| 陈雷,杜湘珂.家族性异染性脑白质营养不良的磁共振成像及磁共振质子波谱表现：附3例报告及文献综述[J].中国医学影像技术,2003,19(6):738~741 |
| 家族性异染性脑白质营养不良的磁共振成像及磁共振质子波谱表现：附3例报告及文献综述 |
| MRI and 1H-MRS Findings in Familial Metachromatic Leukodystrophy: 3 Cases Report and Review of Literatures |
| 投稿时间：2003-03-17 |
| DOI： |
| 中文关键词: 脑白质营养不良 MRI 1H-MRS |
| 英文关键词:Leukodystrophy Magnetic resonance imaging Proton magnetic resonance spectroscopy |
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| 中文摘要: |
| 目的 探讨异染性脑白质营养不良(metachromatic leukodystrophy，MLD)的磁共振成像(MRI)及磁共振质子波谱学(proton magnetic resonance spectroscopy，1H-MRS)表现。方法 3例患者，为一家姐弟3人，年龄分别为1.5、5.5和7.5岁，依靠临床综合表现及血白细胞芳基硫酸酯酶A(arylsulfastase A)明显减低确诊MLD。行颅脑MRI检查，并在左侧基底节区及左侧侧脑室后角旁脑白质内行1H-MRS。另随机选取10例正常儿童作为对照组。结果 3例患者中，2例MRI检查未见异常。1例可见弥漫性，对称性脑白质异常T2高信号，以侧脑室额角和枕角旁显著。3例MLD的1H-MRS中发现脑内NAA/Cr是对照组的52%~70%(基底节区)及46%~77%(脑白质)，mI/Cr是对照组的135%~277%(基底节区)至167%~303％(脑白质)， mI/NAA在病例组较对照组变化更加明显，分别为正常组的212%~444%(基底节区)及193%~660%(脑白质)。结论 MRI和1H-MRS可以反映MLD脑内异常病理及生化改变，尤其是1H-MRS能够在MLD脑组织结构改变之前发现生化代谢的异常，敏感性较好，对于MLD的早期诊断和治疗有积极的意义。 |
| 英文摘要: |
| Objective To evaluate the pathologic and biochemical changes of metachromatic leukodystrophy (MLD) in magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H-MRS). Methods Three children were diagnosed MLD by clinical signs and decreasing arylsulfastase A in leukocyte of blood. The whole brain was scanned by using MRI, and 1H-MRS was performed in the areas of basal ganglia and white matter close to occipital angle of ventricle. Ten matched normal children were randomly choosen as controls. Results In the three cases, two were normal in the clinical and MRI examination, one was found widespread, symmetrical periventricular high signal intensity in the white matter of the cerebral hemispheres on T2WI. In the three cases, NAA/Cr were 52%-70%(areas of basal ganglia) and 46%-77%(white matter close to occipital angle of ventricle),mI/Cr were 135%-277%(basal ganglia) and 167%-303％(white matter),mI/NAA were 212%-444%(basal ganglia) and 193%-660%(white matter) as controls. Conclusion The changes of MRI and 1H-MRS in the MLD's brains can reflect the abnormal pathologic and biochemical process. Especially 1H-MRS can detect early functional changes before structural changes occur. |
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