Narcolepsy with and without cataplexy, idiopathic hypersomnia with and without long sleep time: a cluster analysis

Šonka, Karel; Susta, Marek; Billiard, Michel

doi:10.1016/j.sleep.2014.09.016

Cited by 54 publications

(26 citation statements)

References 8 publications

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“…Fragmented nocturnal sleep, while common in NT1, is no more common in NT2 patients than in IH patients (limited to those NT2 who were HLA DQB1*0602 negative in one study) (1, 25, 35). In cluster analysis (i.e., independent of pre-existing assumptions about diagnosis), symptoms and MSLT findings combined result in three clear clusters: a cluster of those with NT1, a cluster of those with IH and long sleep times, and a cluster containing both those with NT2 and IH without long sleep times (36). …”

Section: Differential Diagnosis Of Ihmentioning

confidence: 99%

Idiopathic Hypersomnia

Trotti

2017

Sleep Medicine Clinics

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Idiopathic hypersomnia (IH) is a chronic neurological disorder that results in daytime sleepiness, frequently accompanied by long nocturnal or daytime sleep, unrefreshing sleep, difficulty in awakening, cognitive dysfunction, and autonomic symptoms. The cause of idiopathic hypersomnia is presently unknown, although a genetic predisposition is suggested by the strong family history of similar symptoms. Dysfunction of autonomic, inflammatory, or immune systems has been proposed, and patients with IH have been found to have an endogenous modulator of GABA-A receptors within their cerebrospinal fluid. Diagnosis of IH involves a careful clinical history, with particular attention to the possibility of other disorders with similar symptomatology, and objective testing with actigraphy, polysomnography, and multiple sleep latency testing. There are no FDA-approved treatments for IH symptoms, which are typically treated with off-label use of medications approved for narcolepsy. Modafinil is first line and supported by two randomized, controlled trials in IH patients. A substantial fraction of IH patients are refractory or intolerant to standard treatments, and different treatment strategies, employing novel therapeutics, are necessary in these cases. Even with current treatment options, quality of life and safety may remain impaired in patients with this challenging chronic disease.

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Section: Differential Diagnosis Of Ihmentioning

confidence: 99%

Idiopathic Hypersomnia

Trotti

2017

Sleep Medicine Clinics

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“…1 The main features of IH are normal nighttime sleep with high sleep efficiency and high percentage of deep (slow wave) nonrapid eye movement (NREM) sleep on polysomnography (PSG), with either decreased mean sleep latencies with <2 sleep onset REM periods (SOREMPs) on the multiple sleep latency test (MSLT) or prolonged time "asleep" on continuous PSG or actigraphy (total sleep time 11 hours on 24-hour monitoring) without sleep apnea or periodic limb movements. [4][5][6] The neurobiology of IH remains unclear. [4][5][6] The neurobiology of IH remains unclear.…”

mentioning

confidence: 99%

“…2,3 IH is rarely the cause of central hypersomnolence disorder, but is instead an exclusion diagnosis, with a broad differential diagnosis including narcolepsy without cataplexy, atypical forms of depression, sleep apnea syndrome, and behaviorally induced insufficient sleep syndrome, although there are overlaps with these conditions. [4][5][6] The neurobiology of IH remains unclear. Whereas wakefulness is largely promoted by neurons from the pons, midbrain, and posterior hypothalamus that release acetylcholine, norepinephrine, dopamine, serotonin, histamine, and orexin/hypocretin, NREM sleep is driven mainly by c-aminobutyric acidergic (GABAergic) sleeppromoting nuclei that are found in the preoptic area (the rostral end of the hypothalamus) that inhibit most compoenents of the arousal system.…”

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confidence: 99%

Absence of γ‐aminobutyric acid‐a receptor potentiation in central hypersomnolence disorders

Dauvilliers

Evangelista

Lopez

et al. 2016

Annals of Neurology

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“…The entity of monosymptomatic narcolepsy is also well established. [19] A detailed family history did not reveal clues to any of the members suffering from narcolepsy or other primary sleep disorder except probable sleep-related breathing disorder. [20] Previous studies have reported sleep disorders in up to 10% of first-degree relatives, which again probably reflects the lack of awareness or rather focus on the pivotal role of sleep and its distortions among our communities.…”

Section: Discussionmentioning

confidence: 99%

Diagnosis and management of narcolepsy in the Indian scenario

Sureshbabu

Muniem

Bhatia³

2016

Ann Indian Acad Neurol

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Introduction:The diagnosis and management of narcolepsy in the Indian context needs to be revisited especially in the wake of concerns raised by sleep medicine experts that the entity could be formidably underdiagnosed, as well as undertreated in our setting.Materials and Methods:The history, clinical records, polysomnographic/multiple sleep latency test data, and treatment records of five hundred consecutive patients attending a dedicated sleep clinic between the years 2013 and 2016 were retrospectively analyzed. The response to treatment measures and improvement in daytime functioning were periodically assessed by personal/telephonic interview and E-mail communication.Results:Thirteen patients were diagnosed with narcolepsy based on the standard criteria of which three had cataplexy. The mean age of presentation was 23.23 years and the male:female ratio was 2.25:1. The mean duration from the onset of symptoms to diagnosis was 4.2 years. Two patients responded to nonpharmacological interventions alone, and six to modafinil, while two patients remained symptomatic and required treatment with methylphenidate. One patient was lost to follow-up, while two others are due for their first follow-up.Conclusion:A refurbished outlook of the diagnostic methodology and treatment paths tailored to our clinical scenario can potentially impact the future of narcolepsy management and research in our country.

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Narcolepsy with and without cataplexy, idiopathic hypersomnia with and without long sleep time: a cluster analysis

Cited by 54 publications

References 8 publications

Idiopathic Hypersomnia

Idiopathic Hypersomnia

Absence of γ‐aminobutyric acid‐a receptor potentiation in central hypersomnolence disorders

Diagnosis and management of narcolepsy in the Indian scenario

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