Zhou Li, Huang Bei, Wang Jing, Chau Steven Wh, Chan Joey Wy, Zhang Jihui, Yu Mandy Wm, Tsang Jessie Cc, Li Shirley Xin, Mok Vincent Ct, Wing Yun Kwok, Liu Yaping
Department of Psychiatry, Faculty of Medicine, The Chinese University of Hong Kong, Shatin, NT, Hong Kong SAR, China; Li Chiu Kong Family Sleep Assessment Unit, Department of Psychiatry, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong SAR, China.
Department of Psychiatry, Faculty of Medicine, The Chinese University of Hong Kong, Shatin, NT, Hong Kong SAR, China; Li Chiu Kong Family Sleep Assessment Unit, Department of Psychiatry, Faculty of Medicine, The Chinese University of Hong Kong, Hong Kong SAR, China; Center for Sleep and Circadian Medicine, The Affiliated Brain Hospital of Guangzhou Medical University, Guangzhou, Guangdong, China.
Sleep Med. 2023 May;105:1-8. doi: 10.1016/j.sleep.2023.03.007. Epub 2023 Mar 7.
Age at onset of neurodegenerative disease has significant implications in differentiating disease profiles. We aimed to determine whether age at onset could identify clinical and neurodegenerative profiles in patients with isolated/idiopathic rapid eye movement sleep behavior disorder (iRBD) - a prodromal stage of α-synucleinopathies.
In this retrospective cohort study, the time of the first episode of dream-enactment behaviors that the patient/bed-partners recalled at the time of the patient's first visit to sleep clinic was collected. The distribution of age at onset was examined and patients were dichotomized into early- and late-onset groups based on the intersection point of underlying two Gaussian distributions of onset age.
A total of 241 patients were included. The intersection of underlying two Gaussian models of onset age was 64.6 years, yielding 168 early- (median onset age: 58.0 years, range: 38.0-64.0) and 73 late-onset patients (median onset age: 70.0 years, range: 65.0-82.0). Among them, 154 of early- and 68 late-onset patients were followed-up. Late-onset patients had milder RBD symptoms, but worse sleep, cognition, olfactory and motor functions, and a higher risk of phenoconversion (adjusted hazard ratio (aHR) = 2.2, 95% confidence interval (CI) = 1.2-3.9), especially to probable dementia with Lewy bodies (DLB) (aHR = 8.9, 95% CI = 3.0-26.2), than early-onset patients.
Late-onset iRBD was associated with a higher level of neurodegenerative markers and a quicker phenoconversion, especially to probable DLB. Age at onset of iRBD could help identify clinical features and predict prognosis of iRBD.
神经退行性疾病的发病年龄对区分疾病特征具有重要意义。我们旨在确定发病年龄是否能够识别孤立性/特发性快速眼动睡眠行为障碍（iRBD）患者的临床和神经退行性特征，iRBD是α-突触核蛋白病的前驱阶段。
在这项回顾性队列研究中，收集了患者/床伴回忆起的患者首次就诊睡眠诊所时梦呓行为首次发作的时间。检查发病年龄的分布情况，并根据发病年龄的两个潜在高斯分布的交点将患者分为早发组和晚发组。
共纳入241例患者。发病年龄的两个潜在高斯模型的交点为64.6岁，产生168例早发患者（中位发病年龄：58.0岁，范围：38.0 - 64.0岁）和73例晚发患者（中位发病年龄：70.0岁，范围：65.0 - 82.0岁）。其中，154例早发患者和68例晚发患者接受了随访。晚发患者的RBD症状较轻，但睡眠、认知、嗅觉和运动功能较差，且发生症状转化的风险较高（调整后风险比（aHR）= 2.2，95%置信区间（CI）= 1.2 - 3.9），尤其是转化为可能的路易体痴呆（DLB）的风险（aHR = 8.9，95% CI = 3.0 - 26.2），高于早发患者。
晚发性iRBD与更高水平的神经退行性标志物以及更快的症状转化相关，尤其是转化为可能的DLB。iRBD的发病年龄有助于识别其临床特征并预测预后。
