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男患,6个月。足月顺产,第一胎胎儿期胎动较弱,出生后哭声弱,无窒息,肢体无力,吃奶尚可。病程进行性日趋严重,无缓解期。4个月时双下肢只能水平移动,头不能直竖,不会翻身。近4天吞咽及呼吸困难,咳嗽、呼吸困难;肢体弛缓性瘫痪。体检、神志清,智力正常,全身湿疹。骨盆肌、躯干肌、肩胛肌、颈肌、上下肢均呈对称性无力,以近端较重。皮下脂肪较丰满,肢体肌萎缩不明显,肌张力明显减弱。双下肢肌力Ⅰ级,双上肢远端肌力Ⅲ级。口周绀,肋间肌麻痹腹式呼吸明显,吸气时可见胸骨下陷,双肺呼吸音弱,心脏正常。肝肋下1.5cm,脾胁下3cm质软。腱反射消失。Hb130g/L WBC15.8×
Male suffering, 6 months. Full-term follow-up, the first fetal fetal movement is weak, crying after birth, no asphyxia, limb weakness, nurturing acceptable. Progressive progressive disease, no remission. 4 months when the lower limbs can only move horizontally, the head can not be erected, will not stand up. Swallowing and breathing difficulties in the past 4 days, cough, difficulty breathing; limb flaccid paralysis. Physical examination, conscious, normal intelligence, body eczema. Pelvic muscle, trunk muscle, scapula, neck muscle, upper and lower limbs were symmetrical weakness to the proximal heavier. More subcutaneous fat, limb muscle atrophy was not obvious, significantly reduced muscle tone. Ⅰ lower limb muscle strength, upper limb distal muscle Ⅲ grade. Perioral cyanosis, intercostal muscle paralysis abdominal breathing, inspiratory sternum can be seen subsidence, lung breath sounds weak, normal heart. Liver ribs 1.5cm, spleen threat 3cm soft quality. Tendon reflex disappears. Hb130g / L WBC15.8 ×