title: Aging and sleep in Williams syndrome: accelerated sleep deterioration and decelerated slow wave sleep decrement.
abstract: 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.
authors.affiliation: Institute of Behavioural Sciences, Semmelweis University, Nagyvárad tér 4, H-1089 Budapest, Hungary; Department of General Psychology, Pázmány Péter Catholic University, Mikszáth tér 1, H-1088 Budapest, Hungary. Electronic address: bodizs.robert@med.semmelweis-univ.hu.
authors.forename: Róbert
authors.initials: R
authors.lastname: Bódizs
authors.affiliation: Department of General Psychology, Pázmány Péter Catholic University, Mikszáth tér 1, H-1088 Budapest, Hungary. Electronic address: fgombos@freemail.hu.
authors.forename: Ferenc
authors.initials: F
authors.lastname: Gombos
authors.affiliation: Department of General Psychology, Pázmány Péter Catholic University, Mikszáth tér 1, H-1088 Budapest, Hungary. Electronic address: pgervan@gmail.com.
authors.forename: Patrícia
authors.initials: P
authors.lastname: Gerván
authors.affiliation: Department of Psychiatry and Psychotherapy, Semmelweis University, Balassa u. 6, H-1083 Budapest, Hungary.
authors.forename: Katalin
authors.initials: K
authors.lastname: Szőcs
authors.affiliation: Department of Psychiatry and Psychotherapy, Semmelweis University, Balassa u. 6, H-1083 Budapest, Hungary. Electronic address: rethelyi.janos@med.semmelweis-univ.hu.
authors.forename: János M
authors.initials: JM
authors.lastname: Réthelyi
authors.affiliation: Department of General Psychology, Pázmány Péter Catholic University, Mikszáth tér 1, H-1088 Budapest, Hungary. Electronic address: dr.ilona.kovacs@gmail.com.
authors.forename: Ilona
authors.initials: I
authors.lastname: Kovács
mesh_terms: D000293:Adolescent; D000328:Adult; D000375:Aging / Q000502:physiology*; D016022:Case-Control Studies; D002648:Child; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008297:Male; D017286:Polysomnography; D012890:Sleep / Q000502:physiology*; D012894:Sleep Stages; D012893:Sleep Wake Disorders / Q000150:complications / Q000503:physiopathology*; D012895:Sleep, REM / Q000502:physiology; D018980:Williams Syndrome / Q000150:complications / Q000503:physiopathology*; D055815:Young Adult
keywords: 7q11.23 microdeletion; Aging; Brain development; Polysomnography; Sleep EEG; Spectral analysis; Williams–Beuren syndrome