MCID: OPS006
MIFTS: 52

Opsoclonus-Myoclonus Syndrome

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 54 60 56 17 74
Kinsbourne Syndrome 54 55 60 74
Paraneoplastic Opsoclonus-Myoclonus-Ataxia Syndrome 54 60
Opsoclonus-Myoclonus-Ataxia Syndrome 54 60
Paraneoplastic Opsoclonus-Myoclonus 54 60
Dancing Eye-Dancing Feet Syndrome 54 60
Ataxo-Opso-Myoclonus Syndrome 54 60
Dancing Eye Syndrome 54 60
Poma Syndrome 54 60
Oma Syndrome 54 60
Oms 54 60
Dancing Eyes-Dancing Feet Syndrome 55
Opsoclonus Myoclonus Syndrome 54
Opsoclonus Myoclonus 55

Characteristics:

Orphanet epidemiological data:

60
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:

MESH via Orphanet 46 D053578
ICD10 via Orphanet 35 G25.3
UMLS via Orphanet 75 C0393626 C1096154 C1721017
Orphanet 60 ORPHA1183

Summaries for Opsoclonus-Myoclonus Syndrome

NIH Rare Diseases : 54 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, prostate and thyroid, and related phenotypes are hematopoietic system and immune system

NINDS : 55 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 : 77 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 118)
# Related Disease Score Top Affiliating Genes
1 myoclonic encephalopathy of infants 11.4
2 myoclonus 11.2
3 ataxia and polyneuropathy, adult-onset 11.0
4 small cell cancer of the lung 10.5
5 lung cancer 10.5
6 encephalitis 10.5
7 dengue virus 10.5
8 neuroblastoma 1 10.4
9 west nile virus 10.4
10 teratoma 10.4
11 atrial standstill 1 10.2
12 heart block, congenital 10.2
13 malaria 10.2
14 chikungunya 10.2
15 meningoencephalitis 10.2
16 lyme disease 10.2
17 benign mesothelioma 10.2
18 malignant pleural mesothelioma 10.2
19 rubella 10.2
20 lupus erythematosus 10.2
21 breast cancer 10.2
22 aceruloplasminemia 10.2
23 small cell carcinoma 10.2
24 lymphoma 10.2
25 melanoma 10.2
26 ovarian germ cell teratoma 10.2
27 allergic encephalomyelitis 10.2 CXCL10 POMC
28 encephalopathy 10.2
29 thyroid carcinoma, familial medullary 10.2
30 thyroid cancer 10.2
31 scleredema adultorum 10.1 CXCL9 POMC
32 myoclonus and ataxia 10.1
33 sydenham chorea 10.1 CXCL10 CXCL9
34 mucinoses 10.1 CXCL9 POMC
35 viral encephalitis 10.1 CXCL10 TNFSF13B
36 apple allergy 10.1 CXCL10 CXCL11
37 epidermolysis bullosa simplex, dowling-meara type 10.1 CXCL10 CXCL9
38 renal cell carcinoma, nonpapillary 10.1
39 multiple system atrophy 1 10.1
40 prostate cancer 10.1
41 myasthenia gravis 10.1
42 prostate cancer, hereditary, 8 10.1
43 anxiety 10.1
44 ovarian cancer 1 10.1
45 prostate cancer, hereditary, 6 10.1
46 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.1
47 diabetes mellitus 10.1
48 hepatitis 10.1
49 sinusitis 10.1
50 lambert-eaton myasthenic syndrome 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:

47
# 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 38)
# 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 50-18-0, 6055-19-2 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 Antineoplastic Agents, Immunological Phase 3,Phase 2,Phase 1
9 Immunoglobulins, Intravenous Phase 3
10 glucocorticoids Phase 3
11 Hormone Antagonists Phase 3
12 Adrenocorticotropic Hormone Phase 3
13 Antineoplastic Agents, Alkylating Phase 3
14 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
15 Immunoglobulin G Phase 3
16 Antineoplastic Agents, Hormonal Phase 3
17 Endorphins Phase 3
18 Corticotropin-Releasing Hormone Phase 3
19 Hormones Phase 3
20 Antibodies Phase 3
21 Rho(D) Immune Globulin Phase 3
22 Immunosuppressive Agents Phase 3,Not Applicable
23 Alkylating Agents Phase 3
24 gamma-Globulins Phase 3
25 Anti-Inflammatory Agents Phase 3
26 Immunoglobulins Phase 3
27 Melanocyte-Stimulating Hormones Phase 3
28 beta-endorphin Phase 3
29 Neurotransmitter Agents Phase 3
30 Autonomic Agents Phase 3
31 Peripheral Nervous System Agents Phase 3
32 Antiemetics Phase 3
33 Gastrointestinal Agents Phase 3
34 HIV Protease Inhibitors Phase 3
35 BB 1101 Phase 3
36
protease inhibitors Phase 3
37
Tacrolimus Approved, Investigational Not Applicable 104987-11-3 6473866 445643 439492
38 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:

