MCID: DYS021
MIFTS: 49

Dysautonomia malady

Neuronal diseases, Rare diseases categories
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Summaries for Dysautonomia

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NINDS:43 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 based summary: 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:65 Dysautonomia (or autonomic dysfunction, autonomic neuropathy) is a condition in which the autonomic... more...

Aliases & Classifications for Dysautonomia

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Sources:
43NINDS, 44Novoseek, 62UMLS
See all sources

Dysautonomia, Aliases & Descriptions:

Name: Dysautonomia 43 44
 
Autonomic Nervous System Disorders 62


Classifications:

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


Related Diseases for Dysautonomia

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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 177)
idRelated DiseaseScoreTop Affiliating Genes
1riley-day syndrome31.3DBH, IKBKAP, NTRK1
2parkinson's disease30.7DBH, SNCA
3pure autonomic failure30.5DBH, SNCA
4autonomic neuropathy30.5NTRK1, IKBKAP, DBH
5multiple system atrophy30.1SNCA, PRNP, DBH
6hereditary sensory neuropathy30.1IKBKAP, NTRK1
7progressive supranuclear palsy29.8SNCA, PRNP
8spinocerebellar ataxia29.8PRNP, SNCA
9pheochromocytoma29.7SNCA, NTRK1, DBH
10neuroblastoma29.5NTRK1, PRNP, DBH
11alzheimer's disease28.8HSPG2, DBH, PRNP, NTRK1, SNCA
12autonomic dysfunction10.5
13brain injury10.5
14traumatic brain injury10.4
15neuropathy10.4
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
25neuroaxonal dystrophy10.1NTRK1, SNCA
26ganglioglioma10.1SNCA, NTRK1
27ganglioneuroma10.1NTRK1, SNCA
28chagas disease10.1
29chronic fatigue syndrome10.1
30autonomic nervous system disease10.1
31esophagitis10.1
32myopathy10.1
33dysautonomia like disorder10.1
34stuve-wiedemann syndrome10.1
35orthostatic intolerance10.1
36follicular adenoma10.1NTRK1, HSPG2
37bipolar disorder10.0DBH, HSPG2
38lewy body dementia10.0SNCA, PRNP
39vascular dementia10.0PRNP, SNCA
40myasthenia gravis10.0
41sinusitis10.0
42mitral valve prolapse10.0
43rett syndrome10.0
44third-degree atrioventricular block10.0
45lepromatous leprosy10.0
46esophageal atresia10.0
47cerebritis10.0
48acute intermittent porphyria10.0
49atrioventricular block10.0
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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Drug clinical trials:

Search ClinicalTrials for Dysautonomia

Search NIH Clinical Center for Dysautonomia

Genetic Tests for Dysautonomia

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Anatomical Context for Dysautonomia

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MalaCards organs/tissues related to Dysautonomia:

32
Brain, Heart, Spinal cord, Testes, Skin, Colon, Bone, Liver, Lung, Salivary gland, Small intestine, Kidney, T cells, Thalamus, Tongue

Animal Models for Dysautonomia or affiliated genes

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Publications for Dysautonomia

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Articles related to Dysautonomia:

