Dystrophia myotonica. A model of combined neural and myopathic muscle atrophy

Reports on clinical, electrophysiological and histological studies in dystrophia myotonica have shown that the peripheral nerves are usually not intact. In a few patients the neuropathy exceeds the myopathy. On the basis of these findings some authors believe that muscle changes in dystrophia myotonica are entirely due to the nerve lesions. However, despite the commonly found evidence of neuropathy, electromyographic and muscle biopsy findings are reported usually as "myopathic". Furthermore, clinical and laboratory findings indicate that the degree of muscle atrophy and peripheral nerve changes are unrelated. This report attempts to explain the discrepancies by suggesting that the "myopathic" and "neuropathic" changes in the muscle fibres in dystrophia myotonica are independent processes.