MCID: HMD003
MIFTS: 32

Hemidystonia

Categories: Neuronal diseases

Aliases & Classifications for Hemidystonia

MalaCards integrated aliases for Hemidystonia:

Name: Hemidystonia 12 15 73

Classifications:



External Ids:

Disease Ontology 12 DOID:0050846
UMLS 73 C1960561

Summaries for Hemidystonia

Disease Ontology : 12 A multifocal dystonia that involves the arm and leg on the same side of the body.

MalaCards based summary : Hemidystonia is related to blepharospasm and dystonia. An important gene associated with Hemidystonia is CAMP (Cathelicidin Antimicrobial Peptide), and among its related pathways/superpathways is Alpha-synuclein signaling. Affiliated tissues include brain, globus pallidus and eye, and related phenotypes are behavior/neurological and mortality/aging

Related Diseases for Hemidystonia

Diseases related to Hemidystonia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 65)
# Related Disease Score Top Affiliating Genes
1 blepharospasm 29.9 CAMP GCH1 THAP1 TOR1A
2 dystonia 29.2 ATP1A3 GCH1 PNKD SGCE TAF1 THAP1
3 hemidystonia-hemiatrophy syndrome 12.2
4 spasmodic dysphonia 10.2 THAP1 TOR1A
5 dystonia 24 10.2 THAP1 TOR1A
6 brain injury 10.2
7 traumatic brain injury 10.2
8 split-hand/foot malformation 1 with sensorineural hearing loss, autosomal recessive 10.2 KRIT1 SGCE
9 multifocal dystonia 10.2 ATP1A3 CAMP TOR1A
10 spasmodic dystonia 10.1 CAMP THAP1 TOR1A
11 anal spasm 10.1 CAMP KANTR
12 paraphimosis 10.1 CAMP KANTR
13 anismus 10.1 CAMP KANTR
14 adie pupil 10.1 KANTR TOR1A
15 early-onset generalized dystonia 10.1 GCH1 THAP1 TOR1A
16 thiamine metabolism dysfunction syndrome 2 10.1 GCH1 TOR1A
17 sternum cancer 10.1 SMUG1 VIM
18 episodic kinesigenic dyskinesia 1 10.1 PNKD PRKD1
19 extratemporal epilepsy 10.0 SMUG1 TOR1A
20 oromandibular dystonia 10.0 CAMP GCH1 THAP1 TOR1A
21 moyamoya disease 1 10.0
22 head injury 10.0
23 segmental dystonia 10.0 CAMP GCH1 THAP1 TOR1A
24 cervical dystonia 10.0 CAMP GCH1 THAP1 TOR1A
25 glossopharyngeal neuralgia 10.0 KANTR VIM
26 migraine with or without aura 1 10.0 ATP1A3 KANTR PNKD
27 epicanthus 10.0 ATP1A3 TWIST1
28 focal hand dystonia 10.0 CAMP THAP1 TOR1A VIM
29 plexopathy 10.0 CAMP SMUG1
30 senile ectropion 10.0 KANTR TWIST1
31 laryngeal tuberculosis 10.0 KANTR SMUG1
32 oculomotor nerve paralysis 10.0 CAMP KANTR SMUG1
33 third cranial nerve disease 10.0 CAMP KANTR SMUG1
34 focal dystonia 10.0 CAMP SGCE TAF1 THAP1 TOR1A
35 dystonia 3, torsion, x-linked 10.0 ATP1A3 GCH1 PNKD TAF1 THAP1
36 gliosarcoma 9.9 GCH1 TWIST1 VIM
37 creutzfeldt-jakob disease 9.9
38 diabetes mellitus, noninsulin-dependent 9.9
39 multiple sclerosis 9.9
40 neurofibromatosis, type i 9.9
41 trigeminal neuralgia 9.9
42 diabetes mellitus 9.9
43 aphasia 9.9
44 arteriovenous malformation 9.9
45 vertebral artery occlusion 9.9
46 choreatic disease 9.9
47 neuronal ceroid lipofuscinosis 9.9
48 antiphospholipid syndrome 9.9
49 herpes zoster 9.9
50 toxoplasmosis 9.9

Graphical network of the top 20 diseases related to Hemidystonia:



