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本文报告用青霉胺治疗2例迅速发展的脊髓性肌萎缩患者获得显著进步。患者均为女性,成年。治疗前,2例均有迅速发展的上楼和梳头困难,不能步行、蹲下和起立,不能从仰卧位坐起,肢带肌萎缩无力,其中1例全部深反射消失。三角肌、前臂及手肌的肌电图显示运动神经之消失。脑脊液和血液的肌酸激酶、硷性磷酸酶及转氨酶均正常,没有炎症及免疫学障碍的证据。鉴于患者的职业和工作环境,2例均有远期或潜在性铅中毒的可能性,但铅的毒性测验未见异常。
This article reports the significant improvement in 2 cases of rapidly developing spinal muscular atrophy with penicillamine. Patients are female, adult. Before treatment, 2 cases of rapid development of the stairs and comb hair difficulties, can not walk, squat and stand up, can not sit from the supine position, limb muscle atrophy weakness, including 1 case of deep reflex disappeared. The electromyography of the deltoid, forearm and hand muscles shows the disappearance of motor nerves. Cerebrospinal fluid and blood creatine kinase, alkaline phosphatase and transaminase were normal, there is no evidence of inflammation and immunological disorders. Given the occupational and working conditions of the patients, both patients had the potential for long-term or potential lead poisoning, but no abnormalities were observed in lead toxicity tests.