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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/07/23 19:47:30」(JST)

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Dysautonomia (or autonomic dysfunction) is a broad term that describes any disease or malfunction of the autonomic nervous system. This includes postural orthostatic tachycardia syndrome (POTS), inappropriate sinus tachycardia (IST), vasovagal syncope, pure autonomic failure, neurocardiogenic syncope (NCS), neurally mediated hypotension (NMH), orthostatic hypotension, orthostatic hypertension, autonomic instability and a number of lesser-known disorders such as cerebral salt-wasting syndrome. Dysautonomia is associated with multiple system atrophy (Shy-Drager syndrome), Ehlers-Danlos syndrome (EDS), and Marfan syndrome for reasons that are not fully understood.

Presentation

Symptoms of dysautonomia are numerous and vary widely from person to person. Since dysautonomia is a full-body condition, a large number of symptoms may be present that can greatly alter a person's quality of life. Each patient with dysautonomia is different—some are affected only mildly, while others are left completely bed-ridden and disabled.

The primary symptoms present in patients with dysautonomia are:

Excessive fatigue
Excessive thirst (polydipsia)
Lightheadedness, dizziness or vertigo
Feelings of anxiety or panic (not mentally induced^{[citation needed]})
Rapid heart rate or slow heart rate
Orthostatic hypotension, sometimes resulting in syncope^[1] (fainting)

Other symptoms frequently associated with dysautonomia include: Gastroparesis (delayed gastric emptying), headaches, pallor, malaise, facial flushing, salt cravings, mydriasis (abnormal dilation of the pupils), constipation, diarrhea, nausea, acid reflux, visual disturbances, orthostatic hypotension, numbness, nerve pain, trouble breathing, chest pains, in some cases loss of consciousness and seizures.^[1] Dysautonomia can also present with orthostatic hypertension.^[2] A full list of symptoms may be found at the Dysautonomia Information Network.^[3]

Causes

Causes of dysautonomias are not fully understood, but they are thought to include:^{[citation needed]}

Autoimmune disorders^{[citation needed]}
Lyme disease^[4]
Diabetes mellitus
Brain injury^[5]
Degenerative neurological diseases such as Parkinson's disease^{[citation needed]}
Genetic factors^{[citation needed]}
Hereditary connective tissue diseases, especially Ehlers-Danlos Syndrome. In a study on orthostatic intolerance and EDS, it is suggested that the co-occurrence of these syndromes can be attributed to the abnormal connective tissue in dependent blood vessels of those with EDS, which permits veins to distend excessively in response to ordinary hydrostatic pressures. This in turn leads to increased venous pooling and its hemodynamic and symptomatic consequences.^[6]
Physical trauma or injury which damages the autonomic nervous system, as with Cerebral salt-wasting syndrome.^[5]
Mitochondrial diseases^{[citation needed]}
Spinal cord injury (autonomic dysreflexia)^[7]
Hereditary forms, often together with a sensory neuropathy (hereditary sensory and autonomic neuropathy, five types)^[8]

Management

In some cases, a cardiac ablation procedure can be performed to stop the heart symptoms completely. It is not recommended in POTS patients, and can in fact worsen tachycardia.^[9] Medications are also used to stabilize the condition on a long-term basis. Benzodiazepines can be used for some of the physical problems such as anxiety. In many cases treatment of primary dysautonomia is symptomatic and supportive. Measures to combat orthostatic intolerance include elevation of the head of the bed, frequent small meals, a high-salt diet, fluid intake, and compression stockings. Drugs such as fludrocortisone, midodrine, ephedrine and SSRIs can also be used to treat symptoms. Treating dysautonomia can be difficult and usually requires a combination of drug therapies.

