CAJ | 학술논문

目的探讨孕产妇合并颅内静脉窦血栓形成可能的病因、临床表现、影像学特征、诊断，总结诊疗策略。 　　方法回顾性分析北京协和医院神经内科1993-2013年连续收治的孕产妇合并危重颅内静脉窦血栓形成患者12例，平均年龄（26±5）岁（19～34岁）。12例患者均在急性/亚急性期行计算机断层扫描（computed tomography，CT）或磁共振成像（magnetic resonance imaging，MRI）检查；9例行磁共振静脉成像（magnetic resonance venography，MRV），6例行数字减影血管造影（digital subtraction angiography， DSA）检查诊断静脉窦血栓形成。分析可能的病因、临床表现、影像学特征、诊断及治疗策略。 　　结果发病时期：孕早期7例，孕晚期1例，产后2周内4例，人工流产后1例；合并疾病包括：蛋白S缺乏 　　1例，高同型半胱氨酸1例，特发性血小板减少性紫癜1例，全身感染1例，贫血1例；临床特征：均为急性或亚急性起病。头痛12例，痫性发作5例，其他局灶性神经系统体征5例（包括肢体无力、病理征、麻木、共济失调、周围性面瘫等），视力下降1例，不同程度意识障碍4例。12例患者中9例存在脑实质异常，其中脑梗死或出血性梗死7例，脑出血1例，脑组织明显肿胀1例。受累静脉窦：横窦、乙状窦9例，上矢状窦8例，直窦、下矢状窦2例，颈静脉2例。治疗：6例妊娠期发病患者均及时终止妊娠，1例因家属不同意终止妊娠，病情加重，自动放弃回家。10例积极尽早给予肝素化抗凝治疗，所有患者均病情好转出院。 　　结论孕产妇出现急性/亚急性头痛、抽搐及其他局灶体征时，需警惕危重颅内静脉窦血栓形成可能；影像学提示符合静脉窦引流区域的脑实质异常信号（出血性梗死为主），可作为及时诊断的重要线索；病情危重时及时选择终止妊娠去除病因可能与良好预后相关，同时需尽早开始抗凝治疗。目的探討孕產婦閤併顱內靜脈竇血栓形成可能的病因、臨床錶現、影像學特徵、診斷，總結診療策略。 　　方法迴顧性分析北京協和醫院神經內科1993-2013年連續收治的孕產婦閤併危重顱內靜脈竇血栓形成患者12例，平均年齡（26±5）歲（19～34歲）。12例患者均在急性/亞急性期行計算機斷層掃描（computed tomography，CT）或磁共振成像（magnetic resonance imaging，MRI）檢查；9例行磁共振靜脈成像（magnetic resonance venography，MRV），6例行數字減影血管造影（digital subtraction angiography， DSA）檢查診斷靜脈竇血栓形成。分析可能的病因、臨床錶現、影像學特徵、診斷及治療策略。 　　結果髮病時期：孕早期7例，孕晚期1例，產後2週內4例，人工流產後1例；閤併疾病包括：蛋白S缺乏 　　1例，高同型半胱氨痠1例，特髮性血小闆減少性紫癜1例，全身感染1例，貧血1例；臨床特徵：均為急性或亞急性起病。頭痛12例，癇性髮作5例，其他跼竈性神經繫統體徵5例（包括肢體無力、病理徵、痳木、共濟失調、週圍性麵癱等），視力下降1例，不同程度意識障礙4例。12例患者中9例存在腦實質異常，其中腦梗死或齣血性梗死7例，腦齣血1例，腦組織明顯腫脹1例。受纍靜脈竇：橫竇、乙狀竇9例，上矢狀竇8例，直竇、下矢狀竇2例，頸靜脈2例。治療：6例妊娠期髮病患者均及時終止妊娠，1例因傢屬不同意終止妊娠，病情加重，自動放棄迴傢。10例積極儘早給予肝素化抗凝治療，所有患者均病情好轉齣院。 　　結論孕產婦齣現急性/亞急性頭痛、抽搐及其他跼竈體徵時，需警惕危重顱內靜脈竇血栓形成可能；影像學提示符閤靜脈竇引流區域的腦實質異常信號（齣血性梗死為主），可作為及時診斷的重要線索；病情危重時及時選擇終止妊娠去除病因可能與良好預後相關，同時需儘早開始抗凝治療。
목적탐토잉산부합병로내정맥두혈전형성가능적병인、림상표현、영상학특정、진단，총결진료책략。 　　방법회고성분석북경협화의원신경내과1993-2013년련속수치적잉산부합병위중로내정맥두혈전형성환자12례，평균년령（26±5）세（19～34세）。12례환자균재급성/아급성기행계산궤단층소묘（computed tomography，CT）혹자공진성상（magnetic resonance imaging，MRI）검사；9례행자공진정맥성상（magnetic resonance venography，MRV），6례행수자감영혈관조영（digital subtraction angiography， DSA）검사진단정맥두혈전형성。분석가능적병인、림상표현、영상학특정、진단급치료책략。 　　결과발병시기：잉조기7례，잉만기1례，산후2주내4례，인공유산후1례；합병질병포괄：단백S결핍 　　1례，고동형반광안산1례，특발성혈소판감소성자전1례，전신감염1례，빈혈1례；림상특정：균위급성혹아급성기병。두통12례，간성발작5례，기타국조성신경계통체정5례（포괄지체무력、병리정、마목、공제실조、주위성면탄등），시력하강1례，불동정도의식장애4례。12례환자중9례존재뇌실질이상，기중뇌경사혹출혈성경사7례，뇌출혈1례，뇌조직명현종창1례。수루정맥두：횡두、을상두9례，상시상두8례，직두、하시상두2례，경정맥2례。치료：6례임신기발병환자균급시종지임신，1례인가속불동의종지임신，병정가중，자동방기회가。10례적겁진조급여간소화항응치료，소유환자균병정호전출원。 　　결론잉산부출현급성/아급성두통、추휵급기타국조체정시，수경척위중로내정맥두혈전형성가능；영상학제시부합정맥두인류구역적뇌실질이상신호（출혈성경사위주），가작위급시진단적중요선색；병정위중시급시선택종지임신거제병인가능여량호예후상관，동시수진조개시항응치료。
