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小儿癫痫的治疗:专家意见,2005年

Treatment of pediatric epilepsy: expert opinion, 2005.

作者信息

Wheless James W, Clarke Dave F, Carpenter Daniel

机构信息

Division of Pediatric Neurology, Le Bonheur Comprehensive Epilepsy Program, University of Tennessee Health Science Center, Memphis, TN 38105, USA.

出版信息

J Child Neurol. 2005 Dec;20 Suppl 1:S1-56; quiz S59-60. doi: 10.1177/088307380502000101.

Abstract

BACKGROUND

Childhood epilepsies are a heterogeneous group of conditions that differ in diagnostic criteria and management and have dramatically different outcomes. Despite increasing data on treatment of epilepsy, research findings on childhood epilepsy are more limited and many clinical questions remain unanswered, so that clinicians must often rely on clinical judgment. In such clinical situations, expert opinion can be especially helpful.

METHODS

A survey on pediatric epilepsy and seizures (33 questions and 645 treatment options) was sent to 41 U.S. physicians specializing in pediatric epilepsy, 39 (95%) of whom completed it. In some questions, the experts were asked to recommend overall treatment approaches for specific syndromes (the order in which they would use certain strategies). Most of the questions asked the experts to rate options using a modified version of the RAND 9-point scale for medical appropriateness. Consensus was defined as a non-random distribution of scores by chi-square test, with ratings used to assign a categorical rank (first line/usually appropriate, second line/equivocal, and third line/usually not appropriate) to each option.

RESULTS

Valproate was treatment of choice for symptomatic myoclonic and generalized tonic-clonic seizures except in the very young, with lamotrigine and topiramate also first line (usually appropriate). Zonisamide was first line only if the child also has myoclonic seizures. For initial monotherapy for complex partial seizures, oxcarbazepine and carbamazepine were treatments of choice, with lamotrigine and levetiracetam also first line. As initial therapy for infantile spasms caused by tuberous sclerosis, viagabatrin was treatment of choice, with adrenocorticotropic hormone (ACTH) also first line. As initial therapy for infantile spasms that are symptomatic in etiology, ACTH was treatment of choice, with topiramate also first line. As initial therapy for Lennox-Gastaut syndrome, valproate was treatment of choice, with topiramate and lamotrigine also first line. For acute treatment of a prolonged febrile seizure or cluster of seizures, rectal diazepam was treatment of choice. For benign childhood epilepsy with centro-temporal spikes, oxcarbazepine and carbamazepine were treatments of choice, with gabapentin, lamotrigine, and levetiracetam also first line. For childhood absence epilepsy, ethosuximide was treatment of choice, with valproate and lamotrigine also first line. For juvenile absence epilepsy, valproate and lamotrigine were treatments of choice. For juvenile myoclonic epilepsy in adolescent males, valproate and lamotrigine were treatments of choice, with topiramate also first line; for juvenile myoclonic epilepsy in adolescent females, lamotrigine was treatment of choice, with topiramate and valproate other first-line options. As initial therapy for neonatal status epilepticus, intravenous phenobarbital was treatment of choice, with intravenous lorazepam or fosphenytoin also first line. As initial therapy for all types of pediatric status epilepticus, lorazepam was treatment of choice, with intravenous diazepam also first line. For generalized tonic-clonic status epilepticus, rectal diazepam and fosphenytoin were also first line; for complex partial status epilepticus, fosphenytoin was also first line; and for absence status epilepticus, intravenous valproate was also first line.

CONCLUSION

The expert panel reached consensus on many treatment options. Within the limits of expert opinion and with the understanding that new research data may take precedence, the experts' recommendations provide helpful guidance in situations where the medical literature is scant or lacking. The information in this report should be evaluated in conjunction with evidence-based findings.

摘要

背景

儿童癫痫是一组异质性疾病,其诊断标准和治疗方法不同,预后也有很大差异。尽管关于癫痫治疗的数据不断增加,但关于儿童癫痫的研究结果更为有限,许多临床问题仍未得到解答,因此临床医生常常必须依靠临床判断。在这种临床情况下,专家意见可能会特别有帮助。

方法

向41名美国儿科癫痫专科医生发送了一份关于小儿癫痫和发作的调查问卷(33个问题和645种治疗选择),其中39名(95%)完成了调查。在一些问题中,要求专家推荐针对特定综合征的总体治疗方法(他们使用某些策略的顺序)。大多数问题要求专家使用RAND医疗适宜性9分制的修改版本对选项进行评分。通过卡方检验将共识定义为分数的非随机分布,评分用于为每个选项分配一个分类等级(一线/通常适宜、二线/模棱两可、三线/通常不适宜)。

结果

丙戊酸盐是有症状性肌阵挛和全身性强直阵挛发作的首选治疗药物,但非常年幼的儿童除外,拉莫三嗪和托吡酯也是一线药物(通常适宜)。只有当儿童也有肌阵挛发作时,唑尼沙胺才是一线药物。对于复杂部分性发作的初始单药治疗,奥卡西平和卡马西平是首选治疗药物,拉莫三嗪和左乙拉西坦也是一线药物。作为结节性硬化症所致婴儿痉挛症的初始治疗,氨己烯酸是首选治疗药物,促肾上腺皮质激素(ACTH)也是一线药物。对于病因有症状的婴儿痉挛症的初始治疗,ACTH是首选治疗药物,托吡酯也是一线药物。作为Lennox-Gastaut综合征的初始治疗,丙戊酸盐是首选治疗药物,托吡酯和拉莫三嗪也是一线药物。对于长时间发热性惊厥或惊厥簇的急性治疗,直肠用安定是首选治疗药物。对于伴有中央颞区棘波的良性儿童癫痫,奥卡西平和卡马西平是首选治疗药物,加巴喷丁、拉莫三嗪和左乙拉西坦也是一线药物。对于儿童失神癫痫,乙琥胺是首选治疗药物,丙戊酸盐和拉莫三嗪也是一线药物。对于青少年失神癫痫,丙戊酸盐和拉莫三嗪是首选治疗药物。对于青春期男性的青少年肌阵挛癫痫,丙戊酸盐和拉莫三嗪是首选治疗药物,托吡酯也是一线药物;对于青春期女性的青少年肌阵挛癫痫,拉莫三嗪是首选治疗药物,托吡酯和丙戊酸盐是其他一线选择。作为新生儿癫痫持续状态的初始治疗,静脉注射苯巴比妥是首选治疗药物,静脉注射劳拉西泮或磷苯妥英也是一线药物。作为所有类型小儿癫痫持续状态的初始治疗,劳拉西泮是首选治疗药物,静脉注射地西泮也是一线药物。对于全身性强直阵挛性癫痫持续状态,直肠用安定和磷苯妥英也是一线药物;对于复杂部分性癫痫持续状态,磷苯妥英也是一线药物;对于失神癫痫持续状态,静脉注射丙戊酸盐也是一线药物。

结论

专家小组在许多治疗选择上达成了共识。在专家意见的范围内,并理解新的研究数据可能优先适用的情况下,专家的建议在医学文献匮乏或缺乏的情况下提供了有用的指导。本报告中的信息应结合循证研究结果进行评估。

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