Psychological aspects of being a parent of an individual with Rett syndrome: A scoping review.

BACKGROUND: Rett syndrome (RTT) causes multiple disabilities with a lifelong need for substantial care, placing a tremendous lifelong responsibility on the parents. Parenting an individual with RTT can therefore be challenging. Research on the psychological aspects of parenting individuals with RTT is limited and unclear. We aimed to identify and map the existing literature on this subject. METHOD: A scoping review was conducted with systematic searches in PubMed, PsycINFO and CINAHL. RESULTS: Eighteen studies were included. Negative and positive psychological aspects were described with the majority focusing on the negative. Three factors seemed to especially affect the parents: severity of the diagnosis, time (increasing age of parents or individual with RTT; years of caretaking), work-status of the mother. CONCLUSIONS: Seemingly, parents are highly affected; however, the literature is scarce and has several gaps. Future research should include older parents, fathers, parents of individuals living in group homes, and positive aspects.

Validation of an accelerometer system for measuring physical activity and sedentary behavior in healthy children and adolescents.

The study aims to assess the concurrent validity of the SENS motion® accelerometer system for device-based measurement of physical activity and sedentary behavior in healthy children and adolescents. Thirty-six healthy children and adolescents (mean ± standard deviation (SD) age, 10.2 ± 2.3 years) were fitted with three SENS sensors while performing standardized activities including walking, fast walking, sitting/lying, and arm movements. Data from the sensors were compared with video observations (reference criteria). The agreement between SENS motion® and observation was analyzed using Student's t-test and illustrated in Bland-Altman plots. The concurrent validity was further evaluated using intraclass correlation coefficient (ICC) and was expressed as standard error of measurement (SEM) and minimal detectable change (MDC). Strong agreement was found between SENS and observation for walking time, sedentary time, and lying time. In contrast, moderate agreement was observed for number of steps, sitting time, and time with and without arm movement. ICC2.1 values were overall moderate to excellent (0.5-0.94), with correspondingly low SEM% for walking time, sedentary time, lying time, and time with arm movement (2-9%). An acceptable SEM% level was reached for both steps and sitting time (11% and 12%). For fast walking time, the results showed a weak agreement between the measurement methods, and the ICC value was poor. CONCLUSION: SENS motion® seems valid for detecting physical activity and sedentary behavior in healthy children and adolescents with strong agreement and moderate to excellent ICC values. Furthermore, the explorative results on arm movements seem promising. WHAT IS KNOWN: • Inactivity and sedentary behavior follow an increasing trend among children and adolescents. • SENS motion® seems to be valid for measuring physical activity and sedentary behavior in adults and elderly patients. WHAT IS NEW: • SENS motion® seems valid with strong agreement between video observations and SENS measurement, and ICC values are moderate to excellent when measuring physical activity and sedentary behavior in healthy children and adolescents. • SENS motion® seems promising for detection of arm movements.

Evaluation Tools Developed for Rett Syndrome.

Rett syndrome (RTT) is a complex neurodevelopmental X-linked disorder associated with severe functional impairments and multiple comorbidities. There is wide variation in the clinical presentation, and because of its unique characteristics, several evaluation tools of clinical severity, behavior, and functional motor abilities have been proposed specifically for it. This opinion paper aims to present up-to date evaluation tools which have specifically been adapted for individuals with RTT often used by the authors in their clinical and research practice and to provide the reader with essential considerations and suggestions regarding their use. Due to the rarity of Rett syndrome, we found it important to present these scales in order to improve and professionalize their clinical work. The current article will review the following evaluation tools: (a) the Rett Assessment Rating Scale; (b) the Rett Syndrome Gross Motor Scale; (c) the Rett Syndrome Functional Scale; (d) the Functional Mobility Scale-Rett Syndrome; (e) the Two-Minute Walking Test modified for Rett syndrome; (f) the Rett Syndrome Hand Function Scale; (g) the StepWatch Activity Monitor; (h) the activPALTM; (i) the Modified Bouchard Activity Record; (j) the Rett Syndrome Behavioral Questionnaire; and (k) the Rett Syndrome Fear of Movement Scale. The authors recommend that service providers consider evaluation tools validated for RTT for evaluation and monitoring to guide their clinical recommendations and management. In this article, the authors suggest factors that should be considered when using these evaluation tools to assist in interpreting scores.

Rett Syndrome Behaviour Questionnaire in Children and Adults With Rett Syndrome: Psychometric Characterization and Revised Factor Structure.

