Quantifying lower limb muscle stiffness in typically developing children and adolescents using acoustic radiation force impulse shear wave elastography (ARFI/SWE)-a pilot study.

OBJECTIVE: To investigate and quantify age-related changes in lower limb muscle stiffness in typically developing children and adolescents using acoustic radiation force impulse shear wave elastography. MATERIALS AND METHODS: Shear wave velocities of bilateral rectus femoris, tibialis anterior, and medial gastrocnemius muscles at rest were obtained in typically developing children and adolescents aged 3 to 18 years. The participants were classified into three age groups: Group 1 (children), 3 to 7 years old; Group 2, 8 to 12 (pre-adolescent); and Group 3 (adolescent), 13 to 18. The shear wave velocities of muscle were compared across the three age groups, as well as compared between right- and left-side limbs. The correlation between shear wave velocities and body weight or body mass index was assessed. RESULTS: Of the 47 participants, 21 were in Group 1, 17 in Group 2, and 9 in Group 3. There were no significant differences among the three age groups' shear wave velocities of bilateral lower limb muscles, and no significant differences between right and left sides. There was no correlation between muscle stiffness and body weight or body mass index. CONCLUSION: The present pilot study applied acoustic radiation force impulse shear wave elastography to quantify lower limb muscle stiffness in typically developing children and adolescents aged 3 to 18 years, suggesting no marked change in muscle stiffness occurs as they develop.

Visual-motor integration in children with unilateral cerebral palsy: application of the computer-aided measure of visual-motor integration.

BACKGROUND: Children with unilateral cerebral palsy (UCP) are encouraged to participate in the regular school curriculum. However, even when using the less-affected hand for handwriting, children with UCP still experience handwriting difficulties. Visual-motor integration (VMI) is a predictor of handwriting quality. Investigating VMI in children with UCP is important but still lacking. Conventional paper-based VMI assessments is subjective and use all-or-nothing scoring procedures, which may compromise the fidelity of VMI assessments. Moreover, identifying important shapes that are predictive of VMI performance might benefit clinical decision-making because different geometric shapes represent different developmental stepping stones of VMI. Therefore, a new computer-aided measure of VMI (the CAM-VMI) was developed to investigate VMI performance in children with UCP and to identify shapes important for predicting their VMI performance. METHODS: Twenty-eight children with UCP and 28 typically-developing (TD) children were recruited. All participants were instructed to complete the CAM-VMI and Beery-Buktenica Developmental Test of Visual-Motor Integration (Beery-VMI). The test items of the CAM-VMI consisted of nine simple geometric shapes related to writing readiness. Two scores of the CAM-VMI, namely, Error and Effort, were obtained by image registration technique. The performances on the Beery-VMI and the CAM-VMI of children with UCP and TD children were compared by independent t-test. A series of stepwise regression analyses were used to identify shapes important for predicting VMI performance in children with UCP. RESULTS: Significant group differences were found in both the CAM-VMI and the Beery-VMI results. Furthermore, Error was identified as a significant aspect for predicting VMI performance in children with UCP. Specifically, the square item was the only significant predictor of VMI performance in children with UCP. CONCLUSIONS: This study was a large-scale study that provided direct evidence of impaired VMI in school-aged children with UCP. Even when using the less-affected hand, children with UCP could not copy the geometric shapes as well as TD children did. The copied products of children with UCP demonstrated poor constructional accuracy and inappropriate alignment. Furthermore, the predictive model suggested that the constructional accuracy of a copied square is an important predictor of VMI performance in children with UCP.

Comparative effects of kinect-based versus therapist-based constraint-induced movement therapy on motor control and daily motor function in children with unilateral cerebral palsy: a randomized control trial.

