MCID: HMD003
MIFTS: 29

Hemidystonia

Categories: Neuronal diseases

Aliases & Classifications for Hemidystonia

MalaCards integrated aliases for Hemidystonia:

Name: Hemidystonia 12 15 71

Classifications:



External Ids:

Disease Ontology 12 DOID:0050846
UMLS 71 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 hereditary dystonia and hemiplegia. An important gene associated with Hemidystonia is THAP1 (THAP Domain Containing 1), and among its related pathways/superpathways is Folate biosynthesis. Affiliated tissues include brain, globus pallidus and thalamus, and related phenotypes are behavior/neurological and nervous system

Related Diseases for Hemidystonia

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

(show top 50) (show all 109)
# Related Disease Score Top Affiliating Genes
1 hereditary dystonia 30.5 GCH1 ATP1A3
2 hemiplegia 30.4 PNKD CACNA1A ATP1A3
3 torticollis 30.4 TOR1A CACNA1A
4 choreatic disease 28.8 SGCE PNKD LGI1 JRK GYPE GCH1
5 dystonia 27.9 TOR1A THAP1 TAF1 SPR SGCE PNKD
6 blepharospasm 27.6 TOR1A THAP1 TAF1 SGCE JRK GNAL
7 movement disease 26.7 TOR1A THAP1 TAF1 SGCE PNKD JRK
8 focal dystonia 26.6 TOR1A THAP1 TAF1L TAF1 SGCE JRK
9 hemidystonia-hemiatrophy syndrome 12.4
10 spasmodic dysphonia 10.4 TOR1A THAP1
11 conversion disorder 10.3 PNKD GCH1
12 dyskinetic cerebral palsy 10.3 PNKD JRK
13 brain injury 10.3
14 head injury 10.3
15 traumatic brain injury 10.3
16 spinocerebellar ataxia 27 10.2 PNKD CACNA1A
17 tetrahydrobiopterin deficiency 10.2 SPR GCH1
18 aromatic l-amino acid decarboxylase deficiency 10.2 SPR GCH1
19 reflex epilepsy 10.2 PNKD LGI1
20 arteriovenous malformation 10.2
21 tremor 10.2
22 lingual-facial-buccal dyskinesia 10.2 LGI1 JRK
23 hemiplegic migraine 10.2 CACNA1A ATP1A3
24 cortical deafness 10.2 LGI1 ATP1A3
25 episodic kinesigenic dyskinesia 1 10.2 PNKD JRK
26 episodic ataxia, type 1 10.2 PNKD CACNA1A
27 mutism 10.1 LGI1 ATP1A3
28 episodic ataxia, type 2 10.1 PNKD CACNA1A
29 moyamoya disease 1 10.1
30 aphasia 10.1
31 antiphospholipid syndrome 10.1
32 cerebrovascular disease 10.1
33 spasticity 10.1
34 hemiparkinsonism-hemiatrophy syndrome 10.1
35 oculogyric crisis 10.1 SPR GCH1 ATP1A3
36 episodic ataxia 10.1 PNKD CACNA1A ATP1A3
37 dystonia, dopa-responsive, due to sepiapterin reductase deficiency 10.1 SPR GCH1
38 cranio-facial dystonia 10.1 TOR1A THAP1 SGCE GCH1
39 lymphatic malformation 5 10.1 TOR1A THAP1 SGCE GCH1
40 hereditary lymphedema ii 10.1 TOR1A THAP1 SGCE GCH1
41 neurodegeneration with brain iron accumulation 10.0 TOR1A THAP1 GCH1 ATP1A3
42 obsessive-compulsive disorder 10.0 TOR1A SGCE GCH1
43 internuclear ophthalmoplegia 10.0 LGI1 JRK CACNA1A
44 photosensitive epilepsy 10.0 LGI1 JRK CACNA1A
45 torsion dystonia 4 10.0 GNAL ANO3
46 creutzfeldt-jakob disease 10.0
47 multiple sclerosis 10.0
48 neurofibromatosis, type iv, of riccardi 10.0
49 sarcoidosis 1 10.0
50 trigeminal neuralgia 10.0