Rett syndrome (RTT) is a severe neurodevelopmental disorder associated with multiple neurobehavioral abnormalities. The Rett Syndrome Behaviour Questionnaire (RSBQ) was developed for pediatric RTT observational studies. Because its application has expanded to adult and interventional studies, we evaluated the RSBQ's psychometric properties in six pediatric (n = 323) and five adult (n = 309) datasets. Total and General Mood subscale scores had good reliability. Clinical severity had no influence on RSBQ scores. Exploratory and confirmatory factor analyses yielded 6 pediatric and 7 adult clinically relevant and psychometrically strong factors including the original Breathing Problems and Fear/Anxiety subscales and the novel Emotional and Disruptive Behavior subscale composed of items from the original General Mood and Nighttime Behaviours subscales. The present findings support additional evaluations and improvements of an important RTT behavioral measure.

Can telehealth increase physical activity in individuals with Rett syndrome? A multicentre randomized controlled trial.

AIM: To evaluate the effects of a physical activity programme on sedentary behaviour and physical activity in ambulant individuals with Rett syndrome (RTT). METHOD: In this multicentre randomized waitlist-controlled trial, we recruited 43 ambulatory individuals with RTT in Australia and Denmark. Adequate baseline data were obtained from 38 participants (mean age 20 years, range 6-41, SD 10 years 6 months, one male). All completed the trial. Participants received 12 weeks of usual care (n = 19) or a goal-based, telehealth-supported programme in which activities occurred in their familiar environments (n = 19). Sedentary time and daily steps were assessed at baseline, post-test, and 12-week follow-up. The data analyst was blinded to group allocation. RESULTS: Sedentary time decreased in the intervention group by 2.7% (95% confidence interval [CI] -6.0 to 0.6) and increased in the control group by 1.3% (95% CI -4.8 to 7.4). Intervention and control groups increased the number of their steps per day by 264.7 (95% CI -72.2 to 601.5) and 104.8 (95% CI -178.1 to 387.7) respectively. No significant differences were found on any outcomes at post-test. There were three minor adverse events. INTERPRETATION: A goal-based telehealth intervention seemed to produce small improvements in physical activity for individuals with RTT. Families require more support to increase these individuals' extremely low physical activity levels. WHAT THIS PAPER ADDS: A telehealth-supported intervention may produce small changes in physical activity in Rett syndrome (RTT). Increasing physical activity in individuals with RTT is challenging for caregivers. Families require substantial out-of-home support to increase their children's activity levels.

Parent and therapist perspectives on "uptime" activities and participation in Rett syndrome.

PURPOSE: People with a disability may spend more time sitting and lying ("downtime") and less time standing and walking ("uptime"). Caregivers and therapists supporting individuals with Rett syndrome were surveyed, aiming to gather insights on how to support participation in "uptime" activities. METHOD: An anonymous online survey including open ended questions about the enablers and barriers to "uptime" was administered to parent/caregivers and therapists/health professionals in an international sample. Responses were coded to the International Classification of Functioning, Health and Disability (ICF) framework identifying barriers, enablers, and strategies for increasing uptime activities. RESULTS: Parents (N = 115) and therapists (N = 49) completed the survey. Barriers and enablers to "uptime" were identified for all ICF domains and additional data coded to enabling access to the physical environment. Strategies to promote "uptime" activities and participation particularly related to the individual's physical capacity and personal factors as well as social and physical environmental factors. CONCLUSIONS: Findings can inform the design of interventions aiming to increase "uptime" in individuals with Rett syndrome. Strategies should create individualised support by considering how to build fitness using activities that are motivating, at the same time creating opportunities for social interactions within a range of environments.IMPLICATIONS FOR REHABILITATION"Uptime" participation comprised a dynamic interaction of "doing" the standing or walking activity, with a sense of self-engagement with the activities and interaction with others.Strategies to promote "uptime" participation should consider how to create support for person-related attributes, including building physical capacity for a greater volume of "uptime" in activities that are enjoyable and motivating.Strategies to promote "uptime participation should also include creating a supportive environment, comprising opportunities for social interaction within a range of environments".

Participation and engagement in family activities among girls and young women with Rett syndrome living at home with their parents - a cross-sectional study.

