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AIM: To study the electromyogram and muscular pathological features of adult spinal muscular atrophy(SMA4). METHODS: 46 cases of SMA4 were evaluated based on clinical, 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 muscular atrophy, and there was a relatively good prognosis. Laboratory found: one-fourth of the disease had elevated serum creatine kinase levels. Eletromyogram revealed neurogenic damages. The muscular pathological changes showed small groups of atrophy of denervation, ATPase reaction showed fibre-type grouping of renervation and hypertrophy in muscle fibers. CONCLUSION: Muscle biopsy was important; it could to help to establish to diagnose the disorder and provided available cases for gene study.