Distal dystrophy--unusual presentations. A report of 2 cases

Two men suffering from progressive muscle wasting and weakness are described. The first patient presented with a rapidly progressive myopathy of the right lower leg characterised on histological examination of muscle biopsy specimens by extensive ringed fibre formation. The second patient exhibited severe muscle weakness and wasting of the lower legs characterised by the accumulation of amorphous sarcoplasmic material and vacuolation seen on histochemical evaluation and confirmed by electron microscopy. There was no clinical or electrophysiological evidence of myotonia in either case.