{"orpha":238624,"name":"Idiopathic intracranial hypertension","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Benign intracranial hypertension","IIH","Pseudotumor cerebri"],"definition":"Idiopathic intracranial hypertension is a neurological disorder characterized by isolated increased intracranial pressure manifesting with recurrent and persistent headaches, nausea, vomiting, progressive and transient obstruction of the visual field, papilledema. Visual loss can be irreversible.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-238624/","orphanet_url":"https://www.orpha.net/en/disease/detail/238624","names":{"fr":{"name":"Hypertension intracrânienne idiopathique","synonyms":["HII","Hypertension intracrânienne bénigne","IIH","Pseudotumeur cérébrale","Pseudotumor cerebri"],"definition":"L'hypertension intracrânienne idiopathique est un trouble neurologique caractérisé par une augmentation isolée de la pression intracrânienne se manifestant par des céphalées récurrentes et persistantes, des nausées, des vomissements, un rétrécissement transitoire et progressif du champ visuel et un papilloedème. La perte de la vision peut être irréversible."},"de":{"name":"Hypertension, idiopathische intrakranielle","synonyms":["IIH","Intrakranielle Hypertension, benigne","Pseudotumor cerebri"],"definition":"Die Idiopathische intrakranielle Hypertension ist eine neurologische Störung und gekennzeichnet durch erhöhten intrakraniellen Druck mit rezidivierenden und persistierenden Kopfschmerzen, Übelkeit, Erbrechen, progredienter oder vorübergehender Obstruktion des Gesichtsfeldes und Papillenödem. Irreversibler Visusverlust ist möglich."},"es":{"name":"Hipertensión intracraneal idiopática","synonyms":["IIH","Pseudotumor cerebri","HII","Hipertensión intracraneal benigna"],"definition":"La hipertensión intracraneal idiopática es un trastorno neurológico caracterizado por un incremento aislado de la presión intracraneal que se manifiesta con dolores de cabeza recurrentes y persistentes, náusea, vómitos, obstrucción progresiva y transitoria del campo visual, papiledema. La pérdida de visión puede ser irreversible."},"it":{"name":"Ipertensione intracranica idiopatica","synonyms":["IIH","Ipertensione intracranica benigna","Pseudotumor cerebri"],"definition":"L'ipertensione intracranica idiopatica è un disturbo neurologico caratterizzato da un aumento isolato della pressione intracranica che si manifesta con cefalee ricorrenti e persistenti, nausea, vomito, ostruzione transitoria e progressiva del campo visivo, papilledema. La perdita della vista può essere irreversibile."},"nl":{"name":"Idiopathische intracraniële hypertensie","synonyms":["Goedaardige intracraniële hypertensie","IIH","Pseudotumor cerebri"],"definition":"Een neurologische aandoening, gekenmerkt door geïsoleerde verhoogde intracraniële druk die zich manifesteert met recidiverende en persisterende hoofdpijn, misselijkheid, braken, progressieve en transiënte obstructie van gezichtsveld, en papiloedeem. Verlies van gezichtsvermogen kan onomkeerbaar zijn."},"pt":{"name":"Hipertensão intracraniana idiopática"},"pl":{"name":"Idiopatyczne nadciśnienie wewnątrzczaszkowe","synonyms":["IIH","Łagodne nadciśnienie wewnątrzczaszkowe","Rzekomy guz mózgu"]},"cs":{"name":"Idiopatická intrakraniální hypertenze","synonyms":["IIH","Pseudotumor cerebri","Benigní intrakraniální hypertenze"]},"uk":{"name":"Ідіопатична внутрішньочерепна гіпертензія","synonyms":["Доброякісна внутрішньочерепна гіпертензія","IIH","Псевдопухлина головного мозку"],"definition":"Idiopathic intracranial hypertension is a neurological disorder characterized by isolated increased intracranial pressure manifesting with recurrent and persistent headaches, nausea, vomiting, progressive and transient obstruction of the visual field, papilledema. Visual loss can be irreversible."},"zh":{"name":"自发性颅内高压","synonyms":["良性颅内高压"],"definition":"特发性颅内高压是一种神经系统疾病，以颅内压升高为特征，表现为反复和持续性头痛、恶心、呕吐、进行性和短暂性视野障碍、视乳头水肿。视力丧失是不可逆的。"},"tr":{"name":"İdiyopatik intrakraniyal hipertansiyon","synonyms":["IIH","Psödotümör serebri","Selim intrakraniyal hipertansiyon"]}},"xrefs":[{"source":"OMIM","code":"243200","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"G93.2","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"8D60.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"815670637"},{"source":"MONDO","code":"0009468","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D011559","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0033845","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"4561","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10078904","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98006,"name":"Rare neurologic disease","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":14.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2016[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-07-02","source":"31213758[PMID]_28013373[PMID]","terms":[{"hpo":"HP:0002315","label":"Headache","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002516","label":"Increased intracranial pressure","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012393","label":"Allergy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001513","label":"Obesity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001085","label":"Papilledema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100851","label":"Abnormal emotion/affect behavior","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000622","label":"Blurred vision","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000651","label":"Diplopia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002018","label":"Nausea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000613","label":"Photophobia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010822","label":"Scintillating scotoma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002360","label":"Sleep abnormality","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000572","label":"Visual loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003418","label":"Back pain","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000716","label":"Depression","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001254","label":"Lethargy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002076","label":"Migraine","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011161","label":"Olfactory auras","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0008629","label":"Pulsatile tinnitus","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002321","label":"Vertigo","frequency":"Very rare (<4-1%)","criteria":null}]}}