Hemimegalencephaly on magnetic resonance imaging scan (MRI) consists of cortical gray matter almost uniformly abnormal, areas of increased thickness of the cortical gray matter (GM), abnormal gyral patterns, blurring of the grey-white matter transition, atrophy or hemispheric hypertrophy, demyelination, gliosis. We present a case of ten-year-old boy with a history of infantile spasms and developmental delay who presented to the pediatric neurology room with an episode of disinhibited behavior in family environment. An MRI was performed and isolated hemimegalencephaly with polymicrogyria of the right occipital lobe was diagnosed.
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From the Research Institute of the McGill University Health Centre (E.K., J.S.-O., N.A.-B., L.M., E.B., K.A.M., J.L.-L., J.-B.R., R.W.D., M.S.); Integrated Program in Neuroscience (E.K.), McGill University; Department of Specialized Medicine (A.A.), McGill University Health Centre; Department of Human Genetics (A.A., J.-B.R.), Faculty of Medicine; Goodman Cancer Centre (G.P., S.-H.K., N.S.), Department of Biochemistry, McGill University; Department of Pediatric Neurosurgery (T.B.-C., A.W.), Centre Hospitalier Universitaire Sainte-Justine, University of Montreal; Division of Pediatric Neurology (G.A., K.A.M., C.C.P., M.S.), Department of Pediatrics, McGill University, Montreal, Quebec, Canada; Department of Pediatrics (G.A.), Unaizah College of Medicine and Medical Sciences, Qassim University, Saudi Arabia; Department of Neurology and Neurosurgery (K.A.M., F.D., J.H., C.C.P., M.S.), McGill University Health Centre; Department of Pathology (J.K., S.A.), McGill University; Division of Neurosurgery (J.-P.F., J.A., R.W.D.), Department of Pediatric Surgery, McGill University Health Center; McGill University (B.R.); Department of Pathology (C.F.-B.), Centre Hospitalier Universitaire Sainte-Justine, University of Montreal, Quebec, Canada.
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