Diseases related to Hemidystonia

Symptoms & Phenotypes for Hemidystonia

MGI Mouse Phenotypes related to Hemidystonia:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.02 ATP1A3 JAG1 PNKD SGCE SNCA THAP1
2 mortality/aging MP:0010768 9.93 ATP1A3 CAMP GCH1 JAG1 KRIT1 PRKD1
3 cardiovascular system MP:0005385 9.92 GCH1 JAG1 KRIT1 PRKD1 SGCE SNCA
4 cellular MP:0005384 9.91 CAMP JAG1 SGCE SMUG1 SNCA THAP1
5 muscle MP:0005369 9.56 ATP1A3 JAG1 KRIT1 PRKD1 SGCE TOR1A
6 nervous system MP:0003631 9.36 ATP1A3 GCH1 JAG1 KRIT1 PNKD SGCE

Drugs & Therapeutics for Hemidystonia

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Multi-Target Pallidal and Thalamic Deep Brain Stimulation for Hemi-Dystonia Unknown status NCT02982304 Phase 1
2 Humanitarian Device Exemption Approved for marketing NCT00580658
3 Studying Childhood-Onset Hemidystonia Completed NCT01432899
4 Functional MRI and DTI in the Preoperative Assessment of Dystonia Recruiting NCT02877836 Not Applicable

Search NIH Clinical Center for Hemidystonia

Genetic Tests for Hemidystonia

Anatomical Context for Hemidystonia

MalaCards organs/tissues related to Hemidystonia:

41
Brain, Globus Pallidus, Eye, Thalamus, Caudate Nucleus, Parietal Lobe

Publications for Hemidystonia

Articles related to Hemidystonia:

