Malignant transformation in a case of megalencephalic leukoe
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Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is an autosomal recessive inherited disorder characterized by macrocephaly, progressive motor disability, seizures, mild cognitive decline, slow progression, and typical magnetic resonance imaging (MRI) findings. Age of onset of symptoms is described from birth to 25 years. Most important clue for diagnosis is the characteristic

MRI changes that include diffuse involvement of subcortical white matter mainly in the frontoparietal region with relative sparing of central white matter along with subcortical cysts mostly in the anterior temporal region. Cysts are usually benign and slowly progressive. Malignant transformation of cysts has not been reported as yet. This article reports a very unusual and probably the first case of MLC who presented to us in a unique manner with late-onset and malignant transformation of cyst in the left temporal region leading to rapid neurological decline.

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