Progression of white matter abnormalities on brain MRI in patients with vanishing white matter disease at different ages of onset

Progression of white matter abnormalities at transverse fluid-attenuated inversion recovery (FLAIR) MRI examinations in patients with different ages at onset.

(A, E) First (A) and last (E) MRI scans (at ages 7 months and 23 months) in a male patient with onset at 7 months. FLAIR hyperintensity of the internal capsule and corpus callosum and minimal rarefaction in the deep cerebral white matter are present on the first MRI scan (repetition time msec/echo time msec/inversion time msec, 6000/120/2000). Complete cystic degeneration of the cerebral white matter is visible on the last MRI scan (9774.8/109/2500). On the last MRI scan, the posterior part of the lateral ventricles is dilated because of white matter collapse; the anterior part of the ventricles has remained narrow, and the cystic white matter looks swollen.

(B, F) First (B) and last (F) MRI scans (at ages 19 months and 30 months) in a male patient with onset at 14 months. The first MRI scan (8500/119/2500) shows diffuse white matter abnormalities with rarefaction in the deep white matter. On the last MRI scan (9000/105/2200), the cerebral white matter is fully replaced by fluid, and the cystic white matter looks swollen.

(C, G) First (C) (9000/105/2200) and last (G) (9500/126.3/2250) MRI scans (at ages 8 years and 25 years) in a female patient with onset at 7 years. The deep white matter contains radiating stripes, reflecting better-preserved perivascular tissue strands within rarefied tissue. The last MRI scan shows a subtle increase in rarefied white matter and atrophy of the cerebral white matter.

(D, H) First (D) and last (H) MRI examinations (at 48 years and 55 years) of a female patient with onset at age 46 years. The first MRI scan (8000/126.3/2347) shows mild rarefaction of the periventricular white matter, gliosis of the deep and subcortical white matter, and cerebral atrophy. The last MRI scan (8000/127.3/2344) displays no evident increase in rarefaction and a slight increase in cerebral atrophy.