癫痫杂志

癫痫杂志

儿童晚发型癫痫性痉挛的临床特点及脑电图改变

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目的探讨儿童晚发型癫痫性痉挛的临床特点及脑电图(EEG)改变。 方法回顾分析总结2010年6月-2015年8月就诊于南方医科大学附属中山市博爱医院儿科的13例临床诊断晚发型癫痫性痉挛患儿的临床资料、治疗、随访及结果。 结果13例入组患儿,其中男9例,女4例,起病年龄为1岁3个月~5岁7个月,病程1年3个月~4年8个月。7例有明确的病因(53.8%),其中2例明确病毒性脑炎,3例娩出时窒息、缺氧,1例新生儿期败血症、急性呼吸窘迫综合征,1例诊断甲基丙二酸血症伴高同型半胱氨酸血症;余6例未明确病因。痉挛发作仍为主要的发作类型,7例合并部分性起源发作,发作以清醒期及醒睡期为著。但易与其他的发作类型并存。发作期EEG为广泛性中-高波幅慢波、尖慢波阵发,复合或不复合低波幅快波,有时伴阵发后电压衰减数秒,成串或孤立性出现。同步双侧三角肌肌电监测可见双侧或单侧同步肌电爆发1~2 s。间歇期EEG为多灶和广泛性放电,仍以尖(棘)慢波连续发放为主。治疗:所有患儿均行促肾上腺皮质激素(Adrenocorticotropic hormone,ACTH)或甲基强的松龙免疫调控治疗,3例行生酮饮食治疗。同时根据病情选择丙戊酸、托吡酯、氯硝西泮、拉莫三嗪、左乙拉西坦等抗广谱抗癫痫药物(AEDs),13例患儿均采取联合用药方式。预后: 13例患儿运用ACTH或甲基强的松龙免疫调控疗程结束后发作减少或控制,随访3~12个月出现临床发作控制不良;3例生酮饮食治疗(现已持续1年3个月~2年5个月),其中1例发作控制、智力改善明显;另2例发作次数减少,智力水平明显改善。 结论围产期因素与某些后天获得性脑损伤是儿童晚发型癫痫性痉挛最为常见的致病病因。发作以痉挛发作为主,与其他发作形式并存。EEG无典型高度失律表现,同步肌电监测可见双侧或单侧同步肌电爆发。对各种AEDs治疗效果欠佳,绝大多数发展为难治性癫痫。生酮饮食治疗或许是一个相对好的选择。

ObjectiveTo investigate the clinical features and changes of EEG in children with late onset epilepsy spasm. MethodsThe clinical data, treatment, follow-up and outcome of 13 patients with late-onset epilepsy spasms were analyzed retrospectively from June 2010 to August 2015 in Bo ai Hospital of Zhong Shan City.Affiliated Southern Medical University ResultsThirteen cases of children were enrolled in the group, including 9 males and 4 females, the onset of age were 1 year 3 months to 5 years 7 months, duration of treatment were 1 year 5 months to 4 years 8months.Seven cases of children had clear cause in 13 patients: 2 cases of viral encephalitis, 3 cases of HIE, 1 case of neonatal sepsis, ARDS, and 1 case of methylmalonic acid hyperchomocysteinemia.Six cases did not clear the cause.Spasm is still the main type of Seizures.Seven cases had seizures with partial origin.the most onset time were awake period and wake up for the time, and coexisted with other types of seizures.EEG in Epileptic seizures period was a broad range of high amplitude slow wave, slow bursts, complex or non-composite low amplitude fast wave, sometimes with the burst after the voltage attenuation of a few seconds, string or isolation occurs.Synchronous bilateral deltoid EMG monitoring showed bilateral or unilateral synchronous EMG 1 ~ 2s Bilateral or unilateral synchronous EMG outbreak1-2s.Intermittent EEG showed multifocal and extensive epileptic discharge, still sharp (spine) slow wave continuous release based.Treatment: All children underwent ACTH or methylprednisolone immunoregulation treatment, 3 cases underwent ketone diet therapy.At the same time choice valproic acid, topiramate, clonazepam, lamotrigine, levarabesilan and other anti-broad-spectrum antiepileptic drugs, according to the history.all children were taken in combination with the way.Prognosis: 13 patients'seizures reduced or controled after the end of the ACTH or methylprednisolone immunotherapy course.followed-up 3 to 12 months, the clinical attack control were failed 3 cases had relatively good prognosis, treated with Ketogenic diet (Lasted for 1 year 3 mothes~2 years 5 mothes), one case of attack control, mental improvement significantly, Another 2 cases, the numbers of episodes were reduced and the level of intelligence were significantly improved. ConclusionPerinatal factors and acquired brain injury are the most common cause of pathogenesis.Spasm as a major form of attack, and other forms of coexistence.EEG is not typical of high degree of performance.Simultaneous EMG monitoring shows bilateral or unilateral synchronous EMG outbreaks.The treatment of various antiepileptic drugs were ineffective.The vast majority of patients developed refractory epilepsy.Ketogenic diet treatment may be a relatively good choice.

关键词: 癫痫性痉挛; 儿童; 脑电图; 临床特点

Key words: Epilepsy spasm; Children; Electroencephalogram; Clinical features

引用本文: 罗序峰, 张胜, 朱建萍, 谢惠源, 刘楠, 周涛, 付四毛. 儿童晚发型癫痫性痉挛的临床特点及脑电图改变. 癫痫杂志, 2017, 3(5): 389-394. doi: 10.7507/2096-0247.20170060 复制

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