Abstract

Dyke–Davidoff–Masson syndrome is a rare condition with classical, clinical and radiological changes – mental retardation, hemiparesis, facial asymmetry, seizures and cerebral hemiatrophy with calvarial changes. Contralateral cerebellar atrophy is rare and occurs if insult occurs after 1 month of age. We report a case of a 6-year-old female child presenting with right-sided hemiparesis, convulsions and left cerebral hemiatrophy with an old infarct in left middle cerebral artery (MCA) territory, ipsilateral calvarial thickening and right (crossed) cerebellar atrophy.

Highlights

  • Dyke–Davidoff–Masson syndrome (DDMS) is a rare disease characterised by cerebral atrophy in one cerebral hemisphere because of an insult in utero or in early childhood characterised by hemiparesis, seizures, mental retardation and facial asymmetry

  • Hemiparesis, seizures and mental retardation were seen in nine patients

  • The classical computed tomography (CT) and Magnetic resonance imaging (MRI) findings of DDMS are seen only if the brain insult occurs before 3 years of age

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Summary

Introduction

Dyke–Davidoff–Masson syndrome (DDMS) is a rare disease characterised by cerebral atrophy in one cerebral hemisphere because of an insult in utero or in early childhood (acquired cases) characterised by hemiparesis, seizures, mental retardation and facial asymmetry. Magnetic resonance imaging (MRI) of the brain showed atrophy of the right cerebellar hemisphere with prominence of the cerebellar folia (Figure 1). Hemiparesis, seizures and mental retardation were seen in nine patients.

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