(show top 50)    (show all 539)
idTitleAuthorsYear
1
A neuron autonomous role for the familial dysautonomia gene ELP1 in sympathetic and sensory target tissue innervation. (24917501)
2014
2
Dysautonomia in narcolepsy: evidence by questionnaire assessment. (25324880)
2014
3
Dysautonomia in Parkinson disease. (24715569)
2014
4
The presence of dysautonomia in different subgroups of myasthenia gravis patients. (25119843)
2014
5
Familial dysautonomia model reveals Ikbkap deletion causes apoptosis of Pax3+ progenitors and peripheral neurons. (24173031)
2013
6
Dysautonomia after pediatric brain injury. (22712762)
2012
7
Cardiovascular dysautonomia in Parkinson disease: from pathophysiology to pathogenesis. (22094370)
2012
8
Dysautonomia: perioperative implications. (22143168)
2012
9
A 10-year follow-up study of patients with severe traumatic brain injury and dysautonomia treated with intrathecal baclofen therapy. (22668125)
2012
10
SIADH and dysautonomia as the initial presentation of Guillain-BarrAc syndrome. (21335571)
2012
11
Deletion of exon 20 of the Familial Dysautonomia gene Ikbkap in mice causes developmental delay, cardiovascular defects, and early embryonic lethality. (22046433)
2011
12
The Movement Disorders task force review of dysautonomia rating scales in Parkinson's disease with regard to symptoms of orthostatic hypotension. (21547951)
2011
13
Evaluation of formalin-fixed ileum as the optimum method to diagnose equine dysautonomia (grass sickness) in simulated intestinal biopsies. (20224086)
2010
14
A comparison of dysautonomias comorbid with cyclic vomiting syndrome and with migraine. (20111731)
2009
15
Neoplasia in familial dysautonomia: a 20-year review in a young patient population. (19914433)
2009
16
Deep breath test for evaluation of autonomic nervous system dysfunction in familial dysautonomia. (20077949)
2009
17
Plasma catechols in familial dysautonomia: a long-term follow-up study. (18357519)
2008
18
Dysautonomia and Clinical Outcome in Vegetative State. (18771395)
2008
19
The molecular basis of familial dysautonomia: overview, new discoveries and implications for directed therapies. (17985250)
2008
20
Perioperative management of familial dysautonomia: a systematic review. (17202006)
2007
21
Cardiovascular dysautonomia as a cause of falls in Parkinson's disease. (16621660)
2006
22
Dysautonomia and salmonellosis in an 11-year-old female llama (Lama glama). (16496946)
2006
23
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
24
Successful pharmacotherapy for an adult case with prolonged symptoms of postganglionic cholinergic dysautonomia. (15159611)
2004
25
Abomasal emptying defect of sheep may be an acquired form of dysautonomia. (15017030)
2004
26
Clinical versus electrophysiological assessment of dysautonomia in obstructive sleep apnea syndrome. (15622130)
2004
27
Dysautonomia in chronic fatigue syndrome: facts, hypotheses, implications. (14962627)
2004
28
Screening for familial dysautonomia in Israel: evidence for higher carrier rate among Polish Ashkenazi Jews. (12885336)
2003
29
Dermal microdialysis provides evidence for hypersensitivity to noradrenaline in patients with familial dysautonomia. (12185162)
2002
30
Transcranial Doppler sonography during head up tilt suggests preserved central sympathetic activation in familial dysautonomia. (11971058)
2002
31
Canine dysautonomia: two clinical cases. (11833820)
2002
32
Dysautonomia and autonomic neuropathies. (11785731)
2002
33
Genetics of familial dysautonomia. Tissue-specific expression of a splicing mutation in the IKBKAP gene. (12102458)
2002
34
Progressive cardiac dysautonomia observed in patients affected by classic Rett syndrome and not in the preserved speech variant. (11392524)
2001
35
Gallbladder contraction in familial dysautonomia. (10229040)
1999
36
Indicators of dysautonomia in severe Guillain-BarrAc syndrome. (10631632)
1999
37
Acute dysautonomia secondary to autoimmune diseases: efficacy of intravenous immunoglobulin and correlation with a stimulation of plasma norepinephrine levels. (10609075)
1999
38
Taste and smell in familial dysautonomia. (9233364)
1997
39
A case of SjAPgren syndrome associated with multiple mononeuritis and dysautonomia including bilateral tonic pupils]. (9311000)
1997
40
A pilot study of motility and tone of the left colon in patients with diarrhea due to functional disorders and dysautonomia. (9040210)
1997
41
An evaluation of the use of cisapride in horses with chronic grass sickness (equine dysautonomia) (8885464)
1996
42
Familial dysautonomia (Riley-Day syndrome). (10829892)
1995
43
Decreased incorporation of D-glucosamine into glycosphingolipids of intact Familial Dysautonomia lymphoblasts. (8746450)
1995
44
Atrial natriuretic peptide response to postural change and medication in familial dysautonomia. (7711467)
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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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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Compounds for genes affiliated with Dysautonomia

