OMS
MCID: OPS006
MIFTS: 48

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 17 72
Kinsbourne Syndrome 53 54 59 72
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:

MESH via Orphanet 45 D053578
ICD10 via Orphanet 34 G25.3
UMLS via Orphanet 73 C0393626 C1096154 C1721017
Orphanet 59 ORPHA1183
UMLS 72 C0393626 C1096154

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 viral encephalitis and neuroblastoma 1, 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 Prednisone 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 : 75 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 197)
# Related Disease Score Top Affiliating Genes
1 viral encephalitis 30.4 TNFSF13B CXCL10
2 neuroblastoma 1 11.9
3 myoclonic encephalopathy of infants 11.8
4 myoclonus 11.3
5 ataxia and polyneuropathy, adult-onset 11.0
6 aceruloplasminemia 10.7
7 encephalitis 10.7
8 myoclonus and ataxia 10.6
9 small cell cancer of the lung 10.5
10 dengue virus 10.5
11 west nile virus 10.5
12 gangliocytoma 10.5
13 teratoma 10.5
14 ganglioneuroblastoma 10.5
15 ganglioneuroma 10.5
16 encephalopathy 10.5
17 tremor 10.5
18 teratoma, ovarian 10.4
19 lung cancer 10.4
20 small cell carcinoma 10.4
21 ovarian germ cell teratoma 10.4
22 lymphoma 10.3
23 mutism 10.3
24 rare movement disorder 10.3
25 allergic encephalomyelitis 10.3 POMC CXCL10
26 heart block, congenital 10.3
27 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.3
28 suppression of tumorigenicity 12 10.3
29 human immunodeficiency virus type 1 10.3
30 malaria 10.3
31 neuroblastoma 3 10.3
32 chikungunya 10.3
33 exanthem 10.3
34 horner's syndrome 10.3
35 benign mesothelioma 10.3
36 differentiating neuroblastoma 10.3
37 malignant pleural mesothelioma 10.3
38 lupus erythematosus 10.3
39 autoimmune disease 10.3
40 breast cancer 10.3
41 ovarian cancer 10.3
42 lymphoma, hodgkin, classic 10.3
43 melanoma 10.3
44 gastroenteritis 10.3
45 breast adenocarcinoma 10.3
46 movement disease 10.3
47 dystonia 10.3
48 acute disseminated encephalomyelitis 10.3
49 b-cell lymphoma 10.3
50 chickenpox 10.3

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 38)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
2
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
3
rituximab Approved Phase 3 174722-31-7 10201696
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 Corticotropin-Releasing Hormone Phase 3
7 Antibodies Phase 3
8 gamma-Globulins Phase 3
9 Immunoglobulins Phase 3
10 Immunoglobulins, Intravenous Phase 3
11 Immunoglobulin G Phase 3
12 Rho(D) Immune Globulin Phase 3
13 Hormone Antagonists Phase 3
14 Antiemetics Phase 3
15 Gastrointestinal Agents Phase 3
16 Anti-Inflammatory Agents Phase 3
17 Alkylating Agents Phase 3
18 Peripheral Nervous System Agents Phase 3
19 HIV Protease Inhibitors Phase 3
20
protease inhibitors Phase 3
21 glucocorticoids Phase 3
22 Hormones Phase 3
23 Antineoplastic Agents, Hormonal Phase 3
24 Antineoplastic Agents, Immunological Phase 3
25 Immunosuppressive Agents Phase 3
26 Autonomic Agents Phase 3
27 BB 1101 Phase 3
28 Antirheumatic Agents Phase 3
29 Immunologic Factors Phase 3
30 Antineoplastic Agents, Alkylating Phase 3
31 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
32 Neurotransmitter Agents Phase 2
33 Adrenocorticotropic Hormone Phase 2
34 Melanocyte-Stimulating Hormones Phase 2
35 beta-endorphin Phase 2
36 Endorphins Phase 2
37
Tacrolimus Approved, Investigational 104987-11-3 445643 439492 6473866
38 Calcineurin Inhibitors

