Skip to main content
. 2023 Jul 26;14:1219324. doi: 10.3389/fneur.2023.1219324

Figure 11.

Figure 11

MRI features of hypomyelinating leukodystrophies. MRI features of an adolescent with spastic ataxic syndrome due to PMLD with T2 axial at 1 year (A) and 7 years (B, C) show near complete absence of myelination on the initial scan with no progression on the second scan. Note the diffuse pontine involvement in image C which is more common in PMLD than PMD.T1 axial image (D) shows hypo intensity of the white matter except in centrum semiovale. 18 q-syndrome in a 15 year old; T2 axial (E) shows reduced myelination than expected for age with a washed out appearance on FLAIR (F) but the preservation of hyperintensity on T1 images (G). Combination of these signal abnormalities on various sequences is highly suggestive of hypomyelination. MRI brain findings of an individual with POLR3-related hypomyelination with T2 (H), T1 (I), and FLAIR (J) axial images showing diffuse hypomyelination with sparing optic radiations and patchy myelin islands. T2 also shows hypo intensity of ventrolateral thalamus due to the relative preservation of myelin.