PURPOSE: To describe the extent of participation and engagement in family activities and explore variables potentially impacting on these factors in family activities among girls and young women with Rett syndrome (RTT) under the age of 21. MATERIALS AND METHODS: The Child Participation in Family Activities (Child-PFA) questionnaire was sent to parents in the target group (n = 42). Additionally, age, number of siblings at home, ambulation level, clinical severity and level of hand function were recorded to explore possible impact. Data were analyzed using descriptive statistics, Fishers exact test and cross-tables. RESULTS: 23 families participated. Highest degrees of participation and engagement were seen in social and stationary family activities. Indoor activities were frequent and showed high levels of participation and engagement, Outdoor activities were infrequent and showed low levels of participation despite a high degree of engagement. Routine activities were frequent but showed moderate to low participation and engagement. A negative association was found between participation in watching a movie and number of siblings living at home, and positive associations between engagement and age in three family activities. CONCLUSION: Therapists working with this target group may benefit from focusing on engagement in routine activities and modification of family activities.IMPLICATIONS FOR REHABILITATIONTherapists may benefit from focusing on engagement in routine activities in the goal setting process and intervention as they occur on a daily basis, giving the opportunity for development of new skills.Therapists may benefit from focusing on assistive devices or other compensatory strategies for outdoor activities and activities that require a certain amount of hand function.Therapists may benefit from modifying the family's activities so that they require more social and mental participation and focus on experiencing different types of sensory input e.g., sound, tactile, visual or vestibular input rather than taking part in the activity by using their hands.

Decline in gross motor skills in adult Rett syndrome; results from a Danish longitudinal study.

Longevity of individuals with neurodevelopmental diseases as Rett syndrome (RTT) has increased and many reach adulthood and old age. There is therefore a need to increase knowledge about the course of RTT in adults in order to improve medical care management and quality of life. We did a longitudinal study to address if a possible decline in motor skills in adults with RTT can be explained by the presence of common medical conditions as epilepsy, breathing disturbance, and scoliosis. Data from the Danish RTT database, medical files, and videos from visits at the national Center for Rett syndrome were reviewed. The study included 24 individuals aged 30-66 years at last visit after a follow-up period of 6-12 years. Results showed a clinically observable and significant decline in gross motor skills using the Rett syndrome Gross Motor Scale (RSGMS) with a tendency of less decline in the individuals with the best motor abilities. The frequencies of comorbidities were high. Decline in RSGMS score was associated with the presence of epilepsy and severe scoliosis that had been conservatively managed. The results emphasize that epilepsy plays a significant role in the adult RTT life and management of severe scoliosis in the younger years has impact on the motor abilities in adulthood.

Implementing telehealth support to increase physical activity in girls and women with Rett syndrome-ActivRett: protocol for a waitlist randomised controlled trial.

INTRODUCTION: Individuals with Rett syndrome (RTT) experience impaired gross motor skills, limiting their capacity to engage in physical activities and participation in activities. There is limited evidence of the effectiveness of supported physical activity interventions. This study aims to evaluate the effects of a telehealth-delivered physical activity programme on physical activity, sedentary behaviour and quality of life in RTT. METHODS AND ANALYSIS: This is a multicentre study, conducted in Australia, Denmark and Israel. It is a randomised waitlist-controlled trial comparing an intervention to support physical activity with usual care. Participants are children and adults with RTT, recruited from the Australian Rett Syndrome Database, the Danish Center for Rett Syndrome and the Rett Syndrome Association of Israel. The intervention duration is 12 weeks, including fortnightly telephone contact to plan, monitor and develop individual activity programmes. Outcomes are measured at baseline, at 13 weeks and then at 25 weeks. The primary outcomes are sedentary behaviour assessed with an activPAL accelerometer and the number of daily steps measured with a StepWatch Activity Monitor. Secondary outcomes include sleep, behaviour and quality of life. Caregiver experiences will be assessed immediately after the intervention using a satisfaction questionnaire. Group differences for each outcome will be evaluated with analysis of covariance, adjusting for baseline values on an intention-to-treat basis. ETHICS AND DISSEMINATION: Ethics approval has been obtained in Western Australia from the Child and Adolescent Health Services (RGS3371), in Denmark from the Capital Region Ethics Committee (H-19040514) and in Israel from the Ariel University Institutional Review Board (AU-HEA-ML-20190331). Manuscripts on the development of the intervention from pilot work and the results of the intervention will be submitted to peer-reviewed journals. Results will be presented at conferences and consumer forums. We will develop an online resource documenting the physical activity programme and available supporting evidence. TRIAL REGISTRATION NUMBER: NCT04167059; Pre-results.

Feasibility and Effectiveness of an Individualized 12-Week "Uptime" Participation (U-PART) Intervention in Girls and Women With Rett Syndrome.

