Functional ability and muscle force in healthy children and ambulant Duchenne muscular dystrophy patients

Beenakker, E. A. C.; Maurits, Natasha M.; Fock, Johanna M.; Brouwer, Oebele F.; Hoeven, Johannes H. van der

doi:10.1016/j.ejpn.2005.06.004

Cited by 84 publications

(90 citation statements)

References 8 publications

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“…Studies on scales for patients with neuromuscular di seases emphasize muscle strength, but postulate that there is no direct relationship between strength and functional per formance 10,17,19,20 . However, Doglio et al reported that some motor strategies may be adopted to compensate for muscle weakness and postpone wheelchair dependency 21 .…”

Section: Discussionmentioning

confidence: 99%

Compensatory movements during functional activities in ambulatory children with Duchenne muscular dystrophy

Martini

Voos

Hukuda

et al. 2014

Arq. Neuro-Psiquiatr.

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Objective: During the transitional phase (ambulatory to non-ambulatory), synergies characterize the evolution of Duchenne muscular dystrophy (DMD). This study was performed to describe and quantify compensatory movements while sitting down on/rising from the floor and climbing up/down steps. Method: Eighty videos (5 children × 4 assessments × 4 tasks) were recorded quarterly in the year prior to gait loss. Compensatory movements from the videos were registered based on the Functional Evaluation Scale for DMD. Results: The most frequently observed compensatory movements were upper limb support on lower limbs/floor/handrail during all the tasks and lumbar hyperlordosis, trunk support on handrail, equinus foot, increased base of support, non-alternated descent, and pauses while climbing up/down steps. Conclusion: Climbing up/down steps showed a higher number of compensatory movements than sitting down on/rising from the floor, which seemed to be lost before climbing up/down steps in ambulatory children with DMD.Keywords: Duchenne muscular dystrophy, evaluation, scales. RESUMOObjetivo: Durante a fase de transição (deambulador -não-deambulador), sinergias musculares caracterizam a evolução da distrofia muscular de Duchenne (DMD). Este estudo visou descrever e quantificar os movimentos compensatórios durante o sentar/levantar do solo, subir/descer degraus. Oitenta vídeos (5 crianças × 4 avaliações × 4 tarefas) foram gravados trimestralmente durante o ano que antecedeu a perda da marcha. Método: Os movimentos compensatórios dos vídeos foram registrados utilizando a Escala de Avaliação Funcional para DMD. Resultados: Os movimentos compensatórios mais frequentemente observados foram apoio de membros superiores nos membros inferiores/solo/corrimão durante todas as tarefas funcionais e hiperlordose lombar, apoio de tronco no corrimão, pés equinos, aumento da base de suporte, descida não alternada e pausas ao subir/descer degraus. Subir/descer degraus apresentou um número maior de movimentos compensatórios do que sentar/levantar do solo. Conclusão: Sentar/levantar do solo foram habilidades perdidas antes de subir/descer degraus em crianças com DMD.Palavras-chave: distrofia muscular de Duchenne, avaliação, escalas.

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Section: Discussionmentioning

confidence: 99%

Compensatory movements during functional activities in ambulatory children with Duchenne muscular dystrophy

Martini

Voos

Hukuda

et al. 2014

Arq. Neuro-Psiquiatr.

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“…cycle ergometry (LeMura et al 2001) are not feasible or too burdensome for children with mitochondrial disorders. Besides, measuring performance in a laboratory situation may not always reflect the disabilities experienced in daily life since the performance in daily life may differ from the abilities of the child (Abel et al 2003;Beenakker et al 2005;Parreira et al 2010).…”

Section: Introductionmentioning

confidence: 99%

Domains of Daily Physical Activity in Children with Mitochondrial Disease: A 3D Accelerometry Approach

et al. 2017

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“…In young healthy children, functional scores, such as running or standing up from a sitting position on the floor, improve as the child ages from 4 to 10 years due to the growth-related physiological increase in strength [23]. In contrast, DMD patients in the same age group not treated with prednisone showed decreasing mean functional scores during childhood, due to progressive muscle weakness [23]. Although we lack a control group, our patients showed an improvement or stabilization on their functional scores for at least 12 months while on the prednisone 10/10 on/off therapeutic regimen (Fig.…”

Section: Discussionmentioning

confidence: 99%

Prednisone 10 days on/10 days off in patients with Duchenne muscular dystrophy

et al. 2009

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Corticosteroids are effective in improving motor function in Duchenne muscular dystrophy (DMD) patients within 6 months-2 years of treatment initiation, but there is as yet no consensus on which treatment scheme is the best. We retrospectively analyzed data of 35 DMD patients who were treated with prednisone 0.75 mg/kg per day intermittently 10 days on/10 days off. Prednisone was started during the ambulant phase at age 3.5-9.7 years (median 6.5 years). The median period of treatment was 27 months (range 3-123 months). The median age at which ambulation was lost was 10.8 years (mean 10.9 years; 95% confidence interval 10.0-11.8 years). Nine patients (26%) had excessive weight gain. Eight boys (21%) had a bone fracture, which was when four of these eight children lost the ability to walk. Treatment was stopped in two obese patients, two hyperactive boys and one patient following a fracture. Our data suggest that prednisone 10 on/10 off has relatively few side effects and extends the ambulant phase by 1 year compared to historical controls.

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Functional ability and muscle force in healthy children and ambulant Duchenne muscular dystrophy patients

Cited by 84 publications

References 8 publications

Compensatory movements during functional activities in ambulatory children with Duchenne muscular dystrophy

Compensatory movements during functional activities in ambulatory children with Duchenne muscular dystrophy

Domains of Daily Physical Activity in Children with Mitochondrial Disease: A 3D Accelerometry Approach

Prednisone 10 days on/10 days off in patients with Duchenne muscular dystrophy

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