中枢型神经纤维瘤病的临床表现与MR影像特征(附4例报告及文献复习)

目的 研究中枢型神经纤维瘤病（神经纤维瘤病2型，NF2）的临床表现与MR影像特征。方法 4例可疑NF2患于临床症状出现或加重后3周至2年内行头颅Gd-DTPA增强前后MRI检查，NF2的MRI诊断得到手术与病理证实。同时，记录了每例患的临床表现。结果 MRI平扫显示，NF2病灶的特征性表现为T1WI上呈低，等信号，而在T2WI上呈高信号，Gd-DTPA增强后，病灶呈明显强化，MRI还证实了4例NF2患均合并脑膜瘤，这些患最常见的症状是进行性耳聋，耳鸣，眩晕，步态不稳。结论 NF2具有恒常的能通过对比增强MR像证实的强化特征，因此，头颅Gd-DTPA增强前后MRI检查应成为诊断MF2的主要手段。

Clinical manifestations and MRI features of central neurofibromatosis: a report of 4 cases and the review of literature

Objective To investigate the clinical manifestations and MRI features of central neurofibromatosis(neurofibromatosis type2,NF2).Methods Four patients with clinically suspected NF 2underwent MRI examinations of the brain with and without Gd -DTPA enhancement within three weeks to tw o years after onset or exacerbation o f clinical symptoms.MRI diagnoses o f NF 2in all patients were confirmed by the operation and the pathology.Simultaneously,clinial m anifestations were reorded for each patient.Results MR Plain seans showed that,all the lesions in 4case s were hypointese,isointense on T 1 WI and hyperintense on T 2 WI.On Gd -DTPA enhanced images,the lesions were obviously enhanced.MRI also demonstrated that.all patien ts complicated with meningioma.The commonest presenting symp-toms of these patients were progressive deafness,tinnitus,dizziness a nd unsteadiness.Condusion NF2have consistent enhancernent fe atures that can be demonstrated with contra st enhanced MR imaging and therefore MRI examination of brain with and without Gd -DTPA en-hancement should be the preferred me ans of diagnosing NF2.