神经纤维瘤病Ⅱ型并脑膜瘤的临床表现(附1例报告及文献复习)

Clinical Manifestatins of Neurofibromatosis Type 2 Complicated by Meningioma

目的探讨神经纤维瘤病Ⅱ型(NF2)的病因、临床特点、诊断及治疗。方法对1例神经纤维瘤病Ⅱ型合并脑膜瘤进行回顾性分析。结果本例患者行脑膜瘤、右侧小脑桥脑角肿瘤切除术,病检示,右顶枕部血管瘤型脑膜瘤,右侧小脑桥脑角神经鞘瘤,术后右耳听力无改变,右侧不完全性周围性面瘫,House-Brackmann分级Ⅱ级。结论NF2为常染色体显性遗传性疾病,临床上以双侧听神经瘤为主要特征,可伴有颅内和脊髓的脑膜瘤、室管膜瘤、神经胶质瘤、迟发性晶状体混浊、神经末梢纤维瘤与皮肤神经鞘瘤等。早期诊断和治疗对患者的听力和生命的保护至关重要。MRI扫描应作为NF2的常规检查。

Objective To study the predisposing factors, clinical manifestations, diagnosis and treatment of neurofibromatosis type 2 （NF2）. Methods One case of NF2 complicatede by meningioma was reported, and its clinical manifestation, Herapentie principle and the worth of MRI in its diagmosis were analysed. Results The patient received operation on the meningioma and right cerebellopontine angles tumor. The tumor were proved to be hemangiomatous meningioma and schwannoma by pathological test. The hearing was not improved and the right facial nerve paralysis （ House-Brackmann Ⅱ ） appeared after opration. Conclusion NF2 is an autosom dominant, highly penetrant disease which is characterized by bilateral vestibular schwannomas. It can be complicated by meningioma, ependymomas, glioma, et al. Early diagnosis and management is very important for survival and heating preservation. MRI should be routine examination in diagnosing this disease.