Narcolepsy in children

Wise, Merrill S.; Lynch, Jennifer

doi:10.1053/spen.2001.29475

Cited by 33 publications

(49 citation statements)

References 24 publications

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“…5,11 Historically, narcolepsy was considered a disorder of adulthood, but accumulating evidence indicates that signs of narcolepsy may start during childhood. [12][13][14] The characteristic tetrad of narcolepsy includes daytime hypersomnia, cataplexy, hypnagogic hallucinations, and sleep paralysis.…”

Section: Clinical Features Of Narcolepsymentioning

confidence: 99%

“…[12][13][14] The characteristic tetrad of narcolepsy includes daytime hypersomnia, cataplexy, hypnagogic hallucinations, and sleep paralysis. 5 The initial and sole manifestation of childhood narcolepsy usually is hypersomnia followed by development of other symptoms months to years later. 5,12,[15][16][17] In addition, sleep fragmentation has been recognized as a fifth but commonly present criterion in the diagnosis of narcolepsy.…”

Section: Clinical Features Of Narcolepsymentioning

confidence: 99%

“…5 The initial and sole manifestation of childhood narcolepsy usually is hypersomnia followed by development of other symptoms months to years later. 5,12,[15][16][17] In addition, sleep fragmentation has been recognized as a fifth but commonly present criterion in the diagnosis of narcolepsy. 10 …”

Section: Clinical Features Of Narcolepsymentioning

confidence: 99%

“…4 Stimulant medications are used with the goal of providing optimal alertness during working or school hours and other social situations. 5,6 Modafinil, a non-amphetamine wakefulness-promoting medication, is now considered the first-line treatment for EDS in adults with narcolepsy. 7 REM-suppressant medications such as tricyclic antidepressants or serotonin reuptake inhibitors can be added to control cataplexy or hypnagogic hallucinations.…”

Section: Introductionmentioning

confidence: 99%

“…7 REM-suppressant medications such as tricyclic antidepressants or serotonin reuptake inhibitors can be added to control cataplexy or hypnagogic hallucinations. 5 Sodium oxybate is a gamma amino butyric acid (GABA) B receptor agonist currently approved by the U.S. Food and Drug Administration (FDA) for the treatment of both cataplexy and EDS in patients with narcolepsy with age more than 16 years. 7,8 The focus of this article is the use of sodium oxybate in narcolepsy.…”

Section: Introductionmentioning

confidence: 99%

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Pharmacotherapy of Narcolepsy: Focus on Sodium Oxybate

Kothare

Kaleyias

2010

Clinical Medicine Insights: Therapeutics

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Narcolepsy is a complex disease with multiple symptoms that include excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, and sleep paralysis. Pharmacologic management of patients with narcolepsy is based on treating these symptoms. Sodium oxybate, a neurotransmitter product of γ-amino butyric acid (GABA), is a Schedule III controlled substance in the US, approved for the treatment of excessive daytime sleepiness (EDS), and cataplexy in patients with narcolepsy.The purpose of this review is to summarize the current knowledge about treatment of narcolepsy with sodium oxybate and to familiarize readers with the safety and efficacy of sodium oxybate, thus enabling clinicians to optimize their management of narcolepsy. A review of the pharmacokinetics, pharmacodynamics, efficacy in different symptoms of narcolepsy, adverse effects, tolerability, and dosing of sodium oxybate is presented. Sodium oxybate administered as an oral solution has a rapid onset of effect, a short duration of action, and nearly complete metabolism with negligible unchanged γ-hydroxybutyrate (GHB) appearing in the urine. There is mounting evidence suggesting that GHB may function as an endogenous neuromodulator/neurotransmitter. The mechanism by which sodium oxybate has a therapeutic effect in narcolepsy is unclear. The efficacy of oral sodium oxybate on the treatment of narcolepsy was evaluated in two randomized, double-blind, placebo-controlled, multicenter, US and International trials. Treatment of patients with narcolepsy-cataplexy with sodium oxybate leads to reduction in the frequency of cataplexy attacks in a dose-related manner, improved EDS, increased level of alertness and ability to concentrate. The major problem with sodium oxybate remains in its potential for non-medical use to elicit altered states of consciousness; however, abuse and misuse of sodium oxybate has been rare over the 7 years since its introduction in the market. Other issues revolve around twice nightly dosing, and salty taste. The most commonly reported adverse events associated with the use of sodium oxybate and occurring with at least 5% greater frequency than seen in placebo-treated patients were dizziness, headache, nausea, pain, night terrors, enuresis, confusion, infection, vomiting, and urinary incontinence. In conclusion, sodium oxybate significantly improves all symptoms in patients with narcolepsy and is well tolerated.

