尼泊尔东部以医院为基础的儿童癫痫综合征临床特征和治疗结果研究。

Clinical profile and treatment outcome of epilepsy syndromes in children: A hospital-based study in Eastern Nepal.

机构信息

Department of Pediatrics and Adolescent Medicine, B.P. Koirala Institute of Health Sciences, Dharan, Nepal.

Department of Psychiatric Nursing, College of Nursing, B.P. Koirala Institute of Health Sciences, Dharan, Nepal.

出版信息

Epilepsia Open. 2021 Feb 8;6(1):206-215. doi: 10.1002/epi4.12470. eCollection 2021 Mar.

DOI:10.1002/epi4.12470

PMID:33681663

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7918298/

Abstract

OBJECTIVE

It is often difficult to diagnose epilepsy syndromes in resource-limited settings. This study was aimed to investigate the prospect of ascertaining the diagnosis, clinical profile, and treatment outcomes of epilepsy syndromes (ESs) among children in a resource-limited setting.

METHODS

This was a descriptive study done from 01/07/2009 to 15/06/2017 among children (1-17 years of age) with unprovoked seizures presenting to the pediatric neurology clinic of a university hospital in eastern Nepal. Diagnosis, classification, and treatment of seizures were based upon International League Against Epilepsy guidelines.

RESULTS

Of 768 children with unprovoked seizures, 120 (15.6%) were diagnosed as ES. The age of onset of seizure was unique for each ES. Developmental delay and cerebral palsy were present in 47.5% and 28.3% children, respectively. Common ESs were West syndrome (WS)-26.7%, generalized tonic-clonic seizures alone (GTCSA)-21.7%, self-limited childhood epilepsy with centrotemporal spikes (SLCECTS)-12.5%, childhood absence epilepsy (CAE)-10.0%, Lennox-Gastaut syndrome (LGS)-10.0%, other developmental and epileptic encephalopathies (DEE)-5.8%, self-limited familial infantile epilepsy (SLFIE)-4.2%, and juvenile myoclonic epilepsy (JME)-3.3%. Among children with known outcomes (87/120), overall response to pharmacotherapy and to monotherapy was observed in 72.4% (63/87) and 57.5% (50/87) children, respectively. All children with GTCSA, SLFIE, genetic epilepsy with febrile seizure plus (GEFS+), CAE, SLCECTS, and JME responded to pharmacotherapy and they had normal computerized tomography scans of the brain. Seizures were largely pharmaco-resistant in progressive myoclonus epilepsy (PME)-100.0%, LGS-73.0%, WS-52.0%, and other DEEs-40%.

SIGNIFICANCE

A reasonable proportion (15.6%) of unprovoked seizures could be classified into specific ES despite limited diagnostic resources. WS was the most common ES. GTCSA, SLCECTS, CAE, and LGS were other common ESs. GTCSA, SLFIE, CAE, SLCECTS, GEFS+, and JME were largely pharmaco-responsive. PME, WS, and LGS were relatively pharmaco-resistant. Electro-clinical diagnosis of certain ES avoids the necessity of neuroimaging.

摘要

目的

在资源有限的环境中，癫痫综合征的诊断往往具有挑战性。本研究旨在探讨在资源有限的环境中确定儿童癫痫综合征（ES）诊断、临床特征和治疗结局的可能性。

方法

这是一项描述性研究，于 2009 年 7 月 1 日至 2017 年 6 月 15 日在尼泊尔东部一所大学医院的儿科神经病学诊所对患有无诱因发作的儿童（1-17 岁）进行。根据国际抗癫痫联盟的指南，对癫痫发作进行诊断、分类和治疗。

结果

在 768 名患有无诱因癫痫发作的儿童中，有 120 名（15.6%）被诊断为 ES。每种 ES 的发病年龄均具有独特性。47.5%的儿童存在发育迟缓，28.3%的儿童存在脑瘫。常见的 ES 包括 West 综合征（WS）-26.7%、单纯全面性强直阵挛发作（GTCSA）-21.7%、自限性儿童期癫痫伴中央颞区棘波（SLCECTS）-12.5%、儿童失神癫痫（CAE）-10.0%、Lennox-Gastaut 综合征（LGS）-10.0%、其他发育性和癫痫性脑病（DEE）-5.8%、自限性家族性婴儿癫痫（SLFIE）-4.2%、青少年肌阵挛癫痫（JME）-3.3%。在已知结局的儿童中（87/120），观察到整体药物治疗反应和单药治疗反应分别在 72.4%（63/87）和 57.5%（50/87）的儿童中。所有 GTCSA、SLFIE、热性惊厥附加遗传癫痫（GEFS+）、CAE、SLCECTS 和 JME 儿童均对药物治疗有反应，且大脑计算机断层扫描正常。进行性肌阵挛癫痫（PME）-100.0%、LGS-73.0%、WS-52.0%和其他 DEEs-40%的癫痫发作对药物治疗反应差。

意义

尽管诊断资源有限，但仍有相当比例（15.6%）的无诱因癫痫发作可归类为特定的 ES。WS 是最常见的 ES。GTCSA、SLCECTS、CAE 和 LGS 是其他常见的 ES。GTCSA、SLFIE、CAE、SLCECTS、GEFS+和 JME 对药物治疗反应良好。PME、WS 和 LGS 对药物治疗反应较差。某些 ES 的电临床诊断可避免神经影像学检查的必要性。