ORPHA:1183
Opsoclonus-myoclonus syndrome
Synonyms: Ataxo-opso-myoclonus syndrome · Dancing eye syndrome · Dancing eye-dancing feet syndrome · Kinsbourne syndrome · OMA syndrome · OMS · Opsoclonus-myoclonus-ataxia syndrome · POMA syndrome · Paraneoplastic opsoclonus-myoclonus · Paraneoplastic opsoclonus-myoclonus-ataxia syndrome
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood, Infancy
- Status
- active
Opsoclonus myoclonus syndrome (OMS) is a rare neuroinflammatory disease of paraneoplastic, parainfectious or idiopathic origin, characterized by opsoclonus, myoclonus, ataxia, and behavioral and sleep disorders.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Opsoclonus-myoclonus syndrome | Ataxo-opso-myoclonus syndrome · Dancing eye syndrome · Dancing eye-dancing feet syndrome · Kinsbourne syndrome · OMA syndrome · OMS · Opsoclonus-myoclonus-ataxia syndrome · POMA syndrome · Paraneoplastic opsoclonus-myoclonus · Paraneoplastic opsoclonus-myoclonus-ataxia syndrome |
| fr | Syndrome d'opsoclonie-myoclonie | OMP · OMS · Opsoclonus-myoclonus paranéoplasique · Syndrome OMA · Syndrome ataxo-opso-myoclonus · Syndrome d'opsoclonie-myoclonie-ataxie · Syndrome de Kinsbourne · Syndrome des yeux dansants · Syndrome néoplasique d'opsoclonie-myoclonie-ataxie · Syndrome opsoclonus-myoclonus · syndrome des yeux et des jambes dansantes |
| de | Opsoklonus-Myoklonus-Syndrom | Kinsbourne-Syndrom · OMA-Syndrom · OMS · Opsoklonie - Myoklonie - Ataxie · Opsomyoklonus-Ataxie-Syndrom · POMA-Syndrom · Paraneoplastische Opsoklonus-Myoklonus-Ataxie · Paraneoplastischer Opsomyoklonus |
| es | Síndrome opsoclono-mioclono | OMS · Opsoclono-mioclono paraneoplásico · Síndrome OMA · Síndrome POMA · Síndrome ataxo-opso-mioclono · Síndrome de Kinsbourne · Síndrome de los ojos bailarines · Síndrome de ojos bailarines-pies bailarines · Síndrome de opsoclono-mioclono-ataxia · Síndrome de opsoclono-mioclono-ataxia paraneoplásico |
| it | Sindrome opsoclono-mioclono | OMS · Opsoclono-mioclono, paraneoplastico · Sindrome OMA · Sindrome POMA · Sindrome da atasso-opso-mioclono · Sindrome degli ''occhi ballerini'' · Sindrome degli occhi e dei piedi ballerini · Sindrome di Kinsbourne · Sindrome opsoclono-mioclono-atassia · Sindrome opsoclono-mioclono-atassia, paraneoplastico |
| nl | Opsoclonus - myoclonus-syndroom | Ataxo-opso-myoclonussyndroom · Dancing eye - dancing feet-syndroom · Dancing eye-syndroom · OMA-syndroom · OMS · Opsoclonus - myoclonus - ataxie-syndroom · POMA-syndroom · Paraneoplastisch opsoclonus - myoclonus - ataxie-syndroom · Paraneoplastische opsoclonus-myoclonus · Syndroom van Kinsbourne |
| pt | Síndrome de opsoclonia-mioclonia | Síndrome Kinsbourne |
| pl | Zespół mioklonii i opsoklonii | OMS · Paraneoplastyczne opsoklonie i mioklonie · Paraneoplastyczny zespół ataksji, opsoklonii i mioklonii · Zespół ataksji, opsoklonii i mioklonii · Zespół Kinsbourne'a · Zespół OMA · Zespół POMA · Zespół tańczącego oka · Zespół tańczącego oka i tańczących stóp |