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Sources:
44Novoseek, 24HMDB, 28IUPHAR, 50PharmGKB, 11DrugBank
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Compounds related to Dysautonomia according to GeneCards/GeneDecks:

(show all 48)
idCompoundScoreTop Affiliating Genes
1methanol44 2410.9DBH, SNCA
26-hydroxydopamine449.8SNCA, DBH
3mptp449.8DBH, SNCA
4propranolol44 28 50 24 1113.7DBH, HSPG2
5atropine44 28 1111.7HSPG2, SNCA
6levodopa44 1110.7DBH, SNCA
7n-ethylmaleimide44 1110.6HSPG2, DBH
8opiate449.6NTRK1, DBH
9capsaicin44 2810.6HSPG2, NTRK1
10c2ceramide449.6NTRK1, HSPG2
11hemocyanin449.6DBH, PRNP
12quinacrine44 1110.5HSPG2, PRNP
13formate449.5PRNP, SNCA
14cocaine44 1110.5SNCA, DBH
15doxycycline44 1110.4PRNP, SNCA
16phosphatidylcholine449.4HSPG2, SNCA, DBH
17guanidine hydrochloride449.4SNCA, PRNP
18catecholamine449.3SNCA, DBH, NTRK1
19gnrh449.3NTRK1, HSPG2, DBH
20pd 98,059449.3HSPG2, DBH, NTRK1
21egcg449.2IKBKAP, HSPG2
22sodium dodecylsulfate449.2SNCA, PRNP
23ganglioside449.2PRNP, NTRK1
24dopamine44 28 24 1112.2DBH, SNCA, NTRK1
25cyclic amp44 2410.2NTRK1, HSPG2, DBH
26arachidonic acid44 28 24 1112.2SNCA, HSPG2, NTRK1
27copper44 2410.1SNCA, PRNP, DBH
28iron44 2410.0SNCA, DBH, PRNP
29hydrogen44 2410.0SNCA, PRNP, DBH
30lysine449.0SNCA, PRNP, DBH
31phorbol449.0PRNP, DBH, NTRK1
32phosphatidylinositol448.9NTRK1, DBH, HSPG2
33heparin44 28 24 1111.9SNCA, HSPG2, PRNP
34paraffin448.9NTRK1, SNCA, PRNP
35nitric oxide44 24 1110.9DBH, SNCA, NTRK1
36choline44 24 1110.8HSPG2, DBH, NTRK1, SNCA
37acetylcholine44 50 28 24 1112.8HSPG2, DBH, NTRK1, SNCA
38adenylate448.8NTRK1, SNCA, HSPG2, DBH
39pge2448.6SNCA, DBH, HSPG2, PRNP
40threonine448.5PRNP, IKBKAP, NTRK1
41oxygen44 249.5SNCA, NTRK1, PRNP, DBH
42lipid448.4PRNP, DBH, SNCA, NTRK1
43h2o2448.0PRNP, DBH, NTRK1, SNCA, HSPG2
44glutamate448.0SNCA, NTRK1, PRNP, DBH, HSPG2
45alanine448.0HSPG2, SNCA, DBH, PRNP, NTRK1
46tyrosine448.0DBH, HSPG2, SNCA, NTRK1, PRNP
47calcium44 50 24 1110.9PRNP, DBH, HSPG2, SNCA, NTRK1
48serine447.6DBH, IKBKAP, HSPG2, SNCA, PRNP, NTRK1

GO Terms for genes affiliated with Dysautonomia

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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
  • Proteins
  • Lysates

Sources for Dysautonomia

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet