特发性过度睡眠

目的 探讨特发性过度睡眠患者临床表现以及多导睡眠图特征.方法 与结果回顾分析4 例特发性过度睡眠患者的临床资料,均以白天过度嗜睡首发,无猝倒、睡眠麻痹、睡前幻觉及睡眠行为障碍,其中2 例伴自主神经功能障碍.4 例患者Epworth 嗜睡量表评分均＞ 11 分;多次小睡潜伏期试验平均睡眠潜伏期明显缩短,未见睡眠起始快速眼动期;例3 患者全夜多导睡眠图监测显示睡眠潜伏期明显缩短,总睡眠时间延长,但夜间睡眠结构正常.结论 明确诊断特发性过度睡眠需结合患者病史资料、临床表现及实验室检查进行综合考虑,多次小睡潜伏期试验和全夜多导睡眠图监测是鉴别诊断特发性过度睡眠与发作性睡病的有效方法.     

多次小睡潜伏期试验在有白天过度嗜睡主诉患者的应用

目的通过多次小睡潜伏期试验对有白天过度嗜睡主诉的患进行临床评价。方法对有白天过度嗜睡主诉的16例患进行小睡睡眠监测，以每2h为间隔，共实施5次小睡睡眠监测。监测参数包括脑电图、颏下肌电图、眼动电图、心电图、呼吸及腿动。计算5次小睡的平均睡眠潜伏期及出现睡眠始发快速眼动睡眠时段次数。结果16例患中14例睡眠潜伏期缩短，提示存在白天过度嗜睡。8例有睡眠始发快速眼动睡眠时段发生，其中7例≥2次，1例年轻女性发作性嗜睡12年，平均睡眠潜伏期2．0min，5次小睡中仅有1次睡眠始发快速眼动睡眠时段，无其他原因可解释其白天过度嗜睡，高度怀疑为发作性睡病，结合临床表现诊断为发作性睡病；1例患无睡眠始发快速眼动睡眠时段，但以猝倒发作为首发症状，符合发作性睡病诊断标准；2例白天过度嗜睡患，在睡眠多导生理仪监测的小睡中，出现频繁的睡眠呼吸暂停，经全夜睡眠多导生理仪监测被确诊为阻塞性睡眠呼吸暂停综合征，3例在白天多次小睡中出现深度睡眠(Ⅲ或Ⅳ期)，结合临床确诊为特发性过度睡眠。2例有神经症性障碍患多次小睡潜伏期试验正常。结论多次小睡潜伏期试验为评价白天过度嗜睡的客观方法，是发作性睡病的重要辅助诊断方法，有助于鉴别真、假嗜睡及评价嗜睡的严重程度。     

克莱恩-莱文综合征1例北大核心CSCD

克莱恩-莱文综合征(Kleine-Levin syndrome,KLS)是一种罕见的复发-缓解周期性过度嗜睡疾病。本文总结分析了我院2022年5月收治的1例KLS患者临床资料,患者表现为典型的周期性睡眠增多,发作间期无症状体征,给予患者完善视频多导睡眠监测、多次睡眠潜伏期试验及基因检测后明确诊断,未给予特殊治疗患者出院,随访临床症状好转。本文旨在提高临床工作者对KLS的识别和认识,避免对周期性过度嗜睡患者的误诊漏诊,对该病的早期诊断和治疗具有重要意义。     

发作性睡病12例临床分析

目的探讨发作性睡病的临床特征、诊断方法及治疗。方法对12例发作性睡病患者的临床资料进行回顾性分析。结果12例患者临床表现为白日过度嗜睡12例,猝倒7例,入睡前幻觉4例。根据不同症状给予利他林、氟西汀等药物及行为治疗有效。结论发作性睡病主要表现为睡眠四联症,进行多次睡眠潜伏期试验可以明确诊断,药物及行为、心理治疗有效。     

32例儿童发作性睡病临床特征分析

目的 加强对儿童发作性睡病临床特征及诊断方法的认识. 方法 回顾性分析自2008年9月至2011年9月北京市儿童医院神经科和解放军总医院儿童医学中心收治的32例发作性睡病患儿资料,同时对相关文献进行回顾分析. 结果 32例发作性睡病患儿均有日间不可抗拒的入睡发作,26例(81.3％)存在猝倒发作,11例(34.4％)存在睡眠幻觉,仅2例(6.25％)存在睡眠瘫痪表现.患儿多以日间睡眠增多为起病症状,大多数患儿有夜间睡眠紊乱、易激惹的临床表现,性格改变、食欲及体重增加、青春期提前也是常见的临床症状.多次小睡睡眠潜伏期试验(MSLT)检查在患儿中阳性率较低,可能与患儿年龄小、病程短等因素有关. 结论 不可抗拒的入睡发作、猝倒发作是中国儿童发作性睡病典型的临床表现,结合多导睡眠图和MSLT可明确诊断.     

发作性睡病的临床特征与多次睡眠潜伏期试验对照研究

目的:探讨多次睡眠潜伏期试验(MSLT)对发作性睡病的诊断价值。方法:总结3 6例发作性睡病患者的临床特征,并进行白天5次MSLT和整夜多导睡眠图(PSG)描记,分析平均睡眠潜伏期(sleeplatency ,SL)、睡眠初次出现REM (sleeponsetrapideyemovementperiods ,SOREMP)次数及夜间睡眠相关参数和3 4名正常对照组进行比较。结果:3 6例均有白日过度嗜睡(10 0 % ) ,2 5例伴猝倒(69.44 .% ) ,16例伴入睡前幻觉(4 4 .44 % ) ,8例伴睡眠瘫痪(2 2 .2 2 % ) ,7例典型的睡眠四联征(19.44 % )。白天5次MSLT显示:2 8例发作性睡病患者SL <5min +SOREMP≥2次(77.78% ) ,SL(4 .12±2 .0 4)缩短和SOREMP≥2 (3 .2 8±0 .67)次,与正常对照组相比有显著性差异(P <0 .0 1)。整夜PSG结果显示:发作性睡病组总睡眠时间(3 3 5 .82±3 4.0 9)min、REM潜伏期缩短(17.19±7.14 )min ,和正常对照组相比有显著性差异(P <0 .0 1)。结论:发作性睡病具有睡眠潜伏期缩短和REM睡眠提前的特征,MSLT对发作性睡病的诊断和鉴别诊断具有重要价值。     

睡眠呼吸暂停低通气综合征患者多导睡眠图特征及日间嗜睡分析

目的 通过对男女睡眠呼吸暂停低通气综合征（OSA）患者多导睡眠图特征及日间嗜睡程度的对比,探究OSA患者的多导睡眠图特征及日间嗜睡程度是否存在性别差异.方法 选取2011年5月～2013年2月在华西医院睡眠中心就诊,年龄在18～65岁之间,经整夜多导睡眠呼吸监测确诊为OSA（睡眠呼吸紊乱指数,AHI≥5次/h）的患者进行回顾性分析.按性别分为男性OSA患者及女性OSA患者两组,并对两组的年龄及AHI进行配对.比较两组患者多导睡眠图所示睡眠结构、缺氧状况、多次小睡潜伏时间试验（MSLT）及Epworth嗜睡量表（ESS）评分的差异.结果 共258名患者纳入研究,其中男性129名,平均年龄（49.4±11.3）岁,平均AHI指数（35.3±26.7）次/h;女性129名,平均年龄（49.7±11.8）岁,平均AHI指数（34.1±26.7）次/h.男女OSA患者相比,女性患者睡眠潜伏期更长[（19.2± 28.1）vs.（12.9±12.9）min],睡眠效率更低[（78.5±14.1）% vs.（84.5±9.7）%],睡后觉醒时间更长[（89.8±63.8）vs.（66.1±48.4）min],总睡眠时间更短[（396.9±78.8）vs.（ 427.6± 56.1）min],多次小睡平均潜伏期更长[（9.9±3.39）vs.（9.3±3.7）min],（P均＜0.05）.男女患者呼吸事件中低通气所占比例[（39.9±26.3）% vs.（53.4±27.7）%],阻塞性呼吸暂停所占比例为[（50.81±25.88）% vs.（41.03±26.72）%],（P均＜0.05）.结论 在AHI严重程度相一致的情况下,女性患者睡眠质量更差,但男性患者日间客观嗜睡程度更重.呼吸事件中女性患者多以低通气为主,而男性患者多以阻塞性呼吸暂停为主.     

海洛因依赖者多导睡眠图与主观评估对比分析

目的:探讨海洛因依赖者美沙酮替代治疗早期的主客观睡眠特征. 方法:选择20例美沙酮替代治疗早期的海洛因依赖者(海洛因依赖组)和20名正常人为对照(对照组),进行全夜多导睡眠图(PSG)的监测,以评估受试者的客观睡眠状况;采用匹兹堡睡眠质鼍指数问卷(PSQI)、爱泼沃斯嗜睡评分量表(ESS)评估受试者的主观睡眠状况. 结果:与对照组比较,海洛因依赖组主客观睡眠时间缩短,睡眠效率降低,主观入睡潜伏期延长(P<0.01);主观睡眠时间、入睡潜伏期、睡眠效率与PSG检测结果差异有统计学意义(P<0.01);海洛因依赖组PSQI及ESS评分均显著高于对照组(P<0.01).ESS评分与慢波睡眠的减少呈显著负相关(r=-0.614,P=0.004). 结论:海洛因依赖者美沙酮替代治疗早期睡眠质量差,主客观睡眠评估不一致,患者白天困倦感与慢波睡眠减少相关.     

帕金森病患者睡眠障碍的多导睡眠图、多次睡眠潜伏期试验分析

目的 使用多导睡眠图、多次睡眠潜伏期试验客观分析帕金森病(PD)患者睡眠障碍特征.方法 对26例临床确诊的PD患者(PD组)和31名无明显中枢神经系统疾病的对照者(对照组)行全夜可移动视频多导睡眠监测及次日多次睡眠潜伏期试验,分析比较2组患者睡眠结构及平均睡眠潜伏期、入睡期快速眼球运动(REM)睡眠(SOREMPs)、睡眠发作(Sas)情况.结果 PD组N2睡眠期百分比(32.8%±13.1%)、REM睡眠期百分比(8.6%±5.3%)、平均睡眠潜伏期[(9.6±4.4)min]较对照组[40.2%±9.1%、11.5%±5.1%、(15.7±3.1)min]明显降低(t=-2.515、-2.054、-6.164,P＜0.05),PD组醒觉指数[(41.8±32.1)次/h]较对照组[(28.6±11.0)次/h]明显升高(t=2.151,P＜0.05).PD患者中出现日间过度瞌睡(EDS)7例(7/26,26.9%),明显高于对照组(1/31,3.2%;×2=4.764,P＜0.05).多元逐步线性回归分析显示校正睡眠效率、呼吸暂停低通气指数、醒觉指数,PD患者平均睡眠潜伏期的缩短与年龄(β=-0.328)、左旋多巴等效剂量(β=-0.008)的增加呈线性相关(t=-2.829、-2.352,均P＜0.05).PD组有5例(5/26,19.2%)出现SOREMPs,3例(11.5%)出现Sas,而对照组均无出现SOREMPs和Sas.结论 PD患者睡眠结构改变和EDS较常见,虽然PD患者中Sas不多见,但临床医师需提高警惕.     

多导睡眠图在发作性睡病诊断中的应用

发作性睡病是一种伴随终生但非进行性加重的一种睡眠疾患，以白日过度嗜睡、猝倒及提前出现的快速眼动睡眠为特征，部分患者可伴有入睡或半醒时出现的幻觉、睡眠瘫痪等。国内相应的文献亦较少，且诊断大多依靠临床表现，而缺乏客观的实验室资料。我中心依据临床表现和多导睡眠图(PSG)检查，自1996年以来收治34例该病患者，现总结如下。     

Hypersomnolence and narcolepsy; a pragmatic diagnostic neurophysiological approach

