Aging and sleep in Williams syndrome: Accelerated sleep deterioration and decelerated slow wave sleep decrement

• Published in: Research in developmental disabilities 2014-12, Vol.35 (12), p.3226-3235
• Main Authors: Bódizs, Róbert, Gombos, Ferenc, Gerván, Patrícia, Szőcs, Katalin, Réthelyi, János M., Kovács, Ilona
• Format: Article
• Language: English
• Subjects: 7q11.23 microdeletion; Adolescent; Adult; Aging; Aging - physiology; Biological and medical sciences; Brain development; Case-Control Studies; Child; Chromosome aberrations; Electroencephalography; Female; Humans; Male; Medical genetics; Medical sciences; Polysomnography; Sleep - physiology; Sleep EEG; Sleep Stages; Sleep Wake Disorders - complications; Sleep Wake Disorders - physiopathology; Sleep, REM - physiology; Spectral analysis; Williams Syndrome - complications; Williams Syndrome - physiopathology; Williams–Beuren syndrome; Young Adult
• ISSN: 0891-4222; 1873-3379
• DOI: 10.1016/j.ridd.2014.07.056

•Sleep impairment of subjects with WS is age-dependent and increasing with age.•Accelerated sleep deterioration supports the concept of premature aging in WS.•Compensatory developmental delay is evident from decelerated SWS decrement.•Disharmonic development of sleep is a clinically relevant aspect of WS. Specific developmental and aging trajectories characterize sleep electroencephalogram (EEG) of typically developing (TD) subjects. Williams syndrome (WS) is marked by sleep alterations and accelerated aging of several anatomo-functional and cognitive measures. Here we test the hypothesis of a premature aging of sleep in WS. Age-related changes of home recorded sleep EEG of 42 subjects (21 WS, 21 age- and gender matched TD subjects, age: 6–29 years) were tested by Pearson correlations and homogeneity-of-slopes analysis. Typical developmental/aging effects of sleep EEGs were observed in TD subjects. Accelerated aging in WS was confirmed by overall sleep/wake measures. Specifically, premature aging was evident in accelerated age-dependent declines in WS subjects’ sleep efficiency, as well as in steeper age-related rises in wakefulness and wake after sleep onset (WASO) of the WS group. In contrast, NREM sleep-related measures indicated atypical decelerations of the developmental trends of WS subjects, characterized by the slowing down of the age-related slow wave sleep (SWS) declines mirrored by the lack of age-dependent increase in Stage 2 (S2) sleep. Age-effects in sleep EEG power spectra were not different among the groups. Objectively measured sleep disruption of subjects with WS is age-dependent and increasing with age. Moreover, these data suggest atypical pre- and postpubertal neural development in WS, with sleep/wake balance and REM sleep time indicating accelerated aging while NREM sleep composition revealing signs of an as yet unidentified, perhaps compensatory developmental delay.