Department of Pediatrics and Neurology, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
Adult and Child Consortium for Health Outcomes Research and Delivery Science, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.
J Child Neurol. 2021 Aug;36(9):752-759. doi: 10.1177/08830738211000514. Epub 2021 Mar 25.
Children with infantile spasms may develop Lennox-Gastaut syndrome. The diagnostic criteria for Lennox-Gastaut syndrome are vague, and many experts use varying combinations of the following criteria for diagnosis: paroxysmal fast activity on electroencephalography (EEG), slow spike and wave on EEG, developmental delay, multiple seizure types, and nocturnal tonic seizures. Our objective was to determine the prevalence of Lennox-Gastaut syndrome in a high-risk cohort of children with a history of infantile spasms and the characteristics of infantile spasms that were associated with the diagnosis of Lennox-Gastaut syndrome.
Children with infantile spasms who were diagnosed and treated at Children's Hospital Colorado between 2012 and 2018 were included. Lennox-Gastaut syndrome was defined as having 3 of 5 of the following characteristics: paroxysmal fast activity, slow spike and wave, current developmental delay, multiple seizure types, or tonic seizures. Descriptive statistics were performed using median and interquartile range. Univariable analysis was performed with Pearson chi-square, Fisher exact, or the Kruskal-Wallis test.
Ninety-seven children met inclusion criteria, and 36% (35/97) met criteria for Lennox-Gastaut syndrome. Developmental delay and history of seizures prior to the onset of infantile spasms were identified as risk factors for the development of Lennox-Gastaut syndrome ( = .003) as was poor response to first treatment for spasms ( = .004). Children with an unknown etiology of infantile spasms were less likely to develop Lennox-Gastaut syndrome ( = .019). Eighty percent (28/35) of the children who met Lennox-Gastaut syndrome criteria lacked a documented diagnosis.
Thirty-six percent of children with infantile spasms met criteria for Lennox-Gastaut syndrome. Risk factors for development of Lennox-Gastaut syndrome were developmental delay and seizures prior to the onset of infantile spasms and poor response to first treatment for infantile spasms. Children with an unknown etiology of infantile spasms were less likely to develop Lennox-Gastaut syndrome. Eighty percent of the children who met our criteria were not given a documented diagnosis of Lennox-Gastaut syndrome, which highlights the fact that many children may not be receiving a diagnosis of Lennox-Gastaut syndrome. We recommend establishing clear guidelines for the diagnosis of Lennox-Gastaut syndrome to ensure that the diagnosis is being made accurately.
患有婴儿痉挛症的儿童可能会发展为 Lennox-Gastaut 综合征。Lennox-Gastaut 综合征的诊断标准较为模糊，许多专家在诊断时使用脑电图（EEG）阵发性快活动、EEG 慢棘慢波、发育迟缓、多种发作类型和夜间强直发作等不同组合的标准。我们的目的是确定具有婴儿痉挛症病史的高危患儿中 Lennox-Gastaut 综合征的患病率，以及与 Lennox-Gastaut 综合征诊断相关的婴儿痉挛症特征。
纳入 2012 年至 2018 年在科罗拉多儿童医院诊断和治疗的婴儿痉挛症患儿。将 Lennox-Gastaut 综合征定义为具有以下 5 种特征中的 3 种：阵发性快活动、慢棘慢波、当前发育迟缓、多种发作类型或强直发作。使用中位数和四分位距进行描述性统计。采用 Pearson 卡方检验、Fisher 确切检验或 Kruskal-Wallis 检验进行单变量分析。
97 名患儿符合纳入标准，其中 36%（35/97）符合 Lennox-Gastaut 综合征标准。发育迟缓以及婴儿痉挛发作前的癫痫发作史被确定为 Lennox-Gastaut 综合征发生的危险因素（=0.003），痉挛首次治疗反应差也是危险因素（=0.004）。婴儿痉挛病因不明的患儿发生 Lennox-Gastaut 综合征的可能性较低（=0.019）。80%（28/35）符合 Lennox-Gastaut 综合征标准的患儿未确诊。
36%的婴儿痉挛症患儿符合 Lennox-Gastaut 综合征标准。Lennox-Gastaut 综合征发生的危险因素是发育迟缓、婴儿痉挛发作前的癫痫发作以及婴儿痉挛首次治疗反应差。婴儿痉挛病因不明的患儿发生 Lennox-Gastaut 综合征的可能性较低。80%符合我们标准的患儿未被明确诊断为 Lennox-Gastaut 综合征，这突出表明许多患儿可能未被诊断为 Lennox-Gastaut 综合征。我们建议制定明确的 Lennox-Gastaut 综合征诊断标准，以确保诊断准确。
