Early motor, cognitive, language, behavioural and social emotional development in infants and young boys with Duchenne Muscular Dystrophy- A systematic review.

Duchenne Muscular Dystrophy (DMD) is an X-linked recessive disorder caused by mutations in the dystrophin gene. Deficiency of the dystrophin protein causes not only motor, but also cognitive, language, behavioural and social emotional problems. This is the first systematic review investigating five early developmental domains in boys with DMD between 0 and 6 years old. Interactions between different domains and links with mutation types and sites were explored. A systematic search was performed in PubMed, Web of Science and Scopus. An adapted version of the Scottish Intercollegiate Guidelines Network (SIGN) Checklists for case-control and cohort studies was used to evaluate quality. Fifty-five studies of high or acceptable quality were included. One was an RCT of level 1b; 50 were cohort studies of level 2b; and four were an aggregation of case-control and cohort studies receiving levels 2b and 3b. We found that young boys with DMD experienced problems in all five developmental domains, with significant interactions between these. Several studies also showed relationships between mutation sites and outcomes. We conclude that DMD is not only characterised by motor problems but by a more global developmental delay with a large variability between boys. Our results emphasise the need for harmonisation in evaluation and follow-up of young boys with DMD. More high-quality research is needed on the different early developmental domains in young DMD to facilitate early detection of difficulties and identification of associated early intervention strategies.

Reference curves of motor function outcomes in young steroid-naïve males with Duchenne muscular dystrophy.

AIM: To investigate functional motor performance in a large cohort of young steroid-naïve males with Duchenne muscular dystrophy (DMD) and typically developing males, and to develop specific reference curves for both groups. Also, to describe associations between anthropometric values and functional motor outcomes. METHOD: Cross-sectional data of 196 steroid-naïve males with DMD aged 4 to 8 years and 497 typically developing males aged 2 years 6 months to 8 years were included. Both groups were evaluated with the time to rise from the floor test, 10-metre walk/run test, 6-minute walk test, and North Star Ambulatory Assessment. Reference curves with centiles 5%, 10%, 25%, 50%, 75%, 90%, and 95% were estimated using quantile regression. RESULTS: Males with DMD scored significantly worse on all functional motor outcomes than age-matched typically developing males (p < 0.001): 89% to 95% of the males with DMD scored below the 5th centile of the typically developing males. No or weak correlations exist between anthropometric values and functional motor outcomes. INTERPRETATION: The estimated reference curves can support consultation with families of young males with DMD and can support the evaluation of treatment for reaching motor skills and functional motor outcomes compared with typically developing males.

A Critical View on Motor-based Interventions to Improve Motor Skill Performance in Children With ADHD: A Systematic Review and Meta-analysis.

OBJECTIVE: To map the effect of motor-based interventions on motor skills in children with ADHD. METHOD: A systematic literature search was performed in Pubmed, Web of Science, and the SCOPUS database (last search: October 30th 2022). Methodological quality was assessed using the PEDro-scale and the quality of evidence was determined with the GRADE-method. Meta-analysis was performed when at least five studies were available. RESULTS: Thirteen studies (7 RCTs) satisfied the inclusion criteria, five of which were eligible for meta-analysis. Only one of the included studies reached the low risk of bias threshold. Comparing different motor-based interventions to any non-motor control intervention showed large motor skill improvements (SMD = 1.46; 95% CI = [1.00;1.93]; I² = 47.07%). The most effective type of motor-based intervention and the optimal treatment parameters could not be determined yet. CONCLUSION: Motor-based interventions in general seem to improve motor skills in children with ADHD. Additional RCTs are needed to increase current low GRADE confidence.

Randomized controlled trial combining constraint-induced movement therapy and action-observation training in unilateral cerebral palsy: clinical effects and influencing factors of treatment response.

