Factors associated with balance ability in Duchenne and Becker muscular dystrophies.

BACKGROUND: Problems such as progressive muscle function loss, postural deteriorations, and contractures seen in patients with Duchenne and Becker muscular dystrophies (D/BMD) may affect children's balance ability, and impaired balance ability may lead to limitations in children's functional level. RESEARCH QUESTION: What factors are associated with balance ability in children with D/BMD? METHODS: Twenty participants with D/BMD were included in the study. Participants' ages were asked; height, body weight, and body mass index (BMI) were recorded. Children's functional level was assessed with the Motor Function Measurement-32 (MFM-32), Brooke and Vignos Scales. Muscle strength of hip flexion and extension, knee flexion and extension, and ankle dorsiflexion was measured with a handheld dynamometer. Balance measurements were performed using the Balance Master System. The relationship between balance and continuous independent variables was determined using Spearman's test. RESULTS: The mean age of the participants was 8.57 ± 3.27 years. The balance abilities of children with BMD were better than those of children with DMD (p < 0.05). The balance diminished with age (p < 0.05), while there was a positive correlation between balance and weight, height, BMI (p < 0.05). There was a positive correlation between the balance and the MFM-32 total and subsection scores. Muscle strength was positively related to balance (p < 0.05). SIGNIFICANCE: The results showed the balance ability in children with D/BMD was affected by age, height, weight, BMI, functional level, and muscle strength. Based on the results of this study, balance and strength training should be an integral part of the rehabilitation of children with D/BMD.

Reliability and validity of the Turkish version of the Upper Limb Short Questionnaire in Duchenne muscular dystrophy.

PURPOSE: This study aimed to evaluate the construct validity and reliability of the Turkish version of the Upper Limb Short Questionnaire in Duchenne muscular dystrophy. MATERIALS AND METHODS: A total of 41 children with Duchenne muscular dystrophy have participated in the study. Upper and lower extremities functional levels were assessed with Vignos Scale and Brooke Upper Extremity Functional Rating Scale, respectively. The construct validity of the questionnaire was determined using the correlation between the Upper Limb Short Questionnaire and ABILHAND-Kids. The Cronbach alpha value was calculated to determine internal consistency. To determine test-retest reliability, 17 randomly selected children were evaluated seven days after the first evaluation, and the 'Intraclass Correlation Coefficient' value was calculated. RESULTS: There was a moderate level of positive correlation between the Upper Limb Short Questionnaire scores and the Vignos Scale (r = 0.52, p < 0.001) and the Brooke Upper Extremity Functional Rating Scale (r = 0.65, p < 0.001). There was a strong correlation between the Upper Limb Short Questionnaire and ABILHAND-Kids in the negative direction (r= -0.80, p < 0.001). Internal consistency of the ULSQ was fairly high (Cronbach's alpha = 0.785) and test-retest reliability was good (ICC = 0.86). CONCLUSION: The Turkish version of Upper Limb Short Questionnaire is a valid and reliable scale for children with Duchenne muscular dystrophy. It can be a useful tool in the UE clinical evaluation of children with Duchenne muscular dystrophy.

The psychometric properties of Turkish version of the Modified Paediatric Mini Mental Scale.

BACKGROUND: Children with neurological diseases suffer from neurocognitive problems due to both the disease and the treatment processes. Therefore, it is necessary that a battery can be used to determine and track the cognitive function of these children. The aim of this study is to establish the Turkish version of the Modified Paediatric Mini Mental Scale (MPMMS), ensure its cultural adaptation, and test the validity and reliability of the Turkish version of the MPMMS. METHODS: Sixty-five children with the neurological condition were enrolled in this methodological study. The subjects' age, height, weight, and body mass index were recorded. The MPMMS and the social function subscale of the Paediatric Evaluation of Disability Inventory (PEDI) were used to assess the participants' cognitive function. The construct validity of the questionnaire was determined by the correlation between the MPMMS and the social function subscale of the PEDI. Cronbach's alpha was calculated to determine internal consistency. To determine test-retest reliability, 32 children were assessed 7-14 days after the initial assessment, and the intraclass correlation coefficient (ICC) was calculated. RESULTS: The mean age of the participants was 9.26 ± 3.87 years. A very strong significant correlation was found between the MPMMS and social function subscale of the PEDI (r = 0.935, p = 0.000). The internal consistency of the MPMMS was excellent (Cronbach's alpha = 0.932). CONCLUSIONS: The Turkish version of the MPMMS has excellent validity and reliability and can be used by professionals in various health care settings to determine children's cognitive abilities.

Functional level and its relationship to upper extremity function, pain, and muscle stiffness in children with Duchenne muscular dystrophy.

BACKGROUND: The progressive symptoms of Duchenne muscular dystrophy (DMD) negatively affect upper extremity skills, and this may have an effect that reduces the independence of daily life. AIMS: The purpose of this study is to investigate the relationship between functional level and upper extremity function, pain, and stiffness in children with DMD. METHODS: A total of 38 children with DMD were participated. The functional level of the upper and lower extremities was assessed using Brooke scale and Vignos scale. Upper extremity function, pain and stiffness were assessed using Upper Limb Short Questionnaire (ULSQ). The correlation between ULSQ and Brooke and Vignos scales was calculated. RESULTS: A moderate positive correlation was calculated between ULSQ total scores and Vignos scale (r = 0.52, p < 0.001) and Brooke scale (r = 0.65, p < 0.001). There was a moderate positive correlation between Vignos scale scores and ULSQ subscores of function (r = 0.42, p < 0.05) and stiffness (r = 0.56, p < 0.001); no significant correlation was found between pain scores and Vignos scale (p = 0.053). There was a moderate positive correlation between the function (r = 0.54, p < 0.001), pain (r = 0.40, p < 0.05), and stiffness (r = 0.62, p < 0.001) subscores of the ULSQ with the Brooke scale. CONCLUSION: In our study, there was a significant relationship between the functional level of patients with DMD and upper extremity function, pain, and muscle stiffness.

The COVID-19 pandemic restrictions affect the healthcare and health status of paediatric patients with neuromuscular diseases: a developing country perspective.

PURPOSE: The COVID-19 pandemic has forced the rapid and unprecedented reorganisation of current practices in the treatment of neuromuscular disorders (NMD). Cessation of care and treatments can worsen the underlying condition, exacerbate symptoms, and increase anxiety, leading to a vicious circle and increased management concerns. This study aims to determine the changes in healthcare and health status of children with NMD from a developing country perspective. MATERIALS AND METHODS: Forty-seven children with NMD were included in this cross-sectional study. The participants were contacted via phone call. The survey conducted for the study was included demographic data, changes and problems in healthcare, perceived health status change, and satisfaction with the services provided. Descriptive statistics were used to characterise the sample. RESULTS: The mean age of the children was 7.86 ± 3.45 years. The participants encountered 24.83 ± 26.54% of difficulties in getting medication care, and there was 69.95 ± 24.47% disruption in accessing routine medical care. The participants' rehabilitation sessions were disrupted in the 78.54 ± 14.93%, and there were 95.83 ± 10.03% deficiencies in therapists' informing. Children with NMD indicated that their perceived health status decreased compared to before pandemic in all parameters. CONCLUSION: This study highlights the unfavourable indirect effect of the COVID-19 pandemic restrictions on healthcare and health status of paediatric patients with NMD. Since the COVID-19 pandemic is an uncertain process, the solutions or modifications should be promptly put into effect to improve the healthcare and health status of children with NMD.

Stimulated biofeedback training for a child with Becker muscular dystrophy and compartment syndrome in the left forearm.

BACKGROUND: Muscular dystrophy negatively affects ambulation, mobility, self-care, and community involvement. Neonatal compartment syndrome (NCS) causes loss of muscle strength, sensory problems, and limb dysfunction. Patients with Becker Muscular Dystrophy (BMD) and/or NCS may benefit from individualized rehabilitation to improve function. PURPOSE: This case report describes stimulated biofeedback training (SBT) to improve the functional level, muscle strength, balance, and hand function in a child with BMD and NCS. CASE DESCRIPTION: An 8-year-old male patient with BMD and NCS in the left forearm received 12-weeks of SBT. The functional level was assessed by the Motor Function Measurement-32 (MFM-32), muscle strength by a hand-held dynamometer, balance by the Neurocom Balance Master, and upper limb function by the Quality of Upper Extremity Skills Test (QUEST) at the initial examination, after 6 weeks and after 12 weeks of treatment. Laboratory tests to monitor changes in serum creatine kinase were performed throughout the episode of care. OUTCOMES: The laboratory values remained within the appropriate range to continue SBT. Functional level, hand function, hip, and knee flexion/extension strength, and dorsiflexion strength improved. CONCLUSIONS: This case report suggests that SBT safely and effectively improved functional level, muscle strength, and hand function in this child with BMD and NCS.