| cs | Syndrom zahrnující opsoklonus a myoklonus | Syndrom ataxoopsomyoklonus · OMA syndrom · OMS · Syndrom zahrnující opsoklonus, myoklonus a ataxii · POMA syndrom · Paraneoplastický opsoklonus a myoklonus · Paraneoplastický syndrom zahrnující opsoklonus, myoklonus a ataxii · Syndrom tančících očí · Syndrom tančících očí a nohou · Kinsbournův syndrom |
| uk | Синдром опсоклонус-міоклонус | Атаксо-опсо-міоклонусний синдром · Синдром танцюючих очей · Синдром танцюючих очей-танцюючих ніг · Синдром Кінсборна · Синдром ОМА · OMS · Синдром опсоклонус-міоклонус-атаксія · Паранеопластичний опсоклонус-міоклонус · Паранеопластичний синдром опсоклонус-міоклонус-атаксія · Синдром POMA |
| zh | 眼球阵挛-肌阵挛综合征 | 副肿瘤性斜视性眼肌阵挛-肌阵挛-共济失调综合征 |
| tr | Opsoklonus-miyoklonus sendromu | OMS · OMA sendromu · POMA sendromu · Kinsbourne sendromu · Dans eden göz sendromu · Atakso-opso-miyoklonus sendromu · Dans eden göz dansı ayak sendromu · Paraneoplastik opsoklonus-miyoklonus · Opsoklonus-miyoklonus-ataksi sendromu · Paraneoplastik opsoklonus-miyoklonus-ataksi sendromu |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | G25.3 | NTBT | attributed | validated |
| ICD-11 | 9C85.02 | NTBT | index | validated |
| MONDO | 0015247 | E | validated | |
| MeSH | D053578 | E | validated | |
| UMLS | C0393626 | E | validated | |
| GARD | 10009 | E | validated | |
| MedDRA | 10053854 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (4):
- ORPHA:102005Brain inflammatory disease
rare neurological diseases - ORPHA:36388Paraneoplastic neurologic syndrome
rare neurological diseases - ORPHA:519341Rare brainstem or cerebellar disorder with ophthalmic involvement as a major feature
rare ophthalmic disorders - ORPHA:306753Rare disease with myoclonus as a major feature
rare neurological diseases
No children.
4
Genes
0 directOrphanet records no gene directly on this code.
5
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.02 / 100 000 | Europe | Value and class | Validated | [EXPERT]_19423368[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Annual incidence | <1 / 1 000 000 | 0.018 / 100 000 | United Kingdom | Value and class | Validated | 19423368[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
6
HPO phenotypes
22 annotations · validated 2021-01-05| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0000570 | Abnormal saccadic eye movements | |
| HP:0001251 | Ataxia | |
| HP:0001336 | Myoclonus | |
| HP:0010543 | Opsoclonus | |
| HP:0002360 | Sleep abnormality | |
| Frequent (79-30%) | ||
| HP:5000002 | Anti-Amphiphysin antibody | |
| HP:0000708 | Atypical behavior | |
| HP:0100543 | Cognitive impairment | |
| HP:0000737 | Irritability | |
| HP:0045084 | Limb myoclonus | |
| HP:0002664 | Neoplasm | |
| HP:0003006 | Neuroblastoma | |
| HP:0002321 | Vertigo | |
| Occasional (29-5%) | ||
| HP:0030057 | Autoimmune antibody positivity | |
| HP:0003002 | Breast carcinoma | |
| HP:0031035 | Chronic infection | |
| HP:0001298 | Encephalopathy | |
| HP:0002861 | Melanoma | |
| HP:0100526 | Neoplasm of the lung | |
| HP:0012226 | Ovarian teratoma | |
| HP:0002063 | Rigidity | |
| HP:0030357 | Small cell lung carcinoma | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 30232989[PMID]_30483882[PMID]_23622330[PMID]
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1183
- This record as JSON: /data/d/1183.json