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Diagnostic MR imaging features of hypomyelination of early myelinating structures: A case report
Tokiko Abe1, Koji Yamashita1, Kazufumi Kikuchi2
1Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, Japan.
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.
Insights
A new "sheep sign" on diffusion-weighted imaging (DWI) may help diagnose Hypomyelination of Early Myelinating Structures (HEMS). This finding combines specific DWI hyperintensity and alternating intensity stripes, offering a potential diagnostic tool for this genetic disorder.
Area of Science:
- Neurology
- Medical Imaging
- Genetics
Background:
- Hypomyelination of Early Myelinating Structures (HEMS) is a recently defined genetic disorder.
- Clinical and MRI characteristics of HEMS are known, but diffusion-weighted imaging (DWI) findings remain understudied.
Purpose of the Study:
- To investigate and describe the specific diffusion-weighted imaging (DWI) findings in HEMS.
- To propose a novel imaging sign, the "sheep sign," for HEMS diagnosis.
Main Methods:
- Analysis of diffusion-weighted imaging (DWI) data from patients with HEMS.
- Identification of characteristic DWI patterns, including medial medullary lamina hyperintensity and internal capsule intensity variations.
Main Results:
- The study proposes a novel "sheep sign" on DWI for HEMS.
- This sign is characterized by DWI hyperintensity in the medial medullary lamina.
- Alternating high-low-high (HLH) intensity stripes in the posterior limb of the internal capsule are also observed.
Conclusions:
- The "sheep sign" on DWI, along with HLH intensity stripes, may be a valuable diagnostic tool for HEMS.
- This imaging finding could aid in the early and accurate diagnosis of this genetic disorder.
