{"orpha":443180,"name":"Spontaneous intracranial hypotension","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Spontaneous cerebrospinal fluid leak"],"definition":"A rare headache resulting from a cerebrospinal fluid (CSF) leak with subsequent lowered CSF pressure, characterized clinically by severe headaches which typically worsen upon standing up and get better when lying down. Additional features may include neck stiffness, nausea, vomiting, vertigo, tinnitus, visual disturbances, and cognitive abnormalities, among others, as sagging and displacement of the brain can lead to a variety of lesions and symptoms.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-443180/","orphanet_url":"https://www.orpha.net/en/disease/detail/443180","names":{"fr":{"name":"Hypotension intracrânienne spontanée","synonyms":["Fuite spontanée de liquide céphalo-rachidien"],"definition":"Céphalée rare résultant d'une fuite de liquide céphalorachidien (LCR) et d'une baisse subséquente de la pression du LCR, caractérisée sur le plan clinique par des céphalées sévères qui s'aggravent généralement en position debout et s'améliorent en position allongée. Les autres caractéristiques peuvent inclure une raideur de la nuque, des nausées, des vomissements, des vertiges, des acouphènes, des troubles visuels et des troubles cognitifs, entre autres, l'affaissement et le déplacement du cerveau étant susceptibles d'entraîner toute une série de lésions et de symptômes."},"de":{"name":"Spontane intrakranielle Hypotension","synonyms":["Spontanes Liquorunterdrucksyndrom"],"definition":"Ein seltene Kopfschmerzerkrankung, die auf ein Liquorleck mit nachfolgendem Liquordruckabfall zurückzuführen ist und klinisch durch starke Kopfschmerzen gekennzeichnet ist, die sich typischerweise beim Aufstehen verschlimmern und im Liegen besser werden. Weitere Merkmale können unter anderem Nackensteifigkeit, Übelkeit, Erbrechen, Schwindel, Tinnitus, Sehstörungen und kognitive Anomalien sein, da die Absenkung und Verlagerung des Gehirns zu einer Vielzahl von Läsionen und Symptomen führen kann."},"es":{"name":"Hipotensión intracraneal espontánea","synonyms":["Pérdida espontánea de líquido cefalorraquídeo"],"definition":"Es una cefalea poco frecuente originada por la pérdida de líquido cefalorraquídeo (LCR) con la consiguiente disminución de la presión del LCR. Está caracterizada clínicamente por cefalea intensa que empeora típicamente en bipedestación y mejora en posición supina. Otros hallazgos adicionales pueden incluir rigidez en el cuello, náusea, vómitos, vértigo, tinnitus, trastornos visuales y anomalías cognitivas, entre otros, ya que el hundimiento y desplazamiento cerebral puede derivar en diferentes lesiones y síntomas."},"it":{"name":"Ipotensione intracranica spontanea","synonyms":["Perdita spontanea di liquido cefalorachidiano"],"definition":"Si tratta di una cefalea rara causata dalla perdita di liquido cerebrospinale, con conseguente riduzione della sua pressione. Dal punto di vista clinico, la malattia è caratterizzata da violente cefalee, che di solito si aggravano quando il paziente si alza in piedi e migliorano quando si sdraia. Può associarsi alla rigidità del collo, nausea, vomito, vertigini, acufeni, disturbi della vista e anomalie cognitive, secondari all'affossamento e allo spostamento del cervello che possono causare varie lesioni e sintomi diversi."},"nl":{"name":"Spontane intracraniële hypotensie","synonyms":["Spontaan lek van cerebrospinaal vocht"],"definition":"Een zeldzame hoofdpijn als gevolg van een lekkage van cerebrospinaal vocht (CSV) met daaropvolgend verlaagde CSV-druk, klinisch gekarakteriseerd door ernstige hoofdpijn die doorgaans erger wordt bij opstaan en beter wordt bij neerliggen. Mogelijke bijkomende kenmerken zijn onder meer stijve nek, misselijkheid, braken, vertigo, tinnitus, visuele stoornissen, en cognitieve afwijkingen, aangezien verzakking en verplaatsing van de hersenen kunnen leiden tot verschillende laesies en symptomen."},"pt":{"name":"Hipotensão intracraniana espontânea"},"pl":{"name":"Spontaniczne niedociśnienie wewnątrzczaszkowe","synonyms":["Spontaniczny wyciek płynu mózgowo-rdzeniowego"]},"cs":{"name":"Spontánní intrakraniální hypotenze","synonyms":["Spontánní únik mozkomíšního moku"]},"uk":{"name":"Спонтанна внутрішньочерепна гіпотензія","synonyms":["Спонтанний витік спинномозкової рідини"],"definition":"A rare headache resulting from a cerebrospinal fluid (CSF) leak with subsequent lowered CSF pressure, characterized clinically by severe headaches which typically worsen upon standing up and get better when lying down. Additional features may include neck stiffness, nausea, vomiting, vertigo, tinnitus, visual disturbances, and cognitive abnormalities, among others, as sagging and displacement of the brain can lead to a variety of lesions and symptoms."},"zh":{"name":"自发性颅内低压","synonyms":["自发性脑脊液漏"],"definition":"由脑脊液（CSF）漏引起的罕见头痛，其后CSF压力降低，临床特征是严重的头痛，通常在站立时会加重，躺下后会好转。其他特征可能包括颈部僵硬，恶心，呕吐，眩晕，耳鸣，视力障碍和认知异常等，脑下垂和移位可能导致其他多种病变和症状。"},"tr":{"name":"Spontan intrakraniyal hipotansiyon","synonyms":["Spontan beyin omurilik sıvısı sızıntısı"]}},"xrefs":[{"source":"ICD-10","code":"G96.0","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"8D61.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1304151002"},{"source":"MONDO","code":"0018624","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0751731","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":98022,"name":"Rare headache","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":5.0,"area":"Worldwide","status":"Validated","source":"32644492[PMID]_ORPHANET"}],"phenotypes":null}