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Pilot Study Randomized Trial of Intravenous Gammaglobulin Therapy for Patients With Neuroblastoma Associated Opsoclonus-Myoclonus-Ataxia Syndrome Treated With Chemotherapy and Prednisone Completed NCT00033293 Phase 3 cyclophosphamide;prednisone;Corticotropin-Releasing Hormone
2 Multinational European Trial for Children With the Opsoclonus Myoclonus Syndrome / Dancing Eye Syndrome 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 A Phase I Clinical Trial of Rituximab for Pediatric Opsoclonus-Myoclonus Syndrome Completed NCT00244361 Phase 1, Phase 2 rituximab
5 An Open Label, Multi-centered, Randomized Phase 2 Study to Evaluate the Safety, Tolerability and Bioactivity of Subcutaneous ACTH GeL in PAtients With Scleritis: The ATLAS Study Recruiting NCT03465111 Phase 2 ACTH (adrenocorticotropic hormone) gel
6 Open Label Study of Safety and Tolerability of Rituximab in Neuromyelitis Optica, Recurrent Transverse Myelitis and Recurrent Bilateral Simultaneous Optic Neuritis Completed NCT00501748 Phase 1 Rituximab
7 Cytokines as Biomarkers and Therapeutic Targets in Paraneoplastic Opsoclonus-Myoclonus Syndrome (OMS) Completed NCT00806182
8 Immunotherapy of the Paraneoplastic Syndromes Completed NCT00378326 Tacrolimus
9 Neuroblastoma Biology Studies Recruiting NCT00904241

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, Breast, T Cells, Prostate

Publications for Opsoclonus-Myoclonus Syndrome

Articles related to Opsoclonus-Myoclonus Syndrome:

