National Reference Centre for Narcolepsy, Hypersomnia and Kleine-Levin Syndrome, Pitie-Salpetriere University Hospital, AP-HP, Paris, France.
Sleep Disorder Unit, Pitie-Salpetriere University Hospital, AP-HP, Paris, France.
J Sleep Res. 2018 Oct;27(5):e12690. doi: 10.1111/jsr.12690. Epub 2018 Apr 14.
In Kleine-Levin syndrome (KLS), episodes of hypersomnia and cognitive, psychiatric and behavioural disturbances alternate with asymptomatic periods in adolescents. We evaluated whether psychiatric disorders would emerge during asymptomatic periods in a naturalistic, uncontrolled clinical cohort. Patients with primary KLS underwent psychiatric interviews at diagnosis and every year for 1-10 years, leading to diagnosis of former and present comorbid psychiatric disorders. Among the 115 patients (65.2% male and aged 16.1 ± 4.8 years at KLS onset), 19 (16.5%) had a history of psychiatric disorder prior to KLS onset, which persisted afterwards in 10. Twenty-five (21%) patients developed a new, comorbid psychiatric disorder 1-6 years after KLS onset, during 'asymptomatic' periods, including mood disorders (n = 14; including major depressive episodes, n = 8; recurrent depressive episodes, n = 2; bipolar I disorder, n = 1; dysthymic disorder, n = 1; adjustment disorder with depressive mood, n = 1; and mood disorder not otherwise specified, n = 1), anxiety disorders (n = 7), eating disorders (n = 2), psychotic disorders not otherwise specified (n = 2), schizoaffective disorder (n = 1) and cannabis dependence (n = 1). Six patients attempted suicide: two before and two after KLS onset, and two during episodes. Female sex, longer disease course, longer time incapacitated (356 ± 223 versus 155 ± 186 days) and more frequent psychiatric symptoms during episodes (but no family or personal history of psychiatric disorders) were associated with emerging psychiatric disorders. Contrary to the alleged benignity of KLS and normality between episodes, one KLS patient in five suffers from emerging psychiatric disorders. These disorders may depend on personal vulnerability and, most probably, on psychiatric symptoms during episodes.
在 Kleine-Levin 综合征(KLS)中,青少年会出现周期性的嗜睡和认知、精神及行为障碍,同时伴有无症状期。我们评估了在自然、非控制的临床队列中,无症状期是否会出现精神障碍。在诊断时以及 KLS 发病后每年进行 1-10 年的随访,对 KLS 患者进行精神科访谈,从而诊断出既往和目前的合并精神障碍。在 115 名患者(65.2%为男性,KLS 发病时年龄为 16.1±4.8 岁)中,19 名(16.5%)在 KLS 发病前有精神障碍史,且之后仍存在该病史。25 名(21%)患者在 KLS 发病后 1-6 年内出现新的合并精神障碍,处于“无症状”期,包括心境障碍(n=14;包括重性抑郁发作,n=8;复发性抑郁障碍,n=2;I 型双相障碍,n=1;心境恶劣障碍,n=1;适应障碍伴抑郁心境,n=1;和未特定的心境障碍,n=1)、焦虑障碍(n=7)、进食障碍(n=2)、未特定的精神病性障碍(n=2)、分裂情感性障碍(n=1)和大麻依赖(n=1)。6 名患者尝试自杀:2 名在 KLS 发病前,2 名在 KLS 发病后,2 名在发作期。女性、较长的病程、较长的无能力期(356±223 天比 155±186 天)、发作期更频繁的精神症状(但无精神障碍家族史或个人史)与新发精神障碍有关。与 KLS 假定的良性和发作间期正常性相反,每 5 名 KLS 患者中就有 1 名患有新发精神障碍。这些障碍可能取决于个人易感性,而且很可能取决于发作期的精神症状。