Electromyogram and pathological features of adult spinal muscle atrophy: analysis of 46 cases

AIM:To study the electromyogram and muscular pathological features of adult sp inal muscular atrophy(SMA4).METHODS:46 cases of SMA4 were evaluated based on cli nical,histopathology,enzyme histochemistry and ultrastructure.RESULTS:A mean age of the patients with SMA4 was 38.7 years,clinical progressed was slowly.Clinic manifestations mainly appeared proximal muscular weakness and progressive muscul ar atrophy,and there was a relatively good prognosis.Laboratory found:one-fourt h of the disease had elevated serum creatine kinase levels.Eletromyogram reveale d neurogenic damages. The muscular pathological changes showed small groups of a trophy of denervation,ATPase reaction showed fibre-type grouping of renervation and hypertrophy in muscle fibers.CONCLUSION:Muscle biopsy was important;it coul d to help to establish to diagnose the disorder and provided available cases for gene study.