孤立性皮质静脉血栓三例分析及文献回顾

Isolated cortical venous thrombosis: analysis of three cases and literature review

孤立性皮质静脉血栓(ICoVT)发病率低,极易误诊.通过分析3例ICoVT患者的临床特点及诊治经过,以提高其诊治水平.方法回顾性分析北京协和医院收治的3例ICoVT患者的临床表现、检验、影像及病理特点.结果 3例患者均为急性起病,症状主要表现有头痛、痫性发作、单肢麻木无力及精神异常.腰椎穿刺颅压均正常,头颅核磁显示皮质脑沟内及皮质下异常信号, T2*或磁敏感加权成像(SWI)均可见病灶内低信号.1例行全脑血管造影检查,示皮质静脉闭塞.2例患者伴蛋白S减低,2例合并高同型半胱氨酸血症,1例合并抗磷脂抗体综合征.3例患者临床及影像学表现均可自发缓解.2例患者行脑活体组织检查(活检),1例因脑膜增厚显著而行脑膜活检得以确诊.结论 ICoVT临床表现及常规影像学检查缺乏特异性,极易被误诊.但ICoVT患者常合并易栓危险因素,磁共振T2*或SWI序列对诊断ICoVT较为敏感,脑活检对诊断有一定提示,也有助于鉴别诊断,但应严格掌握适应证.

Objective Isolated cortical venous thrombosis (ICoVT) has a low incidence and is easily to be misdiagnosed. The clinical characteristics, diagnosis and treatment of three cases of ICoVT were analyzed in order to improve the level of diagnosis and treatment. Methods The clinical manifestations, examination, imaging and pathological features of three patients with ICoVT admitted to our hospital were retrospectively analyzed. Results All the three patients had acute onset. The main symptoms were headache, seizures, numbness of one limb and mental disorders. Intracranial pressure was normal. Brain magnetic resonance imaging (MRI) showed abnormal signals located in cortical sulcus and subcortex. Point and linear hypointensity could be seen on T2* or susceptibility weighted imaging (SWI)-weighted image. Digital subtraction angiography was performed in one case, showing occlusion of cortical veins. Protein S decreased in two patients, two patients with hyperhomocysteinemia and one patient with antiphospholipid antibody syndrome. The clinical and imaging manifestations of three patients were spontaneously alleviated. Brain biopsy was performed in two patients, and meningeal biopsy was performed in one patient due to significant meningeal thickening. Conclusions The clinical manifestations and routine MRI of ICoVT lack specificity and are easy to be misdiagnosed. However, ICoVT patients are often associated with risk factors for thrombophilia. T2* or SWI sequences are sensitive to the diagnosis of ICoVT. Brain biopsy has implications for the diagnosis and it is helpful for differential diagnosis, but the indications should be strictly controlled.