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1 病例介绍患者女,20岁,因不规则发热、腹痛、进行性吞咽无力、言语不清,躯干及四肢皮肤、肌肉萎缩、僵硬1年余,加重3个月,于1991年5月14日入院.患者于1年前因不规则发热、阵发右下腹痛,当地医院拟诊肠结核,经正规抗结核治疗半年无效.发热、腹痛时轻时重,且渐出现吞咽无力、呛咳、言语不清,躯干及四肢指(趾)皮肤、肌肉萎缩、僵硬,指、趾末端发凉、既往健康,无肺结核病史.查体:T38℃,P100次/分,BP13/8kPa.面部皮肤菲薄,毛细血管扩张,皮肤及面肌重度萎缩,触之僵硬,不能捏起.不能张口,言语不清.颈部皮肤、肌肉重度萎缩,触之僵硬,颈项固定,不能转动,粗细如常人手臂.胸、腹、背及四肢指(趾)皮肤、肌肉同面、颈部表现,且活动功能受限.双肺呼吸音低,无干湿罗音.第一心音低钝,律齐,无杂音.腹壁僵硬,轻触痛,肝脾未扪清.四肢
1 case description Female patient, 20 years old, due to irregular fever, abdominal pain, progressive swallowing, speechless, trunk and limb skin, muscle atrophy, stiffness more than 1 year, aggravating 3 months, on May 14, 1991 Admitted to hospital.Patients in a year ago because of irregular fever, paroxysmal right lower abdominal pain, the local hospital intends to diagnose intestinal tuberculosis, after six months of formal anti-TB treatment ineffective fever, abdominal pain when light weight, and gradually swallowing weakness, cough, Speechless, torso and limbs (toe) of the skin, muscle atrophy, stiffness, finger, toe end of the cold, past health, no history of pulmonary tuberculosis. Physical examination: T38 ℃, P100 beats / min, BP13 / 8kPa. , Telangiectasia, severe skin and facial muscle atrophy, stiff touch, can not pinch. Can not mouth, unclear. Neck skin, severe muscle atrophy, stiff touch, the neck fixed, can not rotate, the thickness of the arm as normal. Chest, abdomen, back and limbs (toe) of the skin, muscle with the same side, neck performance, and functional limitations .Life lung sounds low, no wet and dry rales. First heart sound low blunt, law Qi, no Noise. Abdominal wall stiffness, touch the pain, liver and spleen not palpable