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先天性副肌强直症是与受寒关系密切、有家族遗传性的少见病。现报告一家2例如下: 例1,男,34岁。患者于3岁起每逢寒冷季节或一经受寒即出现身体暴露部位肌肉强直,动作缓慢笨拙,感双手无力,握拳后不能立即松开,不能迅速眨眼,大笑之后不能即刻收敛笑容,食冷饮后出现咀嚼困难,言语含糊等。在温暖环境中症状消失。体检:内科检查(-)。若使双手在冷水中浸泡片刻后则见手指关节僵屈呈爪状,双手肌力减退。无肌萎缩,感觉及反射正常。心电图、血钾及血CPK正
Congenital paraneoplastic ankylosis is closely related with the cold, family history of rare disease. A report of two cases is as follows: Example 1, male, 34 years old. From the age of 3, every patient in the cold season or once the cold appeared in the exposed parts of the body muscle rigidity, slow and awkward movements, feeling both hands weakness, can not immediately release after the fist, can not quickly blink, laughter can not immediately convergence smile, cold food After chewing difficulties, vague language. Symptoms disappear in warm conditions. Physical examination: medical examination (-). If hands soaked in cold water after a while see the finger joint stiffening was claw-like, hands muscle strength decline. No muscle atrophy, normal feeling and reflex. ECG, blood potassium and blood CPK positive