OMS
MCID: OPS006
MIFTS: 51

Opsoclonus-Myoclonus Syndrome (OMS)

Categories: Endocrine diseases, Eye diseases, Infectious diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Opsoclonus-Myoclonus Syndrome

MalaCards integrated aliases for Opsoclonus-Myoclonus Syndrome:

Name: Opsoclonus-Myoclonus Syndrome 53 59 55 73
Kinsbourne Syndrome 53 54 59 73
Paraneoplastic Opsoclonus-Myoclonus-Ataxia Syndrome 53 59
Opsoclonus-Myoclonus-Ataxia Syndrome 53 59
Paraneoplastic Opsoclonus-Myoclonus 53 59
Dancing Eye-Dancing Feet Syndrome 53 59
Ataxo-Opso-Myoclonus Syndrome 53 59
Dancing Eye Syndrome 53 59
Poma Syndrome 53 59
Oma Syndrome 53 59
Oms 53 59
Dancing Eyes-Dancing Feet Syndrome 54
Opsoclonus Myoclonus Syndrome 53
Opsoclonus Myoclonus 54

Characteristics:

Orphanet epidemiological data:

59
opsoclonus-myoclonus syndrome
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe),<1/1000000 (United Kingdom); Age of onset: Childhood,Infancy; Age of death: normal life expectancy;

Classifications:



External Ids:

Orphanet 59 ORPHA1183
ICD10 via Orphanet 34 G25.3
MESH via Orphanet 45 D053578
UMLS via Orphanet 74 C0393626 C1096154 C1721017

Summaries for Opsoclonus-Myoclonus Syndrome

NIH Rare Diseases : 53 Opsoclonus-myoclonus syndrome(OMS) is a rare disorder that affects the nervous system. Symptoms include rapid, multi-directional eye movements (opsoclonus), quick, involuntary muscle jerks (myoclonus), uncoordinated movement (ataxia), irritability, and sleep disturbance. The onset of OMS is usually abrupt and often severe. The disease may become chronic. OMS typically occurs in association with tumors (neuroblastomas), or following a viral or bacterial infection. Treatment may include corticosteroids or ACTH (adrenocorticotropic hormone). When there is a tumor present, treatment may include chemotherapy, surgery, and/or radiation. In some cases, when the underlying cause of OMS is treated, symptoms improve.

MalaCards based summary : Opsoclonus-Myoclonus Syndrome, also known as kinsbourne syndrome, is related to myoclonic encephalopathy of infants and myoclonus, and has symptoms including myoclonus and ophthalmoplegia. An important gene associated with Opsoclonus-Myoclonus Syndrome is POMC (Proopiomelanocortin), and among its related pathways/superpathways are ERK Signaling and PEDF Induced Signaling. The drugs rituximab and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include eye, lung and brain, and related phenotypes are hematopoietic system and immune system

NINDS : 54 Opsoclonus myoclonus is a rare neurological disorder characterized by an unsteady, trembling gait, myoclonus (brief, shock-like muscle spasms), and opsoclonus (irregular, rapid eye movements). Other symptoms may include difficulty speaking, poorly articulated speech, or an inability to speak. A decrease in muscle tone, lethargy, irritability, and malaise (a vague feeling of bodily discomfort) may also be present. Opsoclonus myoclonus may occur in association with tumors or viral infections. It is often seen in children with tumors.

Wikipedia : 76 Opsoclonus Myoclonus Syndrome (OMS), also known as Opsoclonus-Myoclonus-Ataxia (OMA), is a rare... more...

