成年起病的自主神经发作癫痫持续状态1例报告并文献复习

Autonomic status epilepticus in adults: A report of one case and review of the literature

目的:分析成年起病的自主神经发作癫痫持续状态患者的临床特征,以加强对该病的认识,减少误诊误治。方法:综合分析宿州市第一人民医院2018年6月收治的1例成年起病的自主神经发作癫痫持续状态患者的临床资料。在PubMed数据库、大医医学搜索数据库以“ Autonomic status epilepticus”关键词进行检索,共检索到41篇英文文献,排除综述及儿童期起病的病例,最终仅入选2篇英文文献。复习并分析3例患者的临床特点。结果:共3例患者,其中男性1例、女性2例,年龄38-56岁,临床表现为剑突下不适、腹部难以描述的不适、里急后重感、心动过速、出汗、恶心、呕吐、面色苍白、瞳孔散大、流涎、尿失禁和意识丧失等。本例患者未寻找到明确病因。文献报道的2例病因分别为双侧外侧裂综合征、LGI1抗体阳性自身免疫性脑炎。本例患者脑电图提示来源于中央颞区的尖波。双侧外侧裂综合征患者脑电图提示来源于顶-颞区的尖波。自身免疫性脑炎患者头颅MRI显示海马、杏仁核呈高信号。本例患者给予丙戊酸钠治疗,目前未见发作。自身免疫性脑炎患者行免疫调节治疗后复查头颅MRI显示病灶消失。双侧外侧裂综合征患者给予奥卡西平联合拉莫三嗪治疗后,持续8年未出现再次发作。当癫痫再次发作时,改用拉莫三嗪联合左乙拉西坦治疗。结论:成年起病的自主神经发作癫痫持续状态极为罕见,临床表现为多种自主神经症状,其发作可能起源于颞叶。

Objective: To analyze the clinical characteristics and enrich the cognition of adults' autonomic status epilepticus so as to avoid the delay in diagnosis and treatment of this disease. Methods: A comprehensive analysis of one case with adult autonomic status epilepticus was performed. The First People's Hospital of Suzhou. Anhui China in June 2018. In the PubMed and DaYiYiXueSouSuo database with " Autonomic status epilepticus" as the keyword, 41 English professional articles were retrieved, excluding literature review and essays about children's cases. Only two articles about the clinical characteristics of 3 cases were enrolled and analyzed. Results: Of the 3 patients, 1 was male and two were female, aged from 38 to 56 years. Clinical manifestations included in disposition of the subxiphoid area, abdominal sensations of vague unpleasant, tenesmus without diarrhea, tachycardia, diaphoresis, nausea, retching, vomiting, pallor, mydriasis, hypersalivation, urine incontinence and unconsciousness. The causes of the patient of our hospital is uncertain. The causes of other two patients mentioned in the literature were bilateral perisylvian syndrome and anti-LGI1 encephalitis. Electroencephalogram of our patient indicate sharp waves arising from mesiotemporal structures and the sharp waves in the parieto-temporal region was showed in the patient of bilateral perisylvian syndrome. Coronal fluid-attenuated inversion recovery sequence shows hyperintensity of the hippocampus and amygdale in the patient of anti-LGI1 encephalitis. Oral sodium valproate is administered for our patient, with no seizures in the follow-up period. Treatment of bilateral perisylvian syndrome of combined therapy of oxcarbazepine and lamotrigine gave complete seizure remission for 8 years. When seizures re-appeared,treatment option of lamotrigine and levetiracetam was followed by a significant clinical improvement. Conclusions: Autonomic status epilepticus is rarely observed in adults. Clinical manifestations include a variety of autonomic dysfunction, seizures may arising from the temporal lobe.