3 = Gen | date = Oct 2011 | title = Paraneoplastic neuropathy: wide-ranging clinicopathological manifestations |url =https://www.ncbi.nlm.nih.gov/pubmed/21799410|journal=Current Opinion in Neurology|volume=24|issue=5 | pages = 504–510|doi=10.1097/WCO.0b013e32834a87b7|issn=1473-6551|pmid=21799410}}</ref> * [[Parkinson's disease]]<ref name="Goldstein2014">{{Cite journal | last = Goldstein | first = David S | date = Apr 2014 | title = Dysautonomia in Parkinson’s disease: neurocardiological abnormalities |url =https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4222515/|journal=Comprehensive Physiology|volume=4|issue=2 | pages = 805–826|doi=10.1002/cphy.c130026|issn=2040-4603|pmc=4222515|pmid=24715569}}</ref> * [[Sarcoidosis]]<ref name="Bakkers2010">{{Cite journal | last = Bakkers | first = M. | last2 = Faber | first2 = C.G. | last3 = Drent | first3 = M. | last4 = Hermans | first4 = M.C.E. | last5 = van Nes | first5 = S.I. | last6 = Lauria | first6 = G. | last7 = De Baets | first7 = M. | last8 = Merkies | first8 = I. S.J. | date = Dec 2010 | title = Pain and autonomic dysfunction in patients with sarcoidosis and small fibre neuropathy| url = https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2993892/|journal=Journal of Neurology|volume=257|issue=12 | pages = 2086–2090|doi=10.1007/s00415-010-5664-7|issn=0340-5354|pmc=2993892|pmid=20644950}}</ref> * [[Sjögren's syndrome]]<ref name="Ng2012">{{Cite journal | last = Ng | first = W.-F. | last2 = Stangroom | first2 = A.J. | last3 = Davidson | first3 = A. | last4 = Wilton | first4 = K. | last5 = Mitchell | first5 = S. | last6 = Newton | first6 = J.L. | date = Dec 2012 | title = Primary Sjogrens syndrome is associated with impaired autonomic response to orthostasis and sympathetic failure | url =https://www.ncbi.nlm.nih.gov/pubmed/22976617|journal=QJM: monthly journal of the Association of Physicians|volume=105|issue=12 | pages = 1191–1199|doi=10.1093/qjmed/hcs172|issn=1460-2393|pmc=3508582|pmid=22976617}}</ref> * Physical [[trauma]] or surgery<ref name="Kanjwal2010-TBI">{{Cite journal | last = Kanjwal | first = Khalil | last2 = Karabin | first2 = Beverly | last3 = Kanjwal | first3 = Yousuf | last4 = Grubb | first4 = Blair P. | date = 2010 | title=Autonomic dysfunction presenting as postural tachycardia syndrome following traumatic brain injury| url = https://www.ncbi.nlm.nih.gov/pubmed/20865679|journal=Cardiology Journal|volume=17|issue=5 | pages = 482–487|issn=1897-5593|pmid=20865679}}</ref><ref name="Billakanty2008">{{Cite journal | last = Billakanty | first = Sreedhar R. | last2 = Kligman | first2 = MarkD. | last3 = Kanjwal | first3 = Yousuf M. | last4 = Kosinski | first4 = Daniel J. | last5 = Maly | first5 = George T. | last6 = Karabin | first6 = Beverly | last7 = Grubb | first7 = Blair P. | date = Jul 2008 | title = New-onset orthostatic intolerance following bariatric surgery| url = https://www.ncbi.nlm.nih.gov/pubmed/18684286|journal=Pacing and clinical electrophysiology: PACE|volume=31|issue=7 | pages = 884–888|doi=10.1111/j.1540-8159.2008.01103.x|issn=1540-8159|pmid=18684286}}</ref> * [[Vitamin]] deficiencies * Patients have also reported dysautonomia caused by [[Viral infection|viruses]] == Treatment == There are currently no cures for dysautonomia, as it is an umbrella term that covers many conditions related to the dysfunction of the autonomic system. Therefore, there are treatments that are used to improve quality of life by treating specific symptoms, or treating the underlying disease that is causing the dysautonomia. Further treatments are used to improve quality of life.<ref name="what-is"/> == Myalgic Encephalomyelitis == [[File:Dysautonomia1.png|thumb|Symptoms of autonomic dysfunction are correlated with severity of fatigue in CFS patients.<ref name="Jones2007"/>]] Many people with [[myalgic encephalomyelitis]] (ME) experience [[autonomic dysfunction]]. Symptoms of autonomic dysfunction in ME include altered blood pressure, heart rate, digestion, body temperature, [[exercise]] intolerance, and [[Nervous system|neurological]] correlates with [[autonomic nervous system]] (ANS) dysfunction (for a complete list, see our [[Autonomic nervous system|Autonomic Nervous System page]]). Some researchers suggest that dysautonomia is central to the pathophysiology of ME.<ref name="Naschitz2004">{{Cite journal | date = Feb 2004 | first = Jochanan E | last = Naschitz | first2 = Daniel | last2 = Yeshurun | first3 = Itzhak | last3 = Rosner | title = Dysautonomia in chronic fatigue syndrome: facts, hypotheses, implications |url =https://www.sciencedirect.com/science/article/pii/S0306987703003311|journal=Medical Hypotheses|language=en|volume=62|issue=2 | pages = 203–206|doi=10.1016/S0306-9877(03)00331-1|issn=0306-9877}}</ref> Various studies have investigated the efficacy of treating ME patients by targeting autonomic symptoms without significant success.<ref name="Straus2001">{{Cite journal | last = Straus | first = Stephen E. | last2 = Wolff | first2 = Mark | last3 = Lucas | first3 = Katherine E. | last4 = Snader | first4 = Sally | last5 = Hohman | first5 = Patricia | last6 = Soto | first6 = Norberto | last7 = Cuccherini | first7 = Brenda A. | last8 = Sharma | first8 = Geetika | last9 = Anand | first9 = Ravinder | date = 2001-01-03 | title = Fludrocortisone Acetate to Treat Neurally Mediated Hypotension in Chronic Fatigue Syndrome: A Randomized Controlled Trial| url = https://jamanetwork.com/journals/jama/fullarticle/193426|journal=JAMA|language=en|volume=285|issue=1 | pages = 52–59|doi=10.1001/jama.285.1.52|issn=0098-7484}}</ref> === Studies on Dysautonomia and ME === One study (Orjatsalo et al. 2018) has looked at autonomic cardiac function in different sleep stages of people with [[chronic fatigue syndrome]] (CFS). They found that people with CFS had higher [[nocturnal systolic]] and mean BP and lower heart rate than controls, suggesting a [[nocturnal dysfunction]] of the [[cardiac]] ANS in CFS. This means that patients have a lower parasympathetic tone and higher sympathetic tone during sleep.<ref name="Orjatsalo2018">{{Cite journal | last = Orjatsalo | first = Maija | last2 = Alakuijala | first2 = Anniina | last3 = Partinen | first3 = Markku | date = 2018-02-15 | title = Autonomic Nervous System Functioning Related to Nocturnal Sleep in Patients With Chronic Fatigue Syndrome Compared to Tired Controls |url =https://www.ncbi.nlm.nih.gov/pubmed/29246267|journal=Journal of clinical sleep medicine: JCSM: official publication of the American Academy of Sleep Medicine|volume=14|issue=2 | pages = 163–171|doi=10.5664/jcsm.6924|issn=1550-9397|pmc=5786834|pmid=29246267}}</ref> [[File:Dysautonomia2.png|thumb|Difference in total autonomic dysfunction (depicted by COMPASS score) between healthy controls (left), CFS patients (middle), and patients with primary biliary cirrhosis (PBC, a condition in which there is strong evidence of a causal link between fatigue and autonomic dysfunction; right). COMPASS scores were significantly higher in the CFS group.<ref name="Jones2007">{{Cite journal | last = Jones | first =D. E.J. | last2 = Shin | first2 = J. | last3 = Seth | first3 = A. | last4 = Sutcliffe | first4 = K. | last5 = Okonkwo | first5 = O. | last6 = Newton | first6 = J.L. | date = 2007-08-01 | title = Symptoms of autonomic dysfunction in chronic fatigue syndrome | url =https://academic.oup.com/qjmed/article/100/8/519/1521818|journal=QJM: An International Journal of Medicine|language=en|volume=100|issue=8 | pages = 519–526|doi=10.1093/qjmed/hcm057|issn=1460-2725}}</ref>]] Another study ([[Kunihisa Miwa|Miwa]] 2016) investigated POTS in patients with both ME and orthostatic intolerance. They hypothesized that the characteristics of sympathetic activation may differ between these patients’ “good days” and “bad days”. Patients were characterized with an active standing test and an [[echocardiography]]. Their results indicated that ME patients may experience exaggerated activation of the sympathetic nervous system. They suggest that sympathetic activation in ME is impaired and is associated with preload failure.