Objective:

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.

Methods:

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.

Results:

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 (P = .003) as was poor response to first treatment for spasms (P = .004). Children with an unknown etiology of infantile spasms were less likely to develop Lennox-Gastaut syndrome (P = .019). Eighty percent (28/35) of the children who met Lennox-Gastaut syndrome criteria lacked a documented diagnosis.

Conclusions:

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.

中文翻译：

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婴儿痉挛到 Lennox-Gastaut 综合征的演变：有什么需要知道的？

客观的：

患有婴儿痉挛症的儿童可能会发展为 Lennox-Gastaut 综合征。Lennox-Gastaut 综合征的诊断标准不明确，许多专家使用以下标准的不同组合进行诊断：脑电图 (EEG) 上的阵发性快速活动、脑电图上的缓慢棘波和波、发育迟缓、多种癫痫发作类型和夜间强直癫痫发作。我们的目标是确定有婴儿痉挛病史的高危儿童队列中 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特征。

方法：

包括 2012 年至 2018 年间在科罗拉多州儿童医院诊断和治疗的婴儿痉挛儿童。Lennox-Gastaut 综合征被定义为具有以下 5 种特征中的 3 种：阵发性快速活动、缓慢的尖峰和波、当前发育迟缓、多种癫痫发作类型或强直性癫痫发作。使用中位数和四分位数范围进行描述性统计。使用 Pearson 卡方检验、Fisher 精确检验或 Kruskal-Wallis 检验进行单变量分析。

结果：

97 名儿童符合纳入标准，36% (35/97) 符合 Lennox-Gastaut 综合征标准。婴儿痉挛发作前的发育迟缓和癫痫病史被确定为发生 Lennox-Gastaut 综合征的危险因素 ( P = .003)，对痉挛的首次治疗反应不佳 ( P = .004) 也是如此。婴儿痉挛病因不明的儿童患 Lennox-Gastaut 综合征的可能性较小 ( P = .019)。80% (28/35) 符合 Lennox-Gastaut 综合征标准的儿童缺乏记录在案的诊断。

结论：

36% 患有婴儿痉挛症的儿童符合 Lennox-Gastaut 综合征的标准。Lennox-Gastaut 综合征的危险因素是婴儿痉挛发作前的发育迟缓和癫痫发作，以及对婴儿痉挛的首次治疗反应不佳。婴儿痉挛病因不明的儿童患 Lennox-Gastaut 综合征的可能性较小。80% 符合我们标准的儿童没有得到 Lennox-Gastaut 综合征的记录诊断，这突出了许多儿童可能没有接受 Lennox-Gastaut 综合征诊断的事实。我们建议制定明确的 Lennox-Gastaut 综合征诊断指南，以确保准确做出诊断。