Résumé
Aims: To evaluate the health-related quality of life (HRQL) and its correlates in children and adolescents with narcolepsy. Methods: We compared the clinical characteristics of control subjects and patients with primary narcolepsy from data collected at the National Reference Centers for Narcolepsy. Results: The cohort included 69 control subjects (29 boys) and 117 patients (65 boys; 59 de novo patients). Cataplexy was present in 81% and DQB1*0602 was positive in 91%. The control children were older (13.5 +/- 3.2 vs. 11.6 +/- 3.1 years, P < 0.001) and less obese (1.4% vs. 60%, P < 0.001). Twenty-five percent of the patients and 15.6% of the control subjects had clinically significant depressive feelings on Children's Depression Inventory (CDI >= 16) (NS). Fifty-three narcoleptic and 43 control adolescents, 31 narcoleptic children and 23 control children filled out the HRQL questionnaires as well as 83 parents of patients and 60 parents of control subjects. Narcolepsy seriously impacts HRQL in terms of vitality, physical well-being, relations with friends and leisure activities, especially in adolescents. Depression was the factor that most affected HRQL in both narcoleptic and control subjects. For the control subjects and the narcoleptic patients, when the CDI score was entered into the multivariable regression model adjusted for gender and age, no other continuous independent variable could significantly increase the likelihood of the model. When the CDI score increased by 1, the mean HRQL score decreased by 1.7 for narcoleptic patients and 1.5 for control subjects. Apnea-hypopnoea index, diagnosis delay, disease duration, obesity, the presence of cataplexy or treatment had no effects on HRQL. Conclusions: Narcoleptic children and adolescents were at high risk for poor HRQL. Depressive symptoms had a major impact on HRQL. We recommend a more thorough assessment and management of psychological health in this population.