强直性肌营养不良较少见,现报告1例如下:男患,47岁,8年前患“伤寒”,愈后1月开始感双手无力;第二年双下肢无力,手、前臂肌萎缩并有脱发。第三年出现下肢、面部、臂部肌萎缩,两手强硬,左手握物不易松开。第五年出现讲话带鼻音,进食易呛咳,急躁,多汗,性欲明显减退。检查:神志清,智力正常,发稀秃顶,斧头状脸,双眼用裂隙灯检查有白内障改变,舌肌中心部轻度肌萎缩,构音欠清,有鼻音,转颈时无力。四股肌力:右手4级,左手3级, Myotonic dystrophy rare, now report a case as follows: Male, 47 years old, 8 years ago suffering from “typhoid fever”, the feeling of weakness began in January; the second year of weakness in both lower extremities, hand, forearm atrophy And hair loss. The third year of lower extremity, facial, arm muscle atrophy, hands hardened, left hand grip is not easy to release. Appeared in the fifth year with nasal tone, easy to eat cough, irritable, sweating, decreased libido. Check: Consciousness, mental normal, hair thin bald, ax-like face, eyes with slit lamp examination cataract changes, mild muscular atrophy of the central part of the tongue, the consonance is not clear, nasal, neck weakness. Four muscular strength: right hand 4, left hand 3,