New techniques in diagnosis, assessment of progression and research in muscular dystrophy

Abstract

A number of imaging techniques has been used in Duchenne muscular dystrophy and other neuromuscular conditions. Ultrasound reflects the changes occurring in muscle and allows differentiation between myopathies and neuropathies. It may also be of help in deciding where to take a needle biopsy. Computerized tomography (CT) has been used to assess progression over a 6 month period in Duchenne dystrophy. The density changes observed on CT scans give an accurate indication of progression and will be a useful tool in clinical trials. CT scans of obligate carriers of Duchenne dystrophy showed lower muscle densities than male controls. Magnetic resonance imaging also accurately reflects pathological changes in muscle. A pilot project to improve respiratory muscle strength and endurance showed that it was possible to improve endurance. A longer term project is currently being carried out to improve respiratory muscle endurance by the use of computer games connected to a thermistor. A certain inspiratory effort is required to continue the games, which provide the motivating factor.