Objectives To characterize the rapid weight gain (RWG) phenotype associated with the onset of childhood narcolepsy and to determine whether it could constitute a marker of severity of the disease. Methods RWG was defined using the BMI z‐score slope reported to one year (>0.67 SD) from symptom onset to disease diagnosis. We compared the clinical, metabolic, and sleep characteristics between patients with or without RWG at diagnosis. Pharmacological management, anthropometric, and clinical progression were also evaluated during the follow‐up. Results A total of 84 de novo narcoleptic pediatric patients were included; their median age at diagnosis was 12.0 years; 59.5% boys, 90.5% cataplexy, and 98.7% HLA‐DQB1*06:02, 57% had RWG profile. RWG patients were younger at diagnosis than non‐RWG patients, despite a shorter diagnostic delay. They had a higher BMI z‐score and a higher prevalence of obesity at diagnosis, but not at symptom onset, and higher adapted Epworth Sleepiness Scale and Insomnia Severity Index scores than non‐RWG patients. No differences on nocturnal polysomnography and multiple sleep latency tests were found between groups at disease diagnosis. After a median follow‐up of 5 years, RWG patients still had a higher BMI z‐score and a higher prevalence of obesity despite benefiting from the same therapeutic management and displaying improvement in sleepiness and school difficulties. Conclusions Narcoleptic RWG patients were younger, sleepier, and the prevalence of obesity was higher at diagnosis despite a shorter diagnostic delay than that of non‐RWG patients. These patients had also a higher risk of developing a long‐term obesity, despite a positive progression of their narcoleptic symptoms. RGW could then represent a maker of a more severe phenotype of childhood narcolepsy, which should inspire a prompt and more offensive management to prevent obesity and its complications.
Study objectives To determine the prevalence of metabolic syndrome (MS) in children with narcolepsy and to evaluate their clinical and sleep characteristics according to the different components of MS. Methods This retrospective study consisted of 58 de novo children with narcolepsy (median age: 12.7 y, 48.3% of boys). The recently published MS criteria in a French population of children were used. Clinical and sleep characteristics were compared between groups with different components of MS. Results MS was present in 17.2% of children with narcolepsy, among whom 79.3% presented with high homeostasis model assessment for insulin resistance (HOMA-IR), 25.9% with high body mass index (BMI), 24.1% with low high-density lipoprotein cholesterol (HDL-C), and 12.1% with high triglycerides. Patients with at least 2 MS components had more night eating behaviors and tended to have lower percentage of slow wave sleep (SWS) and more fragmented sleep. On multiple sleep latency test (MSLT), they had shorter mean sleep latencies to rapid eye movement (REM), non-rapid eye movement (NREM) sleep and tended to have more sleep onset REM periods (SOREMPs) than those with less than 2 MS components. Conclusions Insulin resistance was found to be the core metabolic disturbance in obese as well as in non-obese children with narcolepsy. Children with narcolepsy with at least 2 MS components presented a more severe daytime sleepiness and a higher prevalence of night eating behaviors than those with less than 2 MS components. Such children might benefit from early evaluation and management in order to prevent future complications.
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