{"orpha":157835,"name":"Paroxysmal hemicrania","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare primary headache disorder characterized by multiple attacks of unilateral pain that occur in association with ipsilateral cranial autonomic symptoms. The hallmarks of this syndrome are the relative shortness of the attacks and the complete response to indomethacin therapy.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-157835/","orphanet_url":"https://www.orpha.net/en/disease/detail/157835","names":{"fr":{"name":"Hémicrânie paroxystique","definition":"Céphalée primaire rare caractérisée par de nombreuses crises de douleur unilatérale, associées à des signes autonomes crâniens ipsilatéraux. Les particularités du syndrome sont une durée relativement courte des crises et une réponse complète au traitement par indométhacine."},"de":{"name":"Hemikranie, paroxysmale","definition":"Eine seltene primäre Kopfschmerzerkrankung, die durch multiple einseitige Schmerzattacken gekennzeichnet ist, die in Verbindung mit ipsilateralen kranialen autonomen Symptomen auftreten. Kennzeichnend für dieses Syndrom sind die relativ kurze Dauer der Attacken und das vollständige Ansprechen auf eine Indometacintherapie."},"es":{"name":"Hemicrania paroxística","definition":"Es una cefalea primaria poco frecuente caracterizada por múltiples episodios de dolor unilateral asociados a síntomas autonómicos craneales ipsilaterales. Las características distintivas de este síndrome son la relativa brevedad de las crisis y la respuesta completa al tratamiento con indometacina."},"it":{"name":"Emicrania parossistica","definition":"L'emicrania parossistica (PH) è una cefalea primaria rara, caratterizzata da episodi multipli dolorosi monolaterali, associati a sintomi a carico del sistema autonomo localizzati nella stessa parte del cranio interessata dal dolore. I segni clinici caratteristici comprendono la durata relativamente breve degli episodi e la risposta completa alla terapia con l'indometacina."},"nl":{"name":"Paroxysmale hemicranie","definition":"Een zeldzame, primaire hoofdpijnaandoening, gekarakteriseerd door meervoudige aanvallen van unilaterale pijn die voorkomen in associatie met ipsilaterale craniale autonome symptomen. De typische kenmerken van dit syndroom zijn de relatief korte duur van de aanvallen en de volledige therapeutische respons op indometacine."},"pt":{"name":"Hemicranea paroxística","definition":"A hemicrania paroxística (PH) é uma cefaleia primária caracterizada por ataques múltiplos de dor unilateral associados com sintomas autónomicos cranianos."},"pl":{"name":"Hemikrania napadowa"},"cs":{"name":"Paroxysmální hemikranie"},"uk":{"name":"Пароксизмальна гемікранія","definition":"A rare primary headache disorder characterized by multiple attacks of unilateral pain that occur in association with ipsilateral cranial autonomic symptoms. The hallmarks of this syndrome are the relative shortness of the attacks and the complete response to indomethacin therapy."},"zh":{"name":"阵发性偏头痛","definition":"一种罕见的原发性头痛疾病，其特征是单侧疼痛多次发作，并伴有同侧脑自主神经症状。该综合征的特点是发作时间相对较短，且吲哚美辛的治疗完全有效。"},"tr":{"name":"Paroksismal hemikrani"}},"xrefs":[{"source":"ICD-10","code":"G44.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"8A82","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"959737563"},{"source":"MONDO","code":"0015529","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D051302","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1399352","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10794","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10019461","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":157843,"name":"Trigeminal autonomic cephalalgia","classifications":[181]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Validated","source":"[EXPERT]_29720814[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2018-02-26","source":"24523000[PMID]_23566235[PMID]","terms":[{"hpo":"HP:0002076","label":"Migraine","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002331","label":"Recurrent paroxysmal headache","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030953","label":"Conjunctival hyperemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0009926","label":"Epiphora","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031284","label":"Flushing","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002183","label":"Phonophobia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000613","label":"Photophobia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000508","label":"Ptosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012384","label":"Rhinitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031417","label":"Rhinorrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000819","label":"Diabetes mellitus","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000616","label":"Miosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030833","label":"Neck pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011161","label":"Olfactory auras","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100540","label":"Palpebral edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012452","label":"Restless legs","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025258","label":"Stiff neck","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012199","label":"Cluster headache","frequency":"Excluded (0%)","criteria":null},{"hpo":"HP:0100661","label":"Trigeminal neuralgia","frequency":"Excluded (0%)","criteria":null}]}}