Prognosis

The outlook for patients with dysautonomia depends on the particular diagnostic category. Some forms of dysautonomia resolve over time and are not life threatening, even if life-changing in the form of minor to major limitations in activities of daily living. Patients with chronic, progressive, generalized dysautonomia in the setting of central nervous system degeneration have a generally poor long-term prognosis. Death can occur from pneumonia, acute respiratory failure, or sudden cardiopulmonary arrest in such patients. ^[10]

History

In the nineteenth and earlier twentieth centuries, a diagnosis that was almost solely given to women was "neurasthenia," or a "weak nervous system." (During World War I, doctors began to apply it to men exhibiting symptoms of what is now called post-traumatic stress disorder.) Women would present with symptoms of fatigue, weakness, dizziness and fainting, and the doctor's orders would simply be bed rest. Some of these women died, while many others recovered. No one understood where the problems came from.

With advances in modern medicine, diagnostic criteria and treatment for various forms of dysautonomia have sharpened. Doctors and researchers are including males in their subject population for this disorder.

The prototype of dysautonomia is the ancient scourge of beriberi, a nutritional deficiency disease due to excess of simple carbohydrate and concomitant vitamin B₁ deficiency. In the early stages, this results in loss of functional efficiency in the central control mechanisms of the autonomic nervous system. If the nutritional deficiency continues, there is gradual degeneration of the system. Other vitamin deficiencies have been implicated in causing dysautonomia and, unlike the genetically determined forms of the disease, they are treatable.^[11]

References

^ ^a ^b Tierney, Lawrence M.; McPhee, Stephen J.; Papadakis, Maxine A. (2006). Current Medical Diagnosis and Treatment 2007 (Current Medical Diagnosis and Treatment). McGraw-Hill Professional. p. 1010. ISBN 0-07-147247-9.
^ http://www.medscape.com/viewarticle/543590_4
^ Dinet.org
^ Lymeinfo.net
^ ^a ^b Baguley IJ, Heriseanu RE, Cameron ID, Nott MT, Slewa-Younan S (2008). "A critical review of the pathophysiology of dysautonomia following traumatic brain injury". Neurocrit Care 8 (2): 293–300. DOI:10.1007/s12028-007-9021-3. PMID 17968518.
^ J Pediatr. 1999 Oct;135(4):494-9. Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome. Rowe PC, Barron DF, Calkins H, Maumenee IH, Tong PY, Geraghty MT.
^ Alexander MS, Biering-Sorensen F, Bodner D, et al. (January 2009). "International standards to document remaining autonomic function after spinal cord injury". Spinal Cord 47 (1): 36–43. DOI:10.1038/sc.2008.121. PMID 18957962.
^ Axelrod FB (2002). "Hereditary sensory and autonomic neuropathies. Familial dysautonomia and other HSANs". Clin Auton Res. 12 Suppl 1 (7): I2–14. DOI:10.1007/s102860200014. PMID 12102459.
^ HRSpatients.org
^ "Dysautonomia". NINDS. http://www.ninds.nih.gov/disorders/dysautonomia/dysautonomia.htm. Retrieved 2012-04-03.
^ Lonsdale D., "Dysautonomia, a heuristic approach to a revised model for etiology of disease", eCAM 2009, 6:3-10.

External links

Dysautonomia at the Open Directory Project
dysautonomia at NINDS
Dysautonomia Youth Network of America, Inc.
ndrf.org
12 More Pages-Live & Cope with Dysautonomia
Dysautonomia Information Network
Handbook for Patients with Dysautonomia, by Dr. David S. Goldstein, MD, PhD and Linda J. Smith - Free chapter by chapter PDF download

UpToDate Contents

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1. 起立性低血圧のメカニズム、原因、および評価 mechanisms causes and evaluation of orthostatic hypotension
2. 遺伝性感覚自律神経ニューロパチー hereditary sensory and autonomic neuropathies
3. アシュケナージ系ユダヤ人集団における遺伝的疾患の出生前スクリーニング prenatal screening for genetic disease in the ashkenazi jewish population
4. 多系統萎縮症：臨床的特徴および診断 multiple system atrophy clinical features and diagnosis
5. 小児における不明熱の病因 etiologies of fever of unknown origin in children

English Journal

Distinguishing ischaemic optic neuropathy from optic neuritis by ganglion cell analysis.