BACKGROUND: Constraint-induced movement therapy (CIMT) is a prominent neurorehabilitation approach for improving affected upper extremity motor function in children with unilateral cerebral palsy (UCP). However, the restraint of the less-affected upper extremity and intensive training protocol during CIMT may decrease children's motivation and increase the therapist's workload and family's burden. A kinect-based CIMT program, aiming to mitigate the concerns of CIMT, has been developed. The preliminary results demonstrated that this program was child-friendly and feasible for improving upper extremity motor function. However, whether the kinect-based CIMT can achieve better or at least comparable effects to that of traditional CIMT (i.e., therapist-based CIMT) should be further investigated. Therefore, this study aimed to compare the effects of kinect-based CIMT with that of therapist-based CIMT on upper extremity and trunk motor control and on daily motor function in children with UCP. METHODS: Twenty-nine children with UCP were recruited and randomly allocated to kinect-based CIMT (n = 14) or therapist-based CIMT (n = 15). The intervention dosage was 2.25 h a day, 2 days a week for 8 weeks. Outcome measures, namely upper extremity and trunk motor control and daily motor function, were evaluated before and after 36-h interventions. Upper extremity and trunk motor control were assessed with unimanual reach-to-grasp kinematics, and daily motor function was evaluated with the Revised Pediatric Motor Activity Log. Between-group comparisons of effectiveness on all outcome measures were analyzed by analysis of covariance (α = 0.05). RESULTS: The two groups demonstrated similar improvements in upper extremity motor control and daily motor function. In addition, the kinect-based CIMT group demonstrated greater improvements in trunk motor control than the therapist-based CIMT group did (F(1,28) > 4.862, p < 0.036). CONCLUSION: Kinect-based CIMT has effects comparable to that of therapist-based CIMT on UE motor control and daily motor function. Moreover, kinect-based CIMT helps decrease trunk compensation during reaching in children with UCP. Therefore, kinect-based CIMT can be used as an alternative approach to therapist-based CIMT. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02808195. Registered on 2016/06/21, https://clinicaltrials.gov/ct2/show/NCT02808195 .

Assessment for Tactile Perception in Children With Cerebral Palsy.

IMPORTANCE: Impaired tactile perception frequently accompanies motor deficits in children with cerebral palsy (CP). Assessing tactile perception precisely for children with CP remains challenging because of a lack of assessments with robust psychometric evidence or standard procedures. OBJECTIVE: To develop a standardized assessment tool, the Tactile Perceptual Test (TPT), for measuring tactile perception in children with CP and to examine its psychometric properties. DESIGN: Observational study design. SETTING: University research laboratory and medical center. PARTICIPANTS: Children with CP (n = 100) and typical development (TD; n = 50). OUTCOMES AND MEASURES: The TPT includes four subtests measuring stereognosis, roughness, hardness, and heaviness. Three comparator instruments, Semmes-Weinstein monofilaments, Two-Point Discrimination, and the stereognosis subtest of the Revised Nottingham Sensory Assessment, were used for convergent validity. RESULTS: Good test-retest reliability was confirmed for all of the TPT subtests. The values of minimal detectable change were acceptable. Moderate correlations between the TPT and comparator instruments were found, as expected. For known-groups validity, the significant difference was confirmed between children with CP and those with TD. CONCLUSIONS AND RELEVANCE: The TPT is a reliable and valid measure for multiple subdomains of tactile perception in children with CP. This tactile assessment may help clarify tactile performance to provide appropriate, precise interventions. What This Article Adds: The TPT measures tactile perception in children with CP. It has four subdomains of tactile perception that could facilitate prioritization of tactile treatment of specific subdomains and thereby aid in the provision of appropriate interventions.

Psychometric Properties of the Little Developmental Coordination Disorder Questionnaire-Taiwan.

