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患者,男,16岁,以头痛4月加重并呕吐40d 主诉于1989年3月29日入院。4月前自觉轻度头痛,夜间加重,头痛加剧视物呈双影,走路无力。出生时全身多处皮肤有大片黑斑。家族中其父全身有散在的“黑色素痣”。查体:意识清,反应迟钝,右眼外展受限,水平眼震(+++),左眼球突出。眼底:左侧视乳头略红,界清,血管搏动差。左侧鼻唇沟浅,其余颅神经检查未见异常。四肢肌力Ⅲ级,肌张力略低,肱二头肌腱反射、膝腱反射活跃,病理反射未引出。面部、枕部、胸背、下肢有散在大片状黑色素痣,以双肩、左胸、双股内侧为著,部分痣上有密集毛发(图1)。左颈动脉造影示:侧裂点轻度上移,颈
Patient, male, 16 years old, with headache aggravated and vomited in April 40d Chief complaint was admitted on March 29, 1989. 4 months ago mild headache, aggravate at night, headache increased visual material was double shadow, walking weakness. Multiple parts of the skin at birth have large black spots. The father of the whole family has scattered “melanoma”. Physical examination: conscious, unresponsive, restricted right eye abduction, horizontal nystagmus (+++), prominent left eyeball. Fundus: the left side of the nipple slightly red, clear boundary, poor vascular beat. The left nasolabial fold shallow, the remaining cranial nerve examination showed no abnormalities. Grade Ⅲ limb muscle strength, muscle tension slightly lower, biceps tendon reflex, knee tendon reflex active, pathological reflex did not lead. Face, occiput, chest and back, lower extremity scattered large melanocytic nevus, to the shoulders, left chest, double-stranded as part of the dense hair on the mole (Figure 1). Left carotid angiography showed: lateral fissure slightly upward, neck