MCID: DYS021
MIFTS: 48

Dysautonomia malady

Neuronal diseases, Rare diseases categories

Summaries for Dysautonomia

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45NINDS, 66Wikipedia, 34MalaCards
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NINDS:45 Dysautonomia refers to a disorder of autonomic nervous system (ANS) function. Most physicians view dysautonomia in terms of failure of the sympathetic or parasympathetic components of the ANS, but dysautonomia involving excessive ANS activities also can occur. Dysautonomia can be local, as in reflex sympathetic dystrophy, or generalized, as in pure autonomic failure. It can be acute and reversible, as in Guillain-Barre syndrome, or chronic and progressive. Several common conditions such as diabetes and alcoholism can include dysautonomia. Dysautonomia also can occur as a primary condition or in association with degenerative neurological diseases such as Parkinson's disease. Other diseases with generalized, primary dysautonomia include multiple system atrophy and familial dysautonomia. Hallmarks of generalized dysautonomia due to sympathetic failure are impotence (in men) and a fall in blood pressure during standing (orthostatic hypotension). Excessive sympathetic activity can present as hypertension or a rapid pulse rate.

MalaCards: Dysautonomia, also known as autonomic nervous system disorders, is related to riley-day syndrome and parkinson's disease. An important gene associated with Dysautonomia is IKBKAP (inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase complex-associated protein), and among its related pathways are RNA Polymerase I Promoter Opening and Neuroscience. The compounds methanol and 6-hydroxydopamine have been mentioned in the context of this disorder. Affiliated tissues include brain, heart and spinal cord, and related mouse phenotypes are cardiovascular system and limbs/digits/tail.

Wikipedia:66 Dysautonomia (or autonomic dysfunction, autonomic neuropathy) is a condition in which the autonomic... more...

Aliases & Classifications for Dysautonomia

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45NINDS, 46Novoseek, 63UMLS
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Neuronal diseases


Aliases & Descriptions:

dysautonomia 45 46
autonomic nervous system disorders 63


Related Diseases for Dysautonomia

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18GeneCards, 19GeneDecks
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Diseases in the Dysautonomia family:

Dysautonomia Like Disorder

Diseases related to Dysautonomia via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 172)
idRelated DiseaseScoreTop Affiliating Genes
1riley-day syndrome31.2DBH, IKBKAP, NTRK1
2parkinson's disease30.7DBH, SNCA
3autonomic neuropathy30.5NTRK1, IKBKAP, DBH
4pure autonomic failure30.3DBH, SNCA
5multiple system atrophy30.2SNCA, PRNP, DBH
6hereditary sensory neuropathy29.8IKBKAP, NTRK1
7progressive supranuclear palsy29.8SNCA, PRNP
8spinocerebellar ataxia29.8PRNP, SNCA
9pheochromocytoma29.7SNCA, NTRK1, DBH
10neuroblastoma29.7NTRK1, PRNP, DBH
11alzheimer's disease29.5HSPG2, DBH, PRNP, NTRK1, SNCA
12autonomic dysfunction10.5
13brain injury10.5
14neuropathy10.4
15traumatic brain injury10.4
16neuronitis10.3
17motor neuropathy peripheral with dysautonomia10.3
18fibromyalgia10.3
19fatal familial insomnia10.2
20polyneuropathy10.2
21acute cholinergic dysautonomia10.2
22diabetes mellitus10.2
23hypertension10.2
24leprosy10.2
25insomnia10.2
26chronic fatigue syndrome10.1
27autonomic nervous system disease10.1
28esophagitis10.1
29myopathy10.1
30dysautonomia like disorder10.1
31stuve-wiedemann syndrome10.1
32orthostatic intolerance10.1
33neuroaxonal dystrophy10.0NTRK1, SNCA
34ganglioglioma10.0SNCA, NTRK1
35ganglioneuroma10.0NTRK1, SNCA
36follicular adenoma10.0NTRK1, HSPG2
37bipolar disorder10.0DBH, HSPG2
38lewy body dementia10.0SNCA, PRNP
39vascular dementia10.0PRNP, SNCA
40atrioventricular block10.0
41mitral valve prolapse10.0
42rett syndrome10.0
43third-degree atrioventricular block10.0
44lepromatous leprosy10.0
45esophageal atresia10.0
46hypermobility syndrome10.0
47cerebritis10.0
48chagas disease10.0
49acute intermittent porphyria10.0
50dementia10.0

Graphical network of the top 20 diseases related to Dysautonomia:



Diseases related to dysautonomia

Symptoms for Dysautonomia

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Drugs & Therapeutics for Dysautonomia

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Dysautonomia

Drug clinical trials:

Search ClinicalTrials for Dysautonomia

Search NIH Clinical Center for Dysautonomia

Search CenterWatch for Dysautonomia

Genetic Tests for Dysautonomia

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Anatomical Context for Dysautonomia

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34MalaCards
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MalaCards organs/tissues related to Dysautonomia:

34
Brain, Heart, Spinal cord, Testes, Skin, Colon, Bone, Salivary gland, Lung, Liver, Kidney, Tongue, T cells, Small intestine, Thalamus