Related Diseases for Opsoclonus-Myoclonus Syndrome

Diseases related to Opsoclonus-Myoclonus Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 112)
# Related Disease Score Top Affiliating Genes
1 myoclonic encephalopathy of infants 11.4
2 myoclonus 11.2
3 ataxia and polyneuropathy, adult-onset 10.8
4 neuroblastoma 10.7
5 small cell cancer of the lung 10.5
6 lung cancer 10.5
7 encephalitis 10.5
8 dengue virus 10.5
9 west nile virus 10.4
10 teratoma 10.4
11 viral infectious disease 10.4
12 atrial standstill 1 10.2
13 heart block, congenital 10.2
14 malaria 10.2
15 chikungunya 10.2
16 meningoencephalitis 10.2
17 lyme disease 10.2
18 malignant pleural mesothelioma 10.2
19 rubella 10.2
20 lupus erythematosus 10.2
21 aceruloplasminemia 10.2
22 lymphoma 10.2
23 melanoma 10.2
24 ovarian germ cell teratoma 10.2
25 myoclonus and ataxia 10.1
26 viral encephalitis 10.1 CXCL10 TNFSF13B
27 apple allergy 10.1 CXCL10 CXCL11
28 scleredema adultorum 10.1 CXCL9 POMC
29 mucinoses 10.1 CXCL9 POMC
30 sydenham chorea 10.1 CXCL10 CXCL9
31 breast cancer 10.1
32 renal cell carcinoma, nonpapillary 10.1
33 multiple system atrophy 1 10.1
34 prostate cancer 10.1
35 myasthenia gravis 10.1
36 anxiety 10.1
37 ovarian cancer 1 10.1
38 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.1
39 diabetes mellitus 10.1
40 hepatitis 10.1
41 lambert-eaton myasthenic syndrome 10.1
42 salmonellosis 10.1
43 mumps 10.1
44 japanese encephalitis 10.1
45 locked-in syndrome 10.1
46 squamous cell carcinoma 10.1
47 hepatitis c 10.1
48 malignant fibroxanthoma 10.1
49 gastroenteritis 10.1
50 west nile encephalitis 10.1

Graphical network of the top 20 diseases related to Opsoclonus-Myoclonus Syndrome:



Diseases related to Opsoclonus-Myoclonus Syndrome

Symptoms & Phenotypes for Opsoclonus-Myoclonus Syndrome

UMLS symptoms related to Opsoclonus-Myoclonus Syndrome:


myoclonus, ophthalmoplegia

MGI Mouse Phenotypes related to Opsoclonus-Myoclonus Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.43 CXCL10 CXCR3 GPI POMC TNFSF13 TNFSF13B
2 immune system MP:0005387 9.1 CXCL10 CXCR3 GPI POMC TNFSF13 TNFSF13B

Drugs & Therapeutics for Opsoclonus-Myoclonus Syndrome

Drugs for Opsoclonus-Myoclonus Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 36)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
rituximab Approved Phase 3,Phase 2,Phase 1 174722-31-7 10201696
2
Cyclophosphamide Approved, Investigational Phase 3 6055-19-2, 50-18-0 2907
3
Prednisone Approved, Vet_approved Phase 3,Not Applicable 53-03-2 5865
4
Dexamethasone Approved, Investigational, Vet_approved Phase 3 50-02-2 5743
5
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 3 1177-87-3
6 Immunologic Factors Phase 3,Phase 2,Phase 1,Not Applicable
7 Antirheumatic Agents Phase 3,Phase 2,Phase 1
8 Alkylating Agents Phase 3
9 Antineoplastic Agents, Alkylating Phase 3
10 Hormones Phase 3
11 gamma-Globulins Phase 3
12 Immunoglobulins, Intravenous Phase 3
13 glucocorticoids Phase 3
14 Immunoglobulins Phase 3
15 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
16 beta-endorphin Phase 3
17 Antineoplastic Agents, Hormonal Phase 3
18 Melanocyte-Stimulating Hormones Phase 3
19 Anti-Inflammatory Agents Phase 3
20 Hormone Antagonists Phase 3
21 Antibodies Phase 3
22 Endorphins Phase 3
23 Rho(D) Immune Globulin Phase 3
24 Adrenocorticotropic Hormone Phase 3
25 Neurotransmitter Agents Phase 3
26 Immunosuppressive Agents Phase 3,Not Applicable
27 Corticotropin-Releasing Hormone Phase 3
28
protease inhibitors Phase 3
29 Peripheral Nervous System Agents Phase 3
30 Autonomic Agents Phase 3
31 Antiemetics Phase 3
32 BB 1101 Phase 3
33 HIV Protease Inhibitors Phase 3
34 Gastrointestinal Agents Phase 3
35
Tacrolimus Approved, Investigational Not Applicable 104987-11-3 445643 439492
36 Calcineurin Inhibitors Not Applicable

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Cyclophosphamide and Prednisone With or Without Immunoglobulin in Treating Abnormal Muscle Movement in Children With Neuroblastoma Completed NCT00033293 Phase 3 cyclophosphamide;prednisone;Corticotropin-Releasing Hormone
2 Opsoclonus Myoclonus Syndrome/Dancing Eye Syndrome (OMS/DES) in Children With and Without Neuroblastoma (NBpos and NBneg)Opsoclonus Myoclonus Syndrome/Dancing Eye Syndrome (OMS/DES) in Children With and Without Neuroblastoma (NBpos and NBneg) Recruiting NCT01868269 Phase 3 Dexamethasone acetate;dexamethasone and cyclophosphamide;dexamethasone and rituximab
3 Use of Rituximab in Opsoclonus-Myoclonus in Children With Neuroblastoma Unknown status NCT00202930 Phase 2 anti-CD20 (Rituximab)
4 Effectiveness of Rituximab in Pediatric OMS Patients. Completed NCT00244361 Phase 1, Phase 2 rituximab
5 Study of Cytokines in Children With Opsoclonus-Myoclonus Syndrome Completed NCT00806182
6 Immunotherapy of the Paraneoplastic Syndromes Completed NCT00378326 Not Applicable Tacrolimus

