Factors Influencing Time to Diagnosis in Childhood Narcolepsy Type 1.

OBJECTIVE: To study the variables impacting on time from symptom onset to diagnosis in childhood narcolepsy, including presence of cataplexy, onset in infancy, administration of the H1N1 Pandemrix vaccine, and date of diagnosis following the H1N1 pandemic. DESIGN: A retrospective cohort study of 42 children seen in a single tertiary pediatric neurology center between 1996 and 2016. KEY RESULTS: Onset of symptoms of narcolepsy occurred between infancy and 15 years, with 92.9% of children symptomatic by 13 years. Time from reported symptom onset to diagnosis ranged from 3 months to 11 years, with 51.3% diagnosed within 12 months of symptom onset. Those patients who were reportedly symptomatic from birth had a statistically significant increased time from reported symptom onset to diagnosis. CONCLUSIONS: The symptoms of childhood narcolepsy progress over time. The number of annual diagnoses in this center increased over the study period, but there is no evidence that the time from symptom onset to diagnosis is decreasing. Being reportedly symptomatic from the age of <1 year is associated with an increased time to diagnosis. We recommend raising awareness of narcolepsy among medical students and general practitioners. It is important that clinicians appreciate the clinical relevance of excessive daytime sleepiness.

Psychosocial and intellectual functioning in childhood narcolepsy.

PURPOSE: This study reports the psychological assessment of 12 children referred to a narcolepsy clinic, using quantitative methods to describe intellectual and psychosocial functioning in childhood narcolepsy. METHODS: The participants were six males and six females aged between 7-16 years (median age 10 years). The protocol included a clinical interview; the Wechsler Intelligence Scale for Children-III-UK; and the Parent version of the Achenbach Child Behaviour Checklist (CBCL). RESULTS: Eleven children obtained an IQ in the average range (mean and median =100). However, a significant difference was found between verbal and performance scales in 42% of children, compared to WISC-III normative prevalence rates of 24%. CBCL results revealed that 10/12 children scored in the clinically significant range on the Total Score Index, with 9/12 obtaining scores in the significant range on the Internalizing Index. The majority of children presented with difficulties in discussing and describing distressing physical and psychological symptoms with parents and others. CONCLUSIONS: These findings suggest that the psychosocial impact of narcolepsy extends beyond the effects of excessive sleepiness and that symptoms such as hallucinations can lead to significant psychological morbidity.