Mutti Carlotta, Bernabè Giorgia, Barozzi Noemi, Ciliento Rosario, Trippi Irene, Pedrazzi Giuseppe, Azzi Nicoletta, Parrino Liborio
Department of Medicine and Surgery, Sleep Disorders Center, University of Parma, Parma, Italy.
Unit of Neuroscience & Interdepartmental Center of Robust Statistics, Department of Medicine and Surgery, University of Parma, Parma, Italy.
Front Neurol. 2020 Dec 11;11:600026. doi: 10.3389/fneur.2020.600026. eCollection 2020.
Differential diagnosis between disorders of arousal (DoA) and sleep-related hypermotor epilepsy (SHE) often represents a clinical challenge. The two conditions may be indistinguishable from a semiological point of view and the scalp video-polysomnography is often uninformative. Both disorders are associated with variable hypermotor manifestations ranging from major events to fragments of a hierarchical continuum of increasing intensity, complexity, and duration. Given their semiological overlap we decided to explore the sleep texture of DoA and SHE seeking for similarities and differences. We analyzed sleep macrostructure and CAP (cyclic alternating pattern) parameters in a cohort of 35 adult DoA patients, 40 SHE patients and 24 healthy sleepers, all recorded and scored in the same sleep laboratory. Nocturnal behavioral manifestations included minor motor events, paroxysmal arousals and major attacks in SHE, and simple, rising, or complex arousal movements in DoA. Compared to healthy controls, DoA and SHE showed similar amounts of sleep efficiency, light sleep, deep sleep, REM sleep, CAP subtypes. Both groups also showed slow wave sleep fragmentation and an increased representation of stage N3 in the second part of the night. The only discriminating elements between the two conditions regarded sleep length (more reduced in DoA) and sleep instability (more elevated in SHE). In DoA recordings, all motor episodes arose from NREM sleep: 37% during light NREM stages and 63% during stage N3 (simple arousal movements: 94%). In SHE recordings, 57% of major attacks occurred during stage N3. So far, emphasis has been placed on the differentiation of sleep-related epilepsy and NREM arousal disorders. However, the impressive analogies between DoA and SHE suggest the existence of an underestimated continuum across the conditions, linked by increased levels of sleep instability, higher amounts of slow wave sleep and NREM/REM sleep imbalance. Sleep texture is extremely similar in the two conditions, although CAP metrics disclose quantitative differences. In particular, SHE patients show a higher arousal instability compared to DoA subjects. Given their clinical and epidemiological overlap, a common genetic background is also hypothesized. In such a perspective, we suggest that the consolidated dichotomy DoA vs. SHE should be reappraised.
觉醒障碍(DoA)与睡眠相关的运动过多性癫痫(SHE)之间的鉴别诊断常常是一项临床挑战。从症状学角度来看,这两种病症可能难以区分,而且头皮视频多导睡眠图通常也无法提供有效信息。这两种病症都与多种运动过多表现相关,这些表现涵盖从重大事件到强度、复杂性和持续时间不断增加的分级连续体片段。鉴于它们在症状学上的重叠,我们决定探索DoA和SHE的睡眠结构,寻找异同点。我们分析了35名成年DoA患者、40名SHE患者和24名健康睡眠者队列的睡眠宏观结构和CAP(周期性交替模式)参数,所有数据均在同一睡眠实验室记录并评分。夜间行为表现包括SHE中的轻微运动事件、阵发性觉醒和重大发作,以及DoA中的简单、上升或复杂觉醒运动。与健康对照组相比,DoA和SHE在睡眠效率、浅睡眠、深睡眠、快速眼动睡眠、CAP亚型的量上表现相似。两组还均表现出慢波睡眠片段化以及夜间后半段N3期比例增加。这两种病症之间唯一的鉴别因素在于睡眠时长(DoA中更短)和睡眠不稳定性(SHE中更高)。在DoA记录中,所有运动发作均起源于非快速眼动睡眠:浅非快速眼动阶段占37%,N3期占63%(简单觉醒运动:94%)。在SHE记录中,57%的重大发作发生在N3期。到目前为止,重点一直放在睡眠相关癫痫和非快速眼动觉醒障碍的鉴别上。然而,DoA和SHE之间令人印象深刻的相似之处表明,在这些病症之间存在一个被低估的连续体,其关联因素包括睡眠不稳定性增加、慢波睡眠量更高以及非快速眼动/快速眼动睡眠失衡。尽管CAP指标揭示出定量差异,但这两种病症的睡眠结构极其相似。特别是,与DoA受试者相比,SHE患者表现出更高的觉醒不稳定性。鉴于它们在临床和流行病学上的重叠,还推测存在共同的遗传背景。从这个角度来看,我们建议重新评估已确立的DoA与SHE的二分法。