- Breathing pattern in muscular dystrophies Muscular dystrophies (MD) are associated with loss of muscle function over time. Symptoms of respiratory insufficiency can be subtle and the routinely applied methods are not always capable to unravel early alterations. In this study we compared spirometry, lung function and breathing pattern obtained from optoelectronic plethysmography (OEP) of a large population of patients affected by MD.
- Biomechanical Analysis of Gait in Individuals with Duchenne Muscular Dystrophy - UCLA Center for Duchenne Muscular Dystrophy at UCLA and UCLA Department of Orthopaedic Surgery
- Early signs of pathological pattern evaluated by CGA in DMD patients Duchenne Muscular Dystrophy (DMD) is an Xlinked hereditary disease, caused by a gene mutation that affects dystrophin production. The absence of this protein causes a progressive degeneration of muscular fibres leading to a diffuse progressive hypostenia. An early manifestation of the pathology may occur since 3 years old. In children without symptoms of the disease, clinical functional task assessment tools may not show an early compensatory strategy acted by the child to compensate for the muscular weakness. Our goal was to study the very early sign of the walking alterations by means of gait analysis in a group of young dystrophic child.
- Gait pattern in Duchenne muscular dystrophy We investigated the gait pattern of 21 patients with Duchenne muscular dystrophy (DMD), compared to 10 healthy controls through 3D Gait Analysis. 3D Gait Analysis gives objective and quantitative information about the gait pattern and the deviations due to muscular situation of DMD subjects; being our study a single moment evaluation, it is otherwise unable to unravel changes only detectable through serial analysis during the time course of the disease and, if any, due to the treatment.
- Spinal stability is improved by inducing a lumbar lordosis in boys with DMD: A pilot study The development of scoliosis in boys with Duchenne Muscular Dystrophy (DMD) is a significant, morbid event in the progression of the disease caused by progressive spinal musclulature weakness. As an alternative to muscle activity, the spine can also be stabilised by locking the articular facet joints, which is achieved when the body is supported on a seat tilted anteriorly using a 'wedge', of the kind commonly recommended for low back pain.We tested spinal stability when using a seat tilted 158 anteriorly in eight boys with DMD, without significant scoliosis, by measuring the ability to support a lateral load applied to the thorax through a sling and hawser. All eight boys tolerated lateral loading better with wedged seating and were able to support an average additional load of 95 g per kilogram of body weight compared to normal seating. Lateral load bearing was improved in 10 normal control boys by an average of 40 g per kilogram of body weight. These encouraging pilot findings indicate that there is a need for further studies on the effectiveness of passive mechanical factors in spinal stabilisation to delay the development of scoliosis in boys with DMD.
- Avaliação de pacientes com DMD em laboratório de marcha através do índice GDI Evaluation of patients with Duchenne Muscular Dystrophy in Gait Analysis Laboratory using Gait Deviation Index (GDI)
Duchenne Muscular Dystrophy - DMD
Objetivo: Aplicação e correlação do índice de alteração da marcha (GDI) com questionário de avaliação funcional (FAQ), escala de mobilidade funcional (FMS) e índice de Sutherland (IS) para pacientes com distrofia muscular de Duchenne (DMD). Métodos: Onze crianças com diagnóstico clínico de DMD, idade entre 6-11 anos, deambuladoras, participaram do estudo. O GDI foi correlacionado com o FAQ, FMS e IS, utilizando o coeficiente de correlação rho de Spearman-Rank. O GDI foi calculado com a cinemática dos membros inferiores coletada com seis câmeras Hawks da Motion Analysis Corporation. Resultados: O GDI médio obtido foi de 82,5 (± 13,5), sendo este valor menor do que o esperado para indivíduos sem patologias musculoesqueléticas (i.e. ≥ 100). O GDI apresentou correlação moderada com o FAQ e FMS (50/500m) e forte com IS (rho > 0,83, p ≤ 0,05). Conclusão: O GDI apresenta correlação com classificações, sejam elas clínicas (i.e. FMS), por estudo do movimento (i.e. IS) e com questionário de avaliação funcional (i.e. FAQ).
Objective: Aplication and correlation at Gait Deviation Index (GDI) and Functional Assessment Questionnaire (FAQ), Functional Mobility Scale (FMS) and Sutherland Index (SI) for patients with DMD. Methods: Eleven children with clinical diagnosis at Duchenne Muscular Dystrophy (DMD), between 6 and 11 years old, walkers, participated at study. The GDI was correlated with the FAQ, FMS and IS, using the rho coefficient of correlation of Spearman-Rank. To calculate the GDI, the kinematics were collected by six Hawks cameras (Motion Analysis Corporation). Results: The average GDI achieved was 82.5 (plus or minus 13.5), this value being lower than expected for individuals without musculoskeletal disorders (ie greater than or equal to 100). GDI showed moderate correlation with the FMS and FAQ (50/500m) and strong with IS (rho>0.83, p<= 0.05). Conclusion: The GDI shows correlation with clinical classifications (FMS), gait analysis classifications (IS) and questionnaries of function evaluation (FAQ).