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Alexander disease (infantile type) at 6 months of age (CT) (2)

(A) There is prominent enhancement bilaterally immediately rostral to the tip of the anterior horn of the lateral ventricle and in the head of the caudate nucleus. (B) Both caudate nuclei exhibit enhancement in the head and body. (C) Prominent, bilateral periventricular enhancement is present. (At 3 months of age, similar but less pronounced enhancement had been present.) (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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  • Megalencephaly
  • Mental retardation