Objective To analyze the MRI appearances of 9 patients with rare demyelinating diseases. Methods Totally 5 patients of neuromyelitis optica (NMO), 1 of Balo's concentric sclerosis (BCS), 2 of Marburg type multiple sclerosis (MS) and 1 of tumefactive demyelinating lesion (TDL) were collected in this study. Routine MR and enhanced MR scan,diffusion weighted imaging (DWI), diffusion tensor image (DTI) and MRI spectroscopy (MRS) were performed. Results MRI revealed signal abnormalities in the optic nerves and the spinal cord which extend over three or more segments in NMO. Lesions detected in NMO patients didn't meet the diagnosis of typical MS. The lesions of BCS appeared as dissemination in time and space, which showed concentric appearance. MRI revealed diffuse white matter involved in Marburg type MS. "Black hole" was observed on T1WI. Arcuate fibers were not involved. TDL represented as a solitary lesion with strip-like enhanced along white matter veins with slight mass effect. Characteristic features of these diseases could be found with routine MRI, MRS, DWI and DTI. Conclusion MRI of these patients showed specific features. MRI is helpful to obtain credible diagnosis of some rare demyelinating diseases.%目的 分析9例罕见脱髓鞘疾病的MRI表现.方法 对5例视神经脊髓炎(NMO)、1例Balò's同心圆硬化(BCS)、2例Marburg型多发性硬化(MS)和1例脱髓鞘假瘤(TDL)患者行常规MRI及增强扫描、弥散加权成像(DWI)、扩散张量成像(DTI)及磁共振波谱(MRS)检查.结果 NMO患者可见视神经及脊髓病灶,脊髓病灶纵向可达到或超过3个椎体节段,颅内的病灶不满足典型多发性硬化诊断;BCS患者MRI表现为多发性"同心圆"样改变;Marburg型MS患者MRI表现为广泛的白质病变,T1WI可见"黑洞",病灶不累及U型纤维;TDL患者常规MRI表现为大块状孤立性病变,增强扫描可见与髓静脉分布一致的明显强化条状影,占位效应不明显.本组患者脑内病灶的MRI、MRS、DWI及DTI表现均提示脱髓鞘改变.结论 部分罕见的脱髓鞘疾病具有特征性MRI表现.MR检查有助于提高临床对脱髓鞘疾病的诊断准确性.
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