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1.400014 重庆, 重庆医科大学附属儿童医院:神经外科
2.400014 重庆, 重庆医科大学附属儿童医院:神经内科
3.400014 重庆, 重庆医科大学附属儿童医院:放射科
4.400014 重庆, 重庆医科大学附属儿童医院:病理科
[ 翟瑄, E-mail:zhaixuan@163.com ZHAI Xuan, E-mail:zhaixuan@163.com
收稿:2017-12-22,
修回:2018-04-28,
纸质出版:2018-08-15
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汪迪飞, 翟瑄, 梁平, 等. 38例局灶性皮质发育不良继发癫痫临床分析[J]. 第三军医大学学报, 2018,40(15):1389-1393.
Difei WANG, Xuan ZHAI, Ping LIANG, et al. Epilepsy secondary to focal cortical dysplasia: a clinical report of 38 children[J]. Journal of Third Military Medical University, 2018, 40(15): 1389-1393.
汪迪飞, 翟瑄, 梁平, 等. 38例局灶性皮质发育不良继发癫痫临床分析[J]. 第三军医大学学报, 2018,40(15):1389-1393. DOI: 10.16016/j.1000-5404.201712219.
Difei WANG, Xuan ZHAI, Ping LIANG, et al. Epilepsy secondary to focal cortical dysplasia: a clinical report of 38 children[J]. Journal of Third Military Medical University, 2018, 40(15): 1389-1393. DOI: 10.16016/j.1000-5404.201712219.
目的
2
探讨儿童局灶性皮质发育不良(focal cortical dysplasia,FCD)继发癫痫的临床特征。
方法
2
回顾性分析2014年1月至2016年12月我院神经外科收治的38例FCD继发癫痫患儿的临床表现、影像学特点、脑电图特点以及手术疗效。
结果
2
FCD患儿癫痫发作类型以全身性强直-阵挛发作为主(50%);FCD最多见于颞叶,各病理类型FCD分布部位无特殊性。磁共振波谱分析(MRS)提示病灶区氮-乙酰门东氨酸(NAA)峰降低见于所有类型的FCD,而胆碱复合物(Cho)峰升高仅见于部分FCDⅡb型。发作间期头皮脑电图(EEG)痫样放电部位与FCD病灶生长部位有较高一致性,术中皮层脑电(ECoG)检测与病灶吻合率100%。术后癫痫发作控制总有效率为71%,各型FCD的手术疗效无统计学差异。各型FCD术后1年的神经心理学评分均有明显改善。
结论
2
儿童FCD所致癫痫可表现为全面性发作,发作间期头皮脑电图检测对致痫灶的定位有一定准确性,而ECoG定位的准确性更高,各种病理类型FCD的术后疗效均较满意。
Objective
2
To analyze the clinical characteristics of epilepsy due to focal cortical dysplasia (FCD) in children.
Methods
2
The clinical data of 38 children with FCD-induced epilepsy admitted in our neurosurgical department from January 2012 to December 2016 were collected in this study. Their clinical manifestations
imaging findings
electroencephalogram (EEG) features
and surgical efficacy were analyzed.
Results
2
The most common form (50%) in clinical seizures in these FCD children was generalized tonic-clonic seizures. FCD was mainly observed in the temporal lobe
and its locations of lesions had no significant differences among the children with different FCD types. Magnetic resonance spectrum (MRS) showed that the peak of N-acetylaspartate (NAA) was lowered in all types of FCD
but the choline (Cho) peak was reduced only in some FCD Ⅱb type. EEG recordings at seizure onset indicated that the epileptogenic zones were of high consistence with the lesions of FCD. There was a proper match (100%) between the intraoperative cortical electroencephalogram (ECoG) and FCD lesions. The total effective rate of seizure control was 71% in the FCD children
and no statistical difference was found among the children with different FCD types. Neuropsychological assessment confirmed that all the children with different FCD types had got great improvement in 1 year after surgery.
Conclusion
2
Generalized seizures are most common in epilepsy secondary to FCD in children. Scalp EEG at seizure onset is helpful in epileptic focus location
but ECoG is more accurate. Satisfied curative effect is obtained in the children with different FCD types.
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KWON H E, EOM S, KANG H C, et al . Surgical treatment of pediatric focal cortical dysplasia:Clinical spectrum and surgical outcome[J ] .Neurology, 2016, 87(9):945-951. DOI:10.1212/WNL.0000000000003042.
FAUSER S, ESSANG C, ALTENMVLLER D M, et al . Long-term seizure outcome in 211 patients with focal cortical dysplasia[J ] .Epilepsia, 2015, 56(1):66-76.DOI:10.1111/epi.12876.
KUZNIECKY R. Clinical applications of MR spectroscopy in epilepsy[J].Neuroimaging Clin N Am, 2004, 14(3):507-516. DOI:10.1016/j.nic.2004.04.010.
CEPEDA C, ANDRÉ V M, WU N, et al .Immature neurons and GABA networks may contribute toepileptogenesis in pediatric cortical dysplasia[J ] .Epilepsia, 2007, 48(Suppl 5):79-85.DOI:10.1111/j.1528-1167.2007.01293.x.
COLON A J, HOFMAN P, OSSENBLOK P P, et al .MRS-lateralisation index in patients with epilepsy and focal cortical dysplasia or a MEG-focus using bilateral single voxels[J ] .Epilepsy Res, 2010, 89(1):148-153. DOI:10.1016/j.eplepsyres.2009.11.007.
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KABAT J, KRóL P. Focal cortical dysplasia-review[J].Pol J Radiol, 2012, 77(2):35-43.
VAROTTO G, TASSI L, FRANCESCHETTI S, et al . Epileptogenic networks of type Ⅱ focal cortical dysplasia:a stereo-EEG study[J ] .Neuroimage, 2012, 61(3):591-598. DOI:10.1016/j.neuroimage.2012.03.090.
BARKOVICH A J, GUERRINI R, KUZNIECKY R I, et al . A developmental and genetic classification for malformations of cortical development:update 2012[J ] .Brain, 2012, 135(Pt 5):1348-1369. DOI:10.1093/brain/aws019.
BOSHUISEN K, ARZIMANOGLOU A, CROSS J H, et al . Timing of antiepileptic drug withdrawal and long-term seizure outcome after paediatric epilepsy surgery (TimeToStop):a retrospective observational study[J ] . Lancet Neurol, 2012, 11(9):784-791. DOI:10.1016/s1474-4422(12)70165-5.
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