Search NIH Clinical Center for Opsoclonus-Myoclonus Syndrome

Genetic Tests for Opsoclonus-Myoclonus Syndrome

Anatomical Context for Opsoclonus-Myoclonus Syndrome

MalaCards organs/tissues related to Opsoclonus-Myoclonus Syndrome:

41
Eye, Lung, Brain, B Cells, Prostate, Heart, Endothelial

Publications for Opsoclonus-Myoclonus Syndrome

Articles related to Opsoclonus-Myoclonus Syndrome:

(show top 50) (show all 203)
# Title Authors Year
1
Acute transverse myelitis following an opsoclonus-myoclonus syndrome: An unusual presentation. ( 29773357 )
2018
2
A case of adult-onset poststreptococcal opsoclonus-myoclonus syndrome. ( 29713906 )
2018
3
Opsoclonus-Myoclonus Syndrome-Reply. ( 29522060 )
2018
4
Exaggerated startle in post-infectious opsoclonus myoclonus syndrome. ( 29729591 )
2018
5
Opsoclonus-Myoclonus Syndrome-Additional Clinical Considerations. ( 29522119 )
2018
6
Corrigendum to &amp;quot;Dexamethasone, Intravenous Immunoglobulin, and Rituximab Combination Immunotherapy for Pediatric Opsoclonus-Myoclonus Syndrome&amp;quot; [Pediatric Neurology 73 (2017) 48-56]. ( 29169865 )
2018
7
Multifactorial analysis of opsoclonus-myoclonus syndrome etiology (&amp;quot;Tumor&amp;quot; vs. &amp;quot;No tumor&amp;quot;) in a cohort of 356 US children. ( 29727049 )
2018
8
Kinsbourne syndrome associated with cryptococcosis infection. ( 29174170 )
2018
9
Paraneoplastic Opsoclonus-myoclonus Syndrome Preceding a Mucosal Malignant Melanoma. ( 30281137 )
2018
10
Genomic Profiles of Neuroblastoma Associated With Opsoclonus Myoclonus Syndrome. ( 29135842 )
2018
11
Evaluation of Responsiveness to Reduced-Dose Rituximab in Corticotropin/Intravenous Immunoglobulin/Rituximab Combination Immunotherapy for Opsoclonus-Myoclonus Syndrome. ( 30197220 )
2018
12
Opsoclonus myoclonus syndrome in children: Paraneoplastic versus parainfectious. ( 30232990 )
2018
13
NMDA receptor antibody in teratoma-related opsoclonus-myoclonus syndrome. ( 30340968 )
2018
14
Opsoclonus-Myoclonus Syndrome Associated With West-Nile Virus Infection: Case Report and Review of the Literature. ( 30386288 )
2018
15
Update on opsoclonus-myoclonus syndrome in adults. ( 30483882 )
2018
16
Rapid Resolution of Enterovirus 71-Associated Opsoclonus Myoclonus Syndrome on Intravenous Immunoglobulin. ( 28975137 )
2017
17
Teaching Video NeuroImages: Maneuvers to elicit opsoclonus in opsoclonus-myoclonus syndrome. ( 28416624 )
2017
18
Brain volumetric analysis and cortical thickness in adults with saccadic intrusions (ocular flutter or opsoclonus-myoclonus syndrome). ( 29121544 )
2017
19
Opsoclonus-myoclonus syndrome following long-term use of cyclosporine. ( 28905654 )
2017
20
Longitudinal multi-modal neuroimaging in opsoclonus-myoclonus syndrome. ( 28054126 )
2017
21
Postintervention acute opsoclonus myoclonus syndrome. ( 28432174 )
2017
22
Opsoclonus myoclonus syndrome in a patient with Japanese encephalitis: a case report. ( 29058581 )
2017
23
Opsoclonus-Myoclonus Syndrome during Rituximab Treatment for Autoimmune Autonomic Ganglionopathy. ( 28740856 )
2017
24
Dexamethasone, Intravenous Immunoglobulin, and Rituximab Combination Immunotherapy for Pediatric Opsoclonus-Myoclonus Syndrome. ( 28651977 )
