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Case Reports
. 2024 Dec;37(6):758-760.
doi: 10.1177/19714009231224419. Epub 2023 Dec 25.

Diagnostic MR imaging features of hypomyelination of early myelinating structures: A case report

Tokiko Abe  1 , Koji Yamashita  1 , Kazufumi Kikuchi  2 , Eriko Hatai  3 , Fumihiko Fujii  3 , Pin Fee Chong  3 , Yasunari Sakai  3 , Hirotomo Saitsu  4 , Ken Inoue  5 , Osamu Togao  2 , Kousei Ishigami  1
Affiliations
Case Reports

Diagnostic MR imaging features of hypomyelination of early myelinating structures: A case report

Tokiko Abe et al. Neuroradiol J. 2024 Dec.

Abstract

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.

Keywords: Hypomyelination; diffusion magnetic resonance imaging; magnetic resonance imaging.

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Conflict of interest statement

Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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