2014•Child NeurologyOpen access

Narcolepsy in children

Marcin Żarowski, Barbara Steinborn, Sanjeev V. Kothare

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Abstract

Narcolepsy is a life-long but non-progressive neurological disorder characterized by excessive daytime sleepiness and increased presence of rapid eye movement (REM) sleep due to loss of hypocretin containing neurons located in the lateral hypothalamus. According to the International Classification of Sleep Disorders, Third Edition (ICSD-3), narcolepsy is typically associated with cataplexy and/or other REM sleep phenomena such as hypnagogic or hypnopompic hallucinations and sleep paralysis. Narcolepsy is not an uncommon disorder, but is under-recognized. The age of onset shows two peaks of presentation: in childhood and in adults. Both genetics and environmental factors may be involved in the development of narcolepsy. The history, combined with polysomnography and MSLT, remain the current gold standard in the diagnosis of narcolepsy. The levels of Hypocretin-1 are undetectable in the cerebrospinal fluid in most patients with narcolepsy with cataplexy. Early recognition and treatment can greatly improve the quality of life of patients with narcolepsy. Treatment of narcolepsy includes non-pharmacological treatment (life style changes) and pharmacological (stimulants, modafinil, sodium oxybate) against hypersomnia and anticataplectic drugs (antidepressants and sodium oxybate).

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What this paper is about

Narcolepsy is a life-long but non-progressive neurological disorder characterized by excessive daytime sleepiness and increased presence of rapid eye movement (REM) sleep due to loss of hypocretin containing neurons located in the lateral hypothalamus. According to the International Classification of Sleep Disorders, Third Edition (ICSD-3), narcolepsy is typically associated with cataplexy and/or other REM sleep phenomena such as hypnagogic or hypnopompic hallucinations and sleep paralysis. Narcolepsy is not an uncommon disorder, but is under-recognized. The age of onset shows two peaks of presentation: in childhood and in adults. Both genetics and environmental factors may be involved in the development of narcolepsy. The history, combined with polysomnography and MSLT, remain the current gold standard in the diagnosis of narcolepsy. The levels of Hypocretin-1 are undetectable in the cerebrospinal fluid in most patients with narcolepsy with cataplexy. Early recognition and treatment can greatly improve the quality of life of patients with narcolepsy. Treatment of narcolepsy includes non-pharmacological treatment (life style changes) and pharmacological (stimulants, modafinil, sodium oxybate) against hypersomnia and anticataplectic drugs (antidepressants and sodium oxybate).

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Available abstract

Narcolepsy is a life-long but non-progressive neurological disorder characterized by excessive daytime sleepiness and increased presence of rapid eye movement (REM) sleep due to loss of hypocretin containing neurons located in the lateral hypothalamus. According to the International Classification of Sleep Disorders, Third Edition (ICSD-3), narcolepsy is typically associated with cataplexy and/or other REM sleep phenomena such as hypnagogic or hypnopompic hallucinations and sleep paralysis. Narcolepsy is not an uncommon disorder, but is under-recognized. The age of onset shows two peaks of presentation: in childhood and in adults. Both genetics and environmental factors may be involved in the development of narcolepsy. The history, combined with polysomnography and MSLT, remain the current gold standard in the diagnosis of narcolepsy. The levels of Hypocretin-1 are undetectable in the cerebrospinal fluid in most patients with narcolepsy with cataplexy. Early recognition and treatment can greatly improve the quality of life of patients with narcolepsy. Treatment of narcolepsy includes non-pharmacological treatment (life style changes) and pharmacological (stimulants, modafinil, sodium oxybate) against hypersomnia and anticataplectic drugs (antidepressants and sodium oxybate).

Key concepts: Narcolepsy, Sleep paralysis, Cataplexy, Modafinil, Excessive daytime sleepiness, Polysomnography, Psychology, Sleep disorder

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