Department of Neurology, 1st Faculty of Medicine and Teaching Hospital, Charles University, Prague, Czech Republic.
Eur J Paediatr Neurol. 2011 Jul;15(4):320-5. doi: 10.1016/j.ejpn.2011.01.008. Epub 2011 Feb 22.
Narcolepsy is a life-long disease characterized by abnormal regulation of the sleep-wake cycle and increased penetration of rapid eye movement (REM) sleep. In children, narcolepsy without cataplexy is more frequently seen than in adults. The aim of our study was to evaluate clinical and polysomnographic parameters to verify if cataplexy appearing later in life can be foretold.
30 patients (12 boys), who contracted narcolepsy before the age of 18, were enrolled. All underwent clinical examination, nocturnal polysomnography (PSG), multiple sleep latency test (MSLT), HLA-DQB1∗0602 testing and, most of them Epworth Sleepiness Scale (ESS) rating. The Mann-Whitney rank and Fisher's tests were used for statistical analysis.
Narcolepsy without cataplexy (NwC) was diagnosed in 40% of the patients. The mean age at the first symptoms was 14.0 ± 3.0, at diagnosis 15.6 ± 3.1 years. Narcolepsy was accompanied by hypnagogic hallucinations in 15 and sleep paralysis in 12 patients. Frequent symptoms were sleep inertia during awakening, REM behavior symptoms, behavioral and serious school problems. BMI was higher in patients with narcolepsy-cataplexy (N-C). A high ESS score was indicative of excessive daytime sleepiness (17.1 ± 2.5). Mean MSLT sleep latency was 4.0 ± 3.1 min with 3.2 ± 1.4 sleep onset REM periods (SOREMs) with no difference between the two study groups. HLA typing revealed no differences either. The N-C group showed a higher degree of wakefulness and superficial non-REM (NREM) stage 1 with a lower NREM stage 3 during PSG.
Narcolepsy in childhood leaves very little scope for the prediction of cataplexy later in life.
嗜睡症是一种终身疾病，其特征是睡眠-觉醒周期异常调节和快速眼动（REM）睡眠渗透增加。在儿童中，没有猝倒的嗜睡症比成人更常见。我们的研究目的是评估临床和多导睡眠图参数，以验证以后出现的猝倒是否可以预测。
纳入了 30 名（12 名男性）在 18 岁之前患有嗜睡症的患者。所有患者均接受临床检查、夜间多导睡眠图（PSG）、多次睡眠潜伏期试验（MSLT）、HLA-DQB1∗0602 检测，以及大多数人进行 Epworth 嗜睡量表（ESS）评分。使用曼-惠特尼秩和检验和 Fisher 检验进行统计学分析。
40%的患者被诊断为无猝倒性嗜睡症（NwC）。首发症状的平均年龄为 14.0 ± 3.0 岁，诊断时为 15.6 ± 3.1 岁。15 例患者伴有催眠幻觉，12 例患者伴有睡眠瘫痪。常见症状包括觉醒时睡眠惯性、REM 行为症状、行为和严重的学校问题。嗜睡症伴猝倒（N-C）患者的 BMI 较高。高 ESS 评分表明白天过度嗜睡（17.1 ± 2.5）。平均 MSLT 睡眠潜伏期为 4.0 ± 3.1 min，有 3.2 ± 1.4 个睡眠起始 REM 期（SOREMs），两组之间无差异。HLA 分型也无差异。N-C 组在 PSG 中显示出更高的觉醒度和浅层非快速眼动（NREM）阶段 1，以及更低的 NREM 阶段 3。
儿童嗜睡症几乎没有为以后出现猝倒的预测留下空间。
