Reliability and Validity of the Trunk Control Measurement Scale in Children With Duchenne Muscular Dystrophy.

While trunk control is essential for daily activities and motor function, there is currently sparse information regarding the psychometric properties of the outcome measures used for assessing trunk control in Duchenne muscular dystrophy (DMD), characterized by proximal muscle weakness. The Trunk Control Measurement Scale (TCMS) was developed to assess trunk control in children with cerebral palsy. We aimed to examine the reliability and validity of the Turkish version of the TCMS for children with DMD. We determined the functional level of 52 children with DMD (median age: 8.83 years) with the Vignos Scale, and we further evaluated them with the Motor Function Measure (MFM), a gold standard for determining the validity of the TCMS. Internal consistency of the Turkish TCMS was excellent with a Cronbach's alpha of .95. Intraclass correlation coefficient (ICC) values for intra-rater reliability were between 0.90-0.96 and for inter-rater reliability were between 0.91-0.95. We reported positive moderate to strong correlations between the TCMS and trunk flexors strength (p < .001, r = 0.77), back extensors strength (p < .001, r = 0.68) and MFM (p < .001, r = 0.85). Thus, we found the Turkish version of TCMS to be a reliable and valid instrument for assessing trunk control of children with DMD in clinical and research settings.

Effects of cycling training on balance and gait in children with Duchenne muscular dystrophy: A randomized controlled study.

BACKGROUND AND AIMS: Although aerobic exercises such as cycling and swimming are increasingly being recommended in Duchenne muscular dystrophy (DMD), their effect on gait and balance parameters is unclear. This study was aimed to investigate the effect of cycling training on balance and spatio-temporal gait parameters in children with DMD. METHODS: Ambulant children (age range: 6.17-11.33 years) were randomly divided into two groups: home-based exercise training applied in the control group (n = 12) while 12 weeks of supervised submaximal lower extremity cycling training in addition to home-based exercise training performed in the study group (n = 11). Gait and balance parameters were evaluated using the GAITRite electronic walkway system and the Bertec Balance Check Screener™, respectively. Assessments were applied before and after 12 weeks of training. RESULTS: The mean ages of the children in the study and control groups were 8.20 (SD:1.34) and 8.86 (SD:1.30) years, consecutively (p > 0.05). Considering the baseline values, the balance and spatio-temporal gait parameters of the children were similar except for the antero-posterior postural sway on the perturbed surface with eyes open (p > 0.05). There was a significant time x group interaction effect in favor of the study group for the antero-posterior postural sway of children on the normal surface with eyes open (F (1,58) = 12.62, p = 0.002). It was found that the antero-posterior postural sway on the normal surface with eyes open was improved in the study group within group comparison (F (1,10) = 8.50, p = 0.015). CONCLUSIONS: The study showed that both the cycling and the home-based exercise training groups may maintain gait and balance parameters during the study. Adding a cycling training to the rehabilitation program can also provide additional contribution to improve antero-posterior balance.

Cognitive and emotional-behavioural outcomes of Turkish Duchenne muscular dystrophy population and its association with motor function.

PURPOSE: The aim of this study was to examine the cognitive and emotional-behavioural outcomes of Turkish children with Duchenne muscular dystrophy (DMD) in comparison with healthy peers, to determine its relationship with motor functions, and to analyse the difference of cognitive and emotional-behavioural outcomes according to the site of mutations. METHOD: Children aged 7-16 years with DMD (n = 68) and age-matched typically developing children (n = 33) were included in the study. The cognitive and emotional-behavioural status and the motor functions were assessed in detail. Children with DMD also divided into two groups as "proximal" and "distal" site mutation groups to compare the cognitive and emotional-behavioural outcomes. RESULTS: The children with DMD and typically developing children were similar in terms of age and body mass index (p > 0.05). Significant differences were found between children with DMD and typically developing peers in almost all subtests of both cognitive and emotional-behavioural assessments (p < 0.05). Cognitive and emotional-behavioural parameters were weakly correlated with specific motor parameters responsive to cognitive functioning (p < 0.05). Children with distal site mutation performed significantly worser than those with proximal site mutation in particular cognitive subtest (p < 0.05). CONCLUSIONS: It is concluded that comprehensive and detailed evaluation of cognitive and emotional-behavioural features of children with DMD is essential for better implementation of rehabilitation programs to maintain motor function which especially requires cognitive ability, since a Turkish cohort represented challenges in particular domains of cognitive and emotional-behavioural areas. CLINICAL TRIAL REGISTRATION NUMBER: NCT05661071.

The Impact of Postural and Anthropometric Properties of Foot and Ankle on Physical Performance and Ambulation of Patients with Duchenne Muscular Dystrophy.

BACKGROUND: Abnormal foot anthropometry and posture of patients with Duchenne Muscular Dystrophy (DMD) can be considered as possible risk factors for performance and ambulation. It was aimed to examine the effects of foot posture and anthropometric characteristics, which deteriorated from the early period, on ambulation and performance of patients with DMD. METHODS: The foot arch height (FAH), the metatarsal width (MW), subtalar pronation angle, and ankle limitation degree (ALD) were evaluated to determine the foot anthropometric characteristics of the patients. Foot Posture Index-6 (FPI-6) was used to evaluate foot posture. The performance of the patients was determined by the 6-minute walk test (6MWT), the 10-meter walk test (10MWT), and ascend/descend a standard 4-step test, and the ambulation was determined by the North Star Ambulatory Assessment (NSAA). Spearman's correlation coefficient was calculated to assess the relationship between foot anthropometric characteristics and posture, and performance and ambulation. RESULTS: The sample consisted of 48 patients with DMD aged 5.5 to 12 years. Both of the right and left foot FPI-6 scores were associated with all parameters, except descending 4-step. The left FAH was associated with 6MWT and NSAA, and the left MW was associated with 6MWT. The ALD of right foot was associated with 6MWT, ascending/descending 4 steps, and NSAA, and left ankle limitation was associated with NSAA (p<0.05). There was no relationship between other parameters. CONCLUSIONS: These findings suggest that postural disorders in the foot and ankle may contribute to the decrease in performance and ambulation in patients with DMD.

Sleep quality and daytime sleepiness amongst family caregivers of children with Spinal Muscular Atrophy.

BACKGROUND: While there are limited studies focusing on sleep quality of family caregivers of children with Spinal Muscular Atrophy (SMA), there are no studies on daytime sleepiness in SMA. AIMS: This study aimed a) to compare the sleep quality and daytime sleepiness between caregivers of children with SMA and those of healthy peers and b) to investigate the sleep quality and daytime sleepiness of family caregivers of children with different types of SMA. METHODS AND PROCEDURES: This study included 30 family caregivers of children with SMA (SMA Type 1:12, SMA Type 2:10, and SMA Type 3:8) and 31 family caregivers of healthy peers. Sleep quality and daytime sleepiness of family caregivers were evaluated using the Pittsburg Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (EPS), consecutively. OUTCOMES AND RESULTS: Demographic characteristics of children with SMA and healthy peers were similar (p > 0.05). The mean ages of family caregivers of children with SMA and healthy peers were 36.07 ± 5.84 and 35.26 ± 5.02 years, respectively (p = 0.6). The PSQI scores of family caregivers of children with SMA (7.50 ± 3.90 points) were lower than those of healthy peers (4.09 ± 1.97 points) (p < 0.001). There was no difference in PSQI scores between SMA types (p = 0.8). Also, no difference was found between SMA types and between SMA and healthy peers in terms of EPS (p > 0.05). CONCLUSIONS AND IMPLICATIONS: Family caregivers of children with SMA had poor sleep quality but similar daytime sleepiness compared with those of healthy peers. Among SMA types, family caregivers had similar sleep quality and daytime sleepiness. It was demonstrated that the sleep quality of family caregivers should be taken into consideration in the disease management of SMA.

Motor imagery ability of children with duchenne muscular dystrophy: Reliability and validity of kinesthetic and Visual Imagery Questionnaire-10, and its association with cognitive status.

PURPOSE: To investigate validity and reliability of the Kinesthetic and Visual Imagery Questionnaire-10 (KVIQ-10) in children with Duchenne Muscular Dystrophy (DMD), to compare the motor imagery (MI) ability with age-matched controls, and to examine the relationship between MI ability and cognitive status. METHODS: The research involved 38 children who were diagnosed with DMD, as well as 20 healthy controls aged between 7 and 18 years. The KVIQ-10 was assessed for its test-retest reliability, internal consistency, construct and concurrent validity. The Motor Imagery Questionnaire for Children (MIQ-C) was selected as the gold standard test for concurrent validity. Cognitive function was assessed using the Modified Mini Mental Test (MMMT) and Montreal Cognitive Assessment (MoCA). RESULTS: KVIQ-10 showed excellent test-retest reliability (ICC>0.90) and high internal consistency (Cronbach's alpha>0.70). A moderate-to-strong association was found between KVIQ-10 and MIQ-C subscales (p < 0.001). KVIQ-10 and MIQ-C subscores were statistically lower in the DMD group (p ≤ 0.05). A correlation was found between MoCA and KVIQ-10 in children with DMD (p ≤ 0.05). CONCLUSIONS: The KVIQ-10 is a reliable and valid measure to assess the MI ability of children with DMD whose imagery ability was determined to be impaired. CLINICAL TRIAL REGISTRATION NUMBER AND URL: NCT05559710 (https://classic. CLINICALTRIALS: gov/ct2/show/NCT05559710?term=NCT05559710&draw=2&rank=1).

Development of a new instrument to evaluate gait characteristics of individuals with Duchenne Muscular Dystrophy: Gait Assessment Scale for Duchenne Muscular Dystrophy, and its validity and reliability.

BACKGROUND: Patients with Duchenne Muscular Dystrophy (DMD) have gait disorders. Therefore, specific gait assessment tools are needed. AIMS: The aim of this study was to develop a gait assessment instrument for DMD patients (DMD-GAS), and investigate its validity and reliability. STUDY DESIGN: The scale was developed considering the expert opinions which included 10 physiotherapists who had experience in the management of patients with DMD, and the Content Validity Index (CVI) was calculated. The final version of the DMD-GAS that was agreed upon the experts consisted of 10 items, and each item scored between 0 and 2. The intra-rater reliability was established by the video analysis of children with a 1-month interval and inter-rater reliability was determined by the scores of 3 physiotherapists. SUBJECTS: The study included 56 patients with DMD. OUTCOME MEASURES: The criterion validity was determined by investigating the relationship between the total score of the DMD-GAS and Motor Function Measure (MFM), 6 Minute Walk Test (6MWT), and the data obtained from GAITRite. RESULTS: The CVI of the DMD-GAS was 0.90 (p < 0.05). The construct validity and internal consistency of the DMD-GAS were excellent as well as the intra- and inter-rater reliability (>0.90). Moderate-to-very strong correlations were found between the total score of the DMD-GAS and the MFM-total score (r = 0.78), 6MWT (r = 0.71), gait speed (r = 0.50), stride length (r = 0.56), and base of support (r = -0.70) (p < 0.01). CONCLUSIONS: The results indicated that DMD-GAS was a reliable and valid instrument to determine gait characteristics of the patients with DMD in clinical settings. CLINICAL TRIAL NUMBER: NCT05244395.

Intensive Therapy of the Lower Limbs and the Trunk in Children with Bilateral Spastic Cerebral Palsy: Comparing a Qualitative Functional and a Functional Approach.

Few studies have examined the effect of intensive therapy on gross motor function and trunk control in children with cerebral palsy (CP). This study evaluated the effects of an intensive burst of therapy on the lower limbs and trunk by comparing qualitative functional and functional approaches. This study was designed as a quasi-randomized, controlled, and evaluator-blinded trial. Thirty-six children with bilateral spastic CP (mean age = 8 y 9 mo; Gross Motor Function Classification II and III) were randomized into functional (n = 12) and qualitative functional (n = 24) groups. The main outcome measures were the Gross Motor Function Measure (GMFM), the Quality Function Measure (QFM), and the Trunk Control Measurement Scale (TCMS). The results revealed significant time-by-approach interaction effects for all QFM attributes and the GMFM's standing dimension and total score. Post hoc tests showed immediate post-intervention gains with the qualitative functional approach for all QFM attributes, the GMFM's standing and walking/running/jumping dimension and total score, and the total TCMS score. The qualitative functional approach shows promising results with improvements in movement quality and gross motor function.

Muscular endurance and its association with neck pain, disability, neck awareness, and kinesiophobia in patients with chronic neck pain.

BACKGROUND: Recent studies revealed the connection between the endurance of neck muscles and pain, neck awareness, and fear of movement in patients with chronic neck pain in literature. OBJECTIVES: To investigate the association between cervical, scapular, trunk, and upper extremity muscles' endurance and neck pain, disability, neck awareness, and kinesiophobia in patients with chronic neck pain. DESIGN: Cross-sectional, observational study. METHODS: Thirty-six patients with chronic neck pain (aged 18-65 years) were included in the study. Endurance tests were performed for 9 muscles/muscle groups in the cervical and scapular region, upper limb, and trunk. Pain severity, neck disability, neck awareness, and fear of movement were measured by Visual Analog Scale (VAS), Neck Disability Index (NDI), Fremantle Neck Awareness Questionnaire (FreNAQ), and Tampa Scale of Kinesiophobia (TSK), respectively. RESULTS: Negative, weak-to-moderate relations were found between VAS (at rest and during activity) and the endurance of muscles evaluated in the cervical and scapular regions, upper extremity, and trunk as well as between NDI and endurance of the same muscles, similar to the relations between FreNAQ scores and the endurance of the cervical flexor, anterior trunk flexor, and upper extremity muscles (p < 0.05). No relation was found between the endurance of the muscles and TSK (p > 0.05). CONCLUSIONS: Since the decrease in upper extremity, scapular region, and trunk muscles' endurance may contribute to neck pain and disability, and decrease neck awareness in patients with chronic neck pain; the evaluation of muscular endurance of upper body and trunk should also be considered. CLINICAL TRIALS: NCT05121467.

Lower extremity proprioception and its association with activity and participation in children with unilateral spastic cerebral palsy.

OBJECTIVE: This cross-sectional study aimed to assess lower extremity proprioception and investigate its relationship to activity and participation levels in children with unilateral spastic cerebral palsy (USCP). METHODS: A total of 22 children with USCP between the ages of 5 and 16 years participated in this study. Lower extremity proprioception was evaluated with a protocol that consisted of verbal and location identification, unilateral and contralateral limb matching, and static and dynamic balance tests performed with the impaired and less impaired lower extremities under eyes-open and eyes-closed conditions. Furthermore, the Functional Independence Measure (WeeFIM) and Pediatric Outcomes Data Collection Instrument (PODCI) were used to evaluate the independence levels in daily living activities and participation levels. RESULTS: Children demonstrated proprioceptive loss, as evidenced by an increase in matching errors under the eyes-closed condition compared to the eyes-open condition (p<0.05). Also, the impaired extremity had greater proprioceptive loss than the less impaired extremity (p<0.05). The 5-6-year age group experienced greater proprioceptive deficits than did the 7-11 and 12-16 age groups (p<0.05). Children's lower extremity proprioceptive deficit was moderately associated with their activity and participation levels (p<0.05). CONCLUSIONS: Our findings suggest that treatment programs based on comprehensive assessments, including proprioception, may be more effective in these children.

Validity and reliability of the turkish version of the early activity scale for endurance in preschool children with cerebral palsy.

This study presented the Turkish translation of the Early Activity Scale for Endurance (T-EASE) which was developed to evaluate the endurance for physical activity of preschool children with Cerebral Palsy (CP), and its validity and reliability. Fifty-five children with CP aged between 2 and 5 years were included in the study. The Gross Motor Function Classification Scale (GMFCS) and Pediatric Outcomes Data Collection Instrument (PODCI) were used to determine motor function levels and quality of life of the study population. The T-EASE had good test-retest reliability (ICC = 0.996), internal consistency (α = 0.903), higher construct validity approved by confirmatory factor analysis, and criterion validity supported by the weak-to-strong correlations between T-EASE and GMFCS, and PODCI. The T-EASE scores were significantly different between GMFCS levels (p < .05). The T-EASE was found to be valid and reliable to determine the endurance for physical activity of Turkish pre-school children with CP with the sensitivity to changes in motor function levels.

Examination of the relationship between foot-body posture and balance and gait in Duchenne muscular dystrophy.

BACKGROUND: Although the gait and balance disturbances of Duchenne muscular dystrophy (DMD) patients were evaluated by using different methods in literature, the impact of the foot and body posture on gait and balance has not been clearly described yet in DMD. AIM: The aim of this study was to examine the relationship between foot-body posture and gait and balance in patients with DMD. METHODS: Ambulatory patients with DMD who had > 90° range of motion at ankle joint were included in the study. Foot and body posture were evaluated with the Foot Posture Index (FPI-6) and the New York Posture Rating (NYPR). The limitation degree at the ankle joint (ALD) was recorded. Gait characteristics and balance were evaluated objectively by using the GAITRite system and the Bertec Balance Check Screener™ force platform system, respectively. RESULTS: A total of 38 ambulatory patients with DMD (age: 92.44 ± 17.91 months) were recruited. Both the right and left foot FPI-6 scores were correlated with GAITRite parameters such as ambulation time, gait speed, number of steps, and left and right stride lengths (p < 0.05). A relationship was determined between right foot FPI-6 score and anterior-posterior limits of stability (LoS) obtained by force platform (p < 0.05). NYPR score was also correlated with the postural control parameters such as left and right base of support and anterior posterior LoS (p < 0.05). CONCLUSION: The current findings indicate a contribution of foot and body posture to gait and balance disorders of children with DMD. CLINICAL TRIAL NUMBER: NCT04353167, Date of registration: April 16, 2020.

A New Instrument to Assess Dynamic Balance in Children with Duchenne Muscular Dystrophy: Four Square Step Test and Its Validity, Reliability and Feasibility.

AIM: The aim of this study was to investigate validity, reliability and feasibility of the Four Square Step Test (FSST) in children with Duchenne Muscular Dystrophy (DMD). METHODS: The study included 44 children with DMD aged 5-12 years. The functional level of the children was determined by using the Brooke Lower Extremity Functional Classification (BLEFC). The concurrent validity of the FSST, a dynamic balance assessment, was investigated by analyzing the association between FSST and Timed Up and Go Test (TUG), North Star Ambulatory Assessment (NSAA) and Six-Minute Walk Test (6MWT) distance. The intra- and inter-rater reliability of the FSST was determined by using the Intra Class Correlation Coefficient (ICC) while the feasibility of the test was determined by calculating the percentage of children who successfully completed the test in accordance with the test instructions. RESULTS: The FSST score of the children (mean age 9.25 ± 2.14 years) was 11.91 ± 3.43 and the BLEFC level ranged from Level 1 (65.9%) to Level 3 (9.1%). FSST had positive, moderate relation with BLEFC (r = 0.447; p = .002); positive, strong relation with TUG (rs = 0.623), and negative, moderate correlations with NSAA (rs = -0.529) and 6MWT distance (rs = -0.592) (p < .001). Intra-rater (ICC: 0.965) and inter-rater (ICC: 0.991) reliability was "excellent," and the test was feasible with successful performance of 88% children. DISCUSSION: The FSST is a valid, reliable and feasible instrument to assess dynamic balance as well as having the ability to reflect falling risk and different functional levels of children with DMD.

The Impact of "Fear of Falling" on Physical Performance, Balance, and Ambulation in Duchenne Muscular Dystrophy.

OBJECTIVE: The aim of this study was to investigate falls and the fear of falling (FOF) in children with Duchenne muscular dystrophy (DMD) and to determine the relationships between the FOF and physical performance, balance, and ambulation. METHODS: Thirty-eight ambulatory children with DMD were included in the study. The functional level, falling history, FOF, physical performance, balance, and ambulation were assessed by using Brooke Lower Extremity Functional Classification, History of Falls Questionnaire, Pediatric Fear of Falling Questionnaire (Ped-FOF), timed performance tests, Timed "Up and Go" (TUG) test, and North Star Ambulatory Assessment (NSAA), respectively. RESULTS: Of the 38 children (mean age: 9.00 ± 2.03 years) 97.4% had a history of serious fall last year and 62.2% were injured due to this fall. The Ped-FOF score was 13.79 ± 7.20. Weak to moderate relations were determined between Ped-FOF and functional level (r = 0.33), frequency of falls (r = 0.41), duration of climbing 4-steps (r = 0.38), TUG (r = 0.36), and NSAA (r = -0.32) (p < 0.05). CONCLUSION: Ambulatory children with better performance scores had lower levels of FOF despite their history of serious falls and injuries. FOF tends to increase as the symptoms of the disease progresses. Investigating the history of falls and FOF from the earliest period will guide to take precautions and make necessary interventions on time in treatment programs.

Feasibility and Safety of Applying the Functional Electrical Stimulation to Child with Facioscapulohumeral Dystrophy: A Case Report.

AIMS: This study aims to investigate the feasibility and safety of short-term functional electrical stimulation (FES) training of the quadriceps femoris muscles in a child with facioscapulohumeral muscular dystrophy (FSHD). METHODS: A 7-year-old child with FSHD received treatment due to a decrease in functional performance and difficulty climbing stairs. The child was followed up with a home-based exercise program. FES was applied twice a week during stair climbing for six weeks. Muscle activation of the quadriceps femoris was measured using superficial electromyography, muscle strength was measured with a hand-held dynamometer, and functional performance was assessed with the 6-Minute Walk and the Stair Climb Tests before and after the treatment period. RESULTS: At the end of the treatment, there was an improvement in muscle activation. While muscle strength increased in the quadriceps femoris muscle of the non-dominant side, it remained constant on the dominant side. Functional performance test results also improved. CONCLUSIONS: FES was a feasible and safe tool to use in our case, a child with FSHD.

The effect of aerobic training on motor function and muscle architecture in children with Duchenne muscular dystrophy: A randomized controlled study.

OBJECTIVE: To explore the effects of aerobic training adding to home-based exercise program on motor function and muscle architectural properties in children with Duchenne muscular dystrophy. DESIGN: This is a prospective randomized controlled study. SETTING: Pediatric neuromuscular clinic in a tertiary care center. SUBJECTS: Children with Duchenne muscular dystrophy. INTERVENTIONS: Children were randomly divided into two groups whereby 12-weeks aerobic training was additionally given in treatment group in contrast to the control group which received only home-based exercise program. MAIN MEASURES: Motor Function Measure and Six Minute Walk Test were used for clinical evaluation, and muscle architectural properties (thickness, pennation angle and fascicle length) were measured by ultrasound imaging. Both groups were assessed at baseline and after 12-weeks of training. RESULTS: Median age of children was 7.9 years in the treatment group and 8.6 years in the control group (p > 0.05). Significant improvements were obtained for Motor Function Measure and Six Minute Walk Test from baseline to 12-weeks in the treatment group; Motor Function Measure total score changed from 83.2 (6.1) to 86.9 (4.0) vs. 82.3 (10.2) to 80.4 (9.4) points in the control group (p = 0.006); 6 Minute Walk Test distance changed from 395.3 (46.6) to 413.0 (52.3) vs. 421.7 (64.4) to 393.8 (68.2) meters in the control group (p < 0.001). However, muscle architectural parameters did not change during study period (p > 0.05). CONCLUSION: Aerobic training may be of additional value in improving motor function and performance with no remarkable effect on muscle architectural properties.

Reliability of Isokinetic Strength Assessments of Knee and Hip Using the Biodex System 4 Dynamometer and Associations With Functional Strength in Healthy Children.

Background: This study aimed to analyze the reliability of concentric isokinetic strength assessments (knee and hip) using the Biodex System 4 in healthy children and assess the association with functional strength tests (sit-to-stand [STS], lateral-step-up [LSU]). Methods: 19 children (6-12 years) were included. Knee and hip flexion and extension, and hip abduction and adduction were tested at 60 and 90°/s. Results: Relative and absolute reliability at 60°/s tended to show better results compared to those at 90°/s. Intra class correlations (ICCs) of knee flexion and extension at 60°/s were good (0.79-0.89). For hip flexion, extension, abduction and adduction at 60°/s ICCs were moderate to good (0.53-0.83). The smallest detectable change (SDC) values (expressed in %) were highly variable. The SDC% for knee flexion and extension and hip abduction at 60°/s were around 50%. Positive associations were found between hip extension and abduction isokinetic strength and the STS test. Conclusion: Concentric isokinetic strength assessments in healthy children using the Biodex System 4 were found reliable for knee flexion and extension and hip abduction. Limited associations were found between concentric isokinetic strength tests and functional strength tests.

Ultrasonographic assessment of lower limb muscle architecture in children with early-stage Duchenne muscular dystrophy.

BACKGROUND: Muscle imaging methods such as ultrasound and magnetic resonance imaging have been used for many years to determine the dystrophic process in muscular dystrophies. However, the knowledge regarding muscle architecture in children at early-stage Duchenne muscular dystrophy (DMD) with different functional levels is limited. OBJECTIVE: To explore the effect of functional level on muscle architectural properties in children with early stage DMD and the difference between DMD and typically developing (TD) peers. METHODS: Thirty children with DMD (15 Grade 1 and 15 Grade 2 according to the Vignos Scale) and 5 TD peers were included. Ultrasound imaging was used to measure muscle thickness (MT), fascicle length (FL), and pennation angle (PA) of vastus lateralis (VL) and medial gastrocnemius (MG) muscles bilaterally. RESULTS: The MT and FL values for VL, and MT, FL and PA values for MG muscles were higher in children with DMD compared with those of TD peers (p<0.05). The FL of VL, and MT and FL of GM muscles of children with DMD Grade 2 were higher than those of children with DMD Grade 1 (p<0.05). CONCLUSIONS: MT and FL are increased in children with DMD compared with TD peers. Additionally, muscle architecture seems to be affected even at the early stages of the disease.

The association between trunk control and upper limb functions of children with Duchenne muscular dystrophy.

Introduction: The aim of this study was to investigate whether trunk control is associated with the upper limb function of children with Duchenne Muscular Dystrophy (DMD).Methods: The children included in the study were divided into two groups according to the Trunk Control Measurement Scale total scores. Twenty-five children whose trunk control levels were lower than 30.5 points were included in Group 1 while 31 children who had good levels of trunk control between 30.5 and 58 points were included in Group 2. General functional levels, muscular strength, and function of the upper limb were assessed and compared between the groups.Results: According to the results of the assessments, children in Group 2 were found to have better upper limb function and muscular strength than Group 1 (p < .001).Conclusions: The current study demonstrated better upper limb functional performance of children with DMD that had good-level trunk control thus suggesting the possible association between trunk stability and upper limb function. The results reveal the need to improve and protect trunk muscle strength and stability as well as upper limb strength, and to optimize trunk stability during upper limb movement in rehabilitation programs.

Investigation of surface electromyography amplitude values during stair climbing task in children with Duchenne muscular dystrophy.

OBJECTIVE: The aims of this study were (a) to examine the surface electromyography (sEMG) amplitude values of the lower limb muscles during stair climbing both between different functional levels of Duchenne muscular dystrophy (DMD), in comparison with healthy children, and (b) to investigate the relationships between sEMG amplitudes and physical performance. METHODS: sEMG amplitudes of the lower limbs of twenty-one children with DMD between levels I and III according to the Brooke Lower Extremity Functional Classification Scale and eleven healthy peers were evaluated by using sEMG during stair climbing task. Physical performance was evaluated by 6-min walk test and ascending 4-step timed performance test. RESULTS: The lower limb sEMG amplitude values of children with DMD were statistically higher than healthy children (p < 0.001). sEMG amplitudes of the right (p = 0.01) and left (p = 0.003) biceps femoris, the right (p < 0.001) and left (p = 0.001) gastrocnemius medialis, and the right vastus lateralis (p = 0.02) muscles were higher in children with levels 2-3 than those in level 1. Moderate-to-strong relations were found between the gastrocnemius medialis and biceps femoris sEMG amplitudes and physical performance assessments (p < 0.05). CONCLUSION: Increased sEMG amplitude values in the lower limbs during stair climbing task are thought to be caused by the effort to compensate for progressive muscle weakness and are associated with lower physical performance in children with DMD. Further, sEMG amplitude values are determined to increase as the functional level deteriorates. CLINICAL TRIAL REGISTRATION NUMBER AND URL: NCT04287582 ( https://clinicaltrials.gov/ct2/show/NCT04287582?term=merve+bora&draw=2&rank=1 ).