Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Seizure. 2024 Oct;121:133-140. doi: 10.1016/j.seizure.2024.08.002. Epub 2024 Aug 3.
The study compared real-time motor cortex excitability using transcranial magnetic stimulation (TMS)-derived parameters between children with epileptic encephalopathy with spike-wave activation in sleep (EE-SWAS) and age-matched neurotypical controls. The EE-SWAS group received steroids as standard of care and were longitudinally followed for three months.
MATERIALS & METHODS: Children aged 5-12 years with immunotherapy-naive EE-SWAS (spike-wave-index≥50 %) and neurotypical controls were enrolled. Cognitive and behavioral assessments were performed using valid psychometric tools. Real-time motor cortex excitability was assessed by measuring resting motor threshold (RMT), short intra-cortical inhibition (SICI) and long intra-cortical inhibition (LICI) in both groups. In EE-SWAS group, a follow up evaluation with TMS at 4- and 12-week intervals, EEG, and neurobehavioral assessments at 12-weeks were performed to assess the effect of steroids on cortical excitability and to determine electroclinical outcome.
Forty-eight children with suspected EE-SWAS and 26 neurotypical controls were screened; 20 were enrolled in each group. Children with EE-SWAS (mean age: 8.05 ± 1.76 years) had cognitive and behavioral problems (20/20), and ongoing seizures (12/20). At baseline, the dominant motor cortex was significantly inhibited in the EE-SWAS group compared to neurotypical children{RMT(%)[86.3 ± 6.96 vs 58.05 ± 4.71(p < 0.0001)]; LICI(%)[55.05 ± 4.39 vs 73.9 ± 3.75(p < 0.0001)]; SICI(%)[39.2 ± 4.36 vs 55.45 ± 4.78(p < 0.0001)]}. Reversal of motor cortex inhibition was sequentially observed in EE-SWAS group at 4- and 12-week follow-ups{(RMT[4, 12 weeks]: 71.45 ± 9.83, 63.45 ± 8.48); (LICI[4, 12 weeks]: 66.00 ± 6.26, 74.50 ± 5.36); (SICI[4, 12 weeks]: 49.35 ± 6.24, 56.05 ± 5.57)}[repeated-measures ANOVA: p < 0.0001].
Motor cortex is remotely inhibited in EE-SWAS, which may contribute to neurobehavioral impairment. Steroids can disinhibit/reverse the epilepsy-induced motor cortex inhibition leading to improvement in neurobehavior.
本研究通过测量经颅磁刺激（TMS）衍生参数，比较睡眠中棘波-慢波激活的癫痫性脑病（EE-SWAS）患儿与年龄匹配的神经典型对照组之间的实时运动皮层兴奋性。EE-SWAS 组接受类固醇作为标准治疗，并进行了为期三个月的纵向随访。
纳入 5-12 岁免疫治疗初治的 EE-SWAS 患儿（棘波指数≥50%）和神经典型对照组。使用有效的心理测量工具进行认知和行为评估。在两组中均测量静息运动阈值（RMT）、短程皮质内抑制（SICI）和长程皮质内抑制（LICI），以评估实时运动皮层兴奋性。在 EE-SWAS 组中，在 4 周和 12 周时进行 TMS 随访评估、脑电图和 12 周时的神经行为评估，以评估类固醇对皮质兴奋性的影响，并确定电临床结果。
对 48 例疑似 EE-SWAS 患儿和 26 例神经典型对照组进行了筛查，每组纳入 20 例。EE-SWAS 组患儿（平均年龄：8.05±1.76 岁）存在认知和行为问题（20/20）和持续癫痫发作（12/20）。基线时，EE-SWAS 组优势运动皮层的抑制程度明显高于神经典型对照组{RMT（%）[86.3±6.96 比 58.05±4.71（p<0.0001）]；LICI（%）[55.05±4.39 比 73.9±3.75（p<0.0001）]；SICI（%）[39.2±4.36 比 55.45±4.78（p<0.0001）]}。EE-SWAS 组在 4 周和 12 周随访时依次观察到运动皮层抑制的逆转{[4、12 周的 RMT：71.45±9.83，63.45±8.48）]；[4、12 周的 LICI：66.00±6.26，74.50±5.36）]；[4、12 周的 SICI：49.35±6.24，56.05±5.57）]}[重复测量方差分析：p<0.0001]。
EE-SWAS 中运动皮层存在远程抑制，这可能导致神经行为损伤。类固醇可以解除/逆转癫痫引起的运动皮层抑制，从而改善神经行为。
