Behavior and sleep disturbance in Smith–Magenis syndrome. Issue 2 (March 2019)

• Record Type: Journal Article
• Authors: Shayota, Brian J.; Elsea, Sarah H.
• Is Part Of: Current opinion in psychiatry. Volume 32:Issue 2(2019:Mar.)
• Publication Date: 2019-03
• Subjects: 17p11.2 deletion -- retinoic acid induced 1 -- self-injurious behaviors -- sleep disturbance -- Smith–Magenis syndrome; Psychiatry -- Periodicals; 616.89005
• DOI: 10.1097/YCO.0000000000000474
• Languages: English
• ISSNs: 0951-7367

Abstract

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.