Graphical network of the top 20 diseases related to Hemidystonia:



Diseases related to Hemidystonia

Symptoms & Phenotypes for Hemidystonia

MGI Mouse Phenotypes related to Hemidystonia:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.61 ATP1A3 CACNA1A GNAL LGI1 PNKD SGCE
2 nervous system MP:0003631 9.28 ATP1A3 CACNA1A GCH1 LGI1 PNKD SGCE

Drugs & Therapeutics for Hemidystonia

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Humanitarian Device Exemption for Medtronic Activa Dystonia Therapy Approved for marketing NCT00580658
2 Characteristics and Mechanism of Childhood-Onset Hemidystonia Completed NCT01432899

Search NIH Clinical Center for Hemidystonia

Genetic Tests for Hemidystonia

Anatomical Context for Hemidystonia

MalaCards organs/tissues related to Hemidystonia:

40
Brain, Globus Pallidus, Thalamus, Eye, Cortex, Cerebellum, Parietal Lobe

Publications for Hemidystonia

Articles related to Hemidystonia:

(show top 50) (show all 191)
# Title Authors PMID Year
1
Paroxismal non-kinesigenic dyskinesia and hemidystonia associated with silent celiac disease. 61
31710883 2020
2
Hemidystonia in a Neonate. 61
30922774 2019
3
Gamma Knife Radiosurgical Pallidotomy for Dystonia: Not a Fallen Angel. 61
31857549 2019
4
[Formula: see text]Neurocognitive outcomes in children with unilateral basal ganglia arterial ischemic stroke and secondary hemidystonia. 61
28701080 2018
5
Mineralizing Vasculopathy Causing Motor Delay and Silent Strokes? 61
30937092 2018
6
Mineralizing Angiopathy with Basal Ganglia Stroke after Minor Trauma: Case Series Including Two Familial Cases. 61
30937087 2018
7
Simultaneous Treatment of Epilepsy and Secondary Dystonia After Anterior Temporal Lobectomy and Amygdalohippocampectomy. 61
29649646 2018
8
Atypical Glutaric Aciduria Type I with Hemidystonia and Asymmetric Radiological Findings Misdiagnosed as an Ischemic Stroke. 61
30838298 2018
9
Tractography study in a patient with hemidystonia-hemiatrophy syndrome. 61
30746400 2018
10
Progressive delayed hemidystonia following clinically mild traumatic brain injury. 61
29288226 2017
11
Unilateral thalamic and pallidal deep brain stimulation for idiopathic hemidystonia: results of individual and combined stimulations. Case report. 61
28669294 2017
12
Multimodal Imaging in a Patient with Hemidystonia Responsive to GPi Deep Brain Stimulation. 61
28744382 2017
13
Hemidystonia in a Child With Neurofibromatosis Type 1. 61
27898639 2016
14
Hemidystonia - hemiatrophy syndrome in Domenico Morone's Madonna with child. 61
26736116 2016
15
Recurrent episodes of isolated hemidystonia in a young adult: Dissection-associated stenosis in the middle cerebral artery. 61
27374977 2016
16
Globus Pallidus Internus Deep Brain Stimulation for Traumatic Hemidystonia Following Penetrating Head Injury. 61
27184900 2016
17
Fluctuating off-period hemidystonia with Pisa syndrome in advanced Parkinson's disease. 61
27423577 2016
18
Clinical Heterogeneity in Cerebral Hemiatrophy Syndromes. 61
30713929 2016
19
GPi deep brain stimulation for palliation of hemidystonia and hemibody jerking in a patient with suspected adult onset neuronal ceroid lipofuscinosis. 61
26944153 2016
20
Comment on: Hemidystonia caused by frontal cortical infarction. 61
26607166 2016
21
Author's Reply: Hemidystonia caused by frontal cortical infarction. 61
26538401 2016
22
(Pseudo)hemidystonia associated with anti-glutamic acid decarboxylase antibodies--a case report. 61
25846894 2015
23