BACKGROUND: Girls and women with Rett Syndrome (RTT) have low levels of daily physical activity and high levels of sedentary time. Reducing sedentary time and enhancing "uptime" activities, such as standing and walking, could be an important focus for interventions to address long-term health and quality of life in RTT. OBJECTIVE: The aim of the study was to evaluate the feasibility and health-related effects of an individualized 12-week uptime participation (U-PART) intervention in girls and women with RTT. DESIGN: The study used a single-group pretest-posttest design with 4 assessments (2 baseline, postintervention, and follow-up). METHODS: A participation-based intervention employing a whole-day approach was used. During a 12-week intervention period, individualized programs focused on participation in enjoyable uptime activities in home, school/day center, and community settings. Feasibility was assessed with a study-specific questionnaire. Primary outcome measures were sedentary time and daily step count. Secondary outcomes were gross motor skills, walking capacity, quality of life, and goal attainment scaling. RESULTS: Fourteen girls and women who were 5 to 48 years old and had RTT participated. The U-PART intervention was perceived as feasible by caregivers. Similar scores were observed at baseline assessments in all outcomes. Positive effects with small to medium effect sizes (0.27-0.54) were seen in sedentary time (- 4%), daily step count (+ 689 steps/d), walking capacity (+ 18.8 m), quality of life (+ 2.75 points), and goal attainment scaling after the intervention. Positive effects were maintained in sedentary time (- 3.2%) and walking capacity (+ 12.1 m) at short-term follow-up. LIMITATIONS: This study was limited by the lack of a control group. However, participants acted as their own control, and the stable baseline period partially mitigated this issue. CONCLUSIONS: The U-PART intervention was found to be feasible and effective in the short term in girls and women with RTT.

Patterns of sedentary time and ambulatory physical activity in a Danish population of girls and women with Rett syndrome.

BACKGROUND: Rett syndrome (RTT) is a rare neurodevelopmental disorder leading to multiple disabilities and high dependency on caregivers. This study aimed to: (1) describe the patterns of sedentary time and daily steps and (2) identify the association of individual and environmental characteristics with sedentary time. METHODS: All Danish females with RTT older than 5 years of age and with a MECP2 mutation were invited to participate. The activPAL and StepWatch Activity Monitor (SAM) were worn by participants for at least four days. Sedentary time and step counts were plotted by time to examine daily activity patterns. Associations between sedentary time and individual and environmental covariates were assessed with linear regression models. RESULTS: The median (interquartile range) age of participants was 22.0 (14.3-36.5) years. On average 83.3% (standard deviation 13.9%) of waking hours were spent in sedentary behaviours (n = 48) and the median (interquartile range) daily step count was 5128 (2829-7704) (n = 28). Females older than 33.5 years, and those unable to walk independently were more sedentary. CONCLUSIONS: This study demonstrated high levels of sedentary time and low daily step counts in a Danish population of females with RTT. Advancing age and lower walking skills were associated with higher levels of sedentary time. Implications for Rehabilitation Sedentary lifestyles in individuals with disabilities have a negative impact on health and quality of life. High levels of sedentary time and low daily step counts were demonstrated in a Danish population of females with Rett syndrome. Advancing age and inability to walk independently were strongly associated with higher levels of sedentary time in females with Rett syndrome. Understanding patterns of sedentary behaviour and physical activity can aid health care professionals in developing health-promoting physical activity interventions.

Facilitators and Barriers of Participation in "Uptime" Activities in Girls and Women With Rett Syndrome: Perspectives From Parents and Professionals.

Rett syndrome (RTT) is a rare neurodevelopmental disorder usually affecting females. It is associated with intellectual and multiple disabilities leading to a high level of dependency in all aspects of daily living including participation in physical activities. This study explored facilitators and barriers to "uptime" (non-sedentary) activities in Danish girls and women with RTT as perceived by parents and professionals using focus groups. Through thematic analysis, one central theme emerged: a constant balance to do the best thing for the girl or woman. Within the central theme, five subthemes of facilitators and barriers were identified relating to the individual and the physical, organizational, social, and attitudinal environments. Environmental barriers can be reduced through policy and management-level changes in health promotion and strong advocacy of physical activity by health professionals. Targeting both facilitators and barriers of "uptime" activities enables the planning and implementing of health-promoting interventions in individuals with RTT.

Measurement of Sedentary Behaviors or "Downtime" in Rett Syndrome.

