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      Dyke–Davidoff–Masson syndrome: A rare cause of cerebral hemiatrophy in children

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          Abstract

          Dyke–Davidoff–Masson syndrome (DDMS) is an uncommon condition, in which the diagnosis is mainly done by various clinical presentations along with positive radiological findings. Patients have facial asymmetry, seizures, learning difficulties, and contralateral hemiparesis. The radiological discoveries of the same incorporate cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Here, we report a case of a 10-year-old female child who presented with a single episode of convulsion, mental retardation, and contralateral hemiparesis. Magnetic resonance imaging of the brain showed unilateral atrophy of the left cerebral hemisphere with dilatation of ipsilateral lateral ventricle and ipsilateral sulcal prominence. These findings were suggestive of the diagnosis of DDMS.

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          Disorders of cortical formation: MR imaging features.

          The purpose of this article was to review the embryologic stages of the cerebral cortex, illustrate the classification of disorders of cortical formation, and finally describe the main MR imaging features of these disorders. Disorders of cortical formation are classified according to the embryologic stage of the cerebral cortex at which the abnormality occurred. MR imaging shows diminished cortical thickness and sulcation in microcephaly, enlarged dysplastic cortex in hemimegalencephaly, and ipsilateral focal cortical thickening with radial hyperintense bands in focal cortical dysplasia. MR imaging detects smooth brain in classic lissencephaly, the nodular cortex with cobblestone cortex with congenital muscular dystrophy, and the ectopic position of the gray matter with heterotopias. MR imaging can detect polymicrogyria and related syndromes as well as the types of schizencephaly. We concluded that MR imaging is essential to demonstrate the morphology, distribution, and extent of different disorders of cortical formation as well as the associated anomalies and related syndromes.
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            Dyke-Davidoff-Masson syndrome (DDMS).

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              Dyke–Davidoff–Masson syndrome: Classical imaging findings

              A 15-year-old female presented with seizures, right-sided hemiparesis, hemiatrophy of the right side of the body and mental retardation. MRI brain revealed characteristic features diagnostic of congenital type of cerebral hemiatrophy or Dyke–Davidoff–Masson syndrome.
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                Author and article information

                Journal
                J Pediatr Neurosci
                J Pediatr Neurosci
                JPN
                Journal of Pediatric Neurosciences
                Medknow Publications & Media Pvt Ltd (India )
                1817-1745
                1998-3948
                Jul-Sep 2016
                : 11
                : 3
                : 252-254
                Affiliations
                [1]Department of Pediatrics, Baroda Medical College, Vadodara, Gujarat, India
                Author notes
                Address for correspondence: Dr. Pareshkumar A. Thakkar, Baroda Medical College, Vadodara, Gujarat, India. E-mail: pareshthakkar@ 123456yahoo.com
                Article
                JPN-11-252
                10.4103/1817-1745.193365
                5108134
                27857800
                79ceed14-3e31-400a-ac81-98ebe778a71b
                Copyright: © Journal of Pediatric Neurosciences

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

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                Categories
                Case Report

                Neurosciences
                cerebral hemiatrophy,dyke–davidoff–masson syndrome,hemiparesis
                Neurosciences
                cerebral hemiatrophy, dyke–davidoff–masson syndrome, hemiparesis

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