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The Diagnosis and Treatment of Pediatric Narcolepsy

  • Sleep (M Thorpy, M Billiard, Section Editors)
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Abstract

Narcolepsy in children is a serious disorder marked by a chronic course and lifelong handicap in school performance and choice of employment, by free time activity limitation, and by behavior and personality changes, all of which constitute a major influence on the quality of life. Increased daytime sleepiness may be the only sign at the disease onset, with attacks of sleep becoming longer and lasting up to hours. Also present may be confusional arousals with features of sleep drunkenness. Paradoxically, preschool and young children may show inattentiveness, emotional lability, and hyperactive behavior. Cataplexy may develop after onset of sleepiness and affect mainly muscles of the face. Hypnagogic hallucinations and sleep paralysis are seldom present. Multiple Sleep Latency Test criteria are not available for children younger than 6 years. The haplotype (HLA-DQB1:0602) can be associated with the disorder; however, the best predictor of narcolepsy–cataplexy is hypocretin deficiency. The treatment generally used in adults is regarded as off-label in childhood, which is why the management of pediatric narcolepsy is difficult .

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Acknowledgments

Sona Nevsimalova was supported by PRVOUK-P26/LF1/4 and IGA MZ CR NT 13238-4/2012.

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Conflict of Interest

Sona Nevsimalova has received a grant from First Faculty of Medicine, Charles University, and the Grant Agency of the Ministry of Health, Czech Republic.

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This article does not contain any studies with human or animal subjects performed by the author.

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  1. Department of Neurology, First Faculty of Medicine, Charles University, Katerinska 30, 120 00, Prague 2, Czech Republic

    Sona Nevsimalova

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Correspondence to Sona Nevsimalova.

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A sleep attack in a 7-year-old girl falling asleep to the ground even while playing with her toys. Her awakening is difficult and prolonged, with features of sleep drunkenness. Cataplectic attacks are evoked by a funny TV story and affect predominantly face muscles. Hypotonia of mimic muscles can be confused for a severe sleepiness (MPG 19793 kb)

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Nevsimalova, S. The Diagnosis and Treatment of Pediatric Narcolepsy. Curr Neurol Neurosci Rep 14, 469 (2014). https://doi.org/10.1007/s11910-014-0469-1

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  • DOI: https://doi.org/10.1007/s11910-014-0469-1

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