Is Excessive Daytime Sleepiness Characteristic of Prader-Willi Syndrome?

Harris, James C.

doi:10.1001/archpedi.1996.02170370066011

Cited by 52 publications

(46 citation statements)

References 21 publications

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“…This is in agreement with other diseases of obesity such as the Prader-Willi syndrome, in which it has been shown that sleepiness remains primarily unaffected, even after weight reduction and improvement of sleepdisordered breathing. 53 However, because our control group consisted of primarily lean individuals, the differences in sleepiness may have been slightly enhanced. Thus, the lack of a control group consisting of obese individuals without brain surgery is a limitation of this study.…”

Section: Figmentioning

confidence: 99%

Severe Hypersomnolence After Pituitary/Hypothalamic Surgery in Adolescents: Clinical Characteristics and Potential Mechanisms

Snow¹,

Gozal

Malhotra

et al. 2002

Pediatrics

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ABSTRACT. Objectives. After resection of hypothalamic/pituitary tumors, children are at risk for development of hormonal deficiencies, obesity, and hypersomnolence. However, the prevalence and pathophysiology of these complications are unclear. The purpose of this study was to assess the prevalence and severity of hypersomnolence in children after resection of pituitary tumors and to study the potential factors that contribute to this sleepiness if present. We further hypothesized that decrements in orexin levels may contribute to the sleepiness.Methods. Six children who underwent hypothalamic/ pituitary surgery were identified. Five of these patients and 5 matched control subjects underwent overnight polysomnography followed by a multiple sleep latency test. Children who had a primary sleep disorder (eg, obstructive sleep apnea) underwent treatment and were restudied subsequently (n ‫؍‬ 2). Blood levels of pituitary hormones were measured. Blood and cerebrospinal fluid (CSF) were drawn from 4 patients and 3 control subjects to measure orexin levels.Results. Endocrine control was appropriate in all children. Although patients had longer sleep duration but similar sleep efficiency than control subjects, relatively severe daytime somnolence was present (mean sleep latency: 10.3 ؎ 5.3 minutes vs 26.2 ؎ 1.1 minute in control subjects). Sleepiness did not correlate with body mass index or age. Furthermore, serum and CSF orexin levels did not differ between patients and control subjects.Conclusions. Severe daytime sleepiness is frequent among children who undergo pituitary/hypothalamic surgery and does not seem to result from inappropriate cortisol or thyroxine replacement, disturbed nocturnal sleep, or low levels of orexin in the serum or CSF. We therefore speculate that other, unidentified neurohormonal mechanisms may mediate the excessive sleepiness of these patients. Pediatrics 2002;110(6). URL: http:// www.pediatrics.org/cgi/content/full/110/6/e74; sleep, excessive daytime sleepiness, adolescents, orexin (hypocretin), brain tumors, craniopharyngioma.

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Section: Figmentioning

confidence: 99%

Severe Hypersomnolence After Pituitary/Hypothalamic Surgery in Adolescents: Clinical Characteristics and Potential Mechanisms

Snow¹,

Gozal

Malhotra

et al. 2002

Pediatrics

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“…Willi et al 28 found sleep apnea in 5 of 6 morbidly obese children, and weight loss led to resolution of the apnea. Harris and Allen 29 found that children with Prader-Willi syndrome had OSA. The severity of OSA was related to their weight, and the OSA diminished following weight reduction.…”

Section: Commentmentioning

confidence: 99%

Effect of Treating Obstructive Sleep Apnea by Tonsillectomy and/or Adenoidectomy on Obesity in Children

Soultan

Wadowski²,

Rao³

et al. 1999

Arch Pediatr Adolesc Med

112

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“…We found no correlation between BMI and ESS or mean sleep latency on MSLT. Two studies [Harris and Allen, 1996;Priano et al, 2006] reported no correlation between EDS and obesity, whereas other studies showed that obese patients with PWS had more sleepiness [Vgontzas et al, 1996;Butler et al, 2002;O-'Donoghue et al, 2005]. In this study, patients with sleep disordered breathing likely associated with obesity were excluded.…”

Section: Discussionmentioning

confidence: 87%

“…In addition, excessive daytime sleepiness (EDS), a highly disruptive symptom to the daily routine of children with PWS and their families has been identified as an important issue affecting clinical care and learning [Cotton and Richdale, 2006]. Sleep-disordered breathing and hypothalamic dysfunction seem to contribute to EDS [Nixon and Brouillette, 2002], but sleepiness persists in patients with PWS after weight loss and reduction of pre-existing sleep disordered breathing by nocturnal continuous positive airway pressure treatment [Harris and Allen, 1996]. Growth hormone (GH) treatment, widely used in these children for their short stature and their abnormal body composition, does not improve this symptom.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of modafinil on excessive daytime sleepiness in Prader–Willi syndrome

Cock

Diene

Molinas

et al. 2011

American J of Med Genetics Pt A

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Excessive daytime sleepiness is a frequent and a highly disruptive symptom to the daily routine of children with Prader-Willi Syndrome (PWS) and their families. The objective of the study was to evaluate the efficacy of modafinil, a central stimulant, on excessive daytime sleepiness in children and adolescents with PWS. The efficacy of modafinil was evaluated in this open label pilot study comparing the Epworth sleepiness scale before and after treatment. Ten patients with molecularly confirmed PWS and a complaint of excessive daytime sleepiness underwent a night-time sleep recording and multiple sleep latency tests. One patient was excluded because of severe obstructive sleep apnea syndrome. Nine patients (4 males) with median age of 16 years (8-21) received modafinil at a starting dose of 100 mg/day. We found that all patients had excessive daytime sleepiness with an Epworth sleepiness scale at 14 (11-20) and mean sleep latency on multiple sleep latency tests at 5 (3-6) minutes. Moreover, six patients had at least two sleep-onset rapid eye movement periods showing a narcolepsy-like phenotype. Modafinil significantly improved sleepiness in all patients on the Epworth sleepiness scale from 14 (11-20) to 4 (3-12), (P = 0.007). Body mass index of the patients did not change significantly under treatment. No side effects were reported, and the drug was well-tolerated. We posit that this open label case series shows good efficacy of modafinil in nine children and adolescents with PWS.

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Is Excessive Daytime Sleepiness Characteristic of Prader-Willi Syndrome?

Cited by 52 publications

References 21 publications

Severe Hypersomnolence After Pituitary/Hypothalamic Surgery in Adolescents: Clinical Characteristics and Potential Mechanisms

Severe Hypersomnolence After Pituitary/Hypothalamic Surgery in Adolescents: Clinical Characteristics and Potential Mechanisms

Effect of Treating Obstructive Sleep Apnea by Tonsillectomy and/or Adenoidectomy on Obesity in Children

Efficacy of modafinil on excessive daytime sleepiness in Prader–Willi syndrome

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