Invited Article: An MRI-based approach to the diagnosis of white matter disorders
Invited Article: An MRI-based approach to the diagnosis of white matter disorders
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DOI：
10.1212/01.wnl.0000343049.00540.c8
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发表时间：
2009-02-24
期刊：
影响因子：
9.9
通讯作者：
van der Knaap, Marjo S.
中科院分区：
文献类型：
--
作者：
Schiffmann, Raphael;van der Knaap, Marjo S.
背景：有许多不同的白色物质障碍，遗传性和获得性，因此诊断过程是困难的。建立一个具体的诊断往往被推迟在巨大的情感和经济成本。所涉及的大脑结构的模式，如通过MRI可视化，已被证明往往具有很高的诊断特异性。方法：我们开发了一个全面的实用算法，主要依赖于脑MRI的特点。初始决策点定义了一种低髓鞘化模式，其中大脑白色物质在T1加权图像上相对于皮质是高信号（正常）、等信号或略低信号，与其他病变相比，T1加权图像上大脑白色物质的低信号更为突出。在所有的病理类型中，受累的白色物质在T2加权像上都是高信号，但是，一般来说，与其他病理相比，在髓鞘形成不足时T2高信号较不明显。一些髓鞘生成不足的疾病通常与周围神经受累有关，而另一些则不相关。除髓鞘形成不足以外的其他病理患者的病变可以是融合的或孤立的和多灶性的。在融合性病变的疾病中，异常的分布具有很高的诊断价值。额外的MRI功能，如白色物质稀疏，存在囊肿，对比增强，并存在钙化，进一步缩小诊断possibility.Conclusion：应用系统的决策树在MRI的白色物质疾病有利于特定病因实体的诊断。神经病学（R）2009; 72：750-759
Background: There are many different white matter disorders, both inherited and acquired, and consequently the diagnostic process is difficult. Establishing a specific diagnosis is often delayed at great emotional and financial costs. The pattern of brain structures involved, as visualized by MRI, has proven to often have a high diagnostic specificity.Methods: We developed a comprehensive practical algorithm that relies mainly on the characteristics of brain MRI.Results: The initial decision point defines a hypomyelination pattern, in which the cerebral white matter is hyperintense (normal), isointense, or slightly hypointense relative to the cortex on T1-weighted images, vs other pathologies with more prominent hypointensity of the cerebral white matter on T1-weighted images. In all types of pathology, the affected white matter is hyperintense on T2-weighted images, but, as a rule, the T2 hyperintensity is less marked in hypomyelination than in other pathologies. Some hypomyelinating disorders are typically associated with peripheral nerve involvement, while others are not. Lesions in patients with pathologies other than hypomyelination can be either confluent or isolated and multifocal. Among the diseases with confluent lesions, the distribution of the abnormalities is of high diagnostic value. Additional MRI features, such as white matter rarefaction, the presence of cysts, contrast enhancement, and the presence of calcifications, further narrow the diagnostic possibilities.Conclusion: Application of a systematic decision tree in MRI of white matter disorders facilitates the diagnosis of specific etiologic entities. Neurology (R) 2009; 72: 750-759