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Alexander disease (infantile type) (MRI)

Child at age 7 years 9 months. (A) In this sagittal, T1-weighted image, both frontal lobes and the left parietal lobe show cystic encephalomalacia, and there is some atrophy of the vermis. (B) This axial, proton density image demonstrates a diffuse and abnormal, increased signal consistent with diffuse hypomyelination (demyelination, dysmyelination) in the remaining white matter posteriorly. There is marked ventriculomegaly in both images. (Reprinted with permission. Johnson AB. Alexander disease. In: Moser HW, editor. Neurodystrophies and neurolipidoses. Handbook of clinical neurology. Vol. 66, revised series vol. 22. Amsterdam: Elsevier, 1996:701-10.)

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Associated Disorders

  • Megalencephaly
  • Mental retardation