Vanishing white matter disease

Discussion:

Vanishing white matter disease appears in MRI as diffuse symmetrical and progressive involvement of cerebral white matter with cystic changes, following CSF signal. Low signal intensity on T1W and high signal intensity on T2W sequences. Flair sequence shows white matter high signal intensity with patchy areas of low CSF signal intensity. T1W and Flair sequences demonstrate a radiating appearance of the fine network of remaining white matter strands best appreciated on the sagittal and the coronal views. Basal ganglia are spared. The diagnosis is made on the basis of clinical and MRI appearances.

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