2017
25
Demographic, Clinical, and Immunologic Features of 389 Children with Opsoclonus-Myoclonus Syndrome: A Cross-sectional Study. ( 28959231 )
2017
26
Rituximab, IVIg, and Tetracosactide (ACTH1-24) Combination Immunotherapy (&amp;quot;RITE-CI&amp;quot;) for Pediatric Opsoclonus-Myoclonus Syndrome: Immunomarkers and Clinical Observations. ( 29258131 )
2017
27
Childhood opsoclonus-myoclonus syndrome: A case series from Tunisia. ( 28549713 )
2017
28
Sleep-Related Laryngeal Stridor in Opsoclonus Myoclonus Syndrome. ( 28823798 )
2017
29
Opsoclonus-Myoclonus Syndrome: A New Era of Improved Prognosis? ( 28479124 )
2017
30
Transient symptomatic worsening by atropine in opsoclonus-myoclonus syndrome. ( 28102628 )
2017
31
Benign opsoclonus myoclonus syndrome and prostate cancer. ( 28958399 )
2017
32
Primary pancreatic neuroblastoma presenting with opsoclonus-myoclonus syndrome. ( 26973724 )
2016
33
Clinical and Immunological Features of Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies. ( 26856612 )
2016
34
New Findings in Adult Opsoclonus-Myoclonus Syndrome. ( 26856251 )
2016
35
Adult-onset Opsoclonus-Myoclonus Syndrome Associated With Ganglionic Acetylcholine Receptor Autoantibody. ( 27801769 )
2016
36
Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies: A Message for Clinicians. ( 27159217 )
2016
37
Trends and tenets in relapsing and progressive opsoclonus-myoclonus syndrome. ( 26786246 )
2016
38
Adult-onset opsoclonus-myoclonus syndrome due to West Nile Virus treated with intravenous immunoglobulin. ( 27473195 )
2016
39
Genetic features of neuroblastic tumors associated with opsoclonus-myoclonus syndrome opens up the possibility for detection in peripheral blood. ( 26949993 )
2016
40
Opsoclonus-Myoclonus Syndrome, Neuroblastoma, and Insulin-Dependent Diabetes Mellitus in a Child: A Unique Patient. ( 26778146 )
2016
41
Altered functional brain connectivity in children and young people with opsoclonus-myoclonus syndrome. ( 27658927 )
2016
42
Sensory Processing Difficulties in Opsoclonus-Myoclonus Syndrome: A Pilot Project of Presentation and Possible Prevalence. ( 26994071 )
2016
43
Pediatric opsoclonus-myoclonus syndrome: the role of functional brain connectivity studies. ( 27730631 )
2016
44
Opsoclonus-Myoclonus Syndrome in Primary Central Nervous System Lymphoma. ( 27023309 )
2016
45
Childhood opsoclonus-myoclonus syndrome: diagnosis and treatment. ( 27095464 )
2016
46
Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies-Reply. ( 27159281 )
2016
47
Anesthesia in a child with Kinsbourne syndrome: Does anesthesia technique matters? ( 27833499 )
2016
48
Opsoclonus-myoclonus syndrome associated with a nasopharyngeal tumor in an adult: a case report. ( 26033370 )
2015
49
Opsoclonus-myoclonus syndrome in a patient with an anaplastic oligoastrocytoma. ( 25864100 )
2015
50
18F-FDG PET/CT Brain Imaging on a Patient With Paraneoplastic Opsoclonus-Myoclonus Syndrome Arising Out of a Mature Cystic Teratoma. ( 26545025 )
2015

Variations for Opsoclonus-Myoclonus Syndrome

Expression for Opsoclonus-Myoclonus Syndrome

Search GEO for disease gene expression data for Opsoclonus-Myoclonus Syndrome.

