神经纤维瘤病MR影像表现

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

Magnetic Resonance Imaging Manifestations of Neurofibromatosis

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.