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

Federica Provini, Veronica Di Pisa, 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
Pages (from-to)44-51
Number of pages8
JournalSleep Medicine
Volume61
DOIs
Publication statusPublished - Sep 2019

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    Provini, F., Di Pisa, V., Ubertiello, S., Bonetti, S., Ricci, E., Ivanovski, I., Caraffi, S. G., Giordano, L., Accorsi, P., Savasta, S., Raviglione, F., Boni, A., Grioni, D., Graziano, C., Garavelli, L., & Cordelli, D. M. (2019). Sleep in Mowat-Wilson Syndrome: a clinical and video-polysomnographic study. Sleep Medicine, 61, 44-51. https://doi.org/10.1016/j.sleep.2019.04.011