IMPORTANCE: Early identification of young children at risk of developmental coordination disorder (DCD) can support early intervention and prevent secondary sequelae. OBJECTIVE: To examine the psychometric properties of a translated and cross-culturally adapted version of the Little Developmental Coordination Disorder Questionnaire-Taiwan (LDCDQ-TW). DESIGN: Prospective study. SETTING: Kindergartens and preschools in north, central, and south Taiwan. PARTICIPANTS: In Phase 1 the participants were 1,124 parents of typically developing children ages 36-71 mo. Children with confirmed developmental diagnoses were excluded. Participants in Phase 3 were 162 children who had been recruited in Phase 2. Outcomes and Measures: The LDCDQ-TW, a 15-item parent questionnaire for identifying children at risk for DCD, and the Movement Assessment Battery for Children (2nd ed.; MABC-2), were administered. RESULTS: The findings revealed excellent test-retest reliability (intraclass correlation coefficient [ICC] = .97) and poor interrater reliability (ICC = .47). On the basis of MABC-2 scores, the non-DCD group (≥15th percentile) scored significantly higher than the DCD and suspect-DCD groups on the LDCDQ-TW, but the latter two groups did not differ from one another. Using the 15th percentile as a cutoff for both the MABC-2 and the LDCDQ-TW, sensitivity was .96 and specificity was .68. CONCLUSIONS AND RELEVANCE: Although standardized performance-based assessments are required to confirm a diagnosis of DCD (typically after age 5 yr), the LDCDQ-TW demonstrated sound reliability and validity and can support the early identification of young children at risk of DCD in Taiwan. What This Article Adds: The LDCDQ-TW can facilitate early intervention for DCD and prevent secondary sequelae, improving outcomes for children with DCD.

Reliability and Validity of the Chinese Version of the Infant/Toddler Sensory Profile.

IMPORTANCE: In Chinese-speaking societies, a reliable and valid measure is needed to enable early identification of young children's challenges in sensory processing. OBJECTIVE: To examine the psychometric properties of the Chinese version of the Infant/Toddler Sensory Profile (ITSP-C) when used with Taiwanese children. DESIGN: Prospective study. SETTING: Multiple settings. PARTICIPANTS: Participants were 367 typically developing (TD) infants ages 0-6 mo (mean [M] age = 3.44 mo, standard deviation [SD] = 1.82) and 677 toddlers ages 7-36 mo (M age = 22.33 mo, SD = 8.15), along with 42 toddlers with developmental disabilities (DD; M age = 28.4 mo, SD = 6.7). OUTCOMES AND MEASURES: The ITSP, a caregiver questionnaire that measures how children respond to sensory events in daily life, was translated and cross-culturally adapted into a Chinese version (ITSP-C). RESULTS: Internal consistency was high for the 0-6 mo subscale (α = .80) and the 7-36 mo subscale (α = .82). Test-retest reliability was excellent for the 7-36 mo subscale (intraclass correlation coefficient = .94). Logistic regression analysis demonstrated that the ITSP-C discriminated between children with DD and TD children. CONCLUSIONS AND RELEVANCE: The ITSP was successfully adapted to Chinese with similar reliability and validity to the original ITSP. The 7-36 mo subscale had a higher level of reliability than the 0-6 mo subscale, and quadrant scores were more reliable than section scores. Moreover, the ITSP-C discriminated children with DD from TD children on the basis of their sensory processing patterns. WHAT THIS ARTICLE ADDS: The ITSP-C has sound psychometric properties and support for its use in early identification of sensory processing difficulties in young Chinese-speaking children.

Clinical Evaluation of Duchenne Muscular Dystrophy Severity Using Ultrasound Small-Window Entropy Imaging.

Information entropy of ultrasound imaging recently receives much attention in the diagnosis of Duchenne muscular dystrophy (DMD). DMD is the most common muscular disorder; patients lose their ambulation in the later stages of the disease. Ultrasound imaging enables routine examinations and the follow-up of patients with DMD. Conventionally, the probability distribution of the received backscattered echo signals can be described using statistical models for ultrasound parametric imaging to characterize muscle tissue. Small-window entropy imaging is an efficient nonmodel-based approach to analyzing the backscattered statistical properties. This study explored the feasibility of using ultrasound small-window entropy imaging in evaluating the severity of DMD. A total of 85 participants were recruited. For each patient, ultrasound scans of the gastrocnemius were performed to acquire raw image data for B-mode and small-window entropy imaging, which were compared with clinical diagnoses of DMD by using the receiver operating characteristic curve. The results indicated that entropy imaging can visualize changes in the information uncertainty of ultrasound backscattered signals. The median with interquartile range (IQR) of the entropy value was 4.99 (IQR: 4.98-5.00) for the control group, 5.04 (IQR: 5.01-5.05) for stage 1 patients, 5.07 (IQR: 5.06-5.07) for stage 2 patients, and 5.07 (IQR: 5.06-5.07) for stage 3 patients. The diagnostic accuracies were 89.41%, 87.06%, and 72.94% for ≥stage 1, ≥stage 2, and ≥stage 3, respectively. Comparisons with previous studies revealed that the small-window entropy imaging technique exhibits higher diagnostic performance than conventional methods. Its further development is recommended for potential use in clinical evaluations and the follow-up of patients with DMD.

