Basal ganglia involvement in the late stage of vacuolating megalencephalic leukoencephalopathy

Vacuolating megalencephalic leukoencephalopathy is a recently defined entity characterized by swelling of white matter, subcortical cysts in fronto-temporal regions and slow progression of neurological symptoms that contrasts with the severity of radiological findings. We present a late follow-up of a case with vacuolating megalencephalic leukoencephalopathy. There was an improvement of the brain swelling and associated sulcal enlargement. Of notice was basal ganglia involvement which has not been described previously.