Using brain imaging to study myotonic dystrophy

Myotonic dystrophy is a genetic condition that causes progressive muscle weakness and wasting. It typically affects muscles of movement and, commonly, the electrical conduction system of the heart, breathing muscles, swallowing muscles, bowels, lens of the eye, and brain.  People with type-1 myotonic dystrophy also experience problems of their central nervous system (CNS), for example fatigue and depression, although the contribution of the CNS to symptoms is complex and poorly understood.

Dr Vivekananda aims to investigate functional brain activity in people with type-1 myotonic dystrophy, using a technique known as magnetoencephalography (MEG) to measure whole-brain activity. Alongside this, he will assess cognitive abilities, how people look at – and analyse – objects, working memory and attention. The aim of the project is to identify biomarkers (measurements that can be used to determine prognosis, or can be used in clinical trials) that could be used for clinical assessment of treatments that target the CNS, and to improve patient care.

This project is a study pilot using a limited number of people. If the results from this study show promise, larger-scale studies can be done with the goal of providing relevant measurements of CNS function in people with type-1 myotonic dystrophy. If successful, this technique may be employed in the measurement of other neuromuscular conditions in which there is CNS involvement