Brain MRI of a 16-year-old female with vanishing white matter disease

MR images of Patient 3. Shown are the transverse T2-weighted (A, B), proton density (C), and coronal FLAIR (D) images. The abnormal cerebral hemispheric white matter has a homogeneously high signal on T2-weighted images, but partly high and partly low signals on proton density and FLAIR images. The signal intensity is lowest in the white matter closest to the lateral ventricles (C, D). Note the radiating stripelike pattern in the low-signal intensity areas (C, D). Within the posterior limb of the internal capsule, a double line of signal abnormality is seen (B; dashed arrows). Within the brainstem, the central tegmental tracts (A; arrowheads) and transverse pontine fibers (A; arrows) are involved.