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Section: Clinical Features Of Narcolepsymentioning

confidence: 99%

Section: Clinical Features Of Narcolepsymentioning

confidence: 99%

Section: Clinical Features Of Narcolepsymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pharmacotherapy of Narcolepsy: Focus on Sodium Oxybate

Kothare

Kaleyias

2010

Clinical Medicine Insights: Therapeutics

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Cataplexy features in childhood narcolepsy

et al. 2008

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Cataplexy, the hallmark of narcolepsy, has been well characterized in adults but not in children. This study systematically used structured clinical assessments and video-recordings (49 episodes in eight cases) to evaluate cataplexy in 23 patients diagnosed before the age of 18 years. Forty-three percent of patients had falls as part of their attacks. During cataplexy knees, head, and jaw were the most frequently compromised body segments; eyelids, arms, and trunk being less commonly involved. More rarely, blurred vision, slurred speech, irregular breathing, or a sudden loss of smiling mimics were reported. One-third of the sample presented with a previously unrecognized description of cataplexy that we coined "cataplectic facies," consisting of a state of semipermanent eyelid and jaw weakness, on which partial or complete cataplectic attacks were superimposed. The usual triggering emotions, such as laughter, joking, or anger, were not always present, especially when close to an abrupt onset, hampering diagnosis. Video-recordings of cataplectic attacks may be useful to document the attack, allowing a comparison with archived presentations.

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Narcolepsy in Children: Sleep disorders in children, A rapidly evolving field seeking consensus

Dye

Simakajornboon

2021

Pediatric Pulmonology

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Narcolepsy is a life‐long sleep disorder with two distinct subtypes, narcolepsy type I and narcolepsy type II. It is now well recognized that the loss of hypocretin neurons underlies the pathogenesis of narcolepsy type I, however, the pathogenesis of narcolepsy type II is currently unknown. Both genetic and environmental factors play an important role in the pathogenesis of narcolepsy. There is increasing evidence that autoimmune processes may play a critical role in the loss of hypocretin neurons. Infections especially streptococcus and influenza have been proposed as a potential trigger for the autoimmune‐mediated mechanism. Several recent studies have shown increased cases of pediatric narcolepsy following the 2009 H1N1 pandemic. The increased cases in Europe seem to be related to a specific type of H1N1 influenza vaccination (Pandemrix), while the increased cases in China are related to influenza infection. Children with narcolepsy can have an unusual presentation at disease onset including complex motor movements which may lead to delayed diagnosis. All classic narcolepsy tetrads are present in only a small proportion of children. The diagnosis of narcolepsy is confirmed by either obtaining cerebrospinal fluid hypocretin or overnight sleep study with the multiple sleep latency test (MSLT). There are limitations of using MSLT in young children such that a negative MSLT test cannot exclude narcolepsy. HLA markers have limited utility in narcolepsy, but it may be useful in young children with clinical suspicion of narcolepsy. For management, both pharmacologic and non‐pharmacologic treatments are important in the management of narcolepsy. Pharmacotherapy is primarily aimed to address excessive daytime sleepiness and REM‐related symptoms such as cataplexy. In addition to pharmacotherapy, routine screening of behavioral and psychosocial issues is warranted to identify patients who would benefit from bio‐behavior intervention.

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Narcolepsy in children

Cited by 33 publications

References 24 publications

Pharmacotherapy of Narcolepsy: Focus on Sodium Oxybate

Pharmacotherapy of Narcolepsy: Focus on Sodium Oxybate

Cataplexy features in childhood narcolepsy

Narcolepsy in Children: Sleep disorders in children, A rapidly evolving field seeking consensus

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