Out of a group of 250 consecutive patients who were examined for various disorders of sleep and waking at Ghent University Hospital within a period of 24 months, 30 patients with hypersomnolence associated with a suspected underlying neurological etiology were selected. The population consisted of 15 males and 15 females with mean age of 36 years (range: 16-60 years). Twenty-one patients had had hypersomnolence for more than 2 years. All patients underwent a single night polysomnography (PSG) and a 4-nap multiple sleep latency test (MSLT). PSG was normal in 23 patients. Sleep onset REM period (SOREMP) was defined as the occurrence of REM sleep within 15 min. after initiation of sleep. PSG demonstrated SOREMP's in only 1 patient and showed evidence of obstructive sleep apnea in 4 patients. Two patients had a low sleep efficiency. MSLT demonstrated hypersomnolence in 17 patients of whom 6 showed SOREMP. Significant hypersomnolence was defined as a mean sleep latency < or = 5 min. 4 patients fulfilled the classical clinical and polygraphic criteria (> or = 2 SOREMP) of narcolepsy. In 8 patients the tentative diagnosis of idiopathic CNS hypersomnolence was made. 13 patients did not sleep during MSLT. These results emphasize the relative importance of MSLT. Our limited 4-nap MSLT protocol proved useful in distinguishing narcolepsy from idiopathic CNS hypersomnolence.     

Multiple sleep latency test and polysomnography in patients with central disorders of hypersomnolence

A multiple sleep latency test (MSLT) with occurrence of sleep onset REM periods (SOREMP) is considered one of the central diagnostic criteria for narcolepsy according to the International Classification of Sleep Disorders, but its sensitivity and specificity have been questioned. This study aims to describe MSLT and polysomnography (PSG) findings, including frequency and distribution of SOREMP during the day, in a large cohort of patients with central disorders of hypersomnolence (CDH).We retrospectively analyzed electrophysiological data from MSLT and PSG in 370 consecutive patients with narcolepsy type 1 (NT1, n = 97), type 2 (NT2, n = 31), idiopathic hypersomnia (IH, n = 48), nonorganic hypersomnia (NOH, n = 116) and insufficient sleep syndrome (ISS, n = 78).NT1 and NT2 patients had a significantly shorter mean Sleep Latency (mSL) and REM-Latency (REML) in MSLT and PSG. SOREMP occurred more frequently in narcoleptic vs. non-narcoleptic patients in MSLT and PSG. Occurrence of 3 or more SOREMP in MSLT and a SOREMP in PSG had a very high specificity and positive predictive value (98%/96% and 100% respectively), however relatively low sensitivity (65% and 45% respectively).NT1 more than NT2 patients have shorter mSL and more frequent SOREMP in MSLT and shorter SL as well as REML during nocturnal PSG. Increasing numbers of SOREMP in MSLT and especially SOREMP during PSG increase specificity on the expense of sensitivity in diagnosing narcolepsy. Therefore, frequency of SOREMP in MSLT naps and PSG can help to discriminate but not clearly separate narcoleptic from non-narcoleptic patients.     

Clinical caveat: prior sleep deprivation can affect the MSLT for days

It is common practice to forcibly awaken patients from an all-night polysomnographic study prior to performance of a multiple sleep latency test (MSLT); either for reasons of protocol or for the convenience of the laboratory personnel. We report a case of a young woman who, by formal sleep study criteria, received the erroneous diagnosis of narcolepsy due to the effects of severe sleep deprivation, and document that the effects of prior sleep deprivation require more than one night of ad libitum sleep. Forced awakening prior to MSLT may permit sleep deprivation or delayed sleep phase syndrome to masquerade as narcolepsy or idiopathic central nervous system (CNS) hypersomnia.     

