Leucoencefalopatia megalencefálica com substância branca evanescente e cistos subcorticais Megalencephalic leukoencephalopathy with vanishing white matter and cystic formation

Abstract

Apresentamos três casos de leucoencefalopatia megalencefálica com substancia branca evanescente e cistos subcorticais, diagnosticados através da ressonância nuclear magnética.Os casos estudados apresentam quadro clinico e radiológico de acordo com os critérios diagnósticos estabelecidos na descrição inicial desta enfermidade. São discutidos os aspectos clínicos e neuroradiológicos.We present three cases of megalencephalic leukoencephalopathy with vanishing white matter and cystic formation in both temporal lobes, diagnosed through magnetic resonance imaging. All the cases presented clinical and radiological aspects according to the diagnostic criteria that were established in the initial description of the syndrome. Clinical and radiological aspects are discussed.

Keywords

• leucoencefalopatia
• megalencefalia
• substância branca evanescente
• síndrome de van der Knaap
• leukoencephalopathy
• megalencephaly
• vanishing white matter
• van der Knaap syndrome