Chari Aswin, Hernan Amanda E, Mahoney J Matthew, Thornton Rachel, Tahir M Zubair, Tisdall Martin M, Scott Rod C
Developmental Neurosciences, Great Ormond Street Institute of Child Health, University College London, London, UK.
Department of Neurosurgery, Great Ormond Street Hospital, London, UK.
Sci Rep. 2024 Dec 30;14(1):31654. doi: 10.1038/s41598-024-80634-5.
Network hypersynchrony is emerging as an important system-level mechanism underlying seizures, as well as cognitive and behavioural impairments, in children with structural brain abnormalities. We investigated patterns of single neuron action potential behaviour in 206 neurons recorded from tubers, transmantle tails of tubers and normal looking cortex in 3 children with tuberous sclerosis. The patterns of neuronal firing on a neuron-by-neuron (autocorrelation) basis did not reveal any differences as a function of anatomy. However, at the level of functional networks (cross-correlation), there is a much larger propensity towards hypersynchrony of tuber-tuber neurons than in neurons from any other anatomical site. This suggests that tubers are the primary drivers of adverse outcomes in children with tuberous sclerosis.
网络超同步正成为结构性脑异常儿童癫痫发作以及认知和行为障碍背后的一种重要系统层面机制。我们研究了3名结节性硬化症患儿的结节、结节跨皮质尾以及外观正常的皮质中记录到的206个神经元的单神经元动作电位行为模式。逐个神经元(自相关)的神经元放电模式未显示出因解剖结构不同而存在任何差异。然而,在功能网络层面(互相关),与来自其他任何解剖部位的神经元相比,结节 - 结节神经元的超同步倾向要大得多。这表明结节是结节性硬化症患儿不良后果的主要驱动因素。