Impaired Sleep-Related Learning in Children with Williams Syndrome (original) (raw)
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A Cross-Syndrome Comparison of Sleep-Dependent Learning on a Cognitive Procedural Task
American Journal on Intellectual and Developmental Disabilities
Sleep plays a key role in the consolidation of newly acquired information and skills into long term memory. Children with Down syndrome (DS) and Williams syndrome (WS) frequently experience sleep problems, abnormal sleep architecture, and difficulties with learning; thus, we predicted that children from these clinical populations would demonstrate impairments in sleep-dependent memory consolidation relative to children with typical development (TD) on a cognitive procedural task: The Tower of Hanoi. Children with DS (n = 17), WS (n = 22) and TD (n = 34) completed the Tower of Hanoi task. They were trained on the task either in the morning or evening, then completed it again following counterbalanced retention intervals of daytime wake and night time sleep. Children with TD and with WS benefitted from sleep for enhanced memory consolidation and improved their performance on the task by reducing the number of moves taken to completion, and by making fewer rule violations. We did not f...
Sleep plays an active role in memory consolidation. Because children with Down syndrome (DS) and Williams syndrome (WS) experience significant problems with sleep and also with learning, we predicted that sleep-dependent memory consolidation would be impaired in these children when compared to typically developing (TD) children. This is the first study to provide a cross-syndrome comparison of sleep-dependent learning in school-aged children. Children with DS (n = 20) and WS (n = 22) and TD children (n = 33) were trained on the novel Animal Names task where they were taught pseudo-words as the personal names of ten farm and domestic animals, e.g. Basco the cat, with the aid of animal picture flashcards. They were retested following counterbalanced retention intervals of wake and sleep. Overall, TD children remembered significantly more words than both the DS and WS groups. In addition, their performance improved following night-time sleep, whereas performance over the wake retention interval remained stable, indicating an active role of sleep for memory consolidation. Task performance of children with DS did not significantly change following wake or sleep periods. However, children with DS who were initially trained in the morning continued to improve on the task at the following retests, so that performance on the final test was greater for children who had initially trained in the morning than those who trained in the evening. Children with WS improved on the task between training and the first retest, regardless of whether sleep or wake occurred during the retention interval. This suggests time-dependent rather than sleep-dependent learning in children with WS, or tiredness at the end of the first session and better performance once refreshed at the start of the second session, irrespective of the time of day. Contrary to expectations, sleep-dependent learning was not related to baseline level of performance. The findings have significant implications for educational strategies, and suggest that children with DS should be taught more important or difficult information in the morning when they are better able to learn, whilst children with WS should be allowed a time delay between learning phases to allow for time-dependent memory consolidation, and frequent breaks from learning so that they are refreshed and able to perform at their best.
Research in Developmental Disabilities, 2020
Children with neurodevelopmental disorders commonly experience sleep problems. Williams Syndrome (WS), a rare genetic disorder characterised by a complex, uneven cognitive profile, is no exception. Compared with children with typical development (TD), school-aged children with WS experience significant sleep disruption: shorter sleep duration, more night wakings, greater bedtime resistance and excessive daytime tiredness. In children with TD, sleep problems impede optimal daytime functioning. In WS, this could compound existing difficulties. Few studies have examined sleep in very young children with WS and little is known about the early emergence of sleep problems in this population. To date, studies have been based on parent-report and no studies have objectively assessed sleep patterns using longitudinal approach in toddlers with WS. Thus, the current study sought to objectively explore sleep patterns in toddlers with WS. Parents of 38 children (13 WS, 25 TD) completed the Brief Infant Screening Questionnaire and the Medical and Demographics Questionnaire and sleep patterns were assessed using actigraphy. Data were collected longitudinally at ages 18, 24 and 30 months. Significant sleep disturbances were present in WS from 18 months old. Sleep duration, as measured by actigraphy, was significantly shorter in WS at all ages and, furthermore, parents of children with WS reported more night wakings, longer settling times and high levels of parental involvement. Crucially, whereas actigraphy showed developmental improvements in sleep quality in TD, no longitudinal changes were found in WS. Findings could be instrumental in working towards instigating appropriate, timely sleep management in this group, thus improving outcomes for children and their families. Dear Prof Heaton, On behalf of all authors, I would like to submit the paper "Early manifestation of sleep problems in toddlers with