Sleep in Mowat-Wilson Syndrome: a clinical and video-polysomnographic study.
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.
['Di Pisa|Veronica|V|', 'Provini|Federica|F|', 'Ubertiello|Sara|S|', 'Bonetti|Silvia|S|', 'Ricci|Emilia|E|', 'Ivanovski|Ivan|I|', 'Caraffi|Stefano Giuseppe|SG|', 'Giordano|Lucio|L|', 'Accorsi|Patrizia|P|', 'Savasta|Salvatore|S|', 'Raviglione|Federico|F|', 'Boni|Antonella|A|', 'Grioni|Daniele|D|', 'Graziano|Claudio|C|', 'Garavelli|Livia|L|', 'Cordelli|Duccio Maria|DM|']
D000293:Adolescent D000328:Adult D000367:Age Factors D002648:Child D002675:Child, Preschool D004569:Electroencephalography D019066:Facies D005260:Female D006627:Hirschsprung Disease D006801:Humans D007223:Infant D008607:Intellectual Disability D007558:Italy D008297:Male D008831:Microcephaly D017286:Polysomnography D012890:Sleep D012893:Sleep Wake Disorders D011795:Surveys and Questionnaires D014741:Video Recording D055815:Young Adult
Mowat-wilson syndrome Sleep disturbances ESES Video-polysomnography