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封面摄影：邓敏兴老师 低颅压与静脉窦血栓形成 医院神经内二科郭伟宾译 医院神经内科孙鹏校对 Case1A27-year-oldfemalepatientwasadmittedtoourhospitalwithageneralizedtonic–clonicseizure.Shehadacaesariansectiondeliveryunderepiduralanesthesiawithanatraumaticneedleaweekago.女性，27岁，主因全身性强直阵挛发作入院。一周前，她在硬膜外麻醉下进行剖腹产，应用无损伤缝合针缝合。Shehadbeensufferingfromamoderateheadachewhichstarted3daysaftersurgery.Herheadachegotworseinuprightpositionandsubsidedwhenshelieddown.Herfamilyandbirthhistorieswereunremarkable.Sheonlyhadaniron-deficiencyanemia.Thiswasherthirduneventfulpregnancyanddelivery.术后3天患者出现了中度头痛。直立时加剧，平卧减轻。家族史和生长发育史无特殊。既往患缺铁性贫血。孕3产3。Whenshewasadmittedtotheemergencyroom,shewaspostictalandmildlyconfusedandhadnootherneurologicalsignsorsymptoms.急诊就诊时，处于发作后状态，轻度意识模糊，余神经系统体征无特殊。Contrast-enhancedcranialmagneticresonanceimaging(MRI)revealedaslightlyhemorrhagicvenousinfarctionareaontheleftfrontallobeandbilateralduralthickening,andcontrastenhancementmoreprominentontheleftcerebralhemispheresuggestingthepresenceofintracranialhypotension(Fig.1a).CranialMRIvenographyconfirmedpartialthrombosisoftheanteriorpartofsuperiorsagittalsinus(Fig.1b)头颅增强磁共振成像(MRI)显示左侧额叶有少量静脉性梗死伴出血，双侧硬膜增厚，增强序列示左侧大脑半球强化明显，提示存在低颅压(图1A)。头颅MRV证实上矢状窦前部部分血栓形成(图1B)Fig.1aContrast-enhancedcranialMRIrevealsbilateralduralthickeningandcontrastenhancementwhichismoreprominentontheleftcerebralhemispheresuggestingthepresenceofintracranialhypotension.bCranialMRIvenographyconfirmspartialthrombosisintheanteriorpartofsuperiorsagittalsinus.图1a增强的头颅MRI显示双侧硬膜增厚、强化，左侧大脑半球更明显，提示存在低颅压。B头MRV证实上矢状窦前部部分血栓形成Therewerehypochromicmicrocyticerythrocytesintheperipheralbloodsmearconsistentwithiron-deficiencyanemia.Noabnormalfindingswerepresentinthelaboratoryvaluesincludingprothrombinandpartialthromboplastintimes,antithrombinIII,fibrinogenlevel,serumglucoselevel,electrolytes,liverenzymes,urea,creatinine,protein,vitaminB12,folate,andhomocysteine.Extensivelaboratoryinvestigationsforinfections,vasculiticdisordersandhereditaryandacquiredhypercoagulablestatesrevealednopathologicalfindings.外周血涂片示小细胞低色素性贫血，符合缺铁性贫表现。血清凝血酶原、部分凝血活酶时间、抗凝血酶Ⅲ、纤维蛋白原、血糖、电解质、肝酶、尿素、肌酐、蛋白、维生素B12、叶酸、同型半胱氨酸等指标均无异常。其他实验室检查，包括感染检测、血管病筛查、遗传性及获得性高凝状态测定等均未见明显异常。ThepatientwasdiagnosedwithCVSTduetointracranialhypotensionandwasstartedonstandardheparininfusionalongwithhydrationandbedrestforaweekfollowedwithwarfarintreatmentfor6months.患者诊断为低颅压性颅内静脉窦血栓。治疗方面予肝素输注，水化、卧床。一周后加用华法林抗凝治疗，维持6月。Case2A28-year-oldfemalepatient,whohadatermdeliveryunderepiduralanesthesiaperformedwithanatraumaticneedle10daysago,graduallydevelopedheadache,painandhearinglossintheleftearaweekaftertheoperationandhadbeenadmittedtoahospitalwhereshewasdiagnosedwithotitismedia.女性，28岁，胎儿足月，10天前在硬膜外麻醉下顺产，应用无损伤针缝合，术后一周逐渐出现头痛、左耳疼痛和听力丧失，在外院诊断为中耳炎。