Sleep in Mowat-Wilson Syndrome: a clinical and video-polysomnographic study

Veronica Di Pisa, Federica Provini, Sara Ubertiello, Silvia Bonetti, Emilia Ricci, Ivan Ivanovski, Stefano Giuseppe Caraffi, Lucio Giordano, Patrizia Accorsi, Salvatore Savasta, Federico Raviglione, Antonella Boni, Daniele Grioni, Claudio Graziano, Livia Garavelli, Duccio Maria Cordelli

Research output: Contribution to journalArticle

Abstract

Objective: Sleep disturbances are frequently reported in Mowat-Wilson Syndrome (MWS). The current study aimed to evaluate clinical and video-polysomnographic (VPSG) characteristics of the sleep architecture and abnormal electroencephalogram (EEG) patterns during sleep in MWS. Methods: Sixteen individuals with MWS (range 16 months–25 years), attending the Department of Child Neurology and Psychiatry of the University of Bologna, were included. The “Sleep Disturbances Scale for Children (SDSC)” questionnaire was administered to all parents of MWS patients, and all patients underwent a VPSG recording. Results: The analysis of the SDSC questionnaire revealed disturbances mainly at the sleep–wake transition and in initiating and maintaining sleep. Evaluation of sleep structure in MWS patients showed a significant reduction of total sleep time, an increase of wake after sleep onset and arousal index as compared to normal controls. An EEG pattern characterized by slowing of background activity and poverty of physiological sleep characterisitcs was observed in all patients. Moreover, in patients aged >7 years, anteriorly predominant spike and waves were observed, markedly activated by sleep configuring a sub-continuous or continuous activity. Conclusion: Our data (both clinical and VPSG) documented the presence of significant and clinically relevant sleep disturbances in MWS patients. Moreover, we identified a characteristic age-dependent sleep EEG pattern that could provide a new element to assist in the management of MWS.

Original languageEnglish
JournalSleep Medicine
DOIs
Publication statusPublished - Jan 1 2019

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Sleep
Electroencephalography
Mowat-Wilson syndrome
Video Recording
Child Psychiatry
Poverty
Neurology
Arousal
Parents

Keywords

  • ESES
  • Mowat-wilson syndrome
  • Sleep disturbances
  • Video-polysomnography

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Di Pisa, V., Provini, F., Ubertiello, S., Bonetti, S., Ricci, E., Ivanovski, I., ... Cordelli, D. M. (2019). Sleep in Mowat-Wilson Syndrome: a clinical and video-polysomnographic study. Sleep Medicine. https://doi.org/10.1016/j.sleep.2019.04.011

Sleep in Mowat-Wilson Syndrome : a clinical and video-polysomnographic study. / Di Pisa, Veronica; Provini, Federica; Ubertiello, Sara; Bonetti, Silvia; Ricci, Emilia; Ivanovski, Ivan; Caraffi, Stefano Giuseppe; Giordano, Lucio; Accorsi, Patrizia; Savasta, Salvatore; Raviglione, Federico; Boni, Antonella; Grioni, Daniele; Graziano, Claudio; Garavelli, Livia; Cordelli, Duccio Maria.

In: Sleep Medicine, 01.01.2019.

Research output: Contribution to journalArticle

Di Pisa, V, Provini, F, Ubertiello, S, Bonetti, S, Ricci, E, Ivanovski, I, Caraffi, SG, Giordano, L, Accorsi, P, Savasta, S, Raviglione, F, Boni, A, Grioni, D, Graziano, C, Garavelli, L & Cordelli, DM 2019, 'Sleep in Mowat-Wilson Syndrome: a clinical and video-polysomnographic study', Sleep Medicine. https://doi.org/10.1016/j.sleep.2019.04.011
Di Pisa, Veronica ; Provini, Federica ; Ubertiello, Sara ; Bonetti, Silvia ; Ricci, Emilia ; Ivanovski, Ivan ; Caraffi, Stefano Giuseppe ; Giordano, Lucio ; Accorsi, Patrizia ; Savasta, Salvatore ; Raviglione, Federico ; Boni, Antonella ; Grioni, Daniele ; Graziano, Claudio ; Garavelli, Livia ; Cordelli, Duccio Maria. / Sleep in Mowat-Wilson Syndrome : a clinical and video-polysomnographic study. In: Sleep Medicine. 2019.
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AU - Ricci, Emilia

AU - Ivanovski, Ivan

AU - Caraffi, Stefano Giuseppe

AU - Giordano, Lucio

AU - Accorsi, Patrizia

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AU - Raviglione, Federico

AU - Boni, Antonella

AU - Grioni, Daniele

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