Clinical Importance of Central Nervous System Dysfunction in Myopathy

Multidisciplinary treatments including mechanical ventilation and cardioprotective therapy have improved life expectancy in many neuromuscular disorders such as Duchenne muscular dystrophy. For these patients, central nervous system disturbances such as intellectual and/or developmental disability can hinder social activities and communications. In myotonic dystrophy, the personality and/or cognitive dysfunction affects medical consultation behavior and decreases the efficacy of medical treatments. Understanding central nervous system disturbances in myopathies and providing care keeping in mind the patient burden are critical for improving prognosis and quality of life.