PhD conferral Mr Ruben G.F. Hendriksen, MSc.

“Evidence for a dystrophin-associated encephalopathy in Duchenne Muscular Dystrophy”

Keywords: Duchenne muscular dystrophy, cognitive problems, epilepsy, dystrophin

Duchenne muscular dystrophy is a severe and progressive muscle disease. Despite what the name may suggest, this disease affects more than just the muscles; it also causes cognitive problems. As boys with Duchenne muscular dystrophy get older, their symptoms worsen and can have a serious impact on their quality of life. This dissertation introduces additional evidence to support the idea that the disease affects the brain by increasing the risk of developing epilepsy and associated neurological problems. We describe a theoretical model, examine the preclinical background and determine the influence of medication in the form of corticosteroids.