Epileptic spasm

癫痫性痉挛
  • 文章类型: English Abstract
    OBJECTIVE: To investigate the role of brain functional connectivity and nonlinear dynamic analysis in brain function assessment for infants with controlled infantile spasm (IS).
    METHODS: A retrospective analysis was performed on 14 children with controlled IS (IS group) who were admitted to the Department of Neurology, Anhui Provincial Children\'s Hospital, from January 2019 to January 2023. Twelve healthy children, matched for sex and age, were enrolled as the control group. Electroencephalogram (EEG) data were analyzed for both groups to compare the features of brain network, and nonlinear dynamic indicators were calculated, including approximate entropy, sample entropy, permutation entropy, and permutation Lempel-Ziv complexity.
    RESULTS: Brain functional connectivity showed that compared with the control group, the IS group had an increase in the strength of functional connectivity, and there was a significant difference between the two groups in the connection strength between the Fp2 and F8 channels (P<0.05). The network stability analysis showed that the IS group had a significantly higher network stability than the control group at different time windows (P<0.05). The nonlinear dynamic analysis showed that compared with the control group, the IS group had a significantly lower sample entropy of Fz electrode (P<0.05).
    CONCLUSIONS: Abnormalities in brain network and sample entropy may be observed in some children with controlled IS, and it is suggested that quantitative EEG analysis parameters can serve as neurological biomarkers for evaluating brain function in children with IS.
    目的: 探讨脑功能连接及非线性动力学分析在发作控制的婴儿痉挛症(infantile spasm, IS)患儿脑功能评估中的作用。方法: 回顾性选择2019年1月—2023年1月安徽省儿童医院神经科就诊且发作控制的14例IS患儿为IS组,选择同期性别、年龄匹配的12例健康体检儿童为健康对照组。分析2组患儿的脑电图数据,比较其脑网络特征,同时计算非线性动力学指标,包括近似熵、样本熵、排列熵、LZ复杂度。结果: 功能连接显示,与健康对照组比较,IS组网络连接强度增大,其中Fp2与F8两通道之间的连接强度组间比较差异有统计学意义(P<0.05)。网络稳定性分析发现,在不同长度时间窗口下,IS组网络稳定性均高于健康对照组(P<0.05)。非线性动力学分析显示,IS组Fz电极上样本熵小于健康对照组(P<0.05)。结论: 少数预后良好的IS患儿仍存在脑网络及样本熵异常,推测脑电定量分析指标可成为评价IS患儿脑功能状态的神经生物学标志物。.
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  • 文章类型: Journal Article
    为了分析癫痫发作符号学的影响,评估了癫痫性痉挛(ES)患儿的脑电图(EEG)特征和磁共振成像(MRI)改变对癫痫发生区定位和手术预后的影响。回顾性分析了127例接受手术治疗的难治性癫痫伴ES患者的数据。ES符号学被归类为非横向化,双边不对称,和焦点。发作间癫痫样放电分为弥漫性或多灶性,单边,和焦点。MRI结果显示所有患者可见局部病灶,而癫痫发生区定位的解剖-电-临床价值取决于手术结果。在术前视频脑电图监测期间,在所有127例病例中,53例(41.7%)仅患有ES,46人(36.2%)有ES和局灶性癫痫发作,17人(13.4%)患有ES和全身性癫痫发作,11例(8.7%)有ES伴局灶性和全身性癫痫发作.值得注意的是,35例(27.6%)和92例(72.4%)显示简单和复杂的ES,分别。发作间脑电图显示22例(17.3%)有双侧多灶性放电或心律失常,25人(19.7%)有单方面主导排放,80例(63.0%)有明确的局灶性或区域性放电。71例(55.9%)和56例(44.1%)的明确/侧方出院。手术切除的病灶位于半球(28.3%),额叶(24.4%),颞叶(16.5%),颞顶枕区(14.2%),和后皮质区(8.7%)。术后1年和4年无癫痫发生率分别为81.8%和72.7%,分别。ES的电临床特征与无癫痫发作率之间没有显着差异。在该队列中,大多数患者的手术治疗结果良好。ES的符号学和发作脑电图变化对定位没有影响,而发作间脑电图的局灶性或侧向癫痫样放电可能会影响侧向化和定位。通过MRI确定的癫痫灶的完全切除是与阳性手术结果相关的唯一因素。
    To analyze the influence of seizure semiology, electroencephalography (EEG) features and magnetic resonance imaging (MRI) change on epileptogenic zone localization and surgical prognosis in children with epileptic spasm (ES) were assessed. Data from 127 patients with medically intractable epilepsy with ES who underwent surgical treatment were retrospectively analyzed. ES semiology was classified as non-lateralized, bilateral asymmetric, and focal. Interictal epileptiform discharges were divided into diffusive or multifocal, unilateral, and focal. MRI results showed visible local lesions for all patients, while the anatomo-electrical-clinical value of localization of the epileptogenic zone was dependent on the surgical outcome. During preoperative video EEG monitoring, among all 127 cases, 53 cases (41.7%) had ES only, 46 (36.2%) had ES and focal seizures, 17 (13.4%) had ES and generalized seizures, and 11 (8.7%) had ES with focal and generalized seizures. Notably, 35 (27.6%) and 92 cases (72.4%) showed simple and complex ES, respectively. Interictal EEG showed that 22 cases (17.3%) had bilateral multifocal discharges or hypsarrhythmia, 25 (19.7%) had unilateral dominant discharges, and 80 (63.0%) had definite focal or regional discharges. Ictal discharges were generalized/bilateral in 71 cases (55.9%) and definite/lateralized in 56 cases (44.1%). Surgically resected lesions were in the hemisphere (28.3%), frontal lobe (24.4%), temporal lobe (16.5%), temporo-parieto-occipital region (14.2%), and posterior cortex region (8.7%). Seizure-free rates at 1 and 4 years postoperatively were 81.8 and 72.7%, respectively. There was no significant difference between electroclinical characteristics of ES and seizure-free rate. Surgical treatment showed good outcomes in most patients in this cohort. Semiology and ictal EEG change of ES had no effect on localization, while focal or lateralized epileptiform discharges of interictal EEG may affect lateralization and localization. Complete resection of epileptogenic lesions identified via MRI was the only factor associated with a positive surgical outcome.