Exploring Sensory Processing Dysfunction, Parenting Stress, and Problem Behaviors in Children With Autism Spectrum Disorder.

OBJECTIVE: We examined problem behavior in preschool- and school-age children with autism spectrum disorder and assessed whether sensory processing dysfunction (SPD) and parenting stress were differentially associated with problem behaviors in these groups of children. METHOD: Participants were 101 preschool- and 61 school-age children. An independent t test was used to examine group differences in problem behaviors. Pearson correlation analysis and multiple linear regression were conducted to identify associations among SPD, parenting stress, and problem behaviors. RESULTS: Preschool children had more social withdrawal than school-age children. Moderately negative correlations were found between SPD and problem behaviors among preschool children. Parenting stress was a factor associated with problem behaviors in both groups, whereas SPD was a factor specific to preschoolers. CONCLUSION: Understanding unique problem behaviors and the significant role of SPD and parenting stress in problem behaviors could help occupational therapy clinicians plan interventions accordingly.

Preliminary Efficacy of a Friendly Constraint-Induced Therapy (Friendly-CIT) Program on Motor and Psychosocial Outcomes in Children with Cerebral Palsy.

AIMS: Constraint-induced therapy (CIT) is effective, but concerns have been repeatedly raised regarding the generalizability, feasibility, and potentially intrusive nature of restraining a child's unimpaired upper limb. We examined the feasibility and efficacy of friendly-CIT, which uses home-based model, a caregiver-determined schedule, and gentle restraint, in children with cerebral palsy. METHODS: In a one-group pre-post intervention trial, 15 children (median of age = 8 years, 4 months) participated in 8 weeks of friendly-CIT (36 hours). Motor and psychosocial outcomes were investigated. RESULTS: Participants showed significant improvement on the manual dexterity subtest of the Bruininks-Oseretsky Test of Motor Proficiency (p = 0.004); the how often (p = 0.01) and how well (p = 0.02) scales of the Revised Pediatric Motor Activity Log; and the functional skills score on the Chinese version of the Pediatric Evaluation of Disability Inventory (p = 0.002). For psychosocial outcomes, children with CP consistently demonstrated high engagement and increased playfulness (p < 0.0001) during the 8-week intervention. Parents showed no change in stress status and high satisfaction with friendly-CIT. CONCLUSION: Friendly-CIT is feasible and promising for improving a child's motor and psychosocial outcomes without increasing parental stress.

Psychometric and Clinimetric Properties of the Melbourne Assessment 2 in Children With Cerebral Palsy.

