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shen jing xian wei liu bing xing zhi liao ti hui
Author(s): 
Pages: 33-34
Year: Issue:  z2
Journal: CHINESE JOURNAL OF POSTGRADUATES OF MEDICINE

Keyword:  神经纤维瘤病Ⅱ型神经鞘瘤;
Abstract: 目的 提高对神经纤维瘤病Ⅱ型(NFⅡ)的病因、临床表现、诊断及治疗的认识.方法 结合文献复习,分析和总结收治的1例NFⅡ患者的临床资料.结果 该患者男性24岁,双侧听神经瘤,三叉神经、左侧舌咽神经走行区和鞍上池左侧多发神经鞘瘤,左侧脑室、大脑镰多发脑膜瘤,同时有颈髓内及髓外硬膜下多发神经鞘瘤,左侧颈动脉鞘占位,胸7~9髓外硬膜下神经鞘瘤,胸11~腰1水平髓内多发神经鞘瘤,腰1水平以下多发占位累及脊髓圆锥及马尾神经.结论 NFⅡ为常染色体显性遗传疾病,临床上以双侧听神经瘤为主要特征,常合并脑膜瘤,有的患者以椎管内肿瘤为首发症状,可发生在脊髓的各个水平,但以胸段为最常见.早期诊断和治疗对患者的预后至关重要.
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