Publications by authors named "Rachel Royston"

7 Publications

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Prevalence of anxiety symptomatology and diagnosis in syndromic intellectual disability: A systematic review and meta-analysis.

Neurosci Biobehav Rev 2022 07 2;138:104719. Epub 2022 Jun 2.

The School of Psychology, College of Health and Life Sciences, Aston University, UK. Electronic address:

Individuals with syndromic intellectual disability are at increased risk of experiencing anxiety. Comparing prevalence estimates of anxiety will allow the identification of at-risk groups and inform causal pathways of anxiety. No known study has explored estimates of anxiety symptomatology and diagnosis, including specific anxiety profiles, across groups whilst accounting for methodological quality of studies. This systematic review and meta-analysis aimed to fill this gap. Prior to review completion, methodology and analysis plans were registered and documented in a protocol (CRD42019123561). Data from 83 papers, involving a pooled sample of 13,708 across eight syndromes were synthesised using a random effects model. Anxiety prevalence ranged from 9 % (95 % CI: 4-14) in Down syndrome to 73% in Rett syndrome (95 % CI: 70-77). Anxiety prevalence across syndromic intellectual disability was higher than for intellectual disability of mixed aetiology and general population estimates. Substantial variability between syndromes identified groups at higher risk than others. The identification of high-risk groups is crucial for early intervention, allowing us to refine models of risk and identify divergent profiles.
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July 2022

Anxiety characteristics in individuals with Williams syndrome.

J Appl Res Intellect Disabil 2021 Jul 9;34(4):1098-1107. Epub 2021 Feb 9.

University of Birmingham, Birmingham, UK.

Background: Williams syndrome anxiety research predominantly focuses on disorder prevalence and symptomatology, categorised using standardised mental health classifications. However, the use of these assessments may not fully capture the phenotypic features of anxiety in Williams syndrome. In this study, we examined characteristics of anxiety using a formulation framework.

Method: A semi-structured interview was conducted with thirteen parents of individuals with Williams syndrome (median age: 19, age range: 12-45, 8 females).

Results: Various anxiety triggers were reported, including anxiety triggered by phobias, uncertainty and negative emotions in others. The range of described behaviours was diverse with both avoidant and active coping strategies for anxiety management reported.

Conclusions: Many of the characteristics described were consistent with findings in the intellectual disability and typically developing literature, although novel information was identified. The study demonstrates the utility of a formulation framework to explore anxiety characteristics in atypical populations and has outlined new avenues for research.
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July 2021

Clinical and cost effectiveness of a parent mediated intervention to reduce challenging behaviour in pre-schoolers with moderate to severe intellectual disability (EPICC-ID) study protocol: a multi-centre, parallel-group randomised controlled trial.

BMC Psychiatry 2020 01 30;20(1):35. Epub 2020 Jan 30.

Division of Psychiatry, University College London, 6th Floor Maple House, 149 Tottenham Court Road, London, W1T 7NF, UK.

Background: Children with intellectual disabilities are likely to present with challenging behaviour. Parent mediated interventions have shown utility in influencing child behaviour, although there is a paucity of UK research into challenging behaviour interventions in this population. NICE guidelines favour Stepping Stones Triple P (SSTP) as a challenging behaviour intervention and this trial aims to evaluate its clinical and cost effectiveness in preschool children with moderate to severe intellectual disabilities.

Methods: This trial launched in 2017 at four sites across England, with the aim of recruiting 258 participants (aged 30-59 months). The Intervention Group receive nine weeks of SSTP parenting therapy (six group sessions and three individualised face to face or telephone sessions) in addition to Treatment as Usual, whilst the Treatment as Usual only group receive other available services in each location. Both study groups undergo the study measurements at baseline and at four and twelve months. Outcome measures include parent reports and structured observations of behaviour. Service use and health related quality of life data will also be collected to carry out a cost effectiveness and utility evaluation.

Discussion: Findings from this study will inform policy regarding interventions for challenging behaviour in young children with moderate to severe intellectual disabilities.

Trial Registration Number:, NCT03086876. Registered 22nd March 2017,
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January 2020

Communication in Angelman syndrome: a scoping review.

Dev Med Child Neurol 2019 11 10;61(11):1266-1274. Epub 2019 May 10.

Cerebra Centre for Neurodevelopmental Disorders, School of Psychology, University of Birmingham, Birmingham, UK.

Aim: A scoping review was conducted to examine and evaluate empirical data on the communication profile of Angelman syndrome beyond the described dissociation between receptive language and speech.

Method: Three databases (PsycINFO, Embase, and Web of Science) were searched to retrieve articles investigating communication in Angelman syndrome. Seventeen articles investigating the broader communication profile were found; their methodology was evaluated against quality criteria.

