颅内静脉窦血栓形成伴脑出血及阵发性睡眠性血红蛋白尿1例  

Cerebral venous sinus thrombosis with cerebral hemorrhage and paroxysmal nocturnal hemoglobinuria:a case report

作　　者：李淑杰 李思颉[1] 段建钢[1] 马瑾 唐娜[1] LI Shujie;LI Sijie;DUAN Jiangang;MA Jin;TANG Na(Xuanwu Hospital of Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院,北京100053

出　　处：《中国实用神经疾病杂志》2023年第4期494-497,共4页Chinese Journal of Practical Nervous Diseases

基　　金：医药协同科技创新研究(编号:Z181100001918026)。

摘　　要：目的 分析颅内静脉窦血栓形成(CVST)伴脑出血、阵发性睡眠性血红蛋白尿(PNH)患者的临床特点、影像学表现及治疗手段。方法 总结1例CVST伴脑出血、PNH患者的临床资料,分析发病诱因、临床表现、影像学特点和治疗方法。结果 CVST的病因包括口服避孕药、妊娠、激素替代治疗、肿瘤、感染和系统性红斑狼疮等疾病。CVST临床表现包括头痛、局灶性神经症状、癫痫以及昏迷等,诊断主要依据影像学检查,抗凝是CVST治疗的主要方式,危重患者多联合局部溶栓、机械取栓、机械碎栓和抗凝治疗多种治疗方案。结论 CVST伴脑出血、PNH是一种罕见的脑血管疾病。对于长期口服雄激素和糖皮质激素的患者,当出现意识障碍、头痛和癫痫表现时,用常规的脑血管病不能解释,要考虑CVST的可能,及时给予磁共振静脉造影(MRV)等检查,对明确诊断的CVST患者根据病情给予抗凝或机械取栓等治疗。Objective To investigate the clinical characteristics,imaging findings and treatment of cerebral venous sinus thrombosis(CVST)patients with cerebral hemorrhage and paroxysmal nocturnal hemoglobinuria(PNH).Methods The clinical data of a CVST patient with cerebral hemorrhage and PNH were summarized,and the predisposing factors,clinical manifestations,imaging characteristics and treatment methods were analyzed.Results The causes of CVST included oral contraceptives,pregnancy,hormone replacement therapy,tumor,infection and systemic lupus erythematosus.The clinical manifestations of CVST include headache,focal neurological symptoms,epilepsy,coma,etc.The diagnosis is mainly based on imaging examination.Anticoagulation is the main method of CVST treatment.The combination of local thrombolysis,mechanical thrombectomy,mechanical thrombectomy and anticoagulant therapy is often used in critically ill patients.Conclusion CVST with cerebral hemorrhage and PNH is a rare cerebrovascular disease.For patients with long-term oral androgen and glucocorticoid,when the symptoms of consciousness disturbance,headache and epilepsy appear,the conventional cerebrovascular disease cannot be interpreted.It is necessary to consider the possibility of CVST and give MRV and other examinations in time,Patients with CVST should be treated with anticoagulation or mechanical embolectomy according to their condition.

关 键 词：颅内静脉窦血栓形成 脑出血 阵发性睡眠性血红蛋白尿 意识障碍 头痛 癫痫 抗凝 机械取栓 

分 类 号：R743.32[医药卫生—神经病学与精神病学]