OBJECTIVE: To examine the psychometric and clinimetric properties of the Melbourne Assessment 2 (MA2), an outcome measurement that is increasingly used in clinical studies. DESIGN: Psychometric and clinimetric study. SETTING: Community. PARTICIPANTS: Seventeen children with cerebral palsy (CP) from 5 to 12 years were recruited for the estimation of the test-retest reliability and minimal detectable change (MDC). Thirty-five children with CP were recruited to receive an 8-week intensive neurorehabilitation intervention to estimate the validity, responsiveness, and minimal clinically important difference (MCID). INTERVENTIONS: Thirty-five children with CP received upper limb neurorehabilitation programs for 8 weeks. MAIN OUTCOME MEASURES: The MA2 and the criterion measures, including the Bruininks-Oseretsky Test of Motor Proficiency, 2nd edition (BOT-2), the Box and Blocks Test (BBT), and the Pediatric Motor Activity Log-Revised (PMAL-R), were evaluated at pretreatment and posttreatment. RESULTS: The MA2 has 4 subscales: range of motion, fluency, accuracy, and dexterity. The test-retest reliability of the MA2 is high (intraclass correlation coefficient, .92-.98). The significant relationships between the MA2 and BBT, BOT-2, and PMAL-R support its validity. The significance of paired t test results (P<.001) and large magnitudes of the standardized response mean (1.70-2.00) confirm the responsiveness of the MA2. The MDC values of the 4 subscales of the MA2 are 2.85, 1.63, 1.97, and 1.84, respectively, and the suggested MCID values of these 4 subscales are 2.35, 3.20, 2.09, and 2.22, respectively, indicating the minimum scores of improvement to be interpreted as both statistically significant and clinically important. CONCLUSIONS: The study findings indicate that the MA2 has sound psychometric and clinimetric properties and is thus an adequate measurement for research and clinical applications.

Long-term prognosis of patients with infantile-onset Pompe disease diagnosed by newborn screening and treated since birth.

OBJECTIVE: To determine the benefit of newborn screening for the long-term prognosis of patients with classic infantile-onset Pompe disease (IOPD). STUDY DESIGN: A cohort of patients with classic IOPD were diagnosed by newborn screening, treated with recombinant human acid α-glucosidase (rhGAA), and followed prospectively. Outcome measurements included survival, left ventricular mass, serum creatinine kinase, motor function, mental development, and systemic manifestations. RESULTS: Ten patients who presented with left ventricular hypertrophy at diagnosis received rhGAA infusions starting at a median age of 16 days (6-34 days). All patients were cross-reactive immunologic material-positive. After a median treatment time of 63 months (range 28-90 months), all could walk independently, and none required mechanical ventilation. All patients had motor capability sufficient for participating in daily activities, but muscle weakness over the pelvic girdle appeared gradually after 2 years of age. Ptosis was present in one-half of the patients, and speech disorders were common. Anti-rhGAA antibody titers were low (median maximal titer value 1:1600, range: undetectable ∼ 1:12,800). CONCLUSION: By studying patients treated since birth who have no significant anti-rhGAA antibody interference, this prospective study demonstrates that the efficacy of rhGAA therapy is high and consistent for the treatment of classic IOPD. This study also exposes limitations of rhGAA treatment. The etiology of the manifestations in these early-treated patients will require further study.

Pronator teres transfer for forearm and wrist deformity in cerebral palsy children.

BACKGROUND: Forearm pronation and wrist flexion contracture can be a disability for daily living care and 2-hand function in cerebral palsy (CP) children. It may be beneficial to improve their posture and hand-grip power for better functional outcome. The purpose of our study was to investigate the outcome of pronator transfer in CP children. METHODS: Seventeen spastic CP patients (14 hemiplegic, 3 diplegic; 14 male, 3 female; mean age, 12 y 5 mo) underwent pronator teres transfer for forearm pronation and wrist flexion contractures. The mean follow-up period was 46 months. We recorded Gross Motor Function Classification System level, modified Ashworth scale, forearm and wrist range of motion, forearm resting position, grip power, and 3 basic daily living skills preoperatively and postoperatively. Paired t test was used for statistical analysis. RESULTS: The average forearm active supination gained 80.9 degrees (P<0.05) and the active forearm pronation lost 22 degrees (P<0.05), with average postoperative total active forearm range of motion 130.9 degrees (P<0.05). The average active wrist extension gained 76.9 degrees (P<0.05) and the active wrist flexion lost 31.8 degrees (P<0.05). The average grip power gained 4.5 kg (P<0.05). The average forearm resting position improved to 10 degrees pronation (P<0.05). The basic daily living skills showed great improvement. CONCLUSIONS: Our procedure improved the functional outcome in wrist extension and decreased the forearm pronation. Therefore, significant grip-strength enhancement and better forearm posture was noted. It is an additional armamentarium in the management of upper extremity disability in CP children. LEVEL OF EVIDENCE: Level IV-case series.

