发热感染相关性癫痫综合征12例诊治分析及治疗进展

Diagnosis and treatment of 12 cases of febrile infection-related epilepsy syndrome

目的 探讨发热感染相关性癫痫综合征(FIRES)的临床特点及诊治方法。方法 回顾性分析2015年至2018年本院小儿神经专科收治的12例FIRES患儿的临床资料,对基本信息、临床表现、脑电图、影像学检查、治疗经过及预后进行分析。结果 12例FIRES患儿男7例,女5例,发病年龄1岁4个月至13岁,平均(7. 0±3. 7)岁;住院治疗时间中位数52 d(外院+本院34~86 d)。12例病前均体健,抽搐前均有发热,发热至抽搐发作间隔时间1~7(3. 5±1. 7) d;起病后以癫痫持续状态和意识障碍为主要临床表现,8例入院脑电图提示癫痫持续状态,12例均存在意识障碍;急性期发作形式为局灶性发作(100%,12/12)和全面性强直-阵挛发作(41. 7%,5/12)。所有患儿急性期使用3~5种抗癫痫药物(中位数4种),均接受激素和丙种球蛋白治疗。起病2周内开展生酮饮食治疗患儿4例,起病至脑电图好转时间(19. 2±5. 0) d;而未及时开展生酮饮食患儿8例,起病至脑电图好转时间(29. 9±9. 6) d。结论 FIRES多见于既往正常学龄期儿童,主要表现是急性热性疾病后数天出现难治性癫痫持续状态,发作形式以局灶性发作为主,抗癫痫药物耐药,早期开展生酮饮食能缩短急性期疾病时间。

Objective To investigate the clinical features,diagnosis and treatment of febrile infection-related epilepsy syndrome(FIRES).Methods The clinical data of 12 children with FIRES admitted to Xiangya Hospital of Central South University from 2015 to 2018 were retrospectively analyzed.The basic information,clinical manifestations,electroencephalogram,imaging examination,treatment and prognosis were analyzed.Results Of the 12 patients,7 were male and 5 were female.The age of onset was(7.0±3.7)years(1.3 year to 13 years).The average hospitalization time(34-86 days,median 52 days).Twelve patients were healthy before the disease,and had fever before convulsion.The interval between fever and seizure was(3.5±1.7)days(1-7 days).The status epilepticus and consciousness deficit were the main clinical manifestations.The electrogram of 8 patients showed status epilepticus when admitted.12 patients had disturbance of consciousness;the acute episodes were focal seizures(100%,12/12)and generalized tonic-clonic seizures(41.7%,5/12).All patients used 3-5 antiepileptic drugs(median 4),all treated with hormones and gamma globulin.4 patients with ketogenic diet(KD)were treated within 2 weeks of onset,and the average duration from onset to electroencephalogram(EEG)improvement was(19.2±5.0)days.In 8 patients who did not use KD within 2 weeks of onset,the average duration from onset to EEG improvement was(29.9±9.6)days.Conclusions FIRES is more common in normal children with school age.The main manifestation is refractory status epilepticus in the days after acute fever,focal episodes of seizures,anti-epileptic drug resistance.Early initiation of KD produces a favorable prognosis.