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Review
. 2019 Mar;32(2):73-78.
doi: 10.1097/YCO.0000000000000474.

Behavior and sleep disturbance in Smith-Magenis syndrome

Brian J Shayota  1   2 , Sarah H Elsea  1
Affiliations
Review

Behavior and sleep disturbance in Smith-Magenis syndrome

Brian J Shayota et al. Curr Opin Psychiatry. 2019 Mar.

Abstract

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.

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Conflict of interest statement

Conflicts of interest

Dr. Elsea serves as Chair of the Professional Advisory Board for PRISMS, Inc., Parents and Researchers Interested in Smith-Magenis Syndrome and receives research funding from the Smith-Magenis Syndrome Research Foundation. There are no other conflicts of interest.

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