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例1 男,23岁。自幼缓慢出现左颜面,躯干及上下肢萎缩,出汗少,因经常进行体操运动,而左上,下肢无力不明显。无家族史。体查:左上肢及左侧背部见多处深褐色斑状色素沉着,直径1~5cm不等,有的融合成片,左侧面部、躯干及上下肢皮肤及皮下脂肪菲薄、肌肉萎缩,舌及软腭无萎缩,左上、下肢肌力5°~-,感觉正常,左侧偏身少汗,右侧面部、躯干及上下肢肌力及肌容积正常,两侧反射尚对称,无病理反射。内科检查正常。脑脊液、脑电图及颅脑CT均正常。
Example 1 male, 23 years old. Left a slow appearance since childhood, the trunk and lower extremity atrophy, less sweating, due to regular gymnastics, and left upper limb weakness is not obvious. No family history. Physical examination: The left upper limb and left back see multiple dark brown spotted pigmentation, diameter 1 ~ 5cm range, and some integration into a film, the left side of the face, trunk and upper and lower extremity skin and subcutaneous fat, muscle atrophy, tongue and Soft palate without atrophy, left upper and lower limb muscle strength 5 ° ~ -, feeling normal left sweat less sweat, the right side of the face, upper torso and upper limb muscle strength and muscle volume were normal, bilateral symmetry is still reflected, no pathological reflex. Medical examination was normal. Cerebrospinal fluid, EEG and brain CT were normal.