Yang Mei-Hua, Liu Juan, Zhou Yi-Ling, Yang Hui, Cai Fang-Cheng, Zempel John, Yang Qing-Wu, Liu Shi-Yong
Epilepsy Centre, Department of Neurosurgery, Xinqiao Hospital, Army Military Medical University, Chongqing, China.
Department of Neurology, Xinqiao Hospital, Army Military Medical University, Chongqing, China.
Pediatr Neurosurg. 2020;55(1):26-35. doi: 10.1159/000504513. Epub 2019 Dec 18.
Lennox-Gastaut syndrome (LGS) is a severe subtype of childhood-onset epileptic encephalopathy with drug-resistant and poor surgical prognosis. However, electroencephalogram (EEG) patterns of symptomatic LGS or LG phenotypes with structural brain lesions including focal abnormalities or asymmetric slow-spike-wave (SSW) patterns remain largely unknown. Due to the contradictory lateralization difference between MRI lesions and EEG pattern in symptomatic LGS or LG phenotypes, it is difficult to determine the precise lateralization of epileptic lesions, which is crucial to better surgical prognosis. This study is aim to ascertain the clinical characteristics of the EEG patterns, and its relationship with MRI lesions and to evaluate its prognostic value of surgical treatment in symptomatic LGS or LG phenotypes.
Twenty-four symptomatic LGS cases with asymmetric EEG SSW patterns and contralaterally independent or contralaterally dominant MRI lesions were collected, and their clinical features were analyzed retrospectively.
In this cohort, most of lesions were perinatal or acquired during the first 6 months of life. The most common etiology was intracerebral hemorrhage. The LGS patients with both asymmetric SSW and focal sporadic epileptic waves (SEW) patterns showed the best surgical outcome with Engel class I level. Asymmetric SSW patterns with maximal discharges contralateral to MRI lesions were frequently observed in most of symptomatic LGS or LG phenotypes. Predominantly diffuse destructive lesions led to an attenuated voltage of ipsilateral scalp EEG producing an asymmetric SSW pattern in those patients with symptomatic LGS or LG phenotypes.
Our study reveals a special SEW EEG pattern in symptomatic LG patients with asymmetric SSW and MRI lesions contralateral to the dominant EEG patterns. Contradictory lateralization difference between MRI and EEG probably arises from the relative voltage attenuation presenting in EEG ipsilateral to huge destructive lesions from early life. Our study suggests that the independent focal SEW activity remaining ipsilateral to the MRI lesion can potentially predict better surgical prognosis in symptomatic LGS or LG phenotypes.
Lennox-Gastaut综合征(LGS)是儿童期起病的癫痫性脑病的一种严重亚型,具有耐药性且手术预后较差。然而,有症状的LGS或具有脑结构病变(包括局灶性异常或不对称慢棘波(SSW)模式)的LG表型的脑电图(EEG)模式在很大程度上仍不清楚。由于有症状的LGS或LG表型的MRI病变与EEG模式之间存在矛盾的定位差异,因此难以确定癫痫病灶的精确位置,而这对于改善手术预后至关重要。本研究旨在确定EEG模式的临床特征及其与MRI病变的关系,并评估其对有症状的LGS或LG表型手术治疗的预后价值。
收集24例具有不对称EEG慢棘波模式且MRI病变对侧独立或对侧占优的有症状LGS病例,并对其临床特征进行回顾性分析。
在该队列中,大多数病变是围生期的或在生命的前6个月内获得的。最常见的病因是脑出血。具有不对称慢棘波和局灶性散发性癫痫波(SEW)模式的LGS患者手术效果最佳,达到Engel I级水平。在大多数有症状的LGS或LG表型中,经常观察到与MRI病变对侧放电最大的不对称慢棘波模式。主要为弥漫性破坏性病变导致有症状的LGS或LG表型患者同侧头皮EEG电压衰减,从而产生不对称慢棘波模式。
我们的研究揭示了有症状的LG患者中一种特殊的散发性癫痫波EEG模式,其慢棘波和MRI病变与占主导地位的EEG模式对侧。MRI和EEG之间矛盾的定位差异可能源于早期巨大破坏性病变同侧EEG中出现的相对电压衰减。我们的研究表明,MRI病变同侧残留的独立局灶性散发性癫痫波活动可能预示有症状的LGS或LG表型患者手术预后较好。