Animal Models for Dysautonomia or affiliated genes

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38MGI
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Publications for Dysautonomia

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53PubMed
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Articles related to Dysautonomia:

(show top 50)    (show all 521)
idTitleAuthorsYear
1
Familial dysautonomia model reveals Ikbkap deletion causes apoptosis of Pax3+ progenitors and peripheral neurons. (24173031)
2013
2
Dysautonomia after pediatric brain injury. (22712762)
2012
3
Cardiovascular dysautonomia in Parkinson disease: from pathophysiology to pathogenesis. (22094370)
2012
4
Dysautonomia: perioperative implications. (22143168)
2012
5
A 10-year follow-up study of patients with severe traumatic brain injury and dysautonomia treated with intrathecal baclofen therapy. (22668125)
2012
6
SIADH and dysautonomia as the initial presentation of Guillain-BarrAc syndrome. (21335571)
2012
7
Deletion of exon 20 of the Familial Dysautonomia gene Ikbkap in mice causes developmental delay, cardiovascular defects, and early embryonic lethality. (22046433)
2011
8
The Movement Disorders task force review of dysautonomia rating scales in Parkinson's disease with regard to symptoms of orthostatic hypotension. (21547951)
2011
9
Evaluation of formalin-fixed ileum as the optimum method to diagnose equine dysautonomia (grass sickness) in simulated intestinal biopsies. (20224086)
2010
10
A comparison of dysautonomias comorbid with cyclic vomiting syndrome and with migraine. (20111731)
2009
11
Neoplasia in familial dysautonomia: a 20-year review in a young patient population. (19914433)
2009
12
Deep breath test for evaluation of autonomic nervous system dysfunction in familial dysautonomia. (20077949)
2009
13
Plasma catechols in familial dysautonomia: a long-term follow-up study. (18357519)
2008
14
Dysautonomia and Clinical Outcome in Vegetative State. (18771395)
2008
15
The molecular basis of familial dysautonomia: overview, new discoveries and implications for directed therapies. (17985250)
2008
16
Perioperative management of familial dysautonomia: a systematic review. (17202006)
2007
17
Cardiovascular dysautonomia as a cause of falls in Parkinson's disease. (16621660)
2006
18
Dysautonomia and salmonellosis in an 11-year-old female llama (Lama glama). (16496946)
2006
19
Respiratory aspects of Riley-Day Syndrome: familial dysautonomia. (16798585)
2006
20
Allgrove syndrome with features of familial dysautonomia: a novel mutation in the AAAS gene. (16938764)
2006
21
Necrotizing enterocolitis in a premature infant as the presenting symptom of familial dysautonomia in the neonatal period: case report and review of the literature. (16215920)
2005
22
Successful pharmacotherapy for an adult case with prolonged symptoms of postganglionic cholinergic dysautonomia. (15159611)
2004
23
Abomasal emptying defect of sheep may be an acquired form of dysautonomia. (15017030)
2004
24
Clinical versus electrophysiological assessment of dysautonomia in obstructive sleep apnea syndrome. (15622130)
2004
25
Dysautonomia in chronic fatigue syndrome: facts, hypotheses, implications. (14962627)
2004
26
Screening for familial dysautonomia in Israel: evidence for higher carrier rate among Polish Ashkenazi Jews. (12885336)
2003
27
Dermal microdialysis provides evidence for hypersensitivity to noradrenaline in patients with familial dysautonomia. (12185162)
2002
28
Transcranial Doppler sonography during head up tilt suggests preserved central sympathetic activation in familial dysautonomia. (11971058)
2002
29
Canine dysautonomia: two clinical cases. (11833820)
2002
30
Dysautonomia and autonomic neuropathies. (11785731)
2002
31
Genetics of familial dysautonomia. Tissue-specific expression of a splicing mutation in the IKBKAP gene. (12102458)
2002
32
Progressive cardiac dysautonomia observed in patients affected by classic Rett syndrome and not in the preserved speech variant. (11392524)
2001
33
Dysautonomia and neurocardiogenic syncope. (11224639)
2001
34
Indicators of dysautonomia in severe Guillain-BarrAc syndrome. (10631632)
1999
35
Acute dysautonomia secondary to autoimmune diseases: efficacy of intravenous immunoglobulin and correlation with a stimulation of plasma norepinephrine levels. (10609075)
1999
36
Taste and smell in familial dysautonomia. (9233364)
1997
37
A case of SjAPgren syndrome associated with multiple mononeuritis and dysautonomia including bilateral tonic pupils]. (9311000)
1997
38
A pilot study of motility and tone of the left colon in patients with diarrhea due to functional disorders and dysautonomia. (9040210)
1997
39
An evaluation of the use of cisapride in horses with chronic grass sickness (equine dysautonomia) (8885464)
1996
40
Increased major salivary gland secretion in familial dysautonomia. (8603780)
1996
41
Familial dysautonomia (Riley-Day syndrome). (10829892)
1995
42
Decreased incorporation of D-glucosamine into glycosphingolipids of intact Familial Dysautonomia lymphoblasts. (8746450)
1995
43
Atrial natriuretic peptide response to postural change and medication in familial dysautonomia. (7711467)
1994
44
Dysautonomia with acute sensory motor neuropathy. A new classification of acute autonomic neuropathy. (7945000)
1994
45
Maturational dysautonomia and facial anomalies associated with esophageal atresia: support for neural crest involvement. (8331506)
1993
46
Autonomic neurone degeneration in equine dysautonomia (grass sickness). (1469124)
1992
47
Incidence of familial dysautonomia in Israel 1977-1981. (3652488)
1987
48
Cholinergic dysautonomia and Eaton-Lambert syndrome. (220562)
1979
49
Familial dysautonomia (Riley-Day syndrome). (6036298)
1967
50
The neuropsychiatric aspects of familial dysautonomia (the Riley-Day syndrome). (13332409)
1956

