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例1:男,20个月,1985年5月20日就诊.生后3个月,发育营养尚可。4个月后逐渐变差,8个月时四肢变硬,1岁时双手有不自主运动,不会坐立,不会翻身.18个月时四肢变直,头向后仰.1周来发作性咬自己的舌唇,每次发作3～5分钟,以至将舌唇部分咬掉,经常流血.父母非近亲结婚,其姐9岁,身体健康.患儿大姨的1男孩,2岁死亡,死前4个月四肢变直.头向后仰,死前2个月发作性自毁舌、唇,发病情况与患儿相似.检查,发育迟钝,营养不良III.神志清楚,表情呆滞,方头.下唇左侧有1.5×1.5cm,舌有1×0.5cm缺损伤面.表面有脓性分泌物及渗血.全身呈角弓反张状.颅神经(一),眼底(一),四肢肌张力增高,深反射活跃,有不自主大幅度震颤,双侧Babinski氏征(+),无明显感觉障碍.血尿酸>7mg%,
Example 1: Male, 20 months, May 20, 1985. 3 months after birth, developmental nutrition is acceptable. 4 months after gradual deterioration, limbs hardened at 8 months, 1 year old hands involuntary movements, will not sit, will not stand up .18 months straight limbs, head backwards .1 week Bitch his own tongue, each episode 3 to 5 minutes, as part of the tongue bite, often bleeding. Parents non-relatives get married, his sister 9 years old, good health. , Extremities straightened up 4 months before death head back, 2 months before death paroxysmal self-destruction of the tongue, lip, and children with similar morbidity check, dull, malnutrition III delirious, Square head. Left side of the lower lip 1.5 × 1.5cm, 1 × 0.5cm defect of the tongue surface. Purulent secretions and surface bleeding. Whole body was anti-valleys. Cranial nerves (a), fundus ), Limb muscle tension increased, deep reflex active, involuntary significant tremor, bilateral Babinski’s sign (+), no obvious sensory disturbance .Humanuria> 7mg%