This study aimed to validate measures of sedentary time in individuals with Rett syndrome. Twenty-six individuals (median [IQR] age 16.0 (9.4-20.6) years) wore an activPAL accelerometer during video-taped activities and agreement was determined between sedentary time determined by the activPAL and observation. For 11 individuals (median [IQR] age 14.5 (11.5-25.6) years), linear regression was used to determine the relationship between sedentary time recorded on the modified Bouchard activity record diary card and measured using the activPAL. In comparison to observation, the activPAL accurately measured duration of sedentary time with a mean difference (limit of agreement) of -1.0 (6.3) minutes. The duration of Bouchard activity record downtime accounted for 73% of the variance of sedentary time measured by the activPAL (coefficient 0.762, 95% CI 0.413 to 1.111). These data provide clinicians and caregivers with capacity to investigate strategies that would aim to increase activity in the nonexercise component of the activity continuum.

Functional abilities in aging women with Rett syndrome - the Danish cohort.

Rett syndrome (RTT) is a neurodevelopmental disorder, which mainly affects females and results in multiple disabilities. Many clinical descriptions of the symptoms and functional abilities have been made medically, though mainly in children with RTT. Previous reports have established that even though the syndrome causes severe psychomotor disability, women with RTT can live long into adulthood. PURPOSE: We aim to describe what to expect from aging women with RTT regarding some of the basic functional abilities that are used in daily activities and that could have an impact on quality of life in these women. METHODS: A team of two medical doctors, a physiotherapist and an educational psychological adviser, performed clinical evaluations of 27 women with RTT in Denmark above 30 years of age and confirmed MECP2 mutation. RESULTS: We found that 63% of the women were able to walk outside their homes and only 11% were not able to walk at all. However, 67% could not transfer from sitting to standing position without support. There was profound difficulties communicating, but 85.1% of the women could either consistently point with their hand or eyes to things of their interest. CONCLUSIONS: Women with RTT are very dependent on caregivers who maintain and rehabilitate their functional abilities. They can often walk short distances unassisted, but do have trouble transferring and thus getting up from a chair on their own. They have severe problems communicating and they often perform subtle signs that can be difficult to recognize. Implications for rehabilitation 3/4 of aging RTT women are household ambulators - daily training of motor functions and focus on assisting the initiation of movements are needed lifelong to maintain walking ability and participation in daily activities More than half of aging women with RTT can grab on to things - persons with hand function should be motivated to use this ability in the context of eating Communication is a difficult task especially for the aging RTT women - Communicative signs, their meaning and how to react to them should be written down for every woman in an easy accessible way to all caregivers The majority of aging RTT women can point out things of interest - they should be given the opportunity to participate in choice making.

Building the repertoire of measures of walking in Rett syndrome.

BACKGROUND: The repertoire of measures of walking in Rett syndrome is limited. This study aimed to determine measurement properties of a modified two-minute walk test (2MWT) and a modified Rett syndrome-specific functional mobility scale (FMS-RS) in Rett syndrome. METHODS: Forty-two girls and women with Rett syndrome (median 18.4 years, range 2.4-60.9 years) were assessed for clinical severity, gross motor skills, and mobility. To measure walking capacity, 27 of this group completed a 2MWT twice on two different assessment days. To assess walking performance, the FMS-RS was administered to the total sample of parents (n = 42) on two occasions approximately one week apart. RESULTS: There were negative correlations between clinical severity and 2MWT (r = -0.48) and FMS-RS (r = -0.60-0.66). There were positive correlations between gross motor skills and mobility and 2MWT (r = 0.51, 0.43) and FMS-RS (r = 0.71-0.93, 0.74-0.94), respectively. Test-retest reliability for the 2MWT was good with high intraday and interday correlations (ICC = 0.86-0.98). For the 2MWT, the standard error of measurement was 13.8 m and we would be 95% confident that changes greater than 38 m would be greater than within subject error. There was good test-retest reliability for all three distances on the FMS-RS (ICC = 0.94-0.99). CONCLUSIONS: Walking capacity as measured by the 2MWT showed expected but limited relationships with measures of different constructs, providing some support for concurrent validity. Walking performance as measured with the FMS-RS was more strongly consistent with other clinical measures supporting its concurrent validity. Test-retest reliability was good for both the FMS-RS and the 2MWT. Therefore, these measures have the potential to be used in clinical practice and research. Implications for Rehabilitation Walking is one of the commonest daily physical activities in ambulant girls and women with RTT. Comprehensive knowledge about the walking abilities in this population is limited. Evidence of validity and test-retest reliability have been demonstrated for the modified two-minute walk test (2MWT) and the Rett syndrome-specific functional mobility scale (FMS-RS). The 2MWT and FMS-RS offer detailed information of the capacity and performance of walking, respectively, in girls and women with RTT.