A prospective, randomized, blinded assessment of multitarget thalamic and pallidal deep brain stimulation in a case of hemidystonia. 61
26241157 2015
24
Hemidystonia caused by frontal cortical infarction. 61
25119274 2015
25
Prochlorperazine-Induced Hemidystonia Mimicking Acute Stroke. 61
26265971 2015
26
The reorganization of motor network in hemidystonia from the perspective of deep brain stimulation. 61
24728838 2015
27
Anesthesia for deep brain stimulation in traumatic brain injury-induced hemidystonia. 61
26185655 2015
28
A practical approach to management of focal hand dystonia. 61
26019409 2015
29
Hemidystonia with one eye-of-the-tiger sign. 61
25347119 2014
30
Alternating hemidystonia following traumatic brain injury as an unusual presentation of paroxysmal autonomic instability with dystonia syndrome. 61
25414239 2014
31
Hemiballism-hemidystonia after parietal lobe tumour resection. 61
25128656 2014
32
Mineralizing angiopathy with infantile basal ganglia stroke after minor trauma. 61
24102209 2014
33
Secondary and primary dystonia: pathophysiological differences. 61
23771342 2013
34
Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia. 61
23452222 2013
35
Progressive dystonia. 61
23622412 2013
36
A rat model of hemidystonia induced by 3-nitropropionic acid. 61
24194961 2013
37
Use of pallidal deep brain stimulation in postinfarct hemidystonia. 61
23549056 2013
38
Pallidal deep brain stimulation for a case of hemidystonia secondary to a striatal stroke. 61
23446229 2013
39
Behind the screen: pseudobulbar symptoms after deep brain stimulation. 61
23652655 2013
40
[Treatment of dystonia by deep brain stimulation: a summary of 40 cases]. 61
23074845 2012
41
The long-term surgical outcomes of secondary hemidystonia associated with post-traumatic brain injury. 61
22367408 2012
42
Stereotypies as a manifestation of acute hyperglycemia without ketosis. 61
22123156 2012
43
Long-term outcome of atlas-based lesion of posterior zona incerta in secondary hemidystonia. 61
21641267 2011
44
Hemidystonia in uncontrolled type 2 diabetes mellitus. 61
21555647 2011
45
Intrathecal baclofen and motor function in cerebral palsy. 61
21480874 2011
46
Isolated hemidystonia associated with NMDA receptor antibodies. 61
21412839 2011
47
Clinical phenomenology of dystonia. 61
21907098 2011
48
Genetics and pharmacological treatment of dystonia. 61
21907099 2011
49
Posttraumatic hemidystonia treated with unilateral globus pallidus interna stimulation: Long-term follow-up and radiologic features. 61
21992879 2010
50
Clustering of dystonia in some pedigrees with autosomal dominant essential tremor suggests the existence of a distinct subtype of essential tremor. 61
20670416 2010

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
Show member pathways
10.09 SPR GCH1

GO Terms for Hemidystonia

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

# Name GO ID Score Top Affiliating Genes
1 response to pain GO:0048265 9.32 GCH1 CACNA1A
2 neuromuscular process controlling posture GO:0050884 9.26 PNKD GCH1
3 nitric oxide biosynthetic process GO:0006809 9.16 SPR GCH1
4 cofactor metabolic process GO:0051186 8.96 SPR GCH1
5 tetrahydrobiopterin biosynthetic process GO:0006729 8.62 SPR GCH1

Molecular functions related to Hemidystonia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 TBP-class protein binding GO:0017025 8.96 TAF1L TAF1
2 lysine-acetylated histone binding GO:0070577 8.62 TAF1L TAF1

Sources for Hemidystonia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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