Electromyography was the most helpful laboratory study before genetics were available. The combination of myotonic discharges and myopathic appearing motor units, predominantly in distal muscles and ...

Investigation with needle EMG will reveal myotonic discharges in proximal and distal muscles. These discharges can present either as sustained runs of positive waves, or runs of small triphasic ...

As they get older they may need to use a cane or wheelchair to get around. The symptoms of myotonic dystrophy may be obvious from birth or they can develop later -- during the teenage or adult years.

Electromyography demonstrated myotonic discharges and short-duration low-amplitude motor unit potentials. Based on the typical clinical course described above, DM1 was suspected. In conclusion, ...

Myotonic muscular dystrophy is a common multi-system disorder that affects the skeletal muscles (the muscles that move the limbs and trunk) as well as smooth muscles (the muscles that control the ...

The myotonic dystrophies represent a subset of inherited muscular dystrophy disorders. Myotonic dystrophy, or dystrophia myotonica (DM), is a multisystem disease, and the cardiovascular defects may be ...

unsw.edu.au Objective Myotonic dystrophy type 1 (DM1) is an autosomal-dominant neuromuscular disease with variable severity affecting all ages; however, current care guidelines are adult-focused. The ...

Action myotonia and lid-lag phenomenon were not evocated anymore. At a 1-year follow-up, EMG displayed consistent reduction in number and duration of myotonic discharges, improvement of CMAP ...