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1. X-linked adrenoleukodystrophy

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Figure 18-1

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.

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2. X-linked adrenoleukodystrophy

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Figure 18-2

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.

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3. Metachromatic leukodystrophy

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Figure 18-3

Metachromatic leukodystrophy.

There is diffuse hyperintensity in the cerebral white matter on this T2-weighted image.

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4. Maple syrup urine disease

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Figure 18-4

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.

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5. Phenylketonuria

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Figure 18-5

Phenylketonuria.

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.

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6. Pelizaeus-Merzbacher disease

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Figure 18-6

Pelizaeus-Merzbacher disease.

T2-weighted MR images of a 2½-year-old child with developmental delay show marked deficiency of brain myelination.

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7. Alexander disease

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Figure 18-7

Alexander disease.

There is bilateral frontal lobe hypoattenuation on this unenhanced CT image.

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8. Cockayne syndrome

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Figure 18-8

Cockayne syndrome.

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.

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9. Huntington disease

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Figure 18-9

Huntington disease.

A. A CT image obtained at 3 years of ...

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