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Article Dans Une Revue Journal of Clinical Medicine Année : 2022

Sleep Disorders in Adults with Prader–Willi Syndrome: Review of the Literature and Clinical Recommendations Based on the Experience of the French Reference Centre

Pauline Dodet
  • Fonction : Auteur
Federica Sanapo
  • Fonction : Auteur
Smaranda Leu-Semenescu
  • Fonction : Auteur
Muriel Coupaye
Alice Bellicha
Isabelle Arnulf
  • Fonction : Auteur
Stefania Redolfi
  • Fonction : Auteur

Résumé

Prader–Willi syndrome (PWS) is a rare, genetic, multisymptomatic, neurodevelopmental disease commonly associated with sleep alterations, including sleep-disordered breathing and central disorders of hypersomnolence. Excessive daytime sleepiness represents the main manifestation that should be addressed by eliciting the detrimental effects on quality of life and neurocognitive function from the patients’ caregivers. Patients with PWS have impaired ventilatory control and altered pulmonary mechanics caused by hypotonia, respiratory muscle weakness, scoliosis and obesity. Consequently, respiratory abnormalities are frequent and, in most cases, severe, particularly during sleep. Adults with PWS frequently suffer from sleep apnoea syndrome, sleep hypoxemia and sleep hypoventilation. When excessive daytime sleepiness persists after adequate control of sleep-disordered breathing, a sleep study on ventilatory treatment, followed by an objective measurement of excessive daytime sleepiness, is recommended. These tests frequently identify central disorders of hypersomnolence, including narcolepsy, central hypersomnia or a borderline hypersomnolent phenotype. The use of wake-enhancing drugs (modafinil, pitolisant) is discussed in multidisciplinary expert centres for these kinds of cases to ensure the right balance between the benefits on quality of life and the risk of psychological and cardiovascular side effects.

Dates et versions

hal-04011897 , version 1 (02-03-2023)

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Citer

Pauline Dodet, Federica Sanapo, Smaranda Leu-Semenescu, Muriel Coupaye, Alice Bellicha, et al.. Sleep Disorders in Adults with Prader–Willi Syndrome: Review of the Literature and Clinical Recommendations Based on the Experience of the French Reference Centre. Journal of Clinical Medicine, 2022, 11 (7), pp.1986. ⟨10.3390/jcm11071986⟩. ⟨hal-04011897⟩
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