Neuroimaging and electroencephalographic changes after vagus nerve stimulation in a boy with medically intractable myoclonic astatic epilepsy.

Myoclonic astatic epilepsy (MAE) is characterized by multiple seizure types, which are often refractory. Although vagus nerve stimulation (VNS) is an alternative treatment for medically intractable seizures, its exact mechanism of action remains unclear. Herein, we report the case of a 4-year-old boy with intractable MAE who has been in a seizure-free status for 2 years and 3 months since 6 months after the implantation of a vagus nerve stimulator (Model 103, Cyberonics, Inc., Houston, TX). Various test results 6 months after VNS were compared with those before VNS. Results of an electroencephalograph revealed disappearance of epileptiform discharges and an increased beta-gamma spectrum rhythm. The brain diffusion-tensor imaging showed an increased ratio of fraction anisotropy in the right fimbria-fornix, indicating improved diffusion of the white matter tract, and (18)F-fluorodeoxyglucose positron emission tomography revealed globally improved cerebral glucose metabolism. His cognitive and social-emotional performances also improved at 2 years after VNS. To the best of our knowledge, this is the first report to describe the effects of VNS on fimbria-fornix and glucose metabolism in MAE.

Hybrid QUS Radiomics: A Multimodal-Integrated Quantitative Ultrasound Radiomics for Assessing Ambulatory Function in Duchenne Muscular Dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is a neuromuscular disorder that affects ambulatory function. Quantitative ultrasound (QUS) imaging, utilizing envelope statistics, has proven effective in diagnosing DMD. Radiomics enables the extraction of detailed features from QUS images. This study further proposes a hybrid QUS radiomics and explores its value in characterizing DMD. METHODS: Patients (n = 85) underwent ultrasound examinations of gastrocnemius through Nakagami, homodyned K (HK), and information entropy imaging. The hybrid QUS radiomics extracted, selected, and integrated the retained features derived from each QUS image for classification of ambulatory function using support vector machine. Nested five fold cross-validation of the data was conducted, with the rotational process repeated 50 times. The performance was assessed by averaging the areas under the receiver operating characteristic curve (AUROC). RESULTS: Radiomics enhanced the average AUROC of B-scan, Nakagami, HK, and entropy imaging to 0.790, 0.911, 0.869, and 0.890, respectively. By contrast, the hybrid QUS radiomics using HK and entropy images for diagnosing ambulatory function in DMD patients achieved a superior average AUROC of 0.971 (p < 0.001 compared with conventional radiomics analysis). CONCLUSIONS: The proposed hybrid QUS radiomics incorporates microstructure-related backscattering information from various envelope statistics models to effectively enhance the performance of DMD assessment.

Effects of Intensive Versus Distributed Constraint-Induced Movement Therapy for Children With Unilateral Cerebral Palsy: A Quasi-Randomized Trial.

BACKGROUND: Previous studies have compared the effectiveness of constraint-induced movement therapy (CIMT) by different training doses. However, whether the dosing schedule, that is, intensive or distributed, influences the effectiveness of CIMT in children with unilateral cerebral palsy (CP) is unknown. OBJECTIVE: To investigate the effectiveness of intensive and distributed CIMT for children with unilateral CP. METHODS: Fifty children with unilateral CP were assigned to intensive or distributed CIMT group with a total of 36 training hours. The intensive CIMT was delivered within 1 week, and the distributed CIMT was delivered twice a week for 8 weeks. The outcomes were the Melbourne Assessment 2, Box and Block Test, Pediatric Motor Activity Log-Revised (PMAL-R), Bruininks-Oseretsky test of motor proficiency 2, ABILHAND-Kids and Parenting Stress Index-Short Form. The intensive group was assessed at the initiation of treatment (week 0), at the end of 1 week treatment (week 1), and 8 weeks after the initiation of treatment (week 8). The distributed group was assessed at week 0 and week 8. RESULTS: The within-group analyses demonstrated significant differences on all motor outcomes. There were no significant between-group differences at post-treatment, while the intensive CIMT demonstrated larger improvements than the distributed CIMT did on quality of use of the more-affected hand, as rated by parents on the PMAL-R at week 8. CONCLUSIONS: The 2 dosing schedules of CIMT had similar effectiveness for children with unilateral CP. The intensive CIMT yielded additional improvement on parent rated motor quality of the more-affected hand at 8 weeks after the initiation of treatment. TRIAL REGISTRATION: ClinicalTrials.gov (ID: NCT03128385).

