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Romanian Journal of Neurology, Volume XV, No. 1, 2016
ISSN 1843-8148  |  e-ISSN 2069-6094
ISSN-L 1843-8148
DOI: 10.37897/RJN

Indexată BDI  |  IDB Indexed

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DYKE-DAVIDOFF-MASSON SYNDROME PRESENTING WITH RECURRENT SEIZURES

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 ABSTRACT

Dyke-Davidoff-Masson syndrome (DDMS) is cerebral hemiatrophy occurring following brain insult resulting from infarct, trauma or infection in utero or soon after birth. Clinical features of this syndrome include variable degree of contralateral hemiparesis, facial asymmetry, seizures and mental retardation. Recurrent seizures