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Alexander disease (infantile type) at 28 months of age (CT)

Note megalencephaly and white matter degeneration, with lucency indicative of cystic changes in frontal and high parietal cerebrum. Cystic changes displace the frontal horns of ventricles posteriorly. Occipital and deep temporal regions are less involved. (Reprinted with permission from: Johnson 1996.)

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Associated Disorders

  • Megalencephaly
  • Mental retardation