Objective To discuss the possible cause, clinical symptoms, radiologic characteristics, diagnosis and our treatment strategies of cerebral venous sinus thrombosis (SCVT) in pregnancy. Methods We presented 12 cases of pregnancy with emergent SCVT admitted between 1993 and 2013 in the Department of Neurology, Peking Union Medical College (PUMC) hospital. The average age was (26±5) (19~34). Computed tomography (CT)/magnetic resonance imaging (MRI) was performed in all 12 patients in acute/subacute period; magnetic resonance venography (MRV) in 9 and digital subtraction angiography (DSA) in 6. The possible pathogenesis, clinical symptoms, radiological ifndings, diagnosis and treatment strategies were discussed. Results Of the 12 pregnant or maternity patients with SCVT, 7 got the disease in early pregnancy, 1 in late pregnancy, 4 within 2 weeks after delivery, 1 after abortion. The accompanied diseases were:one with protein S deficiency, one with hyperhomocystinemia, one with idiopathic thrombocytopenia purpura, one with infection, one with anemia. Clinical onset was acute or subacute. Twelve patients had headache, 5 had epilepsy, 5 had focal neurological signs (including weakness, Babinski signs, numbness, ataxia, peripheral facial paralysis), 1 had visual loss, 4 had disturbance of consciousness. All 12 patients had MRI during acute or subacute period, 9 had abnormal parenchymal signals--7 had infarction or hemorrhagic infarction, 1 had cerebral hemorrhage, and 1 had cerebral edema. Transverse sinus and sigmoid sinus were involved in 9 cases, superior sagittal sinus in 8, straight sinus and inferior sagittal sinus in 2, jugular vein in 2. Treatment:pregnancy was timely terminated in 6 pregnant patients; 1 patient whose family disagreed to terminate pregnancy aggravated and went back home. Ten patients accepted heparinized anti-coagulation as soon as possible, and all of them got better when they were discharged. Conclusion When pregnant women had acute/subacute headache, epilepsy and other local signs, the possibility of SCVT should be suspected. Abnormal parenchymal signals in draining area of venous sinus (especially hemorrhagic infarction) could be a clue to diagnosis. Timely termination of pregnancy and anti-coagulation could lead to a good prognosis.