(show top 50) (show all 359)
# Title Authors PMID Year
1
Neurometabolic effects of ACTH on free amino compounds in opsoclonus-myoclonus syndrome. 9 38
18991196 2008
2
Monoaminergic effects of high-dose corticotropin in corticotropin-responsive pediatric opsoclonus-myoclonus. 9 38
9613746 1998
3
[Neurological complications associated with dengue virus infection]. 38
31310001 2019
4
Diagnostic value of whole-body MRI in Opsoclonus-myoclonus syndrome: a clinical case series (3 case reports). 38
31429709 2019
5
An upfront immunomodulatory therapy protocol for pediatric opsoclonus-myoclonus syndrome. 38
31033188 2019
6
Clinical Profile, Prognostic Indicators, and Therapeutic Outcomes of Pediatric Opsoclonus-Myoclonus-Ataxia Syndrome: A Single-Center Experience from South India. 38
31359941 2019
7
Update on opsoclonus-myoclonus syndrome in adults. 38
30483882 2019
8
Corrigendum: Treatment-refractory paraneoplastic opsoclonus-myoclonus syndrome in a patient with small cell carcinoma of the lung. 38
30907284 2019
9
IGF-1 alleviates serum IgG-induced neuronal cytolysis through PI3K signaling in children with opsoclonus-myoclonus syndrome and neuroblastoma. 38
30718793 2019
10
A novel exaggerated "spino-bulbo-spinal like" reflex of lower brainstem origin. 38
30316728 2019
11
Paraneoplastic Opsoclonus-Myoclonus Syndrome Preceding a Mucosal Malignant Melanoma. 38
30281137 2019
12
Paraneoplastic opsoclonus-myoclonus syndrome as a rare presentation of breast cancer. 38
30788093 2019
13
Treatment-refractory paraneoplastic opsoclonus-myoclonus syndrome in a patient with small-cell carcinoma of the lung. 38
30791855 2019
14
Opsoclonus in a child with neuroborreliosis: Case report and review of the literature. 38
30655046 2019
15
Paraneoplastic Opsoclonus Myoclonus in a Patient with Pancreatic Adenocarcinoma. 38
31139479 2019
16
Immune-mediated Cerebellar Ataxias: Practical Guidelines and Therapeutic Challenges. 38
30221603 2019
17
NMDA receptor antibody in teratoma-related opsoclonus-myoclonus syndrome. 38
30340968 2018
18
A case of adult-onset poststreptococcal opsoclonus-myoclonus syndrome. 38
29713906 2018
19
Opsoclonus Myoclonus Syndrome: A Rare Manifestation of Dengue Infection in a Child. 38
30937088 2018
20
Paraneoplastic movement disorders. 38
29561731 2018
21
Acute transverse myelitis following an opsoclonus-myoclonus syndrome: An unusual presentation. 38
29773357 2018
22
Opsoclonus myoclonus syndrome in children: Paraneoplastic versus parainfectious. 38
30232990 2018
23
Evaluation of Responsiveness to Reduced-Dose Rituximab in Corticotropin/Intravenous Immunoglobulin/Rituximab Combination Immunotherapy for Opsoclonus-Myoclonus Syndrome. 38
30197220 2018
24
Multifactorial analysis of opsoclonus-myoclonus syndrome etiology ("Tumor" vs. "No tumor") in a cohort of 356 US children. 38
29727049 2018
25
Relation of intrathecal oligoclonal band production to inflammatory mediator and immunotherapy response in 208 children with OMS. 38
29685330 2018
26
Exaggerated startle in post-infectious opsoclonus myoclonus syndrome. 38
29729591 2018
27
Effect of low-dose cyclophosphamide, ACTH, and IVIG combination immunotherapy on neuroinflammation in pediatric-onset OMS: A retrospective pilot study. 38
29555260 2018
28
Opsoclonus-myoclonus syndrome following long-term use of cyclosporine. 38
28905654 2018
29
Opsoclonus-Myoclonus Syndrome-Additional Clinical Considerations. 38
29522119 2018
30
Opsoclonus-Myoclonus Syndrome-Reply. 38
29522060 2018
31
Rituximab, IVIg, and Tetracosactide (ACTH1-24) Combination Immunotherapy ("RITE-CI") for Pediatric Opsoclonus-Myoclonus Syndrome: Immunomarkers and Clinical Observations. 38
29258131 2018
32
Genomic Profiles of Neuroblastoma Associated With Opsoclonus Myoclonus Syndrome. 38
29135842 2018
33
Autoimmune and paraneoplastic movement disorders: An update. 38
29406902 2018
34
Kinsbourne syndrome associated with cryptococcosis infection. 38
29174170 2018
35
A case report of sphenoid sinusitis causing opsoclonus myoclonus syndrome. 38
30596202 2018
36
Opsoclonus-Myoclonus Syndrome Associated With West-Nile Virus Infection: Case Report and Review of the Literature. 38
30386288 2018
37
Corrigendum to "Dexamethasone, Intravenous Immunoglobulin, and Rituximab Combination Immunotherapy for Pediatric Opsoclonus-Myoclonus Syndrome" [Pediatric Neurology 73 (2017) 48-56]. 38
29169865 2018
38
Immune-mediated ataxias. 38
29891069 2018
39
[Psychological development of children with opsoclonus myoclonus syndrome and its correlation with age and disease onset]. 38
30141794 2018
40
Sleep-Related Laryngeal Stridor in Opsoclonus Myoclonus Syndrome. 38
28823798 2017
41
Brain volumetric analysis and cortical thickness in adults with saccadic intrusions (ocular flutter or opsoclonus-myoclonus syndrome). 38
29121544 2017
42
6-Mercaptopurine modifies cerebrospinal fluid T cell abnormalities in paediatric opsoclonus-myoclonus as steroid sparer. 38
28710878 2017
43
Microglial/macrophage markers CHI3L1, sCD14, and sCD163 in CSF and serum of pediatric inflammatory and non-inflammatory neurological disorders: A case-control study and reference ranges. 38
28991699 2017
44
A Toddler With Uncontrollable Shaking After a Minor Fall. 38
26466152 2017
45
Childhood opsoclonus-myoclonus syndrome: A case series from Tunisia. 38
28549713 2017
46
Opsoclonus myoclonus syndrome in a patient with Japanese encephalitis: a case report. 38
29058581 2017
47
Clinicopathological features of neuroblastic tumors with opsoclonus-myoclonus-ataxia syndrome: Follicular structure predicts a better neurological outcome. 38
28971571 2017
48
Opsoclonus-Myoclonus Syndrome during Rituximab Treatment for Autoimmune Autonomic Ganglionopathy. 38
28740856 2017
49
Benign opsoclonus myoclonus syndrome and prostate cancer. 38
28958399 2017
50
[Effectiveness and safety of rituximab for children with autoimmune diseases of the nervous system]. 38
28881516 2017

