白营养不良成像:诊断困境的启示。

Q1 Medicine
Rajvi N Thakkar, Drashti Patel, Ivelina P Kioutchoukova, Raja Al-Bahou, Pranith Reddy, Devon T Foster, Brandon Lucke-Wold
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引用次数: 0

摘要

白质营养不良症是一组罕见的脱髓鞘疾病,主要影响中枢神经系统。不同类型的白质营养不良症的临床表现可能是非特异性的,因此,核磁共振成像等成像技术可用于更明确的诊断。这些疾病的特点是大脑病变具有特征性脱髓鞘模式,可作为鉴别诊断工具。在这篇综述中,我们将讨论每种白营养不良症的核磁共振成像研究结果、相关遗传学、有助于鉴别的血液检查、新兴诊断方法以及后续成像策略。本文讨论的白营养不良症包括X连锁肾上腺脑白质营养不良症、变色斑性白营养不良症、克拉伯氏病、佩里扎伊斯-梅尔茨巴赫病、亚历山大病、卡纳万病和艾卡迪-古蒂耶斯综合征。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Leukodystrophy Imaging: Insights for Diagnostic Dilemmas.

Leukodystrophies, a group of rare demyelinating disorders, mainly affect the CNS. Clinical presentation of different types of leukodystrophies can be nonspecific, and thus, imaging techniques like MRI can be used for a more definitive diagnosis. These diseases are characterized as cerebral lesions with characteristic demyelinating patterns which can be used as differentiating tools. In this review, we talk about these MRI study findings for each leukodystrophy, associated genetics, blood work that can help in differentiation, emerging diagnostics, and a follow-up imaging strategy. The leukodystrophies discussed in this paper include X-linked adrenoleukodystrophy, metachromatic leukodystrophy, Krabbe's disease, Pelizaeus-Merzbacher disease, Alexander's disease, Canavan disease, and Aicardi-Goutières Syndrome.

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CiteScore
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