| 郭雪梅,谢晟,肖江喜.伴皮层下囊肿的巨脑型脑白质病的MRI特点[J].中国医学影像技术,2010,26(5):822~824 |
| 伴皮层下囊肿的巨脑型脑白质病的MRI特点 |
| MRI features of megalencephalic leukoencephalopathy with subcortical cysts |
| 投稿时间：2009-12-10 修订日期：2009-12-25 |
| DOI： |
| 中文关键词: 皮层下囊肿 脑白质病 磁共振成像 |
| 英文关键词:Subcortical cysts Leukoencephalopathy Magnetic resonance imaging |
| 基金项目: |
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| 中文摘要: |
| 目的 评价伴皮层下囊肿的巨脑型脑白质病的MRI特点。方法 回顾性分析7例临床确诊的伴皮层下囊肿的巨脑型脑白质病患儿的颅脑MRI资料,其中4例有扩散加权成像(DWI)图像,4例有单体素MR波谱(MRS),分析其影像表现特点。结果 7例患儿均存在不同程度的双侧脑白质异常及双侧颞叶的皮层下囊肿,6例伴有皮质脊髓束异常,4例伴有小脑白质异常。7例灰质均未受累。MRS显示N-乙酰天门冬氨酸(NAA)、肌酸、胆碱波峰存在,NAA峰正常或轻度降低,未见明显乳酸峰。DWI显示双侧脑白质呈弥漫低信号,未见明显高信号病灶。结论 伴皮层下囊肿的巨脑型脑白质病的MRI表现具有特征性,MRI能为该疾病的诊断提供帮助。 |
| 英文摘要: |
| Objective To investigate the MRI features of the megalencephalic leukoencephalopathy with subcortical cysts (MLC). Methods MR images of 7 patients with clinical diagnosis of MLC were analyzed retrospectively. There were 4 patients underwent diffusion weighted imaging (DWI) and 4 patients underwent single voxel MR spectroscopy (MRS). Results MRI demonstrated diffused high intensity in bilateral white matter on T2WI and subcortical cysts in bilateral temporal lobes. Six of them had abnormal signal in the corticospinal tract, 4 in the white matter of the cerebellum. No abnormality was found in grey matter. MRS showed mildly reduced or normal N-acetylaspartate (NAA) peak, without obvious lactate peak. DWI showed diffused low signal intensity in the bilateral white matter, with no abnormal high signal. Conclusion MLC has typical features on MR imaging, and MRI may be helpful for the diagnosis of the disease. |
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