Duchenne Muscular Dystrophy: a neurocognitive and behavioral perspective

Abstract

Duchenne muscular dystrophy is a progressive neuromuscular disorder associated with neurocognitive and behavioral difficulties. In the past, the focus of research and clinical care was mainly on the devastating physical consequences of this disease. Recently, more attention goes to the neurocognitive and behavioral aspects that affect boys with Duchenne muscular dystrophy and their families. Boys with Duchenne muscular dystrophy are more vulnerable for cognitive deficits, learning difficulties and behavioral problems. The combination with their physical problems can be a heavy burden for these patients and their families. Research tries to reveal the complex mechanisms between genotype, dystrophin deficiency, brain structure and functioning, and behavior and cognition. Meanwhile, clinical care should focus on early detection and intervention. This paper reflects about what we know thus far about these aspects, which research is ongoing and how these issues can be handled in clinical care.