INTRODUCTION: Constraint-induced movement therapy (CIMT) improves upper limb (UL) motor execution in unilateral cerebral palsy (uCP). As these children also show motor planning deficits, action-observation training (AOT) might be of additional value. Here, we investigated the combined effect of AOT to CIMT and identified factors influencing treatment response. METHODS: A total of 44 children with uCP (mean 9 years 6 months, SD 1 year 10 months) participated in a 9-day camp wearing a splint for 6 h/day and were allocated to the CIMT + AOT (n = 22) and the CIMT + placebo group (n = 22). The CIMT + AOT group received 15 h of AOT (i.e. video-observation) and executed the observed tasks, whilst the CIMT + AOT group watched videos free of biological motion and executed the same tasks. The primary outcome measure was bimanual performance. Secondary outcomes included measures of body function and activity level assessed before (T1), after the intervention (T2), and at 6 months follow-up (T3). Influencing factors included behavioural and neurological characteristics. RESULTS: Although no between-groups differences were found (p > 0.05; η2 = 0-16), the addition of AOT led to higher gains in children with initially poorer bimanual performance (p = 0.02; η2 = 0.14). Both groups improved in all outcome measures after the intervention and retained the gains at follow up (p < 0.01; η2 = 0.02-0.71). Poor sensory function resulted in larger improvements in the total group (p = 0.03; η2 = 0.25) and high amounts of mirror movements tended to result in a better response to the additional AOT training (p = 0.06; η2 = 0.18). Improvements were similar irrespective of the type of brain lesion or corticospinal tract wiring pattern. CONCLUSIONS: Adding AOT to CIMT, resulted in a better outcome for children with poor motor function and high amounts of mirror movements. CIMT with or without AOT seems to be more beneficial for children with poor sensory function. TRIAL REGISTRATION: Registered at ClinicalTrials.gov on 22nd August 2017 (ClinicalTrials.gov identifier: NCT03256357).

Normative data and percentile curves for the three-minute walk test and timed function tests in healthy Caucasian boys from 2.5 up to 6 years old.

The three-minute walk test (3MWT) and timed function tests (TFTs) (rise from floor, 10 m run, climbing and descending four stairs) are currently used to evaluate functional capacity in young boys with neuromuscular disorders. This study aimed to generate normative data in healthy boys aged 2.5 up to 6 years for these tests and to provide percentile curves according to age and height. The relation between the 3MWT, TFTs and anthropometric variables was investigated. In total 179 boys (mean age: 4.1 y ± 1.0) were evaluated across four age (2.5 years; 3 years; 4 years and 5 years) and three height groups: (<100 cm; 100 to <110 cm and ≥110 cm). Three-minute walk distance (3MWD) increased significantly, from 168.4 m (± 18.8) at 2.5 years to 214.5 m (± 26.1) at 5 years and from 172.6 m (±21.8) for children <100 cm to 212.7 m (±26.2) for children ≥110 cm. TFTs times decreased significantly with age and height. Significant correlations between the anthropometric values, 3MWD and TFTs were found (r(s)= 0.55-0.84; p <0.0001). These normative data and percentile curves provide a useful tool in the assessment of functional capacity in young boys. This study also confirms the association between functional tests and anthropometric values.

Time Course of Upper Limb Function in Children with Unilateral Cerebral Palsy: A Five-Year Follow-Up Study.

Knowledge on long-term evolution of upper limb function in children with unilateral cerebral palsy (CP) is scarce. The objective was to report the five-year evolution in upper limb function and identify factors influencing time trends. Eighty-one children (mean age 9 y and 11 mo, SD 3 y and 3 mo) were assessed at baseline with follow-up after 6 months, 1, and 5 years. Passive range of motion (PROM), tone, muscle, and grip strength were assessed. Activity measurements included Melbourne Assessment, Jebsen-Taylor test, Assisting Hand Assessment (AHA), and ABILHAND-Kids. At 5-year follow-up, PROM (p < 0.001) and AHA scores (p < 0.001) decreased, whereas an improvement was seen for grip strength (p < 0.001), Melbourne Assessment (p = 0.003), Jebsen-Taylor test (p < 0.001), and ABILHAND-Kids (p < 0.001). Age influenced the evolution of AHA scores (p = 0.003), with younger children being stable over time, but from 9 years onward, children experienced a decrease in bimanual performance. Manual Ability Classification System (MACS) levels also affected the evolution of AHA scores (p = 0.02), with stable scores in MACS I and deterioration in MACS II and III. In conclusion, over 5 years, children with unilateral CP develop more limitations in PROM, and although capacity measures improve, the spontaneous use of the impaired limb in bimanual tasks becomes less effective after the age of 9 years.

