Maintenance of ambulation in childhood muscular dystrophy

Vignos, Paul J.; Archibald, Kenneth C.

doi:10.1016/0021-9681(60)90105-3

Cited by 138 publications

(74 citation statements)

References 5 publications

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“…In the later, nonambulatory stage of DMD, patients develop more severe contractures, specifically in the lower extremities, which may benefit from surgical or other orthopedic intervention, such as bracing or tendonectomy (149,150,174). Respiratory failure due to severe wasting of the diaphragm muscle typically presents itself following loss of ambulation and is the primary cause of death in 80% to 90% of DMD patients (48,60,71).…”

Section: Clinical Presentation Of Dmdmentioning

confidence: 98%

“…The presence of these enzymes in the serum indicates rupture of the muscle membrane. In the years following initial diagnosis, DMD patients will experience a progressive decline in mobility and the development of Achilles tendon, iliotibial band, and hamstring contractures (22,149,174). Patients typically lose the ability to climb stairs first, followed by loss of ability to stand from a supine position (22).…”

Section: Clinical Presentation Of Dmdmentioning

confidence: 99%

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Exercise and Muscular Dystrophy: Implications and Analysis of Effects on Musculoskeletal and Cardiovascular Systems

Barnabei

Martindale

Townsend

et al. 2011

Comprehensive Physiology

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The muscular dystrophies are a heterogeneous collection of progressive, inherited diseases of muscle weakness and degeneration. Although these diseases can vary widely in their etiology and presentation, nearly all muscular dystrophies cause exercise intolerance to some degree. Here, we focus on Duchenne muscular dystrophy (DMD), the most common form of muscular dystrophy, as a paradigm for the effects of muscle disease on exercise capacity. First described in the mid-1800s, DMD is a rapidly progressive and lethal muscular dystrophy caused by mutations in the dystrophin gene. Dystrophin is a membrane-associated cytoskeletal protein, the loss of which causes numerous cellular defects including mechanical instability of the sarcolemma, increased influx of extracellular calcium, and cell signaling defects. Here, we discuss the physiological basis for exercise intolerance in DMD, focusing on the molecular and cellular defects caused by loss of dystrophin and how these manifest as organ-level dysfunction and reduced exercise capacity. The main focus of this article is the defects present in dystrophin-deficient striated muscle. However, discussion regarding the effects of dystrophin loss on other tissues, including vascular smooth muscle is also included. Collectively, the goal of this article is to summarize the current state of knowledge regarding the mechanistic basis for exercise intolerance in DMD, which may serve as an archetype for other muscular dystrophies and diseases of muscle wasting.

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Section: Clinical Presentation Of Dmdmentioning

confidence: 98%

Section: Clinical Presentation Of Dmdmentioning

confidence: 99%

Exercise and Muscular Dystrophy: Implications and Analysis of Effects on Musculoskeletal and Cardiovascular Systems

Barnabei

Martindale

Townsend

et al. 2011

Comprehensive Physiology

View full text Add to dashboard Cite

show abstract

“…The Vignos scale was developed in 1960 by Vignos and Archibald. 19 It is considered a gold standard in the evaluation of staging of DMD, for it is widely used in various studies, being simple and precise. According to Archibald, the scale is composed of phases of evolution of the motor status, graduated from 0 to 10, with 0 (pre-clinical phase) and 10 (confinement to bed, needing help for all activities).…”

Section: Evaluation On the Groundmentioning

confidence: 99%

“…It is common to find children with DMD who use wheelchairs and yet are able to walk when immersed in water. [14][15][16][17][18][19] It is necessary, based on the organization of evaluation routines, to demonstrate the effects of the various physiotherapy resources that associate ground and water-immersed activities and thereby allow the development of more appropriate intervention protocols and, in the case of children with DMD, less injurious.…”

Section: Introductionmentioning

confidence: 99%

Comparison of motor function in patients with Duchenne muscular dystrophy in physical therapy in and out of water: 2-year follow-up

Ferreira¹,

Goya²,

Ferrari³

et al. 2015

Acta Fisiátrica

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The treatment for patients with Duchenne Muscular Dystrophy (DMD) is multidisciplinary. It is necessary to understand the effects of activities performed on dry ground as well as waterimmersed to allow the development of intervention protocols. Objective: To compare the motor function of children with DMD in physiotherapy carried out on the ground and immersed over a 2-year period. Method: A retrospective study, assisted by the Brazilian Association of Muscular Dystrophy (ABDIM), of 23 patients diagnosed with DMD, 8-24 years of age. We collected assessment data both water-immersed (adaptation to the water, bipedalism, sitting position, cross and longitudinal rotation, swimming, and running) and on the ground (Egan Klassifikation Scale and Vignos Scale) over a 2-year period. Results: Analyzing the ratings between semesters during the 2-year period, there were differences in the performance of water-immersed activities (p < 0.001) and there was no difference on the Egan Klassifikation Scale (p < 0.003) or the Vignos Scale (p < 0.012). Conclusion: Due to the physical properties of the water, patients either improved or maintained their scores for motor function in water immersion. However, their scores for the Egan Klassifikation and the Vignos scales, which represent motor function on the ground, diminished.

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“…Foram coletados dos prontuários, os escores obtidos a partir da aplicação da escalas clínicas de Vignos (Vignos & Archibald, 1960) A FES-DMD é uma escala brasileira que apresentou boa concordância na repetibilidade e reprodutibilidade. É composta por quatro domínios: sentar e levantar da cadeira, sentar e levantar do chão, subir e descer escadas e deambular.…”

Section: Ii-8unclassified

Caracterização da força e da função muscular nas disferlinopatias em amostra brasileira

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Maintenance of ambulation in childhood muscular dystrophy

Cited by 138 publications

References 5 publications

Exercise and Muscular Dystrophy: Implications and Analysis of Effects on Musculoskeletal and Cardiovascular Systems

Exercise and Muscular Dystrophy: Implications and Analysis of Effects on Musculoskeletal and Cardiovascular Systems

Comparison of motor function in patients with Duchenne muscular dystrophy in physical therapy in and out of water: 2-year follow-up

Caracterização da força e da função muscular nas disferlinopatias em amostra brasileira

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