Dyke-Davidoff-Masson Syndrome/ Cerebral Hemiatrophy in an Adult Male : A Rare Imaging Case
Journal Title: Indian Journal of Applied Radiology - Year 2016, Vol 2, Issue 1
Abstract
Dyke Davidoff Masson Syndrome (DDMS) is a rare condition characterized by seizures, hemiparesis/ hemiplegia, facial asymmetry and mental retardation. The underlying etiology is cerebral insult that may occur in uteroIor early in life. All modalities ableto image the brain (x ray skull, ultrasound, CT and MRI) will be able to more or less identify the key features, although MRI will give the best imaging. Radiologically, magnetic resonance (MR) and computed tomography (CT) shows the parenchymal abnormalities of unilateral loss of cerebral volume with ipsilateral ex vacuo dilatation of the lateral ventricle and sulci. There are compensatory bone alterations in the calvarium, such as thickening of the skull vault and enlargement of the paranasal sinuses and mastoid cells as well as elevation of the, orbital roof, petrous ridge and greater wing of the sphenoid bone. This occurs in adaptation to the relative vacuum created by the brain atrophy. Atrophy/hypoplasia of cerebral peduncle, basal ganglia, thalamus and cerebellum (ipsilateral or contra lateral hemisphere) may be present. In this case report, we discuss a case of twenty five year-old married male who presented with seizures, facial asymmetry, mental retardation and right -sided hemiparesis. Classical findings of the Dyke Davidoff Masson Syndrome were present on CT scan, so diagnosis was made on basis of the history, clinical features and radiological findings.
Authors and Affiliations
Seema Sharma
Keywords
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