진행성 근이영양증의 임상적 고찰

A CLINICAL STUDY ON PROGRESSIVE MUSCULAR DYSTROPHY

We performed this prospective study to analyse the clinical findings on twenty patients who were diagnosed as progressive muscular dystrophy(PMD) at department of Pediatrics. Chungnam national university hospital from January, 1988 to February, 1994. The results were as follows : 1. In the age and sex distribution at the diagnosis, all patients were male and the group of 6-10 years of age accounted for 75%. 2. In the age distribution at the onset, the group of 1-5 years of age was 85%. 3. With regard to family history,a positive history was present in 15% of patients. 4. In the clinical symptoms and signs, 3 leading ones in order of frequency were Gower sign, muscular pseudohypertrophy, gait disturbance. 5. In the serum enzyme levels, the muscular enzymes-CPK. LDH. SGOT and SGPT were increased in almost of all patients. 6. Abnormal EKG findings were 62.5% of patients. 7. abnormal muscle biopsy and EMG findings were present characteristics of myopathic changes in all patients. 8. On the classification of progressive muscular dystrophy. Duchenne type were occupied more than 80%. Therefore , we noted that clinical findings, serum enzyme levels. EKG. EMG and muscle biopsy findings were important in the diagnosis of progressive muscular dystrophy.