Theheadacheincreasedwhenshestoodupanddecreasedwhenshelieddown.2dayslatertheheadachelostitsposturalcharacterandbecamemoreintense.Onthethirdday,shehadageneralizedtonic–clonicseizureandwasadmittedtoouremergencyroom.Shewasmildlyconfusedwithoutanyfocalneurologicdeficits.Shehadnoprevioushistoryofdiseaseandherfamilyhistorywasalsounremarkable.头痛站立后加重，平卧减轻。2天后头痛加剧，与体位无关。3天后，患者出现了全身性强直性阵挛发作，急诊就诊。查体意识模糊，无局灶性神经功能缺损体征。既往史和家族史无特殊情况。Contrast-enhancedcranialMRIconfirmedcerebralvenousinfarctiononthelefttemporallobeandrevealedCVSTofthelefttransversesinusandduralcontrastenhancement(Fig.2a).CranialMRIvenographyalsoconfirmedthediagnosis(Fig.2b).增强头颅MRI证实左侧颞叶静脉性梗死，并左侧横窦血栓、硬膜强化(图2A)。头颅MRV进一步证实该诊断(图2B)。Fig.2aContrast-enhancedcranialMRIdepictsduralcontrastenhancement.bCranialMRIvenographyconfirmslefttransversesinusthrombosis图2a增强的头颅MRI显示硬膜增强.b头MRV证实左横窦血栓形成Laboratoryinvestigationsdisclosednoabnormality.EEGshowedfocalepileptiformactivityinthetemporalregionofthelefthemisphere.实验室检查没有发现异常。脑电图显示左半球颞区有局灶性癫痫样活动。Thepatientreceivedstandardheparininfusionfor1weekfollowedbywarfarintreatmentfor6months.Oxcarbazepinemg/daywasaddedtohertreatmentandseizureswerecontrolled.普通肝素静脉滴注1周之后，改为华法林治疗6个月，奥卡西平mg/d，控制癫痫发作。DiscussionLumbarpunctureandepiduralorperiduralanesthesiawithaccidentalduralpuncturemayrarelycauseCVST.SymptomsofCVSTincludefocalorgeneralizedseizuresfollowedbyhemiparesis,aphasia,hemianopiaorotherfocaldeficits,oftenwithoutsignsofelevatedintracranialpressure.Inbothofourcases,changeinthecharacteristicsofheadacheandgeneralizedseizureswasprominentfindingswithoutanyotherfocalneurologicaldeficit.腰椎穿刺、硬膜外麻醉以及意外硬膜穿刺很少引起CVST。CVST的症状包括局灶性或全身性癫痫，其次是偏瘫、失语、偏盲或其他局灶性缺损，通常没有颅内压升高的迹象。在两个病例中，头痛和全身性癫痫发作的是首要症状，而无其他局灶性神经功能缺损体征。Postpartumstateisawell-establishedriskfactorforCVST.Inourpatients,pregnancywasahypercoagulablestateandinthesecondcaseotitismediaandintracranialhypotensionpossiblyworkedtogethertocauseastronghypercoagulativestatethatresultedinvenousthrombosis.CVSTisdifficulttodiagnosebecauseofthelackofagoldstandard;thus,itmightbeoverlooked.RecentreportshaveemphasizedMRIfindingsinintracranialhypotension.产后状态是CVST的一个公认的危险因素。妊娠会导致高凝状态，在第二例中，中耳炎和低颅压可能共同作用造成严重的高凝状态导致静脉血栓形成。由于缺乏金标准，CVST很难诊断；因此，它可能被忽略。最近的报道强调低颅压在MRI上的表现。Inconclusion,CVSTmaybeadiagnosticchallengeafterintracranialhypotensionduetoaccidentalduraldamagefromspinalinterventions.AnychangeinthecharacteristicsofaheadachefollowinglumbarpunctureshouldbeevaluatedtoexcludeCVST.SeizuresshouldalsowarnthephysicianaboutCVSTevenintheabsenceofanyfocalneurologicalsignsandsymptomssuggestingraisedintracranialpressure.综上所述，由于脊柱穿刺、损伤等因素造成硬脑膜损伤，引起低颅压后CVST的诊断具有挑战性。腰椎穿刺后存在头痛性质改变，需警惕CVST。合并癫痫发作也需警惕CVST，即使没有任何局灶性神经体征和提示颅高压的症状。“低颅压综合征导致静脉窦血栓形成的机制：根据Monroe—Kellie定律，在容积一定的颅腔内，脑体积、脑脊液容积和颅内血容量三者之和为一常数。脑体积相对同定，故当脑脊液容积减少时，颅内血容量将代偿性增加，致颅内静脉及静脉窦扩张。有文献报道SIH患者静脉窦的横截面积比正常人大70％。而在层流状态下，经过某一截面的血液流速与截面面积成反比。因此，如果其他条件保持恒定，颅内静脉扩张必然会导致血流速度减慢，血流淤滞，从而增加脑静脉及静脉窦血栓形成的机会。”参考文献李佳,邓晖,吴小坤,等.低颅压综合征伴发颅内静脉窦血栓形成1例报告及文献复习[J].中风与神经疾病杂志,,29(12):-.郭伟宾扫一扫下载订阅号助手，用手机发文章赞赏 长按白瘕风会出现什么状况如何治疗白癜风偏方
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