Summary:The clinical significance of an abnormal signal-averased electrocardiogram (SA-ECG) in time-and frequencydomain analyses was assessed in patients with Duchenne muscular dystrophy (DMD). Twenty-four DMD patients and 20 age-matched healthy volunteers were studied. The SA-ECG was recorded by timedomain signal processing using the vector-magnitude method and frequency-domain signal processing using the fast-Fourier transform. Abnormal SA-ECGs were based on comparison with controls and eight abnormal SAECGs were detected among 24 DMD patients, seven by frequency-domain analysis and four by timedomain analysis. The end-diastolic left ventricular volume was larger in the patients with abnormal SA-ECG than in those with normal SA-ECG (136+_32mlvs.77+_43 rnl,p

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We evaluated the excursion of the diaphragm in five patients with Duchenne muscular dystrophy maintained on a chest respirator. The excursion was determined by chest X-ray in all cases and by fluroscopy in two. Overnight monitoring using a pulse oximeter was performed on every patient. Two patients who demonstrated recurrent desaturation were examined by chest X-ray during sleep. The excursion of the diaphragm was very small in all cases.

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Generally a balanced forearm orthosis (BFO) is used to assist the dystrophic patients with proximal muscle weakness in performing upper extremity activities. It is adjustable so that the arm will run downhill with gravity and will be moved uphill by the muscles.An adjustable not severely weakened and well co-ordinated muscle as power source must be available to drive the ordinary BFO uphill and downhill. However, the patients with severe proximal weakness or paralysis are often unable to drive or control this orthosis.We have recently developed an externally powered balanced forearm orthosis for them. The results of clinical use in two limb-girdle dystrophic patients with severely proximal weakness showed that this device was an effective and useful one.