(show top 50) (show all 87)
# Title Authors Year
1
Progressive delayed hemidystonia following clinically mild traumatic brain injury. ( 29288226 )
2017
2
Unilateral thalamic and pallidal deep brain stimulation for idiopathic hemidystonia: results of individual and combined stimulations. Case report. ( 28669294 )
2017
3
Neurocognitive outcomes in children with unilateral basal ganglia arterial ischemic stroke and secondary hemidystonia. ( 28701080 )
2017
4
Multimodal Imaging in a Patient with Hemidystonia Responsive to GPi Deep Brain Stimulation. ( 28744382 )
2017
5
Hemidystonia in a Child With Neurofibromatosis Type 1. ( 27898639 )
2016
6
GPi deep brain stimulation for palliation of hemidystonia and hemibody jerking in a patient with suspected adult onset neuronal ceroid lipofuscinosis. ( 26944153 )
2016
7
Hemidystonia - hemiatrophy syndrome in Domenico Morone's Madonna with child. ( 26736116 )
2016
8
Recurrent episodes of isolated hemidystonia in a young adult: Dissection-associated stenosis in the middle cerebral artery. ( 27374977 )
2016
9
Fluctuating off-period hemidystonia with Pisa syndrome in advanced Parkinson's disease. ( 27423577 )
2016
10
Globus pallidus internus deep brain stimulation for traumatic hemidystonia following penetrating head injury. ( 27184900 )
2016
11
Comment on: Hemidystonia caused by frontal cortical infarction. ( 26607166 )
2016
12
Author's Reply: Hemidystonia caused by frontal cortical infarction. ( 26538401 )
2016
13
(Pseudo)hemidystonia associated with anti-glutamic acid decarboxylase antibodies - a case report. ( 25846894 )
2015
14
A prospective, randomized, blinded assessment of multitarget thalamic and pallidal deep brain stimulation in a case of hemidystonia. ( 26241157 )
2015
15
Prochlorperazine-Induced Hemidystonia Mimicking Acute Stroke. ( 26265971 )
2015
16
Anesthesia for deep brain stimulation in traumatic brain injury-induced hemidystonia. ( 26185655 )
2015
17
Hemidystonia caused by frontal cortical infarction. ( 25119274 )
2014
18
Hemidystonia With One Eye-of-the-Tiger Sign. ( 25347119 )
2014
19
Alternating hemidystonia following traumatic brain injury as an unusual presentation of paroxysmal autonomic instability with dystonia syndrome. ( 25414239 )
2014
20
The reorganization of motor network in hemidystonia from the perspective of deep brain stimulation. ( 24728838 )
2014
21
Hemiballism-hemidystonia after parietal lobe tumour resection. ( 25128656 )
2014
22
A rat model of hemidystonia induced by 3-nitropropionic acid. ( 24194961 )
2013
23
Use of pallidal deep brain stimulation in postinfarct hemidystonia. ( 23549056 )
2013
24
Pallidal deep brain stimulation for a case of hemidystonia secondary to a striatal stroke. ( 23446229 )
2013
25
The long-term surgical outcomes of secondary hemidystonia associated with post-traumatic brain injury. ( 22367408 )
2012
26
Long-term outcome of atlas-based lesion of posterior zona incerta in secondary hemidystonia. ( 21641267 )
2011
27
Isolated hemidystonia associated with NMDA receptor antibodies. ( 21412839 )
2011
28
Hemidystonia in uncontrolled type 2 diabetes mellitus. ( 21555647 )
2011
29
Posttraumatic hemidystonia treated with unilateral globus pallidus interna stimulation: Long-term follow-up and radiologic features. ( 21992879 )
2010
30
Levodopa responsive hemidystonia associated with contralateral nigral lesion. ( 20127249 )
2010
31
Hemidystonia as an initial manifestation of leptomeningeal metastasis. ( 24868364 )
2009
32
The treatment of persistent vascular hemidystonia-hemiballismus with unilateral GPi deep brain stimulation. ( 19452565 )
2009
33
Hemidystonia-hemiatrophy syndrome. ( 19097182 )
2009
34
Hemidystonia secondary to thalamic hemorrhage treated with GPi stimulation. ( 18661563 )
2008
35
Moyamoya disease presenting with hemichoreoathetosis and hemidystonia. ( 17657809 )
2007
36
Unilateral pallidotomy for hemidystonia. ( 16534757 )
2006
37
Hemidystonia as initial manifestation of sporadic Creutzfeldt-Jakob disease. ( 16796596 )
2006
38
Psychogenic paroxysmal dyskinesia following paroxysmal hemidystonia in multiple sclerosis. ( 16186511 )
2005
39
Hemidystonia precipitated by acute pontine infarct. ( 15935386 )
2005
40
The natural history and treatment of acquired hemidystonia: report of 33 cases and review of the literature. ( 11784827 )
2002
41
Delayed onset of hemidystonia and hemiballismus following head injury: a clinicopathological correlation. Case report. ( 11213970 )
2001
42
Coexistence of hemidystonia and hemiballism in a diabetic patient with striatal hyperintensity on T1-weighted MRI. ( 12013590 )
2001
43
Long-term follow-up study of chronic globus pallidus internus stimulation for posttraumatic hemidystonia. ( 10701534 )
2000
44
Putaminal necrosis presenting with hemidystonia. ( 10734254 )
2000
45
Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study. ( 11009203 )
2000
46
Hemidystonia and hemichoreoathetosis as an initial manifestation of moyamoya disease. ( 11030805 )
2000
47
Symptomatic paroxysmal hemidystonia due to a demyelinating subthalamic lesion. ( 11054143 )
2000
48
Hemidystonia secondary to cervical demyelinating lesions. ( 11054144 )
2000
49
Follow-up findings in regional cerebral blood flow (r-CBF)-SPECT in a case of idiopathic childhood hemidystonia. Functional neuroimaging and pathophysiological implications. ( 10100236 )
1999
50
Benedikt's syndrome with delayed-onset rubral tremor and hemidystonia: a complication of tic douloureux surgery. ( 10435512 )
1999

Variations for Hemidystonia

Expression for Hemidystonia

Search GEO for disease gene expression data for Hemidystonia.

Pathways for Hemidystonia

Pathways related to Hemidystonia according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.13 SNCA TOR1A

GO Terms for Hemidystonia

Biological processes related to Hemidystonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of angiogenesis GO:0045766 9.61 CAMP PRKD1 TWIST1
2 response to interferon-gamma GO:0034341 9.4 GCH1 SNCA
3 aortic valve morphogenesis GO:0003180 9.37 JAG1 TWIST1
4 synaptic vesicle transport GO:0048489 9.32 SNCA TOR1A
5 neuromuscular process controlling posture GO:0050884 9.26 GCH1 PNKD
6 dopamine biosynthetic process GO:0042416 9.16 GCH1 SNCA
7 negative regulation of histone acetylation GO:0035067 8.96 SNCA TWIST1
8 cardiac neural crest cell development involved in outflow tract morphogenesis GO:0061309 8.62 JAG1 TWIST1

Sources for Hemidystonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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