Polysomnography Findings in
Children with Williams Syndrome
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1
The Children's Hospital of Philadelphia, United States
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2
Montefiore Medical Center, United States
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3
University of Pennsylvania, United States
Purpose: Parents of children with Williams Syndrome (WS) often report that their children have sleep difficulties. A prior study of 7 WS subjects at the Children’s Hospital of Philadelphia (CHOP) supported an association between WS and periodic limb movements in sleep, as well as increased wake time, less time in sleep stages 1 and 2, and more time in sleep stages 3 and 4 than control subjects (Arens et al. 1998). We wanted to expand the analysis of sleep in children with WS with a larger sample size, unselected for a history of sleep disturbance, to determine whether particular sleep features may be characteristic of WS.
Methods: Eligible subjects were males and females ages 2-18 years who met clinical criteria for WS, and who had haploinsufficiency for the elastin gene as determined by fluorescent in situ hybridization (FISH). WS patients were recruited from the CHOP Multispecialty Center for Williams Syndrome. An equal number of healthy control subjects without sleep problems (matched for gender, age, race, and ethnicity) were enrolled. The test series included overnight polysomnography as well as parentcompleted questionnaires.
Results: 35 WS subjects and 35 matched control children were studied. In each group, there were 15 males and 20 females; the majority (89%) was white. The median age was 8.4 years. WS subjects had significantly decreased sleep efficiency compared to control children (paired t = -2.67, df = 34, p = 0.01); the mean difference was 4.5% (9.9%). The standardized effect size was -0.45. Children with WS also significantly increased slow wave sleep (non-REM stage 3 and 4 sleep), as a percentage of total sleep time, compared to control children (paired t=-2.67, df=34, p=0.01); the mean difference was 4.6% (10%). The standardized effect size was 0.45. No statistically significant differences were seen in REM sleep latency, REM sleep as a percentage of total sleep time, the obstructive apnea/hypopnea index, or the periodic limb movement index.
Discussion: In this study, children with WS have altered sleep architecture compared to matched controls, with significantly lower sleep efficiency and increased slow wave sleep; for both of these parameters, effect sizes approach the moderate range. It is intriguing to consider that these differences in children with WS may reflect genetic influences on sleep, and may in turn be associated with daytime behavioral features.
Conference:
12th International Professional Conference on Williams Syndrome, Garden Grove,CA, United States, 13 Jul - 14 Jul, 2008.
Presentation Type:
Poster Presentation
Topic:
Multidisciplinary Poster Session
Citation:
Mason
T,
Arens
R,
Sharman
J,
Pack
A and
Kaplan
P
(2009). Polysomnography Findings in
Children with Williams Syndrome.
Conference Abstract:
12th International Professional Conference on Williams Syndrome.
doi: 10.3389/conf.neuro.09.2009.07.052
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Received:
04 May 2009;
Published Online:
04 May 2009.
*
Correspondence:
T. Mason, The Children's Hospital of Philadelphia, Philadelphia, United States, masont@email.chop.edu