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 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10
2
Show member pathways
13.27 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10
3
Show member pathways
13.24 POMC CXCR3 CXCL9 CXCL11 CXCL10
4
Show member pathways
12.99 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10
5
Show member pathways
12.8 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10
6
Show member pathways
12.55 TNFSF13B TNFSF13 CXCL9 CXCL11
7
Show member pathways
12.55 CXCR3 CXCL9 CXCL11 CXCL10
8
Show member pathways
11.88 CXCR3 CXCL9 CXCL11 CXCL10
9
Show member pathways
11.79 CXCR3 CXCL9 CXCL11 CXCL10
10 11.69 TNFSF13B TNFSF13 CXCL10
11 11.54 TNFSF13B TNFSF13
12
Show member pathways
11.43 TNFSF13B TNFSF13
13 11.43 CXCL9 CXCL11 CXCL10
14 11.12 CXCL9 CXCL10
15 11 TNFSF13B TNFSF13
16 10.62 TNFSF13B TNFSF13 CXCL9 CXCL11 CXCL10
17 10.58 TNFSF13B TNFSF13 CXCL9 CXCL11 CXCL10
18 10.46 CXCR3 CXCL9 CXCL11 CXCL10

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 TNFSF13B TNFSF13 POMC GPI CXCL9 CXCL11
2 external side of plasma membrane GO:0009897 9.33 CXCR3 CXCL9 CXCL10
3 extracellular space GO:0005615 9.17 TNFSF13B TNFSF13 POMC GPI CXCL9 CXCL11

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

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 9.93 POMC CXCR3 CXCL9 CXCL11 CXCL10
2 signal transduction GO:0007165 9.87 TNFSF13B TNFSF13 POMC CXCR3 CXCL9 CXCL11
3 inflammatory response GO:0006954 9.8 CXCR3 CXCL9 CXCL11 CXCL10
4 regulation of cell proliferation GO:0042127 9.73 CXCL9 CXCL11 CXCL10
5 cell-cell signaling GO:0007267 9.73 POMC CXCL9 CXCL11 CXCL10
6 cellular response to lipopolysaccharide GO:0071222 9.72 CXCL9 CXCL11 CXCL10
7 chemotaxis GO:0006935 9.71 CXCR3 CXCL9 CXCL11 CXCL10
8 defense response GO:0006952 9.7 CXCL9 CXCL11 CXCL10
9 adenylate cyclase-activating G protein-coupled receptor signaling pathway GO:0007189 9.69 CXCL9 CXCL11 CXCL10
10 neutrophil chemotaxis GO:0030593 9.65 CXCL9 CXCL11 CXCL10
11 antimicrobial humoral immune response mediated by antimicrobial peptide GO:0061844 9.63 CXCL9 CXCL11 CXCL10
12 cell chemotaxis GO:0060326 9.58 CXCR3 CXCL10
13 tumor necrosis factor-mediated signaling pathway GO:0033209 9.57 TNFSF13B TNFSF13
14 positive regulation of release of sequestered calcium ion into cytosol GO:0051281 9.54 CXCL9 CXCL11 CXCL10
15 leukocyte chemotaxis GO:0030595 9.43 CXCL9 CXCL11 CXCL10
16 T cell chemotaxis GO:0010818 9.33 CXCR3 CXCL11 CXCL10
17 chemokine-mediated signaling pathway GO:0070098 9.26 CXCR3 CXCL9 CXCL11 CXCL10
18 immune response GO:0006955 9.1 TNFSF13B TNFSF13 CXCR3 CXCL9 CXCL11 CXCL10

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 TNFSF13B TNFSF13 POMC CXCL10
2 cytokine activity GO:0005125 9.35 TNFSF13B TNFSF13 GPI CXCL9 CXCL10
3 chemokine activity GO:0008009 9.33 CXCL9 CXCL11 CXCL10
4 tumor necrosis factor receptor binding GO:0005164 9.32 TNFSF13B TNFSF13
5 CXCR3 chemokine receptor binding GO:0048248 8.8 CXCL9 CXCL11 CXCL10

Sources for Opsoclonus-Myoclonus Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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