Rehabilitation bei neuromuskulären Erkrankungen: Stellenwert der medizinischen Trainingstherapie

Abstract

Neuromuscular diseases are a heterogeneous population of disorders including the different kinds of muscular dystrophies, myopathies, motor neuron diseases and neuropathies. The leading symptom is the mostly progressive muscle weakness due to myopathy or denervation of muscles. Despite progress of molecular diagnosis and improved therapeutic approaches for the majority of neuromuscular diseases there exists no causal treatment. Symptomatic drug therapy is often not sufficient. There is general agreement on the importance of rehabilitative therapy in neuromuscular disease but the scientific basis for the different methods of rehabilitation is poor, resulting in different opinions on suitable therapy. Especially the benefit or potential damage of active therapy in neuromuscular disease was discussed controversially in the past because of case reports of overwork weakness due to unsupervised exercise training. Persons with neuromuscular disease usually have a sedentary lifestyle resulting in an additional disuse weakness. Secondary deconditioning could be a reversible component of disability in neuromuscular disease. Exercise training improves muscle strength or endurance in healthy people. The response to exercise training may include an element of reversing the effects of disuse as well as altering the natural history of neuromuscular disease itself. However, in neuromuscular disease the different effects of strength or endurance training on each type of disease are not known. Research is lacking in determining whether persons with neuromuscular diseases respond to exercise training in a similar beneficial manner. The effects of exercise training are influenced by the kind, progression and stage of disease and by the type, frequency, intensity and duration of exercise training. Patients must have enough residual muscle strength to be able to respond to an exercise training. The comparative value of strength training versus endurance training in neuromuscular diseases has not been evaluated nor do we know the optimal amount of exercise to maintain or improve muscle function. There are only few studies indicating positive effects of exercise training in slowly progressive neuromuscular diseases, the long-term effects of training are not known. Most studies concerned Duchenne muscular dystrophy, patients with postpoliomyelitis sequelae or a variety of neuromuscular disease. These studies have substantial methodological shortcomings, such as too few patients, a large variety of disorders, a combination of exercise training and other therapeutic interventions and in most studies the lack of a separate nonexercising control group having the same disorder. Only few studies have evaluated changes in functionality. To evaluate the effects of training measurements of functionality and quality of life are necessary and not only measurement of muscle strength or aerobic capacity. The benefit of exercise training may vary between different neuromuscular diseases because of differences in pathophysiology. Research on groups with a variety of neuromuscular disorders is not an appropriate way to evaluate the effects of exercise training. Overwork weakness has not been demonstrated in controlled trials of exercise when training is properly supervised. Because of the lack of controlled studies until today a final assessment is not possible.