Duchenne Muscular Dystrophy Overview of Cognitive Impairment

Abstract:

Over the last 30 years many studies have been published concerning the cognitive function in DMD patients and the possible correlation with an absence of dystrophin protein. However, most of the studies focused on the specific domain of cognition and the correlation between dystrophin mutations and cognitive function was never established .The purpose of this review paper is to define the range of neurofunctional, cognitive and psychiatric disorders in DMD patients. This paper will include the clinical features and the molecular defects mainly responsible for the cognitive defects. A general review of this muscular disease will help us achieve a better understanding of the relationship between the molecular defects and the extent of the cognitive impairment in DMD patients. It will also help us determine the developmental and degenerative aspects of CNS involvement in DMD patients and correlate that to development of muscle function as the disease progresses. Finally, this paper will discuss the effects of early factors such as birth history on the future of neuropsychological development and integration of neuropsychological function with emotional and behavioral functioning.

A comparison of early and more recent studies will help us better understand the baseline profile and time course of the cognitive aspects of Duchenne Muscular Dystrophy.