Abstract

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 1-10years, leading to diagnosis of former and present comorbid psychiatric disorders. Among the 115 patients (65.2% male and aged 16.1±4.8years 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 1-6years 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±186days) 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.

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