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一家族四代10例患颅底凹陷症,有二例在本院手术。例1:21岁女性。自18岁始出现双下肢无力,渐加重。随后吐字不清。近半年来头痛、无恶心呕吐,无呛咳及声音嘶哑,颈部无外伤史。家系调查:姊妹5人;3人患此病,1人死亡。其母及3个姨母亦患此病,母亲因此病死亡。外祖母、外祖母之兄及曾祖父均患此病死亡。检查:步态蹒跚、情感反应差,构音不良。双下肢肌张力高,深反射亢进,共济运动差。颅骨水平侧位片及CT扫描示:颅底凹陷症。手术见:枕骨增厚,右侧明显达1.3cm。环椎后弓
A family of four cases of 10 cases of skull base depression, there are two cases in our hospital surgery. Example 1: 21-year-old woman. Since the age of 18, double-limb weakness began to appear, gradually heavier. Then unclear articulation. Nearly six months headache, no nausea and vomiting, no cough and hoarseness, no history of neck trauma. Family survey: 5 sisters; 3 people suffering from the disease, 1 person died. His mother and three aunt also suffered from the disease and her mother died of the disease. The grandmother, grandmother’s brother and great-grandfather were both suffering from the disease. Check: gait faltering, poor emotional response, poor articulation. Double lower extremity muscular tension, hyperreflexia, mutual aid movement. Skull lateral radiographs and CT scan showed: skull base depression. See surgery: occipital thickening, the right significantly up to 1.3cm. Ring after the bow