Psychosocial Profile and Quality of Life in Children With Type 1 Narcolepsy: A Case-Control Study

Abstract

Study objectives: To investigate behavioral aspects and quality of life in children and adolescents with type 1 narcolepsy (NT1).

Methods: We performed a case-control study comparing 29 patients with NT1 versus sex- and age-matched patients with idiopathic epilepsy (n = 39) and healthy controls (n = 39). Behavior and quality of life were evaluated by self-administered questionnaires (Child Behavior Checklist, Pediatric Quality of Life Inventory). Patient groups were contrasted and scale results were correlated with clinical and polysomnographic parameters, and cerebrospinal fluid hypocretin-1 levels.

Results: Young patients with NT1 showed increased internalizing problems associated with aggressive behavior. Emotional profile in patients with NT1 positively correlated with age at onset, diagnostic delay, and subjective sleepiness, whereas treatment and disease duration were associated with fewer behavioral problems (attention problems, aggressive behavior, and attention deficit/hyperactivity disorder). Psychosocial health domains of pediatric NT1 were worse than in healthy controls, whereas the physical health domains were comparable.

Conclusions: Young NT1 patients show a discrete pattern of altered behavioral, thought, and mood profile in comparison with healthy controls and with idiopathic epilepsy patients thus suggesting a direct link with sleepiness. Further studies investigating behavior in patients with idiopathic hypersomnia or type 2 narcolepsy are needed to disentangle the role of REM sleep dysfunction and hypocretin deficiency in psychiatric disorders. Symptoms of withdrawal, depression, somatic complaints, thought problems, and aggressiveness were common, NT1 children perceived lower school competencies than healthy children, and their parents also reported worse psychosocial health. Our data suggest that early effective treatment and disease self-awareness should be promoted in NT1 children for their positive effect on behavior and psychosocial health.

Keywords: attention deficit/hyperactivity disorder; narcolepsy with cataplexy; psychiatry; quality of life.

Figure 1

Distribution of T-scores in Syndrome scales. Distribution of T-scores for NT1 (gray box), IE patients (dark gray box), and controls (black box) in Syndrome scales. In the box plot graph, box's sides mark the first and third quartiles, the central line represents the median and the whiskers account for the maximum and minimum; outliers points lie outside the fences. The horizontal lines mark clinical/normal boundaries. CBCL, Child Behavior Checklist; IE, idiopathic epilepsies; NT1, narcolepsy type 1.

Figure 2

Distribution of T-scores in Diagnostic and Statistical Manual Oriented scales. Distribution of T-scores for NT1 (gray box), IE patients (dark gray box), and controls (black box) in Diagnostic and Statistical Manual Oriented scales. In the box plot graph, box's sides mark the first and third quartiles, the central line represents the median and the whiskers account for the maximum and minimum; outliers points lie outside the fences. The horizontal lines mark clinical/normal boundaries. ADHD, attention deficit/hyperactivity disorder; CBCL, Child Behavior Checklist; IE, idiopathic epilepsies; NT1, narcolepsy type 1; ODD, oppositional/defiant disorder.