Department of Biomedical and NeuroMotor Sciences (DiBiNeM), University of Bologna, Bologna, Italy.
Sleep Disorders Center, Neurology Service, Hospital Clínic de Barcelona, Barcelona, Spain; Clinical Neurophysiology Group, Institut D'Investigacions Biomediques August Pi I Sunyer (IDIBAPS), Barcelona, Spain; CIBERNED CB06/05/0018-ISCIII, Spain; Universitat de Barcelona, Barcelona, Spain.
Sleep Med. 2023 Oct;110:268-286. doi: 10.1016/j.sleep.2023.08.022. Epub 2023 Aug 28.
Neurodegenerative diseases often alter sleep architecture, complicating the application of the standard sleep scoring rules. There are no recommendations to overcome this problem. Our aim was to develop a scoring method that incorporates the stages previously applied in dementia with Lewy Bodies (DLB), anti-IgLON5 disease, and fatal insomnia, and to test it in patients with alpha-synucleinopathies.
Video-polysomnographies (VPSG) of nine patients (DLB:3, Parkinson's disease (PD):3, and multiple system atrophy (MSA):3) selected for their difficulty in applying standard rules were scored independently by two authors, using additional Sleep/Wake stages. These included Abnormal Wake, Subwake, Undifferentiated NREM sleep (UNREM), Poorly structured N2 (P-S N2) and abnormal REM sleep including REM without atonia (RWA), REM without low-amplitude, mixed-frequency EEG activity (RWL) and REM without rapid eye movements (RWR).
Patients (4 females) had a median age of 74 (range 63-85). Six patients (all with PD or DLB) had abnormal EEG awake and Subwake stage. UNREM sleep was present in all patients, typically at sleep onset, and was the most common sleep stage in five. P-S N2 was recorded only in the three patients with MSA. Periods of normal and abnormal NREM coexisted in three patients. RWA was the predominant REM subtype, RWR occurred mainly in patients with MSA and RWL in those with DLB. Six patients had brief REM episodes into NREM sleep which we termed "Encapsulated RBD".
Our scoring system allows an accurate description of the complex sleep-wake changes in patients with alpha-synucleinopathies.
神经退行性疾病常改变睡眠结构，使标准睡眠评分规则的应用变得复杂。目前尚无克服这一问题的建议。我们的目的是开发一种评分方法，纳入先前在路易体痴呆症（DLB）、抗 IgLON5 病和致死性失眠症中应用的阶段，并在α-突触核蛋白病患者中进行测试。
选择 9 名患者（DLB：3 名，帕金森病（PD）：3 名，多系统萎缩（MSA）：3 名）的视频多导睡眠图（VPSG），这些患者因难以应用标准规则而被两名作者独立评分，使用额外的睡眠/觉醒阶段。这些阶段包括异常觉醒、Subwake、未分化的 NREM 睡眠（UNREM）、结构不良的 N2（P-S N2）和异常 REM 睡眠，包括无动性 REM 睡眠（RWA）、低振幅、混合频率 EEG 活动（RWL）和无快速眼动（RWR）的 REM 睡眠。
患者（4 名女性）的中位年龄为 74 岁（范围 63-85 岁）。6 名患者（均为 PD 或 DLB）有异常 EEG 觉醒和 Subwake 期。所有患者均存在 UNREM 睡眠，通常在睡眠开始时出现，5 名患者最常见的睡眠阶段为 UNREM 睡眠。P-S N2 仅记录在 3 名 MSA 患者中。正常和异常 NREM 睡眠阶段共存于 3 名患者中。RWA 是主要的 REM 亚型，RWR 主要发生在 MSA 患者中，RWL 发生在 DLB 患者中。6 名患者有短暂的 REM 进入 NREM 睡眠的发作，我们称之为“包裹性 RBD”。
我们的评分系统可以准确描述α-突触核蛋白病患者复杂的睡眠-觉醒变化。
