113

FIGURE 113-1

FIGURE 113-2

FIGURE 113-3

FIGURE 113-4

FINDINGS Figure 113-1. Axial T2WI through the basal ganglia. There is diffusely hyperintense white matter (WM) (stars), indicating lack of mature myelin at 1 year of age. Figure 113-2. Corresponding axial T1WI in the same patient. There is normal hyperintense myelin only at the posterior limbs of the internal capsules and along the optic radiations (arrows). Figures 113-3 and 113-4. Axial T2WI with corresponding T1WI through the basal ganglia, respectively, in a companion case. There is diffuse hyperintense myelin on T2WI (stars) and lack of normal hyperintense myelin on T1WI throughout the brain.

DIFFERENTIAL DIAGNOSIS Pelizaeus-Merzbacher disease (PMD), metachromatic leukodystrophy, Lowe syndrome, Salla disease, trichothiodystrophy.

DIAGNOSIS PMD.

DISCUSSION

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