Evolution of functional capacity, assessed with the Egen Klassifikation scale, in the Spanish population with spinal muscular atrophy or Duchenne muscular dystrophy. A three year longitudinal study]

REVISTA DE NEUROLOGIA(2015)

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摘要
Introduction. Spinal muscular atrophy (SMA) and Duchenne muscular dystrophy (DMD) are two neuromuscular diseases which evolve with a progressive loss of muscle strength and, therefore, the loss of functional capacity. The valuation measurement scales are used to understand better and to quantify this involution as well as making treatment to anticipate problems and improve the quality of life of people suffering from these diseases. Aim. To study the changes in the functional capacity of a group of patients with SMA and DMD, over a period of three years. Patients and methods. Nineteen individuals of the Spanish population affected with SMA and DMD, which were assessed with the Egen Klassifikation scale twice, with a time interval of three years. Results. The results show a decrease in the functional capacity of these persons during this time period, with a significant difference in the total amount of the scale (p = 0.003). All scale items showed lower valuations after three years, reaching statistical significance during the assessment, containing the ability to move his hands and coughing. Conclusion. The functional capacity of patients with SMA and DMD decreases significantly within three years.
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关键词
Duchenne muscular dystrophy,EK scale,Functional capacity,Spinal muscular atrophy
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