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The Diagnosis and Treatment of Pediatric Narcolepsy

  • Sona NevsimalovaEmail author
Sleep (M Thorpy, M Billiard, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Sleep

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 .

Keywords

Diagnostic procedure Differential diagnosis Management Treatment 

Notes

Acknowledgments

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

Compliance with Ethics Guidelines

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.

Human and Animal Rights and Informed Consent

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

Supplementary material

Video S1

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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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of Neurology, First Faculty of MedicineCharles UniversityPrague 2Czech Republic

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