42
Eye, Prostate, Thyroid, Brain, B Cells, T Cells, Heart

Publications for Opsoclonus-Myoclonus Syndrome

Articles related to Opsoclonus-Myoclonus Syndrome:

(show top 50) (show all 214)
# Title Authors Year
1
Paraneoplastic opsoclonus-myoclonus syndrome as a rare presentation of breast cancer. ( 30788093 )
2019
2
Paraneoplastic Opsoclonus-Myoclonus Syndrome Preceding a Mucosal Malignant Melanoma. ( 30281137 )
2019
3
Update on opsoclonus-myoclonus syndrome in adults. ( 30483882 )
2019
4
IGF-1 alleviates serum IgG-induced neuronal cytolysis through PI3K signaling in children with opsoclonus-myoclonus syndrome and neuroblastoma. ( 30718793 )
2019
5
Treatment-refractory paraneoplastic opsoclonus-myoclonus syndrome in a patient with small-cell carcinoma of the lung. ( 30791855 )
2019
6
Corrigendum: Treatment-refractory paraneoplastic opsoclonus-myoclonus syndrome in a patient with small cell carcinoma of the lung. ( 30907284 )
2019
7
An upfront immunomodulatory therapy protocol for pediatric opsoclonus-myoclonus syndrome. ( 31033188 )
2019
8
Acute transverse myelitis following an opsoclonus-myoclonus syndrome: An unusual presentation. ( 29773357 )
2018
9
Kinsbourne syndrome associated with cryptococcosis infection. ( 29174170 )
2018
10
Evaluation of Responsiveness to Reduced-Dose Rituximab in Corticotropin/Intravenous Immunoglobulin/Rituximab Combination Immunotherapy for Opsoclonus-Myoclonus Syndrome. ( 30197220 )
2018
11
Opsoclonus myoclonus syndrome in children: Paraneoplastic versus parainfectious. ( 30232990 )
2018
12
NMDA receptor antibody in teratoma-related opsoclonus-myoclonus syndrome. ( 30340968 )
2018
13
Opsoclonus-Myoclonus Syndrome Associated With West-Nile Virus Infection: Case Report and Review of the Literature. ( 30386288 )
2018
14
A case report of sphenoid sinusitis causing opsoclonus myoclonus syndrome. ( 30596202 )
2018
15
Opsoclonus Myoclonus Syndrome: A Rare Manifestation of Dengue Infection in a Child. ( 30937088 )
2018
16
Opsoclonus-myoclonus syndrome following long-term use of cyclosporine. ( 28905654 )
2018
17
Genomic Profiles of Neuroblastoma Associated With Opsoclonus Myoclonus Syndrome. ( 29135842 )
2018
18
Corrigendum to "Dexamethasone, Intravenous Immunoglobulin, and Rituximab Combination Immunotherapy for Pediatric Opsoclonus-Myoclonus Syndrome" [Pediatric Neurology 73 (2017) 48-56]. ( 29169865 )
2018
19
Rituximab, IVIg, and Tetracosactide (ACTH1-24) Combination Immunotherapy ("RITE-CI") for Pediatric Opsoclonus-Myoclonus Syndrome: Immunomarkers and Clinical Observations. ( 29258131 )
2018
20
Opsoclonus-Myoclonus Syndrome-Reply. ( 29522060 )
2018
21
Opsoclonus-Myoclonus Syndrome-Additional Clinical Considerations. ( 29522119 )
2018
22
A case of adult-onset poststreptococcal opsoclonus-myoclonus syndrome. ( 29713906 )
2018
23
Multifactorial analysis of opsoclonus-myoclonus syndrome etiology ("Tumor" vs. "No tumor") in a cohort of 356 US children. ( 29727049 )
2018
24
Exaggerated startle in post-infectious opsoclonus myoclonus syndrome. ( 29729591 )
2018
25
Opsoclonus-Myoclonus Syndrome during Rituximab Treatment for Autoimmune Autonomic Ganglionopathy. ( 28740856 )
2017
26
Opsoclonus myoclonus syndrome in a patient with Japanese encephalitis: a case report. ( 29058581 )
2017
27
Dexamethasone, Intravenous Immunoglobulin, and Rituximab Combination Immunotherapy for Pediatric Opsoclonus-Myoclonus Syndrome. ( 28651977 )
2017
28
Sleep-Related Laryngeal Stridor in Opsoclonus Myoclonus Syndrome. ( 28823798 )
2017
29
Benign opsoclonus myoclonus syndrome and prostate cancer. ( 28958399 )
2017
30
Demographic, Clinical, and Immunologic Features of 389 Children with Opsoclonus-Myoclonus Syndrome: A Cross-sectional Study. ( 28959231 )
2017
31
Rapid Resolution of Enterovirus 71-Associated Opsoclonus Myoclonus Syndrome on Intravenous Immunoglobulin. ( 28975137 )
2017
32
Brain volumetric analysis and cortical thickness in adults with saccadic intrusions (ocular flutter or opsoclonus-myoclonus syndrome). ( 29121544 )
2017
33
Adult-onset opsoclonus-myoclonus syndrome due to West Nile Virus treated with intravenous immunoglobulin. ( 27473195 )
2017
34
Altered functional brain connectivity in children and young people with opsoclonus-myoclonus syndrome. ( 27658927 )
2017
35
Pediatric opsoclonus-myoclonus syndrome: the role of functional brain connectivity studies. ( 27730631 )
2017
36
Longitudinal multi-modal neuroimaging in opsoclonus-myoclonus syndrome. ( 28054126 )
2017
37
Transient symptomatic worsening by atropine in opsoclonus-myoclonus syndrome. ( 28102628 )
2017
38
Teaching Video NeuroImages: Maneuvers to elicit opsoclonus in opsoclonus-myoclonus syndrome. ( 28416624 )
2017
39
Postintervention acute opsoclonus myoclonus syndrome. ( 28432174 )
2017
40
Opsoclonus-Myoclonus Syndrome: A New Era of Improved Prognosis? ( 28479124 )
2017
41
Childhood opsoclonus-myoclonus syndrome: A case series from Tunisia. ( 28549713 )
2017
42
Opsoclonus-Myoclonus Syndrome in Primary Central Nervous System Lymphoma. ( 27023309 )
2016
43
18F-FDG PET/CT Brain Imaging on a Patient With Paraneoplastic Opsoclonus-Myoclonus Syndrome Arising out of a Mature Cystic Teratoma. ( 26545025 )
2016
44
Adult-onset Opsoclonus-Myoclonus Syndrome Associated With Ganglionic Acetylcholine Receptor Autoantibody. ( 27801769 )
2016
45
Rituximab treatment for relapsed opsoclonus-myoclonus syndrome. ( 26375512 )
2016
46
Early Detection and Treatment of Neuroblastic Tumor with Opsoclonus-Myoclonus Syndrome Improve Neurological Outcome: A Review of Five Cases at a Single Institution in Japan. ( 26409265 )
2016
47
Opsoclonus-Myoclonus Syndrome, Neuroblastoma, and Insulin-Dependent Diabetes Mellitus in a Child: A Unique Patient. ( 26778146 )
2016
48
Trends and tenets in relapsing and progressive opsoclonus-myoclonus syndrome. ( 26786246 )
2016
49
New Findings in Adult Opsoclonus-Myoclonus Syndrome. ( 26856251 )
2016
50
Clinical and Immunological Features of Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies. ( 26856612 )
2016

