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患者,女性,35岁。7年前开始静坐后双下肢起步困难,僵硬感,行走后缓解。2年后渐感双手活动困难,并见肌肉萎缩,言语也逐渐含糊不清。病后5年曾作肌电图检查:双手大小鱼际肌插入电位延长及强直电位,提示:强直性肌营养不良症。服用苯妥因钠可使肌僵硬暂时好转。近二月来,四肢乏力加重,双手屈、伸困难,行走不便。家族中无类似患者。检查:神清,言语欠清,长脸,额部发线后移,双侧颞肌萎缩,颈细长。双眼白内障,视力:左0.8右0.8,露齿稍差。双手大、小鱼际肌、骨间肌,下肢胫骨前肌萎缩。双手指、足趾肌力4度,屈指缓慢,伸指动作需3~4秒完成,但未见扣击
Patient, female, 35 years old. Seven years ago began to sit after the lower limbs meditation difficult, stiff sense, ease after walking. 2 years later gradually feel both hands difficult activities, and see muscle atrophy, speech gradually vague. 5 years after the disease has been made for electromyography: hands and the size of the motor muscle into the potential prolongation and tonic potential, suggesting that: myotonic dystrophy. Taking phenytoin sodium can temporarily improve muscle rigidity. In recent months, limbs increased fatigue, both hands flexion, extension difficulties, walking inconvenience. There are no similar patients in the family. Check: God clear, the language is not clear, long face, forehead hairline shift, bilateral temporal muscle atrophy, slender neck. Eyes cataracts, visual acuity: left 0.8 right 0.8, a little exposed teeth. Large hands, small intertidal muscle, interosseous muscle, lower extremity anterior tibial muscle atrophy. Two fingers, toe muscle strength 4 degrees, flexor slowly, stretching finger action to be completed within 3 to 4 seconds, but no buckle