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Behaviours that challenge (BtC) are common in people with intellectual disability (ID) and associated with negative long-term outcomes. Reliable characterisation of BtC and behavioural function is integral to person-centred interventions. This systematic review and meta-analytic study quantitatively synthesised the evidence-base for the internal consistency, inter-rater reliability, and test-retest reliability of measures of BtC and behavioural function in people with ID (PROSPERO: CRD42021239042). Web of Science, Embase, PsycINFO and MEDLINE were searched from inception to March 2024. Retrieved records (n = 3691) were screened independently to identify studies assessing eligible measurement properties in people with ID. Data extracted from 83 studies, across 29 measures, were synthesised in a series of random-effects meta-analyses. Subgroup analyses assessed the influence of methodological quality and study-level characteristics on pooled estimates. COSMIN criteria were used to evaluate the measurement properties of each measure. Pooled estimates ranged across measures: internal consistency (0.41–0.97), inter-rater reliability (0.29–0.93) and test-retest reliability (0.52–0.98). The quantity and quality of evidence varied substantially across measures; evidence was frequently unavailable or limited to a single study. Based on current evidence, candidate measures with the most evidence for internal consistency and reliability are discussed; however, continued assessment of measurement properties in ID populations is a key priority.

Behaviours that challenge (BtC) are common in people with intellectual disability (ID) and associated with negative long-term outcomes. Reliable characterisation of BtC and behavioural function is integral to person-centred interventions. This systematic review and meta-analytic study quantitatively synthesised the evidence-base for the internal consistency, inter-rater reliability, and test-retest reliability of measures of BtC and behavioural function in people with ID (PROSPERO: CRD42021239042). Web of Science, Embase, PsycINFO and MEDLINE were searched from inception to March 2024. Retrieved records (n = 3691) were screened independently to identify studies assessing eligible measurement properties in people with ID. Data extracted from 83 studies, across 29 measures, were synthesised in a series of random-effects meta-analyses. Subgroup analyses assessed the influence of methodological quality and study-level characteristics on pooled estimates. COSMIN criteria were used to evaluate the measurement properties of each measure. Pooled estimates ranged across measures: internal consistency (0.41–0.97), inter-rater reliability (0.29–0.93) and test-retest reliability (0.52–0.98). The quantity and quality of evidence varied substantially across measures; evidence was frequently unavailable or limited to a single study. Based on current evidence, candidate measures with the most evidence for internal consistency and reliability are discussed; however, continued assessment of measurement properties in ID populations is a key priority.

Rubinstein–Taybi syndrome (RTS) is a rare genetic syndrome associated with growth delay, phenotypic facial characteristics, microcephaly, developmental delay, broad thumbs, and big toes. Most research on RTS has focused on the genotype and physical phenotype; however, several studies have described behavioral, cognitive, social, and emotional characteristics, elucidating the behavioral phenotype of RTS. The reporting of this review was informed by PRISMA guidelines. A systematic search of CINAHL, Medline, and PsychINFO was carried out in March 2021 to identify group studies describing behavioral, cognitive, emotional, psychiatric, and social characteristics in RTS. The studies were quality appraised. Characteristics reported include repetitive behavior, behaviors that challenge, intellectual disability, mental health difficulties, autism characteristics, and heightened sociability. Findings were largely consistent across studies, indicating that many characteristics are likely to form part of the behavioral phenotype of RTS. However, methodological limitations, such as a lack of appropriate comparison groups and inconsistency in measurement weaken these conclusions. There is a need for multi-disciplinary studies, combining genetic and psychological measurement expertise within single research studies. Recommendations are made for future research studies in RTS.

Background There is a need for evidence-based approaches to reduce anxiety experienced by autistic children with severe to profound intellectual disability (ID). Avoidance of anxiety triggers, as a response to pronounced anxiety, occurs irrespective of age, background and neurodiversity. When avoidance is unhelpful, evidence-based anxiety reduction approaches aim to reduce it gradually (graded exposure), subsequently reducing anxiety. Combining graded exposure with emotional regulation techniques may be effective and acceptable for autistic children with severe to profound ID, if sensitive to needs and characteristics of autistic children. We have developed a 16-week, parent-led intervention (LADDERS) to reduce anxiety in this population of autistic children. LADDERS consists of psychoeducation, graded exposure-based tasks, and skills building, delivered utilising a person-centred approach. This study aims to assess whether LADDERS 1) reduces anxiety and 2) whether autistic children and parents find it acceptable and feasible. Method A single-site, multiple baseline, single case experimental study will be conducted. Participants will be parents of autistic children aged between 4-15 years. A minimum of 8 participants will be recruited through a research participant database, the Autistica Discover Network and social media. Once eligibility is confirmed, participants will be assessed at baseline, during the intervention and at a 2-month follow-up (week 24). The primary outcome measure will be a daily diary that assesses child anxiety. Discussion The study will provide preliminary evidence of whether LADDERS reduces anxiety in autistic children with severe to profound ID. Qualitative data from parents and child engagement will provide data on acceptability and feasibility.

Background There is a need for evidence-based approaches to reduce anxiety experienced by autistic children with severe to profound intellectual disability (ID). Avoidance of anxiety triggers, as a response to pronounced anxiety, occurs irrespective of age, background and neurodiversity. When avoidance is unhelpful, evidence-based anxiety reduction approaches aim to reduce it gradually (graded exposure), subsequently reducing anxiety. Combining graded exposure with emotional regulation techniques may be effective and acceptable for autistic children with severe to profound ID, if sensitive to needs and characteristics of autistic children. We have developed a 16-week, parent-led intervention (LADDERS) to reduce anxiety in this population of autistic children. LADDERS consists of psychoeducation, graded exposure-based tasks, and skills building, delivered utilising a person-centred approach. This study aims to assess whether LADDERS 1) reduces anxiety and 2) whether autistic children and parents find it acceptable and feasible. Method A single-site, multiple baseline, single case experimental study will be conducted. Participants will be parents of autistic children aged between 4-15 years. A minimum of 8 participants will be recruited through a research participant database, the Autistica Discover Network and social media. Once eligibility is confirmed, participants will be assessed at baseline, during the intervention and at a 2-month follow-up (week 24). The primary outcome measure will be a daily diary that assesses child anxiety. Discussion The study will provide preliminary evidence of whether LADDERS reduces anxiety in autistic children with severe to profound ID. Qualitative data from parents and child engagement will provide data on acceptability and feasibility.

Rubinstein–Taybi syndrome (RTS) is a rare genetic syndrome associated with growth delay, phenotypic facial characteristics, microcephaly, developmental delay, broad thumbs, and big toes. Most research on RTS has focused on the genotype and physical phenotype; however, several studies have described behavioral, cognitive, social, and emotional characteristics, elucidating the behavioral phenotype of RTS. The reporting of this review was informed by PRISMA guidelines. A systematic search of CINAHL, Medline, and PsychINFO was carried out in March 2021 to identify group studies describing behavioral, cognitive, emotional, psychiatric, and social characteristics in RTS. The studies were quality appraised. Characteristics reported include repetitive behavior, behaviors that challenge, intellectual disability, mental health difficulties, autism characteristics, and heightened sociability. Findings were largely consistent across studies, indicating that many characteristics are likely to form part of the behavioral phenotype of RTS. However, methodological limitations, such as a lack of appropriate comparison groups and inconsistency in measurement weaken these conclusions. There is a need for multi-disciplinary studies, combining genetic and psychological measurement expertise within single research studies. Recommendations are made for future research studies in RTS.