{"orpha":499107,"name":"Idiopathic optic perineuritis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Idiopathic OPN"],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-499107/","orphanet_url":"https://www.orpha.net/en/disease/detail/499107","names":{"fr":{"name":"Périnévrite optique idiopathique","definition":"Maladie ophtalmique rare caractérisée par une inflammation orbitaire idiopathique dans laquelle le tissu cible spécifique est la gaine du nerf optique. Les patients présentent généralement une douleur oculaire, une douleur lors des mouvements des yeux, des troubles visuels avec perte de la vision progressive sur plusieurs semaines, une dyschromatopsie et des anomalies variables du champ visuel. On peut observer des signes cliniques et des symptômes d'une atteinte orbitaire, notamment un ptosis, une ophtalmoplégie et une exophtalmie. Dans la plupart des cas, on observe un oedème de la papille optique. La maladie est généralement unilatérale."},"de":{"name":"Optikusperineuritis, idiopathische","synonyms":["OPN, idiopathische"],"definition":"Eine seltene Augenerkrankung, die durch eine idiopathische Entzündung der Augenhöhle gekennzeichnet ist, bei der das spezifische Zielgewebe die Sehnervenscheide ist. Die Patienten zeigen typischerweise Augenschmerzen, Schmerzen bei Augenbewegungen, visuelle Symptome mit über mehrere Wochen fortschreitendem Sehverlust, Dyschromatopsie und variable Gesichtsfeldausfälle. Orbitale Anzeichen und Symptome können vorhanden sein und umfassen Ptosis, Ophthalmoplegie und Exophthalmus. In den meisten Fällen wird ein Ödem des Sehnervenkopfes beobachtet. Die Erkrankung ist in der Regel unilateral."},"es":{"name":"Perineuritis óptica idiopática","synonyms":["OPN idiopática"],"definition":"Es un trastorno oftálmico poco frecuente caracterizado por enfermedad inflamatoria orbitaria idiopática en la que el tejido diana específico es la vaina del nervio óptico. Los pacientes suelen presentar dolor ocular, dolor al mover los ojos, síntomas visuales con pérdida de visión que progresa durante varias semanas, discromatopsia y defectos variables del campo visual. También puede ir acompañado de signos y síntomas orbitarios, incluyendo ptosis, oftalmoplejía y exoftalmos. En la mayoría de los casos se observa edema del disco óptico. La entidad suele ser unilateral."},"it":{"name":"Perineurite ottica idiopatica","definition":"Si tratta di una malattia oculare rara, caratterizzata da un'infiammazione idiopatica dell'orbita che interessa la guaina del nervo ottico. Di solito i pazienti presentano dolore oculare, dolore al movimento oculare, sintomi visivi con perdita della vista che progredisce nell'arco di alcune settimane, discromatopsia e difetti variabili del campo visivo. I pazienti possono presentare anche sintomi orbitali (ptosi, oftalmoplegia ed esoftalmo). La maggior parte dei pazienti presenta un edema del disco ottico. In genere la malattia è monolaterale."},"nl":{"name":"Idiopathische perineuritis optica","synonyms":["Idiopathische OPN","Idiopathische optische perineuritis"],"definition":"Een zeldzame aandoening van oog, gekarakteriseerd door idiopathische inflammatie van orbita waarbij specifiek zenuwschede van nervus opticus aangetast wordt. Patiënten vertonen doorgaans pijn in oog, pijn bij oogbeweging, visuele symptomen met progressie naar verlies van gezichtsvermogen gedurende meerdere weken, dyschromatopsie, en variabele defecten van gezichtsveld. Orbitale verschijnselen en symptomen kunnen aanwezig zijn, waaronder ptose, oftalmoplegie, en exoftalmie. Papiloedeem wordt in de meeste gevallen waargenomen. De aandoening is meestal unilateraal."},"pt":{"name":"Perineurite óptica idiopática"},"pl":{"name":"Idiopatyczne okołonerwowe zapalenie nerwu wzrokowego","synonyms":["Samoistne okołonerwowe zapalenie nerwu wzrokowego"]},"cs":{"name":"Idiopatická perineuritida optiku","synonyms":["Idiopathic OPN","Idiopatická OPN"]},"uk":{"name":"Ідіопатичний периневрит зорового нерва","synonyms":["Ідіопатичний OPN"],"definition":"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."},"zh":{"name":"特发性视神经周围炎","synonyms":["Idiopathic OPN"]},"tr":{"name":"İdiyopatik optik perinevrit","synonyms":["İdiyopatik OPN"]}},"xrefs":[{"source":"ICD-10","code":"H05.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"9C40.3","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"349248275"},{"source":"MONDO","code":"0044690","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"44690","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680093","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":499047,"name":"Autoimmune/inflammatory optic neuropathy","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}