1. X-linked adrenoleukodystrophy
A, B. T2-weighted (A) and FLAIR (B) images of a 5-year-old boy show hyperintensity in the splenium and adjacent occipital white matter. C. Contrast enhancement indicates active inflammation.
2. X-linked adrenoleukodystrophy
A, B. White matter hyperintensity on T2-weighted images indicates severe demyelination in the occipital and parietal lobes, with sparing of most of the frontal lobes. The splenium is also hyperintense.
3. Metachromatic leukodystrophy
There is diffuse hyperintensity in the cerebral white matter on this T2-weighted image.
4. Maple syrup urine disease
Maple syrup urine disease.
This 9-month-old infant with classic type maple syrup urine disease suffered an acute decompensation following a viral illness. A. There is abnormal hyperintensity in the globi pallidi and internal capsules on this T2-weighted MR image. B. A diffusion-weighted image shows hyperintensity in the internal capsules, globi pallidi, cerebral peduncles, and brainstem.
A. There is deficient myelination throughout most of the cerebral white matter on this T2-weighted image of a 4-year-old child. B. An axial FLAIR image shows more pronounced signal abnormality in the frontal and posterior parietal regions, suggesting gliosis. C. The corpus callosum and the brainstem are small.
6. Pelizaeus-Merzbacher disease
T2-weighted MR images of a 2½-year-old child with developmental delay show marked deficiency of brain myelination.
There is bilateral frontal lobe hypoattenuation on this unenhanced CT image.
There are dense calcifications in the basal ganglia and periventricular white matter on this unenhanced CT image. The ventricles are enlarged in association with generalized cerebral atrophy/hypoplasia.
A. A CT image obtained at 3 years of ...
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