佐匹克隆及三唑仑对失眠症患者多导睡眠图影响的研究

目的探讨短半衰期催眠药对失眠症患者夜间多导睡眠图（PSG）和白天多次睡眠期测定（MSLT）的影响。方法将22例非器质性失眠症患者随机分成佐匹克隆组（15mg）和三唑仑组（0.5mg）,各11例。对两组用药前后进行夜间PSG和白天MSLT检测和比较。结果用药后夜间PSG显示睡眠效率提高、觉醒和S     

Demographic, clinical, and polysomnographic features in patients with narcolepsy: an experience of 181 patients with narcolepsy from a Turkish sleep center

The present study was designed to describe the socio-demographic, clinical, and polysomnographic features of patients diagnosed with narcolepsy in our sleep center. This retrospective cross-sectional study was conducted on 181 patients diagnosed with narcolepsy based on the results of clinical evaluation, polysomnography (PSG), and multiple sleep latency test (MSLT) between 1993 and 2009. Approximately 70% of the patients had cataplexy, whereas 42% had hallucinations and 55.8% had sleep paralysis. Although sleep efficiency was higher (91.28?±?5.89%) in patients with narcolepsy, they woke frequently during the night, and their percentages of deep sleep were low (stage 3, 5.12?±?3.08%, stage 4, 9.60?±?7.10%). Our study group was divided into two based on age: individuals aged <30?years (n?=?152) and >30?years (n?=?29). REM latency on PSG was shorter (t?=?2.96, p?=?0.004) and sleep onset REM (SOREM) on MSLT was higher (t?=?2.56, p?=?0.011) in the older group than in the younger group. Cataplexy is seen in most patients with narcolepsy. In older patients, REM latency on PSG is shorter and the number of SOREM on MSLT is higher.     

Utility of the sleep stage sequence preceding sleep onset REM periods for the diagnosis of narcolepsy: a study in a Japanese cohort

BackgroundThe minimum narcolepsy criteria “mean sleep latency (MSL) ≤8 min and ≥2 sleep onset rapid eye movement (REM) periods (SOREMPs) on polysomnography (PSG) and the multiple sleep latency test (MSLT),” according to The International Classification of Sleep Disorders, Third Edition (ICSD-3), are not specific to narcolepsy. Recently, the characteristic sleep stage sequences preceding SOREMPs in narcolepsy have received attention, but their diagnostic utility remains unclear.MethodsWe retrospectively reviewed PSG/MSLT records and chart data for 102 Japanese patients with hypersomnia and at least one SOREMP. We examined the sporadic rates of two sleep stage sequences preceding the SOREMPs—wakefulness or stage 1 to REM (W/S1→R) and stage 2 to REM (S2→R)—comparing these between patient groups with narcolepsy type 1 (N = 28), narcolepsy type 2 (N = 19), and other hypersomnia (N = 55). We also examined the utility of three simple indices using the occurrence of W/S1→R SOREMPs for distinguishing between narcolepsy and other hypersomnia in patients who satisfied the minimum narcolepsy criteria.ResultsW/S1→R SOREMPs were significantly more frequent in narcolepsy than in other hypersomnia, and this tendency was also observed even in the patients who satisfied the minimum narcolepsy criteria. The three indices had moderate sensitivities and specificities for distinguishing between narcolepsy and other hypersomnia in patients satisfying the minimum narcolepsy criteria.ConclusionsThe W/S1→R pattern was observed significantly more frequently in narcolepsy than in other hypersomnia, suggesting it may help with differentiating narcolepsy from other hypersomnia in patients demonstrating the narcolepsy criteria, although its ability to do so may be modest.     

Homeostatic sleep regulation in patients with idiopathic hypersomnia.

OBJECTIVE: To determine whether the spectral activity during sleep of patients with idiopathic hypersomnia (IH) differs from that of healthy subjects. METHODS: Spectral analysis of the electroencephalogram (EEG) was performed in 10 patients with IH and in 10 age-matched control subjects. We compared the time course of absolute power for slow wave activity (SWA: 0.75-4.5 Hz), and for theta, alpha, sigma and beta bands for the first 4 non-rapid-eye movement (NREM) episodes. RESULTS: Compared to controls, IH patients had less SWA across the night although the exponential decay was preserved. The fall in SWA was statistically significant for the first two NREM episodes only. The lower power of SWA was related to lower amounts of stages 3 and 4 of NREM sleep during the sleep episodes. No correlation was found between SWA during the night and the mean sleep latency on the multiple sleep latency test (MSLT). CONCLUSIONS: These results showed that, in IH patients, the homeostatic sleep regulatory mechanisms are preserved but the sleep pressure, indicated by SWA, is lower.     

A six year-old case of narcolepsy

We report here a 6-year-old boy with narcolepsy. The diagnostic criteria were met by the clinical symptoms including excessive daytime sleepiness and cataplexy, and by the results of overnight polysomnography (PSG), multiple sleep latency test (MSLT), and human leukocyte antigen (HLA). PSG showed increased ratio of sleep stages 1 and 2 due to frequent awakening. All the five test session of MSLT showed a sleep onset REM period. HLA typing was positive for DRB1* 1501 and DQB1* 0602. Though the present case had very early onset, all the clinical symptoms and results of sleep studies met the criteria of narcolepsy. The CSF orexin level was far below the lower limit of the control values. It is very useful to measure CSF orexin for the diagnosis of early onset narcolepsy.     

Difference in the characteristics of subjective and objective sleepiness between narcolepsy and essential hypersomnia

The present study was conducted to investigate the difference in the characteristics of daytime sleepiness between narcolepsy and essential hypersomnia and to identify the relationship between the Epworth Sleepiness Scale (ESS) and the Multiple Sleep Latency Test (MSLT) in patients with these two disorders. Subjects consisted of 34 patients with essential hypersomnia (32.4 +/- 11.0 years old), 52 patients with narcolepsy (29.0 +/- 13.8 years old), and 45 control subjects (33.3 +/- 6.6 years old). The subjects completed the ESS and underwent MSLT following a regular sleep-wake schedule for over 2 weeks. The ESS scores were pathologically high and mean sleep latency on MSLT was short, not only in narcolepsy but also in essential hypersomnia. With respect to sleep latencies on each MSLT session, both essential hypersomnia and control subjects had the smallest value at 14:00, while narcolepsy lacked any statistical change at this time period. The correlation between ESS and mean sleep latency on MSLT was higher in essential hypersomnia than in narcolepsy, and the correlation was strongest for the session performed at 14:00. Based on the ESS and MSLT results, the severity of excessive daytime sleepiness was significantly milder in essential hypersomnia compared with that in narcolepsy. The results also indicate that diurnal variation of sleepiness was maintained, and the correlation between subjective and objective sleepiness was relatively maintained in essential hypersomnia compared to narcolepsy. It is suggested that the mild disease severity of essential hypersomnia contributed to the formation of these characteristics.     

Impact of acute administration of sodium oxybate on nocturnal sleep polysomnography and on multiple sleep latency test in narcolepsy with cataplexy

ObjectiveTo analyze the acute effects of sodium oxybate (SO) on polysomnographic night-time recordings (PSG) and multiple sleep latency test (MSLT) on patients with narcolepsy with cataplexy (NC).MethodsSixteen NC adult patients were recruited, together with 16 normal controls. Two consecutive PSG followed by two MSLT sessions were carried out, before and during the first night of SO assumption, respectively.ResultsThe administration of SO was followed by a significant decrease in number of stage shifts and awakenings, wakefulness after sleep onset, and percentage of sleep stage 1. Sleep efficiency and slow wave sleep percentage increased. REM latency decreased significantly from 73 to 12 min. Cyclic alternating pattern (CAP) rate remained unchanged but the percentage of CAP A3 subtypes decreased. The number of CAP A3 subtypes per hour of NREM sleep decreased significantly, whereas that of A1 remained unchanged. The duration of A1 and A3 subtypes was slightly increased. Chin muscle tone was not modified by SO as well as periodic leg movements during sleep, but their periodicity index decreased, becoming similar to that of controls. MSLT sleep latency also significantly improved after SO intake.ConclusionsThe administration of SO in NC patients is followed by immediate important and complex effects on PSG parameters and MSLT, including an evident (over)increase in slow wave sleep, which does not display a physiological microstructure, a moderate decrease in periodic and isolated LMs, possibly mediated by a disinhibited dopaminergic neuronal activity, and an improvement on daytime mean sleep latency at the MSLT.