Physical models of rehabilitation in neuromuscular disease

Abstract

Physical methods of treatment for neuromuscular diseases constitute the mainstay of current management. The overall goals of management are the maintenance of independent ambulation and the optimal functional state consistent with the disease process. Maintenance of muscle strength requires regular daily physical activity. An active exercise program can give limited increases of strength in muscular dystrophy dependent on the severity of the disease. Active exercise programs do not result in overwork weakness when properly supervised. Endurance exercise training does not appear to be suitable for Duchenne muscular dystrophy but may have value in the less rapidly progressive neuromuscular diseases. contracture development can be retarded by passive stretching and splinting initiated early in the disease course. Weight control is important both for ambulatory and wheelchair-bound patients. Reliable assessment of the results of physical therapy programs has been improved by the introduction of newer, more objective methods for measuring muscle strength.

Pompe disease diagnosis and management guideline

“…Rehabilitation management of Pompe disease should be comprehensive and preventative, based on an understanding of the pathokinesiology of disease progression and on individual assessment 74. It should optimize and preserve motor and physiological function, prevent or minimize secondary complications, promote and maintain the maximum level of function, and maximize the benefits of ERT and other therapies when they become available.…”

Physical therapy management of Pompe disease

“…28 Compensations can also limit the use of existing strength by placing muscles at a mechanical disadvantage, compromise length-tension relationships, and limit opportunities to increase or maintain strength, leading to additional disuse atrophy. 33 Proportional increases in weakness may occur with growth due to biomechanical disadvantage as height, weight, or mass increase without the ability of the muscles to cope with the increased workload, as in DMD and SMA. 32 Respiratory involvement, and cardiac involvement, if present, can further compromise function and endurance as in other disorders, 1,2,34 as can undernutrition due to feeding problems from oral-motor weakness.…”

“…28,33,46 The key to management lies in understanding the interaction between the presence, progression, and potential remediation of weakness; the biomechanics of efficient movement; the risks for development of contracture and deformity and strategies for prevention and remediation; the risks and possible remediation of osteoporosis; and function. Muscle weakness presents and progresses in generally predictable patterns with predictable compensations used to cope with weakness.…”

Aquatic therapy for children with Duchenne muscular dystrophy: a pilot feasibility randomised controlled trial and mixed-methods process evaluation

“…28 They are intended to maintain dorsiflexion and hip flexion range, among other targets, with a view to postponing the onset of contractures and prolonging the length of time the child can walk independently. 33 There are no clear guidelines to specific exercise prescription, but regular submaximal exercise is recommended to maintain existing muscle strength and avoid secondary disuse atrophy, 36,37 along with general advice on regular activity such as walking, cycling and swimming. Although there is still the need for further research, there is general agreement that exercise that contains a substantial eccentric component (such as trampolining, stair descending) should be avoided because of the risk of exacerbating muscle damage.…”

“…Stretches (ingredient) provide mechanical traction for, 76 and relax, 37 muscles (mechanisms), which tend to tighten with the progression of DMD, thereby maintaining muscle length and soft tissue structure, while deferring the development of contractures (target). 37 Muscle training and general aerobic, submaximal exercises (ingredients) create biochemical adaptations that increase muscle mass by means of muscle fibre hypertrophy 37 and maintained stroke volume 77 (mechanisms) to prevent disuse atrophy and maintain or improve skills such as sit to stand, getting up from the floor and stair climbing (targets). Warm water (ingredient) creates haemodynamic changes 78 and offers properties such as buoyancy and turbulence (mechanisms) 47 that are not available in land-based physiotherapy, thereby reducing mechanical stress during muscle strengthening and allowing activation of muscles in ways not possible on land (target).…”