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 18)
# 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
13.24 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 11.53 TNFSF13 TNFSF13B
11
Show member pathways
11.52 CXCL10 CXCL11 CXCL9 CXCR3
12
Show member pathways
11.43 TNFSF13 TNFSF13B
13 11.43 CXCL10 CXCL11 CXCL9
14 11.12 CXCL10 CXCL9
15 11 TNFSF13 TNFSF13B
16 10.62 CXCL10 CXCL11 CXCL9 TNFSF13 TNFSF13B
17 10.58 CXCL10 CXCL11 CXCL9 TNFSF13 TNFSF13B
18 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 19)
# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 9.95 CXCL10 CXCL11 CXCL9 CXCR3 POMC
2 signal transduction GO:0007165 9.91 CXCL10 CXCL11 CXCL9 CXCR3 POMC TNFSF13
3 inflammatory response GO:0006954 9.81 CXCL10 CXCL11 CXCL9 CXCR3
4 cell-cell signaling GO:0007267 9.76 CXCL10 CXCL11 CXCL9 POMC
5 regulation of cell proliferation GO:0042127 9.74 CXCL10 CXCL11 CXCL9
6 cellular response to lipopolysaccharide GO:0071222 9.73 CXCL10 CXCL11 CXCL9
7 chemotaxis GO:0006935 9.73 CXCL10 CXCL11 CXCL9 CXCR3
8 defense response GO:0006952 9.71 CXCL10 CXCL11 CXCL9
9 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.7 CXCL10 CXCL11 CXCL9
10 neutrophil chemotaxis GO:0030593 9.67 CXCL10 CXCL11 CXCL9
11 antimicrobial humoral immune response mediated by antimicrobial peptide GO:0061844 9.65 CXCL10 CXCL11 CXCL9
12 cell chemotaxis GO:0060326 9.58 CXCL10 CXCR3
13 tumor necrosis factor-mediated signaling pathway GO:0033209 9.58 TNFSF13 TNFSF13B
14 positive regulation of release of sequestered calcium ion into cytosol GO:0051281 9.58 CXCL10 CXCL11 CXCL9
15 leukocyte chemotaxis GO:0030595 9.5 CXCL10 CXCL11 CXCL9
16 chemokine-mediated signaling pathway GO:0070098 9.46 CXCL10 CXCL11 CXCL9 CXCR3
17 T cell chemotaxis GO:0010818 9.43 CXCL10 CXCL11 CXCR3
18 immune response GO:0006955 9.43 CXCL10 CXCL11 CXCL9 CXCR3 TNFSF13 TNFSF13B
19 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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