Variations for Dysautonomia

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Expression for genes affiliated with Dysautonomia

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Dysautonomia

Search GEO for disease gene expression data for Dysautonomia.

Pathways for genes affiliated with Dysautonomia

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51PathCards, 56Reactome, 31KEGG, 5Cell Signaling Technology
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Compounds for genes affiliated with Dysautonomia

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46Novoseek, 25HMDB, 30IUPHAR, 52PharmGKB, 12DrugBank
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Compounds related to Dysautonomia according to GeneCards/GeneDecks:

(show all 48)
idCompoundScoreTop Affiliating Genes
1methanol46 2510.9SNCA, DBH
26-hydroxydopamine469.8DBH, SNCA
3mptp469.8SNCA, DBH
4propranolol46 30 52 25 1213.7DBH, HSPG2
5atropine46 30 1211.7SNCA, HSPG2
6levodopa46 1210.7DBH, SNCA
7n-ethylmaleimide46 1210.6DBH, HSPG2
8opiate469.6NTRK1, DBH
9capsaicin46 3010.6HSPG2, NTRK1
10c2ceramide469.6NTRK1, HSPG2
11hemocyanin469.6PRNP, DBH
12egcg469.5IKBKAP, HSPG2
13quinacrine46 1210.5PRNP, HSPG2
14formate469.5SNCA, PRNP
15doxycycline46 1210.4SNCA, PRNP
16phosphatidylcholine469.4SNCA, DBH, HSPG2
17guanidine hydrochloride469.4PRNP, SNCA
18catecholamine469.3SNCA, NTRK1, DBH
19gnrh469.3HSPG2, DBH, NTRK1
20pd 98,059469.3NTRK1, DBH, HSPG2
21sodium dodecylsulfate469.2SNCA, PRNP
22ganglioside469.2NTRK1, PRNP
23dopamine46 30 25 1212.2SNCA, NTRK1, DBH
24cocaine46 1210.2SNCA, DBH
25cyclic amp46 2510.2HSPG2, DBH, NTRK1
26arachidonic acid46 30 25 1212.2SNCA, NTRK1, HSPG2
27copper46 2510.1SNCA, PRNP, DBH
28iron46 2510.0SNCA, PRNP, DBH
29hydrogen46 2510.0DBH, PRNP, SNCA
30lysine469.0DBH, PRNP, SNCA
31phorbol469.0NTRK1, PRNP, DBH
32phosphatidylinositol468.9NTRK1, DBH, HSPG2
33heparin46 30 25 1211.9SNCA, PRNP, HSPG2
34paraffin468.9PRNP, NTRK1, SNCA
35nitric oxide46 25 1210.9DBH, NTRK1, SNCA
36choline46 25 1210.8SNCA, NTRK1, DBH, HSPG2
37acetylcholine46 52 30 25 1212.8HSPG2, DBH, NTRK1, SNCA
38adenylate468.8HSPG2, DBH, NTRK1, SNCA
39pge2468.6HSPG2, DBH, PRNP, SNCA
40threonine468.5NTRK1, IKBKAP, PRNP
41oxygen46 259.5DBH, PRNP, NTRK1, SNCA
42lipid468.4DBH, PRNP, NTRK1, SNCA
43h2o2468.0HSPG2, DBH, PRNP, NTRK1, SNCA
44glutamate468.0HSPG2, DBH, PRNP, NTRK1, SNCA
45alanine468.0SNCA, NTRK1, PRNP, DBH, HSPG2
46tyrosine468.0HSPG2, DBH, PRNP, NTRK1, SNCA
47calcium46 52 25 1210.9HSPG2, DBH, PRNP, NTRK1, SNCA
48serine467.6HSPG2, DBH, PRNP, IKBKAP, NTRK1, SNCA

GO Terms for genes affiliated with Dysautonomia

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17Gene Ontology
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Biological processes related to Dysautonomia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of neuron apoptotic processGO:0435249.3SNCA, NTRK1
2learning or memoryGO:0076119.0NTRK1, PRNP
3negative regulation of protein phosphorylationGO:0019339.0SNCA, PRNP
4agingGO:0075689.0SNCA, NTRK1

Molecular functions related to Dysautonomia according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1copper ion bindingGO:0055078.7SNCA, PRNP, DBH

Products for genes affiliated with Dysautonomia

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  • Antibodies
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Sources for Dysautonomia

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet