Lower extremity joint contracture according to ambulatory status in children with Duchenne muscular dystrophy

Choi, Young Ah; Chun, Seong Min; Kim, Yale; Shin, Hyung Ik

doi:10.1186/s12891-018-2212-6

Cited by 24 publications

(20 citation statements)

References 22 publications

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“…The muscle fibers are initially hypertrophic, albeit of poor quality [7]. Additionally, contrary to the quadriceps, the GAS is often prone to muscle contractures [48]. This increased stiffness could contribute to the net force production during gait [49], which would explain the decreased weight of the GAS in synergy one and the lack of a clear association between GAS weights.…”

Section: Plos Onementioning

confidence: 99%

Muscle weakness has a limited effect on motor control of gait in Duchenne muscular dystrophy

et al. 2020

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Our aim was to determine if synergy weights and activations are altered in Duchenne muscular dystrophy (DMD) and if these alterations could be linked to muscle weakness. Methods In 22 children with DMD and 22 typical developing (TD) children of a similar age, surface electromyography (sEMG) of the gluteus medius, rectus femoris (REF), medial hamstrings, tibialis anterior, and medial gastrocnemius (GAS) were recorded during gait. Muscle weakness was assessed with maximal voluntary isometric contractions (MVIC). Synergies were calculated with non-negative matrix factorization. The number of synergies explaining �90% of the variance in the sEMG signals (N90), were extracted and grouped with k-means cluster analysis. We verified differences in weights with a Mann-Whitney U test. Statistical non-parametric mapping (Hotelling's T 2 test and two-tailed t-test) was used to assess group differences in synergy activations. We used Spearman's rank correlation coefficients and canonical correlation analysis to assess if weakness was related to modifications in weights and activations, respectively. Results For both groups, average N90 was three. In synergy one, characterized by activity at the beginning of stance, the DMDs showed an increased REF weight (p = 0.001) and decreased GAS weight (p = 0.007). Synergy activations were similar, with only a small difference detected in mid-swing in the combined activations (p<0.001). Weakness was not associated with these differences.

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Section: Plos Onementioning

confidence: 99%

Muscle weakness has a limited effect on motor control of gait in Duchenne muscular dystrophy

et al. 2020

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“…Also, enlarged calf muscles (pseudo hypertrophy) are always noticed [4]. From the age of 7-12, the cases become more deteriorated, and the patients start to suffer from scoliosis [5], and joint contracture [6]. Also, patients will have an apparent reduction in bone-mineral density and will have hypocalciuria and osteoporosis [7].…”

Section: Methods For Dmd Diagnosis 21 Clinical Picturementioning

confidence: 99%

Duchenne Muscular Dystrophy (DMD) Diagnosis: Past and Present Perspectives

Mousa¹,

Osman²,

Fahmy³

et al. 2020

Rare Diseases

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Duchenne muscular dystrophy (DMD) is a fatal X-linked disorder, characterized by progressive skeletal muscle wasting. The disease is caused by various types of mutations in the dystrophin gene (DMD). The disease occurs at a frequency of about 1 in 5000 male births, making it the most common severe neuro-muscular disease. In addition to clinical examinations of muscle strength and function, diagnosis of DMD usually involves a combination of immunological assays using muscle biopsies, typically immunohistochemistry and western blotting, and molecular techniques such as DMD gene sequencing or Multiplex Ligation Dependent Probe Amplification (MLPA) using blood samples. In fact, precise molecular diagnosis is a prerequisite for determining the appropriate personalized therapeutic approach such as exon-skipping, gene therapy or stem cell-based therapies in conjunction with gene editing techniques like CRISPR-Cas9. However, the quest for reliable biomarkers with high sensitivity and specificity for DMD from liquid biopsy is still a hotspot of research, as such non-invasive biomarker(s) would not only facilitate disease diagnosis but would also help in carrier detection, which will eventually result in better disease management. In this chapter, we will illustrate the detailed current and prospect strategies for disease.

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“…Contractures of hip flexors, knee flexors, and ankle plantar flexors are most common in DMD patients. It is known that the loss of range of motion becomes severe when there is deterioration of ambulatory function [20]. A daily preventive home stretching program should begin before the loss of passive range of motion.…”

Section: Original Articlementioning

confidence: 99%

Establishing a telerehabilitation program for patients with Duchenne muscular dystrophy in the COVID-19 pandemic

Sobierajska-Rek

Mański

Jabłońska-Brudło

et al. 2020

Wien Klin Wochenschr

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Summary Background Duchenne muscular dystrophy is a genetic disease characterized by gradual loss of motor function, respiratory failure and cardiomyopathy. During the time of the global coronavirus pandemic, maintenance of social distancing and self-isolation might complicate regular multidisciplinary care of patients with Duchenne muscular dystrophy but on the other hand may lead to new medical care telehealth solutions. The aim of the study was to investigate patients’ situation regarding rehabilitation in the pandemic, to establish an online rehabilitation program and motor assessment and to determine the needs of telerehabilitation in this group. Methods The project involved 69 boys with Duchenne muscular dystrophy. The rehabilitation program was presented during online workshops for patients and caregivers. The same program was recorded on video and published in the internet. The online motor assessment tool consisted of six motor tests, caregivers were asked to perform the tasks and share a photograph of the patient’s posture using a designed app. Results In the nonambulant group the emphasis was placed on chest physiotherapy, stretching of upper extremities, positioning and wheelchair ergonomics. The program for the ambulant group focused on lower extremities stretching and full body exercises. Response rate for the workshops for ambulant patients was 29.7%, and only 9.0% for nonambulant patients. Videos showing exercises were displayed 132 times within a month. Conclusion With the physiotherapist guidance (online communication or video) patients with caregivers’ help can continue home based rehabilitation. Online videos/instructions/video guidelines are more acceptable by parents/caregivers of patients with Duchenne muscular dystrophy than live workshops.

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Lower extremity joint contracture according to ambulatory status in children with Duchenne muscular dystrophy

Cited by 24 publications

References 22 publications

Muscle weakness has a limited effect on motor control of gait in Duchenne muscular dystrophy

Muscle weakness has a limited effect on motor control of gait in Duchenne muscular dystrophy

Duchenne Muscular Dystrophy (DMD) Diagnosis: Past and Present Perspectives

Establishing a telerehabilitation program for patients with Duchenne muscular dystrophy in the COVID-19 pandemic

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