Hemicraniacontinua(HC)isaprimaryheadachedisorderwithpathognomonictreatmentresponsetoindomethacin.Itpresentsclinicallywithabaselinecontinuousunilateralheadacheformonthsthatintermittentlyexacerbateswithassociatedautonomicfeatures.HCwasfirstdescribedinbyMedinaandDiamondasaclusterheadachevariant.[1]andtheterm“hemicraniacontinua”wasfirstcoinedinbySjaastadandSpierings.[2]
持续性偏头痛(HC)是一种原发性头痛,症状治疗一般选用吲哚美辛。临床表现为持续数月的单侧头痛,间歇性加重并伴有相关自主神经特征。HC在年被Medina和Diamond首次描述为一种丛集性头痛。年,Sjaastad和Spierings首次创造了“持续性偏头痛”这个词。
HChasbeenplacedundertheheadingoftrigeminalautonomiccephalalgias(TACs)inthethirdeditionoftheInternationalClassificationofHeadacheDisorder(ICHD-3).OtherprimaryheadachedisordersincludedinTACsareclusterheadache(CH),paroxysmalhemicrania(PH),short-lastingunilateralneuralgiformheadacheattackswithcranialautonomicsymptoms(SUNA),andshort-lasting,unilateral,neuralgiformheadacheattackswithconjunctivalinjectionandtearing(SUNCT).[3]
HC在第三版国际头痛疾病分类(ICHD-3)中被列为三叉自主神经性头痛(TACs)。TACs包括的其他原发性偏头痛,包括丛集性偏头痛(CH)、阵发性偏头痛(PH)、伴自主神经症状的短期单侧神经性头痛发作(SUNA)和伴流泪和短期撕裂样单侧神经性头痛发作(SUNCT)
EtiologyHemicraniacontinuaisaprimaryheadachedisorderwithoutasecondaryorganiccause.OurunderstandingofTACs,includingHC,islimited.Multipletheorieshavebeenproposedthatincludecavernoussinusinflammation,intracranialarterialvasodilation,upregulationofvasopeptides,particularlycalcitoningene-relatedpeptide(CGRP)andvasoactiveintestinalpeptide(VIP),andtrigeminalnerveautonomicdysregulationresultingintheincreasedcranialparasympatheticoutflow.Otherintracranialareasarealsoinvolvedaspainisnotalwaysrestrictedtotheareasuppliedbythetrigeminalnerve.Secondly,thetransectionofthetrigeminalnervedoesnotrelievesymptomsinallthepatients.[4]
持续性偏头痛是一种原发性头痛,没有继发器质性原因。我们对包括HC在内的很多类型偏头痛的理解是有限的。目前已有多种理论提出,包括海绵窦炎症、颅内动脉血管舒张、血管肽(特别是降钙素基因相关肽(CGRP)和血管活性肠肽(VIP)上调、三叉神经自主调节失调导致颅副交感神经兴奋性增加等。且疼痛并不总是局限于三叉神经供应的区域,也累及其他颅内区域。因此,截断三叉神经并不能缓解所有患者的症状。
EpidemiologyHemicraniacontinuawasinitiallyconsideredarareheadachedisorder,andonly18caseswerereportedintheliteratureinthefirst7yearsafterthediscoveryandaboutcasesinthefirst17years(-).[5][6]Althoughlargeepidemiologicstudiesarelackingtooutlinetheexactprevalenceofthiscondition,itisestimatedthatHCcasesconstituteabout1%oftotalheadachecases.[7][8]Thisnumberisstillconsideredunderreportedandunderdiagnosed.HCismoreprevalentinyoungadultsintheirthirdandfourthdecadeswithameanageof30years.However,therangevariesfromthefirsttoseventhdecades.HCismore