目的:分析儿童发作性睡病(NRL)临床特征.方法:2015年1月至2016年12月就诊于院神经科门诊的56例诊断为NRL患儿,进行常规EEG、长程视频脑电图(VEEG)及多次睡眠潜伏试验(MSLT)检查;对曾经误诊病例,探讨其误诊原因.结果:56例均有不同程度多睡.38例(67.9％)有猝倒表现,4例(7.1％)伴睡前幻觉,2例(3.6％)伴醒时幻觉.2例(3.6％)伴睡眠瘫痪.56例EEG背景活动均正常,VEEG排除癫痫发作.MSLT结果:所有患儿平均睡眠潜伏期缩短,快速眼动相睡眠(REM)睡眠潜伏期缩短,56例均<5min,均有2次或2次以上睡眠开始时快速眼动相睡眠(sleep onset REM pe-riods,SOREMP).误诊分析:8例(14.3％)曾被误诊为眼肌型重症肌无力,12例(21.4％)曾被误诊为癫痫,2例(3.6％)曾被误诊为精神分裂症.28例轻症患儿予神经科门诊随访.2例存在多睡、猝倒、睡眠幻觉,家长未允其接受药物治疗,仅予行为指导.26例严重多睡患儿给予哌甲酯或哌甲酯缓释剂治疗,并结合行为指导.结论:儿童NRL容易误诊.应密切随访患儿临床症状变化,结合VEEG及MSLT等多种辅助检查进行分析,减少误诊.%Objective:To analyze the clinical characteristics of narcolepsy. Methods:The clinical data of 56 narcoleptic children were analyzed. Video-EEG monitoring and multiple sleep latency tests were per-formed in all patients. Results:Excessive daytime sleepiness was manifested in all the patients, with disrupted nocturnal sleep. Cataplexy appeared in 38cases, and hypnagogic hallucination in 4, hypnopompic hallucina-tion in 2, and sleep paralysis in 2, respectively. EEG and VEEG of all patients were normal. The multiple sleep latency test demonstrated a short sleep la-tency (<5 minutes) and two or more sleep onset REM periods ( SOREMPs) in all patients. Four patients were misdiag-nosed with myasthenia gravis. Six children were mis-diagnosed with epilepsy. Two patients were misdiagnosed with schizo-phrenia. Twenty-eight mild patients were followed up at the neurological out-patient department. Two cases of sleepiness, cata-plexy and sleep halluci-nation was given just behavior guidance because the parents refused to receive drug treatment. Twenty six chil-dren with severe sleepiness was treated with methylphenidate along with behavior guidance. Conclusion:Nar-colepsy is likely to be misdiagnosed because of its mildsymptoms at onset and atypical clinical manifestations. Misdiagnosis can be reduced by means of VEEG and MSLT.
展开▼