Validity and cross-cultural differences of the Bayley Scales of Infant and Toddler Development, Third Edition in typically developing infants.

BACKGROUND: The Dutch translation of Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III-NL) is a comprehensive tool assessing cognitive, language and motor development in children up to 42 months. AIMS: The first aim of this study was to evaluate concurrent validity of the Bayley-III-NL Gross Motor Scale (GMS) in relation to the Alberta Infant Motor Scale (AIMS). Secondly, divergent validity between the other subscales mutually and the AIMS was investigated. Finally, the importance of population-specific reference values was examined. METHODS AND PROCEDURES: A sample of 122 healthy, typically developing Flemish children (mean age: 9 months 7 days), born full term was assessed with the Bayley-III-NL and the AIMS. OUTCOMES AND RESULTS: Concurrent validity of the Bayley-III-NL GMS and the AIMS was moderate to high (0.59-0.98; p < 0.001). In addition, weak correlations (-0.10-0.27) between the non-motor-Bayley-III-NL subscales and the AIMS were found. Finally, significant differences were found between Bayley-III scores based on Flemish and American norms (p < 0.001), except for fine motor skills (p = 0.11). CONCLUSIONS AND IMPLICATIONS: This study provides support for the concurrent validity of the Bayley-III-NL GMS and divergent validity of the different Bayley-III-NL subscales. Secondly, population-specific reference values should be used to avoid over- and under estimation of infant's development.

Diagnostic criteria for DCD: Past and future.

The aim of this review was to gather information on how well authors comply to DSM criteria in their description and selection of children with DCD. We investigated which selection criteria were used in experimental and intervention studies published in the last 5 years (2010-2014). Results on 176 papers are summarized. Compliance to the DSM criteria has improved over this time period. In general, detailed information was provided on motor performance using standardized test scores and cut-off values are reported. Method sections were far less detailed about other DSM criteria (i.e., whether motor coordination problems interfered with activities of daily living and how IQ, other medical conditions or co-occurring disorders were checked). Views on how the new DSM-5 criteria could be specified for clinical and research use are discussed.

Randomized trial of modified constraint-induced movement therapy with and without an intensive therapy program in children with unilateral cerebral palsy.

BACKGROUND: Constraint-induced movement therapy (CIMT) has gained emerging evidence and popularity in children with unilateral cerebral palsy (CP). However, many issues remain unanswered regarding the best approach. OBJECTIVE: This study investigated the additional effects of an intensive therapy program to promote hand function combined with home-based modified CIMT (m-CIMT). METHODS: . Fifty-one children (mean age 8 years 9 months) were randomized to m-CIMT alone or m-CIMT with intensive therapy (IT). All children had to wear a constraint on the unaffected hand for 1 hour, 5 days/week for 10 weeks. Children in the m-CIMT + IT group also received 3 sessions of 45 minutes weekly of intensive therapy for distal muscle strengthening and hand function, using unimanual and bimanual activities. The Assisting Hand Assessment (AHA) was the primary outcome measure. Secondary outcome measures were muscle tone, strength, Melbourne Assessment, Jebsen-Taylor test, and ABILHAND-Kids questionnaire. Assessments were administered at baseline, after intervention, and at 10-week follow-up. RESULTS: Significant between-group differences in AHA were in favor of the m-CIMT + IT group (P = .04). Both groups demonstrated comparable improvements in muscle tone (P = .002), strength (P < .0001), grip strength (P = .02), and unimanual capacity (Melbourne Assessment and Jebsen-Taylor, P < .0001). Younger children and children with poorer hand function benefited from both interventions, whereas older children and children with better hand function only benefited from the combined approach. CONCLUSIONS: The combination of m-CIMT with an intensive therapy program on distal hand function and strength enhances the effects of m-CIMT alone for improving bimanual performance.