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本病为家族性遗传性锥体束变性疾病,在兄弟姐妹间可有数人发病,发病年龄多在3～15岁。主要表现为逐渐进展的两下肢痉挛性瘫痪,其后还可累及两上肢,严重者可出现构音障碍和吞咽困难,一般无感觉障碍。终则因肌肉无力和挛缩而卧床不起,多死于继发感染。对本病一般尚无有效疗法。作者用左旋多巴和安坦治疗同一家族3例病人,随诊已二年之久,效果确切,满意。现报告如下。
The disease is a familial hereditary pyramidal tract disease, there may be several brothers and sisters disease onset age more than 3 to 15 years old. Mainly for the gradual progress of the lower extremity spastic paralysis, followed by involvement of both upper extremities, severe dysarthria and dysphagia, generally no sensory disturbances. Finally, due to muscle weakness and contracture and bedridden, and more died of secondary infection. The disease is generally no effective therapy. The author with levodopa and An Tan treatment of the same family of 3 patients, followed up for two years, the exact effect, satisfaction. The report is as follows.