Results: Despite the absence of speech, individuals with Angelman syndrome have a wide repertoire of non-verbal communicative behaviours, mainly characterized by gestures, although advanced forms such as symbolic communication are used by some individuals. The use of communicative forms differs between the genetic aetiologies of Angelman syndrome; individuals with non-deletion aetiologies typically have greater communicative abilities.

Interpretation: The broader communication profile of Angelman syndrome is characterized by diverse and multimodal abilities, including some use of symbolic forms of communication that appears atypical given the absence of speech. This is suggestive of a probable dissociation between speech and other expressive forms of communication, indicating an isolated speech production impairment. This highlights a need in this population for alternative communication and specific input from services tailored to support the nuances of the communication profile of Angelman syndrome.

What This Paper Adds: Although absent speech is near universal, a diverse profile of other communicative abilities has been reported. Parental reporting has been predominantly used to assess the communication profile of Angelman syndrome. Literature that investigates the specificities and possible dissociations in such a communication profile is limited.
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November 2019

Williams syndrome: recent advances in our understanding of cognitive, social and psychological functioning.

Curr Opin Psychiatry 2019 03;32(2):60-66

Institute of Psychiatry, Psychology and Neuroscience, King's College London, London, UK.

Purpose Of Review: Since the last review of Williams syndrome in Current Opinion (2001) there have been many advances in knowledge about the cognitive, social and psychological impairments that characterize the disorder. The present review focuses on current research in these areas.

Recent Findings: Williams syndrome is associated with a wide range of cognitive, linguistic, social and other difficulties. When young, these deficits may appear relatively mild - for example, many children are highly sociable and talkative - but with age the impact of these difficulties becomes more evident. Thus, inappropriate social behaviours can significantly increase the risk of social exclusion and vulnerability to abuse. Their superficially good speech can lead to educational and other services failing to understand the true extent of impairments or the need for specialist support. Mental health problems, especially related to anxiety, often become an increasing challenge from adolescence onwards.

Summary: The core difficulties associated with Williams syndrome have a cascading effect on many areas of development over time. However, specialist provision is rare and intervention trials are almost nonexistent. Longitudinal research is needed to identify factors associated with cognitive, social and emotional problems and to develop more effective ways of minimizing and treating difficulties.
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March 2019

A Multifaceted Quality Improvement Programme to Improve Acute Kidney Injury Care and Outcomes in a Large Teaching Hospital.

BMJ Qual Improv Rep 2017 25;6(1). Epub 2017 May 25.

Central Manchester University Hospitals NHS Foundation Trust.

Acute kidney injury (AKI) is now widely recognised as a serious health care issue, occurring in up to 25% of hospital in-patients, often with worsening of outcomes. There have been several reports of substandard care in AKI. This quality improvement (QI) programme aimed to improve AKI care and outcomes in a large teaching hospital. Areas of documented poor AKI care were identified and specific improvement activities implemented through sequential Plan-Do-Study-Act (PDSA) cycles. An electronic alert system (e-alert) for AKI was developed, a Priority Care Checklist (PCC) was tested with the aid of specialist nurses whilst targeted education activities were carried out and data on care processes and outcomes monitored. The e-alert had a sensitivity of 99% for the detection of new cases of AKI. Key aspects of the PCC saw significant improvements in their attainment: Detection of AKI within 24 hours from 53% to 100%, fluid assessment from 42% to 90%, drug review 48% to 95% and adherence to nine key aspects of care from 40% to 90%. There was a significant reduction in variability of delivered AKI care. AKI incidence reduced from 9% of all hospitalisations at baseline to 6.5% (28% reduction), AKI related length of stay reduced from 22.1 days to 17 days (23% reduction) and time to recovery (AKI days) 15.5 to 9.8 days (36% reduction). AKI related deaths also showed a trend towards reduction, from an average of 38 deaths to 34 (10.5%). The number of cases of hospital acquired AKI were reduced by 28% from 120 to 86 per month. This study demonstrates significant improvements related to a QI programme combining e-alerts, a checklist implemented by a nurse and education in improving key processes of care. This resulted in sustained improvement in key patient outcomes.
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May 2017

Palliative care for patients with mesothelioma.

Br J Hosp Med (Lond) 2017 Apr;78(4):219-225

Consultant, Palliative Care Department, North Bristol NHS Trust, Southmead Hospital, Bristol.

Mesothelioma is a highly symptomatic aggressive cancer affecting quantity and quality of life. Excellent palliative care is essential from diagnosis. This article discusses commonly encountered issues and management strategies including the rationale for early referral to specialist palliative care.
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April 2017