<ref name="Miwa2016">{{Cite journal | last = Miwa | first = Kunihisa | authorlink = Kunihisa Miwa | date = Sep 2016 | title = Variability of postural orthostatic tachycardia in patients with myalgic encephalomyelitis and orthostatic intolerance | url =https://www.ncbi.nlm.nih.gov/pubmed/26374335|journal=Heart and Vessels|volume=31|issue=9 | pages = 1522–1528|doi=10.1007/s00380-015-0744-3|issn=1615-2573|pmid=26374335|quote=|via=}}</ref><ref name="Malfait2016"/> [[Julia Newton|Newton]] et al. aimed to determine the prevalence of autonomic dysfunction in CFS using the Composite Autonomic Symptom Scale (COMPASS) and the [[Fatigue Impact Scale]] (FIS). The study included a healthy control group and a control group of patients with primary biliary cirrhosis (PBC; where 60% of patients experience significant fatigue and some experience autonomic dysfunction). Their results indicated that symptoms of autonomic dysfunction were strongly associated with the presence of CFS or PBC (in some but not all patients), and that symptoms correlated with severity of fatigue. COMPASS scores were significantly higher for the CFS group than for the PBC group and healthy control group.<ref name="Jones2007"/> == Ehlers-Danlos Syndrome == A study of 39 female Hypermobile EDS patients showed increased sympathetic activity at rest and reduced sympathetic reactivity to stimuli. It also showed an increased rate of [[orthostatic intolerance]] (74% in hEDS v. 34% in controls).<ref name="DeWandele2014">{{Cite journal | last = De Wandele | first = Inge | last2 = Rombaut | first2 = Lies | last3 = Leybaert | first3 = Luc | last4 = Van de Borne | first4 = Philippe | last5 = De Backer | first5 = Tine | last6 = Malfait | first6 = Fransiska | last7 = De Paepe | first7 = Anne | last8 = Calders | first8 = Patrick | date = 2014-08-01 | title = Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers–Danlos syndrome | url =http://www.sciencedirect.com/science/article/pii/S0049017213002928|journal=Seminars in Arthritis and Rheumatism|volume=44|issue=1 | pages = 93–100|doi=10.1016/j.semarthrit.2013.12.006|issn=0049-0172}}</ref> A study of hEDS patients found that 74.4% had Orthostatic Intolerance.<ref name="Malfait2016">{{Cite journal | last = Malfait | first = Fransiska | last2 = Calders | first2 = Patrick | last3 = De Paepe | first3 = Anne | last4 = De Mits | first4 = Sophie | last5 = Da Silva | first5 = Hellen | last6 = Peersman | first6 = Wim | last7 = De Backer | first7 = Tine | last8 = Rombaut | first8 = Lies | last9 = De Wandele | first9 = Inge | date = 2016-08-01 | title = Orthostatic intolerance and fatigue in the hypermobility type of Ehlers-Danlos Syndrome | url =https://academic.oup.com/rheumatology/article/55/8/1412/2566504|journal=Rheumatology|language=en|volume=55|issue=8 | pages = 1412–1420|doi=10.1093/rheumatology/kew032|issn=1462-0324}}</ref> A study of 48 patients meeting the 1998 [[Brighton criteria]] found that 78% had orthostatic intolerance versus 10% of controls.<ref name="Gazit2003">{{Cite journal | last = Gazit | first = Yael | last2 = Nahir | first2 = A. Menahem | last3 = Grahame | first3 = Rodney | last4 = Jacob | first4 = Giris | date = 2003-07-01 | title = Dysautonomia in the joint hypermobility syndrome | url =http://www.sciencedirect.com/science/article/pii/S0002934303002353|journal=The American Journal of Medicine|volume=115|issue=1 | pages = 33–40|doi=10.1016/S0002-9343(03)00235-3|issn=0002-9343}}</ref> == Fibromyalgia == A symptom survey study comparing patients with EDS and fibromyalgia found that fibromyalgia patients had a much higher autonomic symptom burdens than controls, but similar to patients with EDS.<ref name="DeWandeleCalders2014">{{Cite journal | last = De Wandele | first = Inge | last2 = Calders | first2 = Patrick | last3 = Peersman | first3 = Wim | last4 = Rimbaut | first4 = Steven | last5 = De Backer | first5 = Tine | last6 = Malfait | first6 = Fransiska | last7 = De Paepe | first7 = Anne | last8 = Rombaut | first8 = Lies | date = 2014-12-01 | title = Autonomic symptom burden in the hypermobility type of Ehlers–Danlos syndrome: A comparative study with two other EDS types, fibromyalgia, and healthy controls |url =http://www.sciencedirect.com/science/article/pii/S0049017214001073|journal=Seminars in Arthritis and Rheumatism|volume=44|issue=3 | pages = 353–361|doi=10.1016/j.semarthrit.2014.05.013|issn=0049-0172}}</ref> == See also == * [[Autonomic nervous system]] * [[Dizziness]] * [[Hypotension]] * [[Neurally mediated hypotension]] * [[Orthostatic intolerance]] * [[Postural orthostatic tachycardia syndrome]] == References== {{Reflist}} [[Category:Diagnoses]] [[Category:Neurology]] [[Category:Cardiology]] [[Category:Cardiovascular diseases and disorders]] [[Category:Autonomic nervous system disorders]]
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