神经纤维瘤病I型的MRI研究

目的：回顾神经纤维瘤病I型（NF1）患者MRI表现，分析MR扫描序列及其诊断价值，以建立合适的MR成像方案，为NF1影像诊断提供有价值的依据。方法：对30例临床确诊为NF1患者采用本组MR成像方案进行扫描，主要包括：轴面SE序列T2WI；平扫矢状面SE脉冲序列T1WI；增强轴面或矢状面SE脉冲序列T1WI；轴面或冠状面液体衰减反转恢复（FLAIR）序列，同时分析病变的发病部位、数目、形态、信号的变化和病变的强化情况等。结果：MRI可见下列3种表现：（1）多发性脑内错构瘤：30例中25例在SE脉冲序列T2WI和FlAIR脉冲序列见高信号病灶，病灶主要位于苍白球、小脑和脑干。另外，25例中20例可见海马回、海马旁回等区晕状高信号改变。（2）视通道或下丘脑胶质瘤：视神经、视交叉增粗、扭曲；视交叉或下丘脑肿块，SE脉冲序列T2WI和FlAIR序列表现为不规则分叶状混杂信号肿块，在增强SE脉冲序列T1WI有明显不规则强化。（3）脊柱多发性神经纤维瘤：SE脉冲序列T2WI和脂肪抑制短时反转恢复（STIR）序列显示高信号沿脊神经分布的多发性肿瘤。结论：MRI能够作为1种 常规的影像检查方法对NF1患者进行诊断和追踪。本组MR成像方案能较好地显示NF1的多发性或多灶性病变。

MRI study of neurofibromatosis type I

Objective To review the MR image findings in patients with neurofibromatosis type 1 (NF1) and to analyze the MR sequences and their diagnostic value so as to establish an adequate MR imaging protocol to supply the valuable imaging data for the diagnosis of NF1 Method Thirty patients fulfilling the NIH diagnostic criteria for NF1 were examined with the following MR imaging protocol, which mainly included: Axial SE T 2WI; Sagittal SE T 1WI without contrast; Axial or Sagittal SE T 1WI with contrast; Axial or Coronal FLAIR At the same time the characteristics of the disease including sites, numbers, shapes, and changes of intensity and enhancement of the lesions were recorded and analyzed Results There were three forms of lesions being demonstrated on MRI: (1) Multiple intracranial hamartomas: Hyperintense lesions on T 2WI and FLAIR images in 25 out of 30 patients (83%), which mainly located in the globus pallidus, cerebellums, and brainstems There was no definite enhancement with Gadolinium 20 out of 25 patients (80%) showed diffuse higher intensity in the region of hippocampus or parahippocampus gyrus, thalamus, and around the aqueduct (2) Optic pathway or hypothalamus glioma: Enlargement or/and elongation of the optic nerves and optic chiasms on STIR images; Masses in the region of the optic chiasm or hypothalamus with mixture high intensity on T 2WI and FlAIR images Significant and irregular enhancement was seen on SE T 1WI (3) Multiple neurofibromas in the spine: Multiple bulky tumours extending along the spinal nerves were shown on SE T 2WI and STIR images with higher signals Conclusion MR is suitable for routine imaging investigation in diagnosis and follow up of NF1 Our MR imaging protocol for NF1 could show multiple abnormalities of NF1 better