Validating the Rett Syndrome Gross Motor Scale.

Rett syndrome is a pervasive neurodevelopmental disorder associated with a pathogenic mutation on the MECP2 gene. Impaired movement is a fundamental component and the Rett Syndrome Gross Motor Scale was developed to measure gross motor abilities in this population. The current study investigated the validity and reliability of the Rett Syndrome Gross Motor Scale. Video data showing gross motor abilities supplemented with parent report data was collected for 255 girls and women registered with the Australian Rett Syndrome Database, and the factor structure and relationships between motor scores, age and genotype were investigated. Clinical assessment scores for 38 girls and women with Rett syndrome who attended the Danish Center for Rett Syndrome were used to assess consistency of measurement. Principal components analysis enabled the calculation of three factor scores: Sitting, Standing and Walking, and Challenge. Motor scores were poorer with increasing age and those with the p.Arg133Cys, p.Arg294* or p.Arg306Cys mutation achieved higher scores than those with a large deletion. The repeatability of clinical assessment was excellent (intraclass correlation coefficient for total score 0.99, 95% CI 0.93-0.98). The standard error of measurement for the total score was 2 points and we would be 95% confident that a change 4 points in the 45-point scale would be greater than within-subject measurement error. The Rett Syndrome Gross Motor Scale could be an appropriate measure of gross motor skills in clinical practice and clinical trials.

Mosaicism for c.431_454dup in ARX causes a mild Partington syndrome phenotype.

A common in frame duplication in ARX (c.431_454dup24) was found in a five year-old boy who presented with mild Partington syndrome. The duplication was detected by PCR amplification followed by fragment length analysis and was located in exon 2 spanning the two polyalanine tracts commonly seen to expand. Detection of the duplication by DNA sequencing was difficult due to preferential sequencing of the normal allele, demonstrating the superiority of fragment length analysis in mosaic cases. The clinical symptoms were mild to moderate developmental delay with only the hand dystonia to suggest Partington syndrome. This patient is the first male reported to be mosaic for the duplication, and his clinical features are subtle. This study shows that in males with a phenotype of mild Partington syndrome and in heterozygous females fragment length analysis should be preferred over DNA sequencing.

Discriminative validity of the Danish version of the Pediatric Evaluation of Disability Inventory (PEDI).

The Pediatric Evaluation of Disability Inventory (PEDI) assesses functional status in children with disabilities aged 0.5-7.5 years. The purpose of this study was to examine if the Danish version of the PEDI was able to discriminate between nondisabled children and children with cerebral palsy (CP) or juvenile idiopathic arthritis (JIA). Convenience sampling was used to select the clinical samples that consisted of 22 children with CP and 14 children with JIA aged 1.5-7.5 years. An additional sample, consisting of 224 nondisabled children aged 1.0-5.9 years, was obtained on the basis of random sampling. The PEDI was administered as a questionnaire completed by the parents. Results from receiver operating characteristics (ROC) analysis showed that in the case of discrimination between children with CP and nondisabled children, the sensitivity and specificity were 77.3 and 97.8%, respectively. In the discrimination between children with JIA and nondisabled children, the sensitivity and specificity were 99.8 and 81.7%, respectively. Study results indicate that the Danish version of the PEDI shows good discriminative validity. Further studies of the discriminative validity are recommended.

Applicability and intrarespondent reliability of the pediatric evaluation of disability inventory in a random Danish sample.

PURPOSE: To examine the applicability of US reference data from the Pediatric Evaluation of Disability Inventory (PEDI) in a random Danish sample and to assess intrarespondent reliability. METHODS: The PEDI was administered as a mailed questionnaire that was completed by the parents. Linear regression models and comparisons of item difficulty were performed in the data analysis. Intraclass correlation coefficients were used in the intrarespondent analysis. RESULTS: US and Danish children differed on 3 of the 6 subscales of the PEDI. Danish children had significantly lower scores on the caregiver assistance subscales (self-care and mobility) and significantly higher scores on the functional skills subscale (social function). Item difficulty was found to be comparable (r = 0.82-0.97) on all 6 subscales. Intrarespondent reliability was good (intraclass correlation coefficient = 0.62-0.97). CONCLUSION: The PEDI reference values seem partly applicable in a Danish population. In a Danish rehabilitation setting, interpreting PEDI scores with regard to detecting functional delays must be done cautiously.