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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。
Objective To explore the possible etiology, clinical manifestations, imaging features, diagnosis, and diagnosis and treatment strategies of maternal complicated intracranial venous sinus thrombosis. Methods The clinical data of 12 consecutive pregnant women with severe intracranial venous sinus thrombosis who were admitted to Department of Neurology, Peking Union Medical College Hospital from 1993 to 2013 were retrospectively analyzed. The average age was (26 ± 5) years old (19 to 34 years). All 12 patients were examined by computed tomography (CT) or magnetic resonance imaging (MRI) in acute / subacute stage. Magnetic resonance venography (MRV) was performed in 9 patients and in 6 Digital subtraction angiography (DSA) was used to diagnose sinus thrombosis. Analysis of possible causes, clinical manifestations, imaging features, diagnosis and treatment strategies. Results The onset period was 7 in the first trimester, 1 in the third trimester, 4 in 2 weeks postpartum and 1 after abortion. The combined diseases included 1 case of protein S deficiency, 1 case of homocysteine, 1 case of idiopathic platelet 1 case of purpura reduction, 1 case of systemic infection and 1 case of anemia. Clinical characteristics: Both acute and subacute onset. Headache in 12 cases, epileptic seizures in 5 cases, other focal neurological signs in 5 cases (including limb weakness, pathological signs, numbness, ataxia, peripheral facial paralysis, etc.), decreased visual acuity in 1 case, varying degrees of disturbance of consciousness in 4 cases . Nine of 12 patients had abnormal brain parenchyma, including 7 cases of cerebral infarction or hemorrhagic infarction, 1 case of cerebral hemorrhage and 1 case of obvious brain swelling. Involved sinus: transverse sinus, sigmoid sinus in 9 cases, 8 cases of superior sagittal sinus, straight sinus, sagittal sinus in 2 cases, 2 cases of jugular vein. Treatment: 6 cases of patients with pregnancy termination of pregnancy were promptly terminated, 1 case of termination of pregnancy because of family disagree, aggravating, give up home. 10 cases of positive early heparinized anticoagulant therapy, all patients improved condition and discharged. Conclusions When pregnant women have acute / subacute headache, convulsions and other focal signs, it is necessary to be alert to the possibility of severe intracranial venous sinus thrombosis. The imaging findings are in line with abnormal signals of the brain parenchyma in the sinus drainage area (mainly hemorrhagic infarcts) Can be used as a timely diagnosis of important clues; critically ill timely choice to terminate the cause of pregnancy may be associated with good prognosis, at the same time as soon as possible to start anticoagulant therapy.