【摘要】 目的　分析并探讨神经纤维瘤病的分型及各型的MR影像学表现。方法　回顾性地分析 8例神经纤维瘤病患者的临床及影像学资料 ,所有病例均常规行MRI及钆剂强化扫描 ,归纳所有患者的临床表现及影像学特点。结果　根据NIH的诊断标准 ,8例中 ,4例属NF -1型 ,均在脑内胶质瘤、椎管内神经纤维瘤或脑内对称部位的异常信号基础上 ,合并有皮肤的咖啡牛乳色斑或多发神经纤维瘤 ,其中 1例有家族史 ;4例属NF -2型 ,均患双侧听神经瘤 ,2例合并髓内星型细胞瘤。结论　NF -1型在所有神经纤维瘤病中占大多数 ,且多合并有皮肤损害 ;NF -2型较少见 ,典型表现为双侧听神经瘤 ,皮损少见。MRI及强化扫描对检出神经纤维瘤病中枢神经系统的损害方面有优势更多还原

【Abstract】 Objective To analyze the classification and imaging manifestations of neurofibromatosis.Methods The clinical data and MR features of 8 patients with neurofibromatosis were evaluated retrospectively.MR plain scan and Gd-DTPA enhanced scan were performed in all cases.Results According to the diagnostic standard of NIH,4 cases were NF-1,of them one with multiple cerebral parenchymal gliomas,two patients with intravertebral neurofibromas,and one girl with symmetric abnormal high signal intensity lesions at bilateral cerebellar peduncles,globus pallidus and thalamus,cutaneous multiple cafe au-lait spots or neurofibromas could be found in 4 cases with NF-1,one case had family history;4 patients with bilateral acoustic neuromas were belonged to NF-2. Conclusion NF-1 is more common in neurofibromatosis and always associated with cutaneous lesions,while NF-2 is always associated with bilateral acoustic schwannomas.MR plain scan and Gd-DTPA enhanced scan are of important value in detecting the lesions of CNS.更多还原