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Behavior and sleep disturbance in Smith–Magenis syndrome

Shayota, Brian J.a,b; Elsea, Sarah H.a

Current Opinion in Psychiatry: March 2019 - Volume 32 - Issue 2 - p 73–78
doi: 10.1097/YCO.0000000000000474
NEURODEVELOPMENTAL DISORDERS: Edited by James C. Harris
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Purpose of review To provide an update of the most recent studies on Smith–Magenis syndrome (SMS) with a focus on the unique pattern of behavioral and sleep disturbances associated with the condition.

Recent findings The recent literature on SMS has focused on the characteristic severe behavioral and sleep disturbances. A better understanding of the underlying pathophysiological mechanisms and common clinical course has helped further characterize SMS, while much is left to be discovered in regard to effective treatment/management.

Summary SMS is a difficult to manage genetic condition defined by pervasive and progressive behavioral and sleep disturbances with a unique pattern that can often be easily discerned from other neurodevelopmental disorders. Common behavioral features include maladaptive/self-injurious, aggressive, stereotypic, and the newly appreciated food seeking behaviors associated with SMS. In addition, there is a sleep disturbance defined by an altered circadian rhythm with frequent nighttime waking and daytime sleepiness, causing patients and families significant distress. Small studies have suggested some treatment/management approaches to the behavioral and sleep disturbances, however, much remains to be discovered.

aDepartment of Molecular and Human Genetics, Baylor College of Medicine

bTexas Children's Hospital, Houston, Texas, USA

Correspondence to Sarah H. Elsea, PhD, FACMG, Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, BCM225, Houston, TX 77030, USA. Tel: +1 713 798 5484; e-mail: elsea@bcm.edu

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