Narcolepsy in children
- Suresh Kotagal, MD
Suresh Kotagal, MD
- Professor of Neurology, Mayo Clinic
- Consultant in Neurology, Pediatrics, and Sleep Medicine
- Section Editors
- Thomas E Scammell, MD
Thomas E Scammell, MD
- Section Editor — Hypersomnias
- Professor of Neurology, Beth Israel Deaconess Medical Center
- Boston Children's Hospital, and Harvard Medical School
- Ronald D Chervin, MD, MS
Ronald D Chervin, MD, MS
- Editor-in-Chief — Sleep Medicine
- Section Editor — Pediatric Sleep Medicine
- Professor of Neurology
- University of Michigan
Narcolepsy is a chronic neurologic disorder characterized by excessive and irresistible sleepiness, cataplexy, hypnagogic hallucinations (vivid dreams at sleep onset), and sleep paralysis (a momentary inability to move the body as one is drifting off to sleep). Although relatively rare in general, it is one of the more common causes of disabling daytime sleepiness beginning in adolescence or early adulthood.
Narcolepsy can present in children as young as five or six years of age, and delays in diagnosis are common. Early-onset narcolepsy has some unique clinical features compared with later onset cases, including cataplexy with prominent oculobuccofacial involvement and daytime sleepiness manifesting primarily as habitual napping or irritability and hyperactivity.
This topic will review the clinical features, diagnosis, and management of narcolepsy in children. Narcolepsy in adults is reviewed separately. (See "Clinical features and diagnosis of narcolepsy in adults" and "Treatment of narcolepsy in adults".)
FORMS OF NARCOLEPSY
The International Classification of Sleep Disorders, Third Edition (ICSD-3), recognizes two forms of narcolepsy :
●Type 1 (previously called narcolepsy with cataplexy) – Patients with narcolepsy type 1 generally show cataplexy along with sleepiness at the very onset of the disorder or within the first several years of the onset of sleepiness. The underlying pathophysiology is a deficiency of central nervous system hypocretin (orexin), which is a peptide essential for maintaining alertness. (See 'Pathophysiology' below.)To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- FORMS OF NARCOLEPSY
- CLINICAL FEATURES
- Daytime sleepiness
- Hypnagogic hallucinations and sleep paralysis
- Other sleep disturbances
- Neuropsychiatric symptoms
- Obesity and precocious puberty
- DIFFERENTIAL DIAGNOSIS
- Diagnostic criteria
- Polysomnography and MSLT
- CSF hypocretin-1 testing
- Other tests
- Behavioral and lifestyle modification
- - Daytime sleepiness
- - Cataplexy
- Emerging therapies
- SUMMARY AND RECOMMENDATIONS