Early and late brain MRIs of a patient with autopsy-confirmed juvenile Alexander disease, obtained at ages 20 months and nine years

Early (A) and late (B-D) MR studies of a patient with autopsy-confirmed juvenile Alexander disease, obtained at ages 20 months (A) and 9 years (B-D). The early T2-weighted image (A) shows extensive cerebral white matter abnormalities with partial sparing of the occipital region. There is a thin periventricular rim of low signal intensity (arrows, A). The basal ganglia and thalamus have an increased signal intensity. The putamen and caudate nucleus are mildly swollen (A). On follow-up, the extent of the cerebral white matter abnormalities is more or less the same; the occipital white matter is still partially spared (D). The basal nuclei are dark and atrophic on the T2-weighted images (D). A thin periventricular rim of low signal intensity is visible (arrows, D). The proton density-weighted image (C) shows enormous cysts in the frontoparietal white matter, a large cavum vergae, and enlarged lateral ventricles. A lesion is seen in the posterior part of the medulla (B).