Erlich-Malona N1, Mendoza-Santiesteban CE1,2,3, Hedges TR 3rd1, Patel N1, Monaco C1, Cole E1.
Acta ophthalmologica.Acta Ophthalmol.2016 Jul 1. doi: 10.1111/aos.13128. [Epub ahead of print]
PURPOSE: To determine whether a pattern of altitudinal ganglion cell loss, as detected and measured by optical coherence tomography (OCT), can be used to distinguish non-arteritic ischaemic optic neuropathy (NAION) from optic neuritis (ON) during the acute phase, and whether the rate or severity of
PMID 27364519

Musculoskeletal manifestations of Fabry disease: A retrospective study.

Lidove O1, Zeller V2, Chicheportiche V3, Meyssonnier V2, Sené T2, Godot S2, Ziza JM2.
Joint, bone, spine : revue du rhumatisme.Joint Bone Spine.2016 Jul;83(4):421-6. doi: 10.1016/j.jbspin.2015.11.001. Epub 2015 Dec 14.
OBJECTIVES: Fabry disease is a rare X-linked metabolic disorder characterized by a deficiency in the enzyme alpha-galactosidase A. Both males and females can be affected. The main presenting symptom is pain in the extremities, whereas at a more advanced stage, the manifestations include hypertrophic
PMID 26697993

The clinical spectrum of Caspr2 antibody-associated disease.

van Sonderen A1, Ariño H2, Petit-Pedrol M2, Leypoldt F2, Körtvélyessy P2, Wandinger KP2, Lancaster E2, Wirtz PW2, Schreurs MW2, Sillevis Smitt PA2, Graus F2, Dalmau J2, Titulaer MJ2.
Neurology.Neurology.2016 Jul 1. pii: 10.1212/WNL.0000000000002917. [Epub ahead of print]
OBJECTIVE: To report a large cohort of patients with antibodies against contactin-associated protein-like 2 (Caspr2) and provide the clinical spectrum of this disorder.METHODS: Serum and CSF samples were assessed at 2 neuroimmunology centers in Barcelona and Rotterdam. Patients were included if Casp
PMID 27371488

Japanese Journal

トランスクリプトーム創薬の試み

日本毒性学会学術年会 45.1(0), S11-2, 2018-08-10
NAID 130007431954

ICT教育の推進とスクリーニングとしての視力検査の充実に関する研究(2)ビジョントレーニング・身体活動による視力向上効果を有する学校体育の有効活用 (清水真一教授退任記念号国松夏紀教授退任記念号 GONZALEZ Dario教授退任記念号)

人間文化研究 = Journal of humanities research, St. Andrew's University (8), 37-52, 2018-02
NAID 120006460998

Nocturnal Hypertension in Multiple System Atrophy May Cause Posterior Reversible Encephalopathy Syndrome

Intern. Med., 2018
NAID 130007384621

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★リンクテーブル★

リンク元	「自律神経障害」「自律神経失調」「disorders of the autonomic nervous system」「autonomic nervous system disease」「autonomic nervous system disorder」
拡張検索	「familial dysautonomia」「acute pandysautonomia」

「自律神経障害」

Dysautonomia
	Classification and external resources
ICD-10	G90
ICD-9	337.9
MeSH	D001342

　　[★]

英: autonomic neuropathy、autonomic dysfunction、dysautonomia、disorders of the autonomic nervous system, autonomic disturbance

関: 純粋自律神経不全症、自律神経ニューロパチー、自律神経性ニューロパチー、自律神経ニューロパシー、自律神経系疾患、自律神経機能異常、自律神経異常症、自律神経機能不全

臓器毎の自律神経障害

眼	瞳孔の異常 (縮瞳,散瞳)、眼瞼下垂
循環器	起立性低血圧、食事性低血圧、不整脈
呼吸器	睡眠時呼吸障害
消化器	便秘、便失禁、下痢
泌尿器	神経因性膀胱(頻尿,排尿困難,残尿など)
生殖器	勃起障害
皮膚	発汗低下、皮膚栄養障害