早期髓鞘化结构发育不良的磁共振成像诊断特征：病例报告。
Diagnostic MR imaging features of hypomyelination of early myelinating structures: A case report.
机构信息
Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Japan.
Department of Molecular Imaging and Diagnosis, Graduate School of Medical Sciences, Kyushu University, Japan.
出版信息
Neuroradiol J. 2024 Dec;37(6):758-760. doi: 10.1177/19714009231224419. Epub 2023 Dec 25.
Hypomyelination of early myelinating structures (HEMS) has recently been defined as a new genetic disorder accompanied by clinical and MR imaging characteristics. However, no studies have focused on diffusion-weighted imaging (DWI) findings of HEMS. We would like to propose a "sheep sign," which is formed by DWI hyperintensity in the medial medullary lamina along with alternating high-low-high (HLH) intensity stripes in the posterior limb of the internal capsule. We believe the presence of the "sheep sign" on DWI in combination with alternating HLH intensity stripes may be a valuable tool for diagnosing HEMS.
早期髓鞘形成结构发育不良（HEMS）最近被定义为一种新的遗传疾病，伴有临床和磁共振成像特征。然而，目前还没有研究关注 HEMS 的弥散加权成像（DWI）表现。我们提出了一个“羊征”，它由 DWI 中沿内侧髓板的高信号和内囊后肢的高低高（HLH）强度条纹交替组成。我们认为 DWI 上出现“羊征”并伴有 HLH 强度条纹交替可能是诊断 HEMS 的一种有价值的工具。
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