论文部分内容阅读
目的总结并分析新发难治性癫持续状态(NORSE)的临床特点及预后。方法采用回顾性研究分析2011至2013年收治的10例NORSE患者的临床资料,并对其预后进行随访,平均随访时间1.5年。结果 9例患者发热后出现难治性癫持续状态(RSE)。急性期癫发作以部分发作为首发表现8例,继发全面性发作6例。急性期头颅MRI未见异常4例,颅内囊肿2例,余4例提示颅内信号异常。所有患者均接受正规抗癫药物治疗(3~9种),其中使用大剂量苯巴比妥4例、咪达唑仑7例、激素5例、静脉注射免疫球蛋白5例、生酮饮食治疗3例。随访期内仅1例应用3种抗癫药物14个月后无明确临床发作,7例仍有临床发作;1例处于植物状态,1例死亡。格拉斯哥预后评分5分1例,4分6例,3分1例,2分1例,1分1例。结论 NORSE是一组病因不明易发展为RSE的疾病,多种治疗常无效,预后多不良并遗留认知行为障碍。
Objective To summarize and analyze the clinical features and prognosis of newly diagnosed refractory epileptic seizures (NORSE). Methods The clinical data of 10 patients with NORSE admitted from 2011 to 2013 were analyzed retrospectively. The prognosis was followed up for an average of 1.5 years. Results Nine patients developed refractory epileptic persistence (RSE) after fever. Acute epileptic seizures in some episodes as the first performance in 8 cases, secondary to comprehensive attack in 6 cases. Acute head skull MRI showed no abnormalities in 4 cases, intracranial cyst in 2 cases, the remaining 4 cases prompted intracranial signal abnormalities. All patients received regular anti-epileptic drug therapy (3-9 kinds), which used large doses of phenobarbital in 4 cases, midazolam in 7 cases, hormone in 5 cases, intravenous immunoglobulin in 5 cases, ketogenic diet Treatment in 3 cases. During the follow-up period, only one patient with three antiepileptic drugs did not have a definite clinical attack after 14 months, and seven patients still had clinical seizures. One patient was in a vegetative state and one patient died. Glasgow prognosis score 5 points in 1 case, 4 points in 6 cases, 3 points in 1 case, 2 points in 1 case, 1 point in 1 case. Conclusion NORSE is a group of diseases whose etiology is not easy to develop into RSE. Many kinds of treatment are often ineffective, with poor prognosis and cognitive impairment.