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  • 文章类型: Journal Article
    目的:分析临床特点,治疗,维生素B6依赖性癫痫的癫痫性痉挛(ES)的预后,包括由ALDH7A1突变引起的吡哆醇依赖性癫痫(PDE)患者,吡啶(AM)-5'-磷酸氧化酶(PNPO)缺乏症,PLPBP缺乏症。方法:我们分析了54例PDE患者的队列数据,13例PNPO缺乏症,2例PLPBP缺乏症,并在其中寻找ES的表现。结果:共收集到11例有ES发作表现的患者。其中,四名患者携带ALDH7A1突变,六名携带PNPO突变,其余1例携带PLPBP突变。该队列的分析确定了分布在三种疾病中的9例表现为婴儿痉挛的病例和2例表现为被诊断为PDE和PNPO缺乏症的Ohtahara综合征的病例。分别。在PDE和PLPBP缺乏症组中,癫痫发作由吡哆醇单药治疗控制,其余患者因继发性脑萎缩而出现难治性癫痫发作。在PNPO缺乏的群体中,一名患者在接受PLP联合丙戊酸治疗时表现为无癫痫发作,通过PLP单一疗法或吡哆醇或PLP联合其他抗癫痫药物治疗的3例癫痫发作仍然很少,两人死亡。在两个表现为大田原综合征的病例中,常规治疗后,一个显示没有癫痫发作,其他人的癫痫发作频率明显下降,他们的脑电图都有改善.意义:ES可能是PNPO缺乏症中常见的癫痫发作形式,脑电图表现为心律失常或突发抑制模式。吡哆醇难以控制继发性脑损伤引起的频繁发作。在我们的PNPO缺乏症队列中,婴儿痉挛患者对PLP的反应并不比吡哆醇好.及时正确的治疗可以防止儿童疾病从大田原综合征和婴儿痉挛转变为随后的癫痫性脑病或难治性癫痫。
    Objective: To analyze the clinical feature, treatment, and prognosis of epileptic spasms (ES) in vitamin B6-dependent epilepsy, including patients with pyridoxine-dependent epilepsy (PDE) caused by ALDH7A1 mutation, pyridox(am)ine-5\'-phosphate oxidase (PNPO) deficiency, and PLPBP deficiency. Methods: We analyzed data from a cohort of 54 cases with PDE, 13 cases with PNPO deficiency, and 2 cases with PLPBP deficiency and looked for the presentation of ES among them. Results: A total of 11 patients with the seizure presentation of ES have been collected. Among them, four patients carried mutations in ALDH7A1, six carried mutations in PNPO, and the remaining one carried mutation in PLPBP. The analysis of this cohort identified nine cases presenting as infantile spasms distributed in the three diseases and two cases presenting as Ohtahara syndrome diagnosed with PDE and PNPO deficiency, respectively. In the PDE and PLPBP deficiency groups, seizures were controlled by pyridoxine monotherapy, and the remaining one had refractory seizures due to secondary brain atrophy. In the groups with PNPO deficiency, one patient showed seizure-free when treated by PLP combined with valproic acid, three still had infrequent seizures treated by PLP monotherapy or pyridoxine or PLP combined with other antiseizure medications, and two died. In two cases presenting as Ohtahara syndrome, after regular treatment, one showed seizure-free, the others showed a marked decrease in seizure frequency, and they both showed an improvement in EEG. Significance: ES might be a common form of seizures in PNPO deficiency, and EEG presented as hypsarrhythmia or a burst suppression pattern. It is difficult for pyridoxine to control frequent seizures caused by secondary brain injury. In our PNPO deficiency cohort, patients with infantile spasms did not respond better to PLP than pyridoxine. Timely and correct treatment could prevent the transformation of the child\'s disease from Ohtahara syndrome and infantile spasms to subsequent epileptic encephalopathy or refractory epilepsy.
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