Tolosa-Hunt syndrome
Synonyms: Painful ophthalmoplegia
- Type
- Disease
- Group
- Disorder
- Age of onset
- All ages
- Status
- active
Tolosa-Hunt syndrome is an ophthalmoplegic syndrome, affecting all age groups, characterized by acute attacks (lasting a few days to a few weeks) of periorbital pain, ipsilateral ocular motor nerve palsies, ptosis, disordered eye movements and blurred vision usually caused by a non-specific inflammatory process in the cavernous sinus and superior orbital fissure. It has an unpredicatable course with spontaneous remission occurring in some and recurrence of attacks in others.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Tolosa-Hunt syndrome | Painful ophthalmoplegia |
| fr | Syndrome de Tolosa-Hunt | Ophtalmoplégie douloureuse |
| de | Tolosa-Hunt-Syndrom | Ophthalmoplegie, schmerzhafte |
| es | Síndrome de Tolosa-Hunt | Oftalmoplejía dolorosa |
| it | Sindrome di Tolosa-Hunt | Oftalmoplegia dolorosa |
| nl | Syndroom van Tolosa-Hunt | Pijnlijke oftalmoplegie |
| pt | Síndrome de Tolosa-Hunt | Oftalmoplegia dolorosa |
| pl | Zespół Tolosa i Hunta | Oftalmoplegia bolesna |
| cs | Tolosův-Huntův syndrom | Bolestivá oftalmoplegie · Tolosa-Huntův syndrom |
| uk | Синдром Толоса-Ханта | Больова офтальмоплегія |
| zh | 痛性眼肌麻痹综合征 | 痛性眼肌麻痹 |
| tr | Tolosa-Hunt sendromu | Ağrılı oftalmopleji |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H49.8 | NTBT | attributed | validated |
| ICD-11 | 8A85 | NTBT | index | validated |
| MONDO | 0018983 | E | validated | |
| MeSH | D020333 | E | validated | |
| UMLS | C0040381 | E | validated | |
| GARD | 7777 | E | validated | |
| MedDRA | 10051526 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:522510Rare genetic ophthalmic disorder with cranial nerve involvement
rare genetic diseases - ORPHA:519349Rare ophthalmic disorder with cranial nerve involvement
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.1 / 100 000 | Worldwide | Value and class | Validated | 29083745[PMID]_ORPHANET |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | 29083745[PMID]_ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/64686
- This record as JSON: /data/d/64686.json