Selective motor control correlates with gross motor ability, functional balance and gait performance in ambulant children with bilateral spastic cerebral palsy.

BACKGROUND: Selective motor control (SMC) is a fundamental component of typical human motion. As a result of brain damage, impaired SMC often leads to difficulties with coordination, balance, gait efficiency and symmetry. RESEARCH QUESTION: What is the association between impaired SMC and lower limb motor ability, functional balance and gait performance in children with bilateral spastic cerebral palsy (CP)? METHODS: Thirty-six children (aged 5-16 years) with spastic bilateral CP in Gross Motor Function Classification System (GMFCS) level I to II were included in this study. SMC was assessed using Selective Control Assessment of the Lower Extremity (SCALE). Gross motor function was assessed using Gross Motor Function Measure-88 items D and E dimension (GMFM-88 D&E). Functional balance was assessed using Pediatric Balance Scale (PBS) and Timed Up and Go Test (TUG). Gait quality was assessed using Edinburg Visual Gait Score (EVGS) and 10-Meter Walk Test (10MWT). Spearman's rank correlation analyses were used to determine the association between SMC and other factors. RESULTS: Correlation analyses showed that SCALE was strongly positively correlated with GMFM-88 (D&E) (rs=0.756, p < 0.001), PBS (rs=0.769, p < 0.001), and height-normalized fast walking speed (rs=0.632, p < 0.001), and strongly negatively correlated with TUG (rs=-0.766, p < 0.001) and EVGS (rs=-0.893, p < 0.001). SIGNIFICANCE: Lower extremity SMC deficits are associated with poor gross motor function and balance control, more severe overall gait deviations and decreased fast walking speed in children with bilateral spastic CP. Physical therapy should include interventions that promote selective motor control in order to improve overall functional ability.

Rasch analysis of the 32-item motor function measure in ambulant patients with Duchenne muscular dystrophy.

OBJECTIVE: This study analyzed the 32-item Motor Function Measure in a cohort of ambulatory patients with Duchenne muscular dystrophy using Rasch measurement methods. DESIGN: This is a psychometric study. SETTING: Rehabilitation centre of a large public children's hospital in Shenzhen, China. PARTICIPANTS: Data from 176 genetically confirmed ambulant patients with Duchenne muscular dystrophy (mean age 7.3 years, SD 2.3 years, range 3.1-13.1 years) were analyzed. RESULTS: Rasch analyses supported the Motor Function Measure domain D1 as a reliable (person reliability = 0.88, person separation index = 2.71) and valid (acceptable targeting, little misfit, minimal category disordering) measure in ambulant patients with Duchenne muscular dystrophy. Remodelling the domain D1 by collapsing item 25 from 4 to 3 response categories addressed the problematic disordered thresholds, resulting in a rebuilt domain D1 with enhanced measurement properties. However, findings for domains D2 and D3 did not fulfil most Rasch model expectations. There were disordered thresholds for most items in domains D2 and D3, with low reliability coefficients, item mistargeting and misfit, and large ceiling effects. CONCLUSION: Rasch analyses confirmed that the Motor Function Measure domain D1 was reliable and valid and provided a unidimensional measure for motor function in ambulant Duchenne muscular dystrophy patients. Accuracy of measurement had been enhanced through remodelling, and a rebuilt domain D1 with category collapsing for item 25 was proposed. The analysis revealed multiple limitations of the domains D2 and D3 that certain essential psychometrics were poorly met and, therefore, should be used with caution in this patient group.

Chronic pain, psychological distress, and quality of life in males with Duchenne muscular dystrophy.

AIM: To describe chronic pain in Duchenne muscular dystrophy (DMD) from children's/adolescents' perspectives, explore patient variables associated with self-reported pain, and examine the relationship between chronic pain, psychological functioning, and health-related quality of life (HRQoL). METHOD: This observational study included a paediatric cohort (aged 8-18 years; median age 9 years 4 months) with DMD under multidisciplinary care (n = 45). Clinical data of the latest visit were extracted from the electronic health record and assessment of pain, psychological distress, and HRQoL were performed during the same visit. RESULTS: Thirty-two patients reported pain during the previous 4 weeks, and 18 reported persistent or recurrent chronic pain. Average pain intensity of chronic pain was mild, with regions of the legs (n = 12), lower back (n = 6), hips (n = 6), and shoulder (n = 6) most frequently affected. Older age, higher body mass index, being non-ambulatory, wheelchair dependency, and spinal deformities were contextual variables related to the presence of chronic pain. Furthermore, chronic pain was significantly associated with psychological distress and reduced HRQoL in paediatric patients with DMD. INTERPRETATION: Chronic pain in paediatric DMD is frequent and widespread, highlighting the need for pain to be addressed in the routine care of affected young people. Chronic pain may make a significant contribution to psychological distress and impaired HRQoL in paediatric patients with DMD. WHAT THIS PAPER ADDS: Chronic pain differs in aetiology, scope, and nature compared with acute pain in paediatric Duchenne muscular dystrophy (DMD). Older age, higher body mass index, being non-ambulatory, wheelchair dependency, and spinal deformities are important patient variables. Chronic pain is significantly associated with psychological distress and reduced health-related quality of life in paediatric DMD.

Responsiveness and Minimal Clinically Important Difference of the Chinese Version of the Motor Function Measure-32 in Children and Adolescents with Duchenne Muscular Dystrophy.

PURPOSE: This study aimed to examine the responsiveness and minimal clinically important difference of the Motor Function Measure 32-Chinese version (MFM 32-CN) in children and adolescents with Duchenne muscular dystrophy (DMD). METHODS: This observational, prospective cohort study assessed changes in motor function over 12 months using the MFM 32-CN in 108 pediatric patients with DMD. RESULTS: Moderate to high internal responsiveness was found for MFM total, D1, and D2 scores (SRM = 0.46-0.83). Sufficient external responsiveness was observed in all MFM scores, as determined by a good correlation with the Patient Global Impression of Change (PGIC) ratings (r = 0.54-0.72, p < .01). The estimated MCID values derived from different anchor-based methods ranged from 4.7 to 6.0, with a median of 5.0 points (%). CONCLUSION: The MFM 32-CN demonstrates sufficient internal and external responsiveness as a measure of motor function in children and adolescents with DMD. The present study established the MCID of MFM-32 in pediatric patients with DMD.

The Prevalence, Characteristics and Impact of Chronic Pain in People With Muscular Dystrophies: A Systematic Review and Meta-Analysis.

Chronic pain is a frequent, yet under-recognized and under-assessed problem in people with muscular dystrophies (MDs). Knowledge of the prevalence and characteristics of chronic pain, and its impact on function and quality of life is limited and lacks systematic exploration. This article aims to systematically review and synthesize existing literature that addresses chronic pain prevalence, characteristics and impact in people with different types of MDs. The present meta-analysis showed that the estimated prevalence of chronic pain in MDs is high and appears to be similar across different diagnostic groups: 68% (95% CI: 52%-82%) in FSHD, 65% (95% CI: 51%-77%) in DM, 62% (95% CI: 50%-73%) in BMD/DMD, and 60% (95% CI: 48%-73%) in LGMD, although it should be noted that heterogeneity was high in some diagnostic groups. On average, people with FSHD and DM present with moderate pain intensity. The lumbar spine, shoulders and legs are the most frequent sites of chronic pain among people with FSHD, DM, BMD/DMD, and LGMD, with little variation. Diffuse pain across multiple body sites was reported by a notable proportion of these individuals. Chronic pain has a negative impact on daily life activities in people with MDs, and may also contribute to decreased quality of life. The protocol for this review has been published on PROSPERO (CRD42020168096). PERSPECTIVES: This is the first systematic review and meta-analysis exploring the prevalence, and nature and impact of chronic pain in people with MDs. The present study demonstrates how common chronic pain is across various MD populations and highlights the need for better recognition and understanding of the nature and impact of pain from health professionals.

Cross-cultural Adaptation and Multi-centric Validation of the Motor Function Measure Chinese Version (MFM-32-CN) for Patients with Neuromuscular Diseases.

Aim: This study aims to produce a Chinese translation and adaptation of the MFM-32 and examine its measurement parametric properties in Chinese population with neuromuscular diseases. Methods: The MFM-32-CN was validated by testing reliability, internal consistency, and construct validity. Results: The Interrater and intrarater reliability of MFM-32-CN were moderate to excellent for each item, and excellent for the total score and the subscores. The internal consistency was high for the global scale and subscales. The total score was strongly related to the VAS and Vignos grade, and to a lesser degree to the Brooke grade. The discriminant validity was good. Conclusion: MFM-32-CN has satisfactory reliability, internal consistency, convergent validity and discriminant validity in youngsters with DMD, and good psychometric properties have also been observed for the whole population tested combining different NMD groups, but future study with a larger number of participants in each disease group is needed to confirm the present results.