{"orpha":64686,"name":"Tolosa-Hunt syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Painful ophthalmoplegia"],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-64686/","orphanet_url":"https://www.orpha.net/en/disease/detail/64686","names":{"fr":{"name":"Syndrome de Tolosa-Hunt","synonyms":["Ophtalmoplégie douloureuse"],"definition":"Le syndrome de Tolosa-Hunt est un syndrome d'ophtalmoplégie douloureuse, atteignant tous les groupes d'âge, caractérisé par des crises aiguës (d'une durée allant de quelques jours à quelques semaines) de douleurs périorbitaires, une paralysie du nerf oculomoteur homolatéral, un ptosis, des mouvements désordonnés des yeux et une vision brouillée généralement secondaires à un processus inflammatoire non spécifique du sinus caverneux ou de la fissure orbitaire supérieure. Son évolution est imprévisible avec des remissions spontanées dans certains cas et des crises récidivantes dans d'autres cas."},"de":{"name":"Tolosa-Hunt-Syndrom","synonyms":["Ophthalmoplegie, schmerzhafte"],"definition":"Das Tolosa-Hunt-Syndrom ist ein in allen Altersgruppen auftretendes ophthalmoplegisches Syndrom. Symptome sind (i) akute (wenige Tage bis wenige Wochen dauernde) Attacken mit periorbitalen Schmerzen, (ii) ipsilaterale Lähmungen der motorischen Augenmuskeln mit Ptose und gestörten Augenbewegungen und (iii) verschwommener Visus, der in der Regel durch unspezifische entzündliche Prozesse im Sinus cavernosus und in der oberen Orbitalfissur verursacht ist. Der Verlauf kann nicht vorausgesagt werden: Möglich sind spontane Remissionen bei einigen Patienten oder Rezidive der Attacken bei anderen."},"es":{"name":"Síndrome de Tolosa-Hunt","synonyms":["Oftalmoplejía dolorosa"],"definition":"El síndrome de Tolosa-Hunt es un síndrome oftalmopléjico, caracterizado por ataques agudos (que duran desde unos pocos días a unas pocas semanas) de dolor periorbital, parálisis ipsilateral del nervio motor ocular, ptosis, movimientos oculares desordenados y visión borrosa normalmente causado por un proceso inflamatorio no específico en el seno cavernoso y la fisura orbital superior. Afecta a todos los grupos de edad. Tiene un curso impredecible con remisiones espontáneas en algunos casos y recurrencia de los ataques en otros."},"it":{"name":"Sindrome di Tolosa-Hunt","synonyms":["Oftalmoplegia dolorosa"],"definition":"La sindrome di Tolosa-Hunt è una sindrome oftalmoplegica, che colpisce tutte le età; è caratterizzata da attacchi acuti (che durano da pochi giorni a poche settimane) di dolore periorbitale, paralisi ipsilaterali dei nervi motori oculari, ptosi, movimenti oculari disordinati e vista appannata, di solito a causa di un processo infiammatorio non specifico nel seno cavernoso e nella fessura orbitale superiore. Ha un decorso imprevedibile: in alcuni casi, si verifica la remissione spontanea, mentre in altri gli attacchi possono ripetersi."},"nl":{"name":"Syndroom van Tolosa-Hunt","synonyms":["Pijnlijke oftalmoplegie"],"definition":"Een oftalmoplegisch syndroom, dat alle leeftijdsgroepen treft, en gekenmerkt wordt door acute aanvallen (die enkele dagen tot weken duren) van periorbitale pijn, ipsilaterale oculaire motorische zenuwverlammingen, ptosis, verstoorde oogbewegingen en troebel zicht, meestal veroorzaakt door een aspecifiek inflammatoir proces in sinus cavernosus en bovenste orbitaspleet. Het ziekteverloop is onvoorspelbaar, met spontane remissie bij sommigen en recidief van aanvallen bij anderen."},"pt":{"name":"Síndrome de Tolosa-Hunt","synonyms":["Oftalmoplegia dolorosa"]},"pl":{"name":"Zespół Tolosa i Hunta","synonyms":["Oftalmoplegia bolesna"]},"cs":{"name":"Tolosův-Huntův syndrom","synonyms":["Bolestivá oftalmoplegie","Tolosa-Huntův syndrom"]},"uk":{"name":"Синдром Толоса-Ханта","synonyms":["Больова офтальмоплегія"],"definition":"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."},"zh":{"name":"痛性眼肌麻痹综合征","synonyms":["痛性眼肌麻痹"]},"tr":{"name":"Tolosa-Hunt sendromu","synonyms":["Ağrılı oftalmopleji"]}},"xrefs":[{"source":"ICD-10","code":"H49.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8A85","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"969826782"},{"source":"MONDO","code":"0018983","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D020333","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0040381","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7777","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10051526","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97966,"name":"Rare ophthalmic disorder"},"parents":[{"orpha":522510,"name":"Rare genetic ophthalmic disorder with cranial nerve involvement","classifications":[156]},{"orpha":519349,"name":"Rare ophthalmic disorder with cranial nerve involvement","classifications":[189]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.1,"area":"Worldwide","status":"Validated","source":"29083745[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"29083745[PMID]_ORPHANET"}],"phenotypes":null}