最近,作者见到2例成年期起病的进行性脊髓肌萎缩症,其突出的和较先出现的症状为横膈麻痹所致呼吸机能不全。以后陆续出现的其他症状为:消瘦,发音障碍,吞咽困难,嗜睡,肢体肌肉无力和萎缩(以远端为主,例一并伴有舌肌萎缩和纤维颤动);例二的病程中曾有颅内压增高体征,但随呼 Recently, the authors have seen 2 cases of progressive spinal muscular dystrophy onset in adulthood, with prominent and first-mentioned symptoms of respiratory insufficiency due to diaphragmatic paralysis. Other symptoms gradually appeared later: weight loss, dysphonia, dysphagia, drowsiness, weakness and atrophy of the limbs (mainly distal, accompanied by tongue atrophy and fibrillation); the course of the second had Increased intracranial pressure signs, but with call