{"orpha":284388,"name":"Reversible cerebral vasoconstriction syndrome","type":"Clinical syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["RCVS"],"definition":"A rare cerebrovascular disorder characterized by severe headaches with or without focal neurological deficits or seizures, and a reversible segmental and multifocal vasoconstriction of cerebral arteries. It may occur spontaneously or be provoked by various precipitating factors, the most common being postpartum and exposure to various vasoactive substances such as illicit drugs and selective serotonin-reuptake inhibitors. The major complication is ischemic or hemorrhagic stroke.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-284388/","orphanet_url":"https://www.orpha.net/en/disease/detail/284388","names":{"fr":{"name":"Syndrome de vasoconstriction cérébrale réversible","synonyms":["RCVS","SVCR"],"definition":"Maladie cérébrovasculaire rare caractérisée par des céphalées sévères, accompagnées ou non de déficits neurologiques focaux ou de convulsions, et une vasoconstriction segmentaire et multifocale réversible des artères cérébrales. Elle peut survenir spontanément ou être provoquée par divers facteurs déclenchants, les plus courants étant le post-partum et l'exposition à diverses substances vasoactives telles que les substances illicites et les inhibiteurs sélectifs de la recapture de la sérotonine. La complication majeure est l'accident vasculaire cérébral ischémique ou hémorragique."},"de":{"name":"Reversibles zerebrales Vasokonstriktionssyndrom","synonyms":["Call-Fleming-Syndrom","RCVS"],"definition":"Das reversible zerebrale Vasokonstriktionssyndrom (RCVS) ist eine seltene zerebrovaskuläre Störung, die durch starke Kopfschmerzen mit oder ohne fokale neurologische Defizite oder Krampfanfälle und eine reversible segmentale und multifokale Vasokonstriktion der zerebralen Arterien gekennzeichnet ist. Sie kann spontan auftreten oder durch verschiedene auslösende Faktoren provoziert werden, am häufigsten nach der Geburt und nach dem Kontakt mit verschiedenen vasoaktiven Substanzen wie illegalen Drogen und selektiven Serotonin-Wiederaufnahmehemmern. Die Hauptkomplikation ist ein ischämischer oder hämorrhagischer Schlaganfall."},"es":{"name":"Síndrome de vasoconstricción cerebral reversible","synonyms":["SVCR"],"definition":"Es un trastorno cerebrovascular poco frecuente caracterizado por fuertes cefaleas con o sin déficits neurológicos focales o crisis, y vasoconstricción segmentaria y multifocal reversible de las arterias cerebrales. Puede ocurrir de forma espontánea o estar desencadenada por diversos factores precipitantes, siendo los más comunes el posparto y la exposición a diversas sustancias vasoactivas como las drogas ilícitas y los inhibidores selectivos de la recaptación de serotonina. La principal complicación es el ictus isquémico o hemorrágico."},"it":{"name":"Sindrome da vasocostrizione cerebrale reversibile","synonyms":["RCVS"],"definition":"La sindrome da vasocostrizione cerebrale reversibile (RCVS) è una malattia cerebrovascolare rara, caratterizzata da forti cefalee, associate o meno a deficit neurologici focali o crisi epliettiche, secondarie alla vasocostrizione segmentale e multifocale reversibile delle arterie cerebrali."},"nl":{"name":"Reversibele cerebrale vasoconstrictie-syndroom","synonyms":["RCVS"],"definition":"Een zeldzame cerebrovasculaire aandoening, gekarakteriseerd door ernstige hoofdpijn met of zonder focale neurologische functiestoornissen of insulten, en een reversibele segmentale en multifocale vasoconstrictie van cerebrale arteriën. De aandoening kan spontaan voorkomen of uitgelokt worden door verschillende bevorderende factoren, voornamelijk postnatale blootstelling aan verscheidene vasoactieve stoffen zoals illegale drugs en selectieve serotonine-heropnameremmers. De voornaamste complicatie is ischemische of hemorragische beroerte."},"pt":{"name":"Síndrome de vasoconstrição cerebral reversível"},"pl":{"name":"Zespół odwracalnego kurczu naczyń mózgowych","synonyms":["RCVS"]},"cs":{"name":"Syndrom reverzibilní cerebrální vazokonstrikce","synonyms":["RCVS","Call-Flemmingův syndrom"]},"uk":{"name":"Синдром зворотного звуження судин головного мозку","synonyms":["RCVS"],"definition":"A rare cerebrovascular disorder characterized by severe headaches with or without focal neurological deficits or seizures, and a reversible segmental and multifocal vasoconstriction of cerebral arteries. It may occur spontaneously or be provoked by various precipitating factors, the most common being postpartum and exposure to various vasoactive substances such as illicit drugs and selective serotonin-reuptake inhibitors. The major complication is ischemic or hemorrhagic stroke."},"zh":{"name":"可逆性脑血管收缩性综合征","synonyms":["RCVS"],"definition":"可逆性脑血管收缩综合征（RCVS）是一种罕见的脑血管疾病，其特征是严重头痛伴或不伴局灶性神经功能缺损或癫痫发作，及可逆性脑动脉节段性和多灶性血管收缩。"},"tr":{"name":"Tersinir serebral vazokonstriksiyon sendromu","synonyms":["RCVS"]}},"xrefs":[{"source":"ICD-10","code":"I67.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8B22.2","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1474881372"},{"source":"MONDO","code":"0017291","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3544214","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12768","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10073240","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":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-03-31","source":"31582040[PMID]_31869187[PMID]_27015869[PMID]","terms":[{"hpo":"HP:0030907","label":"Thunderclap headache","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0025637","label":"Vasospasm","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0410263","label":"Brain imaging abnormality","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002076","label":"Migraine","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002331","label":"Recurrent paroxysmal headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001892","label":"Abnormal bleeding","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009145","label":"Abnormal cerebral artery morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000622","label":"Blurred vision","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001342","label":"Cerebral hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004944","label":"Dilatation of the cerebral artery","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004302","label":"Functional motor deficit","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002140","label":"Ischemic stroke","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002352","label":"Leukoencephalopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002183","label":"Phonophobia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000613","label":"Photophobia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002138","label":"Subarachnoid hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002326","label":"Transient ischemic attack","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012158","label":"Carotid artery dissection","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002181","label":"Cerebral edema","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030746","label":"Intraventricular hemorrhage","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100309","label":"Subdural hemorrhage","frequency":"Very rare (<4-1%)","criteria":null}]}}