{"orpha":499085,"name":"Chronic relapsing inflammatory optic neuritis","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["CRION"],"definition":"A rare inflammatory optic neuropathy characterized by severe and persistent pain followed by subacute visual loss, a relapsing-remitting course, and steroid-dependence. Involvement of both optic nerves is common and is usually sequential. Serum antibodies against aquaporin 4 are absent in most cases. Magnetic resonance imaging shows contrast enhancement of the acutely inflamed optic nerves.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-499085/","orphanet_url":"https://www.orpha.net/en/disease/detail/499085","names":{"fr":{"name":"Névrite optique idiopathique récurrente chronique","synonyms":["CRION","Neuropathie optique inflammatoire isolée chronique récurrente","Neuropathie optique inflammatoire récidivante"],"definition":"Neuropathie optique inflammatoire rare caractérisée par une douleur aiguë et persistante suivie d'une baisse visuelle subaiguë, une évolution de type poussée-rémission et une dépendance aux stéroïdes. L'atteinte des deux nerfs optiques est fréquente et généralement séquentielle. Les anticorps sériques contre l'aquaporine 4 sont absents dans la plupart des cas. L'imagerie par résonance magnétique avec renforcement du contraste permet de visualiser les nerfs optiques en cas d'inflammation aiguë."},"de":{"name":"Optikusneuritis, chronisch rezidivierende, inflammatorische","synonyms":["CRION","Optikusneuritis, chronisch rezidivierende, isolierte"],"definition":"Eine seltene entzündliche Optikusneuropathie, die durch starke und anhaltende Schmerzen, gefolgt von subakutem Sehverlust, einem schubförmig-remittierenden Verlauf und Steroidabhängigkeit gekennzeichnet ist. Beide Sehnerven sind häufig betroffen und treten in der Regel nacheinander auf. Serumantikörper gegen Aquaporin 4 sind in den meisten Fällen nicht vorhanden. Die Magnetresonanztomographie zeigt eine Kontrastverstärkung der akut entzündeten Sehnerven."},"es":{"name":"Neuropatía óptica inflamatoria recurrente crónica","synonyms":["CRION","Neuritis óptica aislada recurrente crónica"],"definition":"Es una neuropatía óptica inflamatoria poco frecuente caracterizada por dolor intenso y persistente seguido de pérdida visual subaguda, curso remitente-recurrente y dependencia de esteroides. La afectación de ambos nervios ópticos es frecuente y suele ser secuencial. Los anticuerpos séricos contra la aquaporina 4 están ausentes en la mayoría de los casos. La resonancia magnética muestra un realce de contraste de los nervios ópticos con inflamación aguda."},"it":{"name":"Neuropatia ottica infiammatoria cronica recidivante","definition":"Si tratta di una neuropatia ottica infiammatoria rara, caratterizzata da dolore grave e persistente seguito dalla perdita subacuta della vista, con un decorso recidivante-remittente e dipendenza dagli steroidi. Di solito la malattia interessa entrambi i nervi ottici in maniera sequenziale. Nella maggior parte dei pazienti non sono presenti nel siero gli anticorpi diretti contro l'acquaporina 4. La risonanza magnetica mostra un rinforzo dei nervi ottici con infiammazione acuta dopo la somministrazione del mezzo di contrato."},"nl":{"name":"Chronische recidiverende inflammatoire opticusneuropathie","synonyms":["CRION","Chronische recidiverende inflammatoire optische neuropathie","Chronische terugkerende geïsoleerde opticusneuritis"],"definition":"Een zeldzame inflammatoire opticusneuropathie, gekarakteriseerd door ernstige en aanhoudende pijn, gevolgd door subacuut verlies van gezichtsvermogen, een recidiverend-herstellend verloop, en steroïdafhankelijkheid. Betrokkenheid van beide oogzenuwen is gangbaar en meestal sequentieel. Antilichamen tegen aquaporine 4 in serum zijn in de meeste gevallen afwezig. Beeldvorming met magnetische resonantie toont contrastversterking van acuut ontstoken oogzenuwen."},"pt":{"name":"Chronic relapsing inflammatory optic neuropathy","synonyms":["CRION","Neuropatia óptica inflamatória recorrente crónica"]},"pl":{"name":"Przewlekła nawracająca zapalna neuropatia tarczy nerwu wzrokowego","synonyms":["Przewlekłe nawracające zapalenie nerwu wzrokowego"]},"cs":{"name":"Chronická návratná zánětlivá neuropatie optiku","synonyms":["CRION","Chronic recurrent isolated optic neuritis","Chronická recidivující izolovaná optická neuritida"]},"uk":{"name":"Хронічний рецидивуючий запальний неврит зорового нерва","synonyms":["CRION"],"definition":"A rare inflammatory optic neuropathy characterized by severe and persistent pain followed by subacute visual loss, a relapsing-remitting course, and steroid-dependence. Involvement of both optic nerves is common and is usually sequential. Serum antibodies against aquaporin 4 are absent in most cases. Magnetic resonance imaging shows contrast enhancement of the acutely inflamed optic nerves."},"zh":{"name":"慢性复发性炎症性视神经病变","synonyms":["慢性复发性孤立性视神经炎"]},"tr":{"name":"Kronik tekrarlı inflamatuar optik nöropati","synonyms":["CRION","Kronik tekrarlayan izole optik nörit"]}},"xrefs":[{"source":"ICD-10","code":"H46","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0044687","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5567234","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":499096,"name":"Isolated optic neuritis","classifications":[181,189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}