Acoustic radiation force impulse shear wave elastography quantifies upper limb muscle in patients with Duchenne muscular dystrophy.

We investigated whether the upper limb muscle stiffness quantified by the acoustic radiation force impulse shear wave elastography (ARFI/SWE) is a potential biomarker for age-related muscle alteration and functional decline in patients with Duchenne muscular dystrophy (DMD). 37 patients with DMD and 30 typically developing controls (TDC) were grouped by age (3-8, 9-11, and 12-18 years). ARFI/SWE measured the biceps and deltoid muscle's shear wave velocities (SWVs). Performance of Upper Limb Module (PUL 1.2 module) assessed muscle function in DMD patients. Mann Whitney test compared muscle SWVs between DMD and TDC, stratified by three age groups. We used analysis of variance with Bonferroni correction to compare muscle SWVs between DMD and TDC and correlated muscle SWVs with PUL results in the DMD group. Results showed that the SWVs of biceps differentiated DMD patients from TDC across age groups. Younger DMD patients (3-8 years) exhibited higher SWVs (p = 0.013), but older DMD patients (12-18 years) showed lower SWVS (p = 0.028) than same-aged TDC. DMD patients had decreasing biceps SWVs with age (p < 0.001), with no such age effect in TDC. The SWVs of deltoid and biceps positively correlated with PUL scores (r = 0.527 âˆ¼ 0.897, P < 0.05) and negatively correlated with PUL timed measures (r = -0.425 âˆ¼ -0.542, P < 0.05) in DMD patients. Our findings suggest that ARFI/SWE quantifying the SWVs in upper limb muscle could be a potential biomarker to differentiate DMD from TDC across ages and that DMD patients showed age-related muscle alteration and limb functional decline.

Multidimensional Development and Adaptive Behavioral Functioning in Younger and Older Children With Rett Syndrome.

OBJECTIVE: The purpose of this study was to examine clinical severity, multidimensional development, and adaptive behavioral functioning in younger and older children with Rett syndrome (RTT) in the pseudostationary stage (stage III). METHODS: Fourteen younger (≤10 years of age) and 15 older (11-18 years of age) children with confirmed stage III RTT (assigned to young-RTT and old-RTT groups, respectively) participated in this study. Clinical severity was determined using the Clinical Severity Score (CSS) scale for RTT. The children's cognitive, language, motor, and sociocommunicative development was assessed using the Mullen Scales of Early Learning (MSEL) and the Early Social Communication Scale (ESCS). Their adaptive behavioral and daily functional skills were assessed using the Vineland Adaptive Behavior Scales-Chinese version (VABS-C) and Pediatric Evaluation of Disability Inventory-Chinese version (PEDI-C). RESULTS: Compared with the young-RTT group, the old-RTT group had higher severity of scoliosis on the CSS scale, poorer fine motor scores on the MSEL, reduced eye contact, reduced alternating eye gaze, and reduced turn-taking during social interaction on the ESCS. However, none of the VABS-C or PEDI-C subscale scores differed significantly between the groups. Higher CSSs were significantly correlated with lower scores in several subscales of MSEL, ESCS, VABS-C, and PEDI-C, especially for gross motor, mobility, and socialization functioning in all children with RTT. CONCLUSION: Age-related differences in fine motor and sociocommunicative skills were observed between the young-RTT and old-RTT group, as measured using standardized assessments. Greater severity of RTT was correlated with poor motor, sociocommunicative, adaptive behavioral, and daily functional skills in stage III RTT. IMPACT: Practitioners should be aware of clinical severity and the differences of developmental and adaptive behavioral functioning between younger and older children in the pseudostationary stage of RTT to provide specific age-related treatments. LAY SUMMARY: With an understanding of severity and differences of developmental and adaptive behavioral functioning between younger and older children, clinical professionals can provide specific age-related treatments.