Pathways for Opsoclonus-Myoclonus Syndrome

Pathways related to Opsoclonus-Myoclonus Syndrome according to GeneCards Suite gene sharing:

(show all 17)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.48 CXCL10 CXCL11 CXCL9 CXCR3 TNFSF13 TNFSF13B
2
Show member pathways
13.27 CXCL10 CXCL11 CXCL9 CXCR3 TNFSF13 TNFSF13B
3
Show member pathways
13.22 CXCL10 CXCL11 CXCL9 CXCR3 POMC
4
Show member pathways
12.99 CXCL10 CXCL11 CXCL9 CXCR3 TNFSF13 TNFSF13B
5
Show member pathways
12.8 CXCL10 CXCL11 CXCL9 CXCR3 TNFSF13 TNFSF13B
6
Show member pathways
12.55 CXCL11 CXCL9 TNFSF13 TNFSF13B
7
Show member pathways
12.55 CXCL10 CXCL11 CXCL9 CXCR3
8
Show member pathways
11.88 CXCL10 CXCL11 CXCL9 CXCR3
9 11.69 CXCL10 TNFSF13 TNFSF13B
10
Show member pathways
11.52 CXCL10 CXCL11 CXCL9 CXCR3
11
Show member pathways
11.43 TNFSF13 TNFSF13B
12 11.43 CXCL10 CXCL11 CXCL9
13 11.12 CXCL10 CXCL9
14 11 TNFSF13 TNFSF13B
15 10.62 CXCL10 CXCL11 CXCL9 TNFSF13 TNFSF13B
16 10.58 CXCL10 CXCL11 CXCL9 TNFSF13 TNFSF13B
17 10.46 CXCL10 CXCL11 CXCL9 CXCR3

GO Terms for Opsoclonus-Myoclonus Syndrome

Cellular components related to Opsoclonus-Myoclonus Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.5 CXCL10 CXCL11 CXCL9 GPI POMC TNFSF13
2 external side of plasma membrane GO:0009897 9.33 CXCL10 CXCL9 CXCR3
3 extracellular space GO:0005615 9.17 CXCL10 CXCL11 CXCL9 GPI POMC TNFSF13

Biological processes related to Opsoclonus-Myoclonus Syndrome according to GeneCards Suite gene sharing:

(show all 20)
# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 9.96 CXCL10 CXCL11 CXCL9 CXCR3 POMC
2 signal transduction GO:0007165 9.95 CXCL10 CXCL11 CXCL9 CXCR3 POMC TNFSF13
3 inflammatory response GO:0006954 9.83 CXCL10 CXCL11 CXCL9 CXCR3
4 cell-cell signaling GO:0007267 9.78 CXCL10 CXCL11 CXCL9 POMC
5 chemotaxis GO:0006935 9.76 CXCL10 CXCL11 CXCL9 CXCR3
6 regulation of cell proliferation GO:0042127 9.75 CXCL10 CXCL11 CXCL9
7 cellular response to lipopolysaccharide GO:0071222 9.74 CXCL10 CXCL11 CXCL9
8 defense response GO:0006952 9.72 CXCL10 CXCL11 CXCL9
9 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.71 CXCL10 CXCL11 CXCL9
10 neutrophil chemotaxis GO:0030593 9.69 CXCL10 CXCL11 CXCL9
11 antimicrobial humoral immune response mediated by antimicrobial peptide GO:0061844 9.67 CXCL10 CXCL11 CXCL9
12 positive regulation of release of sequestered calcium ion into cytosol GO:0051281 9.61 CXCL10 CXCL11 CXCL9
13 cell chemotaxis GO:0060326 9.59 CXCL10 CXCR3
14 tumor necrosis factor-mediated signaling pathway GO:0033209 9.58 TNFSF13 TNFSF13B
15 leukocyte chemotaxis GO:0030595 9.54 CXCL10 CXCL11 CXCL9
16 positive regulation of cAMP-mediated signaling GO:0043950 9.5 CXCL10 CXCL11 CXCL9
17 chemokine-mediated signaling pathway GO:0070098 9.46 CXCL10 CXCL11 CXCL9 CXCR3
18 T cell chemotaxis GO:0010818 9.43 CXCL10 CXCL11 CXCR3
19 immune response GO:0006955 9.43 CXCL10 CXCL11 CXCL9 CXCR3 TNFSF13 TNFSF13B
20 regulation of signaling receptor activity GO:0010469 9.17 CXCL10 CXCL11 CXCL9 GPI POMC TNFSF13

Molecular functions related to Opsoclonus-Myoclonus Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 signaling receptor binding GO:0005102 9.56 CXCL10 POMC TNFSF13 TNFSF13B
2 cytokine activity GO:0005125 9.35 CXCL10 CXCL9 GPI TNFSF13 TNFSF13B
3 chemokine activity GO:0008009 9.33 CXCL10 CXCL11 CXCL9
4 tumor necrosis factor receptor binding GO:0005164 9.32 TNFSF13 TNFSF13B
5 CXCR3 chemokine receptor binding GO:0048248 8.8 CXCL10 CXCL11 CXCL9

Sources for Opsoclonus-Myoclonus Syndrome

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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