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Society for Pediatric Radiology – Poster Archive

Vanishing White Matter
Showing 1 Abstract.

Lorentz Liam

Final Pr. ID: Poster #: CR-020


Leukodystrophy with vanishing white matter (VWM), previously known as childhood ataxia with central hypomyelination (CACH) is considered one of the most prevalent inherited white matter disorders.<span style="font-size:11px"> </span>The incidence of VWM is not well defined, however, there appears to be a predilection for Caucasians.

Clinical history

We report a 20 month old male with a two week history of right hemiplegia and ataxic gait. Infant is well grown with no previous medical or familial history of note. Normal development and unremarkable birth history.

MRI findings

Brain MRI demonstrates multiple T2 hyperintense periventricular deep white matter cavitating lesions with cortical sparing (fig 2, top arrow). These lesions were of CSF signal intensity on FLAIR and demonstrated differential water content; central hypointensity with peripheral hyperintesity (fig 3). There is no restricted diffusion centrally, however, the peripheral areas that are hyperintense on FLAIR were restricting. The temporal lobes are unaffected. Single, non-enhancing cerebellar white matter hyperintensity (non-cystic) within right hemisphere (fig 2, bottom arrow); no cerebellar or brainstem atrophy. White matter involvement of the corpus callosum with outer rim sparing (fig 1). There is no hydrocephalus and no basal ganglia involvement.
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Authors:  Lorentz Liam

Keywords:  Leukodystrophy, Vanishing White Matter