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

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.