MEGALENCEPHALIC LEUKOENCEPHALOPATHY WITH SUBCORTICAL CYSTS; REPORT OF A RARE CASE

Abstract

Megalencephalic leukoencephalopathy with subcortical cysts is a hereditary autosomal recessive condition characterized by distinctive MRI findings and a varied but mild clinical course. Frontal and temporal subcortical cysts are the distinguishing features. It is typically accompanied with pyramidal and cerebellar symptoms. Our case, a four year old child, on MRI showed bilateral extensive deep white matter T2 hyperintensities involving the subcortical white matter as well as the subcortical U-fibers with relatively large subcortical cysts. Additionally, mild to moderate dilatation of the ventricular system as well as prominent cortical sulci and basal cisterns were seen. Megalencephaly is frequently discovered at an early stage. Seizures may occur, although they are normally readily managed.