Williams Syndrome using a mixed method longitudinal approach" for the special issue in memory of Annette Karmiloff-Smith. Kind Regards, Dagmara Dimitriou Cover Letter Longitudinal and objective sleep patterns in young children with Williams Syndrome Sleep problems in Williams Syndrome emerge and manifest in early childhood Shorter sleep durations in Williams Syndrome; based on objective measures Different developmental sleep trajectory in toddlers with Williams Syndrome Limited sleep support provided for parents who consider sleep problematic *Highlights (for review) *Manuscript Click here to view linked References include aspects of language, social interaction and good facial recognition skills, in conjunction with clear difficulties with visuospatial skills and, in most incidences, mild to moderate ID (Martens, Wilson, & Reutens, 2008; Pober, 2010). Consistent with other neurodevelopmental disorders, studies exploring sleep in individuals with WS indicate that sleep difficulties are highly prevalent. Annaz, Hill, Ashworth, Holley, and Karmiloff-Smith (2011) found that, based on parent-report, 97% of school-aged children experienced sleep problems; these included more night waking, bedtime resistance, restless sleep and insufficient sleep compared with children with TD. Subsequent studies report similar findings, supporting the notion that children with WS experience disturbed sleep. Based on actigraphy and parentreport, Ashworth and colleagues found that children with WS experienced long sleep latencies, short sleep durations and frequent night waking (Ashworth, Hill, Karmiloff-Smith, & Dimitriou, 2013). Polysomnography studies show that children with WS spend more time awake after sleep onset than children with TD (Arens et al., 1998); in addition, individuals with WS experience reduced sleep efficiency and increased slow wave sleep and sleep disruption (Gombos, Bodizs, & Kovacs, 2011; Mason et al., 2011). It has been proposed that sleep disruptions, such as delayed sleep onset and disturbed sleep cycles, may be related to abnormal levels of sleep-related hormones, melatonin and cortisol, which help regulate sleep-wake cycles (Santoro,
Behavioral Sleep Medicine, 2014
In typically developing (TD) children, sleep problems have been associated with day-time attentional difficulties. Children with developmental disabilities often suffer with sleep and attention problems, yet their relationship is poorly understood. The present study investigated this association in school-aged children with Down syndrome (DS) and Williams syndrome (WS). Actigraphy and pulse oximetry assessed sleep and sleep-disordered breathing respectively, and attention was tested 20 using a novel visual Continuous Performance Task (CPT).Attentional deficits were evident in both disorder groups. In the TD group, higher scores on the CPT were related to better sleep quality, higher oxyhemoglobin saturation (SpO 2 ), and fewer desaturation events. Sleep quality, duration, and SpO 2 variables were not related to CPT performance for children with DS and WS.
Sleep in children with Williams Syndrome
Sleep Medicine, 2011
ObjectiveTo analyze sleep in children with Williams Syndrome (WS) compared to normal healthy controls in order to determine whether particular sleep features are characteristic of WS, and to explore associations between disturbed sleep and behavior.
Background Williams syndrome ( WS) is a neurodevelopmental genetic disorder characterised by physical abnormalities and a distinctive cognitive profile with intellectual disabilities (IDs) and learning difficulties. Methods In our study, nine adolescents and young adults with WS and 9 age-and sex-matched typically developing (TD) participants underwent polysomnography. We examined sleep architecture, leg movements and the electroencephalogram (EEG) spectra of specific frequency bands at different scalp locations. Results We found an atypical, WS characteristic sleep pattern with decreased sleep time, decreased sleep efficiency, increased wake time after sleep onset, increased non-rapid eye movement percentage, increased slow wave sleep, decreased rapid eye movement sleep percentage, increased number of leg movements and irregular sleep cycles. Patients with WS showed an increased delta and slow wave activity and decreased alpha and sigma activity in the spectral analysis of the EEG.
Atypical sleep architecture and altered EEG spectra in Williams syndrome
Journal of Intellectual Disability Research, 2010
Background Williams syndrome (WS) is a neurodevelopmental genetic disorder characterised by physical abnormalities and a distinctive cognitive profile with intellectual disabilities (IDs) and learning difficulties. Methods In our study, nine adolescents and young adults with WS and 9 age-and sex-matched typically developing (TD) participants underwent polysomnography. We examined sleep architecture, leg movements and the electroencephalogram (EEG) spectra of specific frequency bands at different scalp locations. Results We found an atypical, WS characteristic sleep pattern with decreased sleep time, decreased sleep efficiency, increased wake time after sleep onset, increased non-rapid eye movement percentage, increased slow wave sleep, decreased rapid eye movement sleep percentage, increased number of leg movements and irregular sleep cycles. Patients with WS showed an increased delta and slow wave activity and decreased alpha and sigma activity in the spectral analysis of the EEG. Conclusions Sleep maintenance and organisation are significantly affected in WS, while EEG spectra suggest increases in sleep pressure.