Idiopathic optic perineuritis
Synonyms: Idiopathic OPN
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
A rare ophthalmic disorder characterized by idiopathic orbital inflammation in which the specific target tissue is the optic nerve sheath. Patients typically present with ocular pain, pain on eye movement, visual symptoms with loss of vision progressing over several weeks, dyschromatopsia, and variable visual field defects. Orbital signs and symptoms may be present and include ptosis, ophthalmoplegia, and exophthalmos. Optic disc edema is observed in most cases. The condition is usually unilateral.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic optic perineuritis | Idiopathic OPN |
| fr | Périnévrite optique idiopathique | |
| de | Optikusperineuritis, idiopathische | OPN, idiopathische |
| es | Perineuritis óptica idiopática | OPN idiopática |
| it | Perineurite ottica idiopatica | |
| nl | Idiopathische perineuritis optica | Idiopathische OPN · Idiopathische optische perineuritis |
| pt | Perineurite óptica idiopática | |
| pl | Idiopatyczne okołonerwowe zapalenie nerwu wzrokowego | Samoistne okołonerwowe zapalenie nerwu wzrokowego |
| cs | Idiopatická perineuritida optiku | Idiopathic OPN · Idiopatická OPN |
| uk | Ідіопатичний периневрит зорового нерва | Ідіопатичний OPN |
| zh | 特发性视神经周围炎 | = en |
| tr | İdiyopatik optik perinevrit | İdiyopatik OPN |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H05.0 | NTBT | attributed | validated |
| ICD-11 | 9C40.3 | E | specific | validated |
| MONDO | 0044690 | E | validated | |
| MONDO | 44690 | E | validated | |
| UMLS | C5680093 | 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 (1):
- ORPHA:499047Autoimmune/inflammatory optic neuropathy
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/499107
- This record as JSON: /data/d/499107.json