Commercial Exergaming in Home-Based Pediatric Constraint-Induced Therapy: A Randomized Trial.

Constraint-induced therapy (CIT) is highly effective yet not accessible to many families. Integrating commercial exergaming in home-based CIT may support the availability and attainability of the intervention. The study compared the effects of supplementary use of Nintendo Wii in home-based CIT with dose-equivalent conventional CIT. Eighteen children with cerebral palsy were randomly assigned to 8 weeks of CIT (CIT) or 4 weeks of CIT, followed by 4 weeks of Wii-augmented CIT (CIT-Wii). Outcome measures included the Bruininks-Oseretsky Test of Motor Proficiency (Manual Dexterity), the ABILHAND-Kids, the WeeFIM (Self-Care), the Test of Playfulness, the Engagement Questionnaire, and the Parenting Stress Index-Short Form. Both groups significantly improved motor outcomes and playfulness. The CIT group demonstrated greater improvement in self-care skills, whereas parental stress decreased only in the CIT-Wii group. CIT-Wii yields no significant difference in treatment effects from conventional CIT and may provide psychosocial benefits.

Measurement properties of the box and block test in children with unilateral cerebral palsy.

This study aimed to examine the reliabilities (test-retest reliability and measurement error), construct validity, and the interpretability (minimal clinically important difference) of the Box and Block Test (BBT) to interpret test scores precisely for children with UCP. A total of 100 children with UCP were recruited and 50 children from the whole sample assessed the BBT twice within 2-week interval. The BBT, the Melbourne Assessment 2, the Bruininks-Oseretsky Test of Motor Proficiency, 2nd Edition, and the Pediatric Motor Activity Log Revised were measured before and immediately after a 36-h intensive neurorehabilitation intervention. Measurement properties of the BBT were performed according to the COnsensus-based Standards for the selection of health Measurement INstruments checklist. The test-retest reliability of the BBT was high (intraclass correlation coefficient = 0.98). The measurement error estimated by the MDC95 value was 5.95. Construct validity was considered good that 4 of 4 (100%) hypotheses were confirmed. The interpretability estimated by the MCID ranged from 5.29 to 6.46. The BBT is a reliable and valid tool for children with UCP. For research and clinical applications, an improvement of seven blocks on the BBT is recommended as an indicator of statistically significant and clinically important change.

Quantifying Lower Limb Muscle Stiffness as Ambulation Function Declines in Duchenne Muscular Dystrophy with Acoustic Radiation Force Impulse Shear Wave Elastography.

Duchenne muscular dystrophy (DMD) is a progressive muscular disease, but validated imaging tools to quantify muscle microstructure alteration as mobility declines are lacking. We aimed to determine the feasibility of using acoustic radiation force impulse shear-wave elastography (ARFI/SWE) in the quantitative assessment of lower limb muscle stiffness in DMD patients. Shear wave velocities (SWVs) of lower limbs were measured in 39 DMD patients and 36 healthy controls aged 3-20 y. Mean SWV values of the controls and of the DMD patients at different ambulatory stages were compared using analysis of variance with Bonferroni correction. The DMD group had increased lower limb muscle stiffness compared with controls. Stiffness of the tibialis anterior and medial gastrocnemius muscle decreased from ambulatory to early non-ambulatory stages, whereas stiffness of the rectus femoris muscle increased from ambulatory to late non-ambulatory stages. We describe how SWV changes in lower limb muscles have the potential to predict ambulatory decline in DMD.