Nocturnal REM Sleep Without Atonia Is a Diagnostic Biomarker of Pediatric Narcolepsy

Bin-Hasan, Saadoun; Videnović, Aleksandar; Maski, Kiran

doi:10.5664/jcsm.6944

Cited by 31 publications

(14 citation statements)

References 21 publications

(31 reference statements)

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“…Sleep drunkenness has not been well-quantified among people with NT2 but is reported to occur in 47% of those with NT2 [ 16 ]. Objective testing in NT2 also shows substantial heterogeneity, particularly in rates of the objective REM features, including nocturnal REM sleep-onset latency [ 17 ], number of daytime SOREMs [ 18 ], and REM without atonia [ 18 , 19 ].…”

Section: Challenges With Current Classificationmentioning

confidence: 99%

To split or to lump? Classifying the central disorders of hypersomnolence

Fronczek

Arnulf

Baumann

et al. 2020

Sleep

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The classification of the central disorders of hypersomnolence has undergone multiple iterations in an attempt to capture biologically meaningful disease entities in the absence of known pathophysiology. Accumulating data suggests that further refinements may be necessary. At the 7th International Symposium on Narcolepsy, a group of clinician-scientists evaluated data in support of keeping or changing classifications, and as a result suggest several changes. First, idiopathic hypersomnia with long sleep durations appears to be an identifiable and meaningful disease subtype. Second, idiopathic hypersomnia without long sleep time and narcolepsy without cataplexy share substantial phenotypic overlap and cannot reliably be distinguished with current testing, and so combining them into a single disease entity seems warranted at present. Moving forward, it is critical to phenotype patients across a wide variety of clinical and biological features, to aid in future refinements of disease classification.

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Section: Challenges With Current Classificationmentioning

confidence: 99%

To split or to lump? Classifying the central disorders of hypersomnolence

Fronczek

Arnulf

Baumann

et al. 2020

Sleep

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“…2 In these children, RBD occurs in correlation with decreased muscle atonia during REM sleep, 2 and indeed REM sleep without atonia (RSWA) is a sensitive and specific biomarker for NT1 in children. 3 Since Sodium Oxybate (SO), a first line treatment of NT1, has been recently reported to be effective in idiopathic RBD (iRBD), 4,5 and in modulating muscle activity during REM sleep in A C C E P T E D narcolepsy, 6,7 the aim of this study was to evaluate the effect of this drug also on nocturnal motor activity/behavior during REM sleep in NT1 children.…”

Section: Introductionmentioning

confidence: 99%

“… 2 In these children, RBD occurs in correlation with decreased muscle atonia during REM sleep 2 ; indeed, REM sleep without atonia (RSWA) is a sensitive and specific biomarker for NT1 in children. 3 …”

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confidence: 99%

REM Sleep Behavior Disorder in Children With Type 1 Narcolepsy Treated With Sodium Oxybate

et al. 2021

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Objective:The aim was to study the effect of stable treatment with Sodium Oxybate (SO) on nocturnal REM sleep behavior disorder (RBD) and REM sleep without atonia (RSWA) that severely affected children with type 1 narcolepsy (NT1.Methods:Nineteen NT1 children and adolescents (nine females; mean age 12.5±2.7, mean disease duration: 3.4±1.6 years) underwent neurological investigations and video-polysomnography (v-PSG) at baseline and after three months of stable treatment with SO.v-PSG was independently analysed by two sleep experts, in order to rate RBD episodes. RSWA was automatically computed by means of the validated REM sleep atonia index (RAI).Results:Compared to baseline, RAI significantly improved (p< 0.05) and complex movements during REM sleep were remarkably reduced after stable treatment with SO. Compared to baseline, children also reported improvement in clinical complaints and showed a different nighttime sleep stage architecture.Conclusions:RBD and RSWA improved after treatment with SO, pointing to a direct role of the drug in modulating motor control during REM sleep.

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“…Vivid or lucid dreams (ie, dreams that a person is consciously aware of dreaming) are frequently reported by people with narcolepsy 31 ; occasionally, dreams are so realistic that they produce false memories or dream-reality confusion 32 . Either clinical dream enactment behavior or REM sleep without atonia seen on polysomnogram can be present in 20% to 60% of people with narcolepsy 33,34 . Other comorbid sleep disorders include obstructive sleep apnea, periodic limb movement disorder, and restless legs syndrome 35 .…”

Section: Primary Chronic Central Nervous System Disorders Of Hypersom...mentioning

confidence: 99%

Central Disorders of Hypersomnolence

Blattner,

Maski

2023

CONTINUUM: Lifelong Learning in Neurology

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Objective The goals of this article are to describe the clinical approach to and management of patients with central disorders of hypersomnolence, and to understand and differentiate available diagnostic tools. Latest Developments Updated clinical practice guidelines for the treatment of central disorders of hypersomnolence and narcolepsy specifically highlight new treatment options. Approval for a lower-sodium oxybate formulation that contains 92% less sodium than the standard sodium oxybate for the treatment of narcolepsy and idiopathic hypersomnia adds to the number of medications available for these disorders, allowing for a more tailored management of symptoms. Essential Points Central disorders of hypersomnolence are characterized by excessive daytime sleepiness that impacts daily functions. These disorders can be differentiated by obtaining a detailed clinical sleep history and by a thoughtful interpretation of sleep diagnostic testing. Tailoring treatment approaches to meet the needs of individuals and accounting for medical and psychiatric comorbidities may improve quality of life.

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Nocturnal REM Sleep Without Atonia Is a Diagnostic Biomarker of Pediatric Narcolepsy

Abstract: The nRWA index is a very good diagnostic biomarker of pediatric narcolepsy. Depending on the clinical cutoffs utilized, this biomarker can identify more children/adolescents with narcolepsy using just the PSG or reduce false-positive diagnostic results.

Cited by 31 publications

References 21 publications

To split or to lump? Classifying the central disorders of hypersomnolence

To split or to lump? Classifying the central disorders of hypersomnolence

REM Sleep Behavior Disorder in Children With Type 1 Narcolepsy Treated With Sodium Oxybate

Central Disorders of Hypersomnolence

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