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RSS发作性睡病患者猝倒持续状态的临床特征与多导睡眠图表现
Clinical and Polysomnographic Characteristics of Narcoleptic Patients with Status Cataplecticus
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摘要:目的 探讨发作性睡病患者猝倒持续状态的临床特征和多导睡眠图表现。方法 2002年1月至2011年12月间在北京协和医院神经科就诊的115例发作性睡病患者中, 收集5例伴猝倒持续状态患者的病例资料。该5例患者年龄46~53岁, 平均49.1岁, 病程3~9个月, 均完成神经系统查体、头部磁共振检查、多次小睡潜伏期试验, 其中3例完成多导睡眠图检查。结果 5例患者中, 首发症状为白天嗜睡4例, 猝倒1例。所有患者均因频繁猝倒就诊。其他症状包括:睡眠幻觉2例, 睡眠麻痹3例。多次小睡潜伏期试验平均睡眠潜伏期0.5~2.5 min。4例可见2次以上睡眠始发快速动眼睡眠, 1例可见1次。多导睡眠图可见猝倒发作时, 肌电活动明显抑制, 脑电图显示为清醒状态。5例患者均给予氯米帕明治疗, 猝倒症状消失。结论 发作性睡病患者猝倒频繁发作, 可诊断猝倒持续状态, 其多导睡眠图表现支持猝倒发作是快速动眼睡眠的分离现象, 即肌电图显示肌张力明显降低, 而脑电图仍显示为清醒状态。Abstract:Objective To explore the clinical and polysomnographic characteristics of narcoleptic patients with status cataplecticus.Methods We collected the clinical data of 5 patients with status cataplecticus out of 115 narcoleptic patients admitted to Department of Neurology in Peking Union Medical College Hospital from January 2002 to December 2011. The 5 patients had a mean age of 49.1 years (range, 46-59 years) at diagnosis. Their disease course ranged from 3 to 9 months. All of them underwent physical and neurological examinations, neuroimaging, and multiple sleep latency test (MSLT). Three of them underwent polysomnography (PSG).Results The first symptom was excessive daytime sleepiness in 4 patients, cataplexy in 1 patient. All patients had frequent cataplectic attacks, diagnosed as status cataplecticus. Other symptoms included hypnagogic hallucinations (2/5) and sleep paralysis (3/5). The MSLT showed a mean sleep latency from 0.5 to 2.5 minutes, and 4 patients had more than 2 sleep-onset rapid eye movement period (SOREMP), and 1 patient had 1 SOREMP. PSG showed that a waking electroencephalography (EEG) existed during cataplectic attacks. Electromygraphy showed suppressions of activity. All patients were treated with clomipramine, which completely resolved the attacks.Conclusions Cataplexy in narcoleptic patients showing the recurrent episodes can be diagnosed as status cataplecticus. PSG supports that cataplectic attacks represent dissociated rapid eye movement phenomena in which there is only the somatic component (atonia) without the cerebral one (sleep).
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