Adequate managment of patients with dystrophinopathies (muscular dystrophy Duchenne/Becker): objective scales and additional diagnostic methods

Abstract

There are still no guidlines on managment of Duchenne/Becker myodystrophy in domestic medical practice. It leads to decrease of quality of life and, what is more important, lifespan of patients. In this article we have described our Western coleagues lаst decade experience, including consensus guidelines published in 2010 on mаnаgment of Duchenne myodystrophy, supplemented with our practicle experience. We have described standardized motor development scale and muscle tone score for patients with MDD/MDB, and algorithm of multidiscipline care with focus on prevention, diagnosis and treatment of main disease and steroid therapy complications: cardiovascular, orthopedics, respirator etc. These recommendations not only improve quality of live and extend lifespan of MDD/MDB patients, but allow to take part in multicentre trials on searching of pathognomonic and symptomatic treatment.

Keywords

• дистрофинопатии
• мышечная дистрофия дюшенна
• мышечная дистрофия беккера
• нейромышечные заболевания
• шкала хаммерсмит
• шкала мануального тестирования мышц хаммерсмит
• осложнения мышечной дистрофии дюшенна
• осложнения мышечной дистрофии беккера