National Reference Network for Narcolepsy, Department of Neurology, Hôpital Gui-de-Chauliac, CHU Montpellier, Montpellier, France.
PLoS One. 2012;7(4):e33525. doi: 10.1371/journal.pone.0033525. Epub 2012 Apr 25.
Narcolepsy with cataplexy (NC) is a disabling sleep disorder characterized by early loss of hypocretin neurons that project to areas involved in the attention network. We characterized the executive control of attention in drug-free patients with NC to determine whether the executive deficits observed in patients with NC are specific to the disease itself or whether they reflect performance changes due to the severity of excessive daytime sleepiness.
Twenty-two patients with NC compared to 22 patients with narcolepsy without cataplexy (NwC) matched for age, gender, intellectual level, objective daytime sleepiness and number of sleep onset REM periods (SOREMPs) were studied. Thirty-two matched healthy controls were included. All participants underwent a standardized interview, completed questionnaires, and neuropsychological tests. All patients underwent a polysomnography followed by multiple sleep latency tests (MSLT), with neuropsychological evaluation performed the same day between MSLT sessions.
Irrespective of diagnosis, patients reported higher self-reported attentional complaints associated with the intensity of depressive symptoms. Patients with NC performed slower and more variably on simple reaction time tasks than patients with NwC, who did not differ from controls. Patients with NC and NwC generally performed slower, reacted more variably, and made more errors than controls on executive functioning tests. Individual profile analyses showed a clear heterogeneity of the severity of executive deficit. This severity was related to objective sleepiness, higher number of SOREMPs on the MSLT, and lower intelligence quotient. The nature and severity of the executive deficits were unrelated to NC and NwC diagnosis.
We demonstrated that drug-free patients with NC and NwC complained of attention deficit, with altered executive control of attention being explained by the severity of objective sleepiness and global intellectual level. Further studies are needed to explore whether medications that promote wakefulness can improve the executive functions in narcolepsy.
猝倒性睡眠发作伴发的发作性睡病(narcolepsy with cataplexy,NC)是一种使人丧失能力的睡眠障碍,其特征是下丘脑分泌素神经元过早丧失,这些神经元投射到参与注意力网络的区域。我们对未经药物治疗的 NC 患者的注意力执行控制进行了描述,以确定 NC 患者中观察到的执行功能缺陷是疾病本身特有的,还是由于日间过度嗜睡的严重程度而导致的表现变化。
22 例 NC 患者与 22 例无猝倒的发作性睡病(narcolepsy without cataplexy,NwC)患者相匹配,这些患者的年龄、性别、智力水平、客观日间嗜睡和睡眠起始快速眼动期(sleep onset REM periods,SOREMPs)数量相匹配。还纳入了 32 例匹配的健康对照者。所有参与者都接受了标准化访谈、问卷调查和神经心理学测试。所有患者都进行了多导睡眠图检查和多次睡眠潜伏期试验(multiple sleep latency test,MSLT),神经心理学评估在 MSLT 检查之间的同一天进行。
无论诊断如何,患者都报告了与抑郁症状强度相关的更高的自我报告注意力抱怨。与 NwC 患者相比,NC 患者在简单反应时任务中的表现更慢且更不稳定,而 NwC 患者与对照组没有差异。NC 患者和 NwC 患者在执行功能测试中通常比对照组反应更慢、变化更大且错误更多。个体分析显示,执行功能缺陷的严重程度存在明显的异质性。这种严重程度与客观的嗜睡、MSLT 中更多的 SOREMPs 以及更低的智商有关。执行功能缺陷的性质和严重程度与 NC 和 NwC 诊断无关。
我们证明,未经药物治疗的 NC 患者和 NwC 患者抱怨注意力缺陷,注意力执行控制的改变可以通过客观嗜睡的严重程度和整体智力水平来解释。需要进一步的研究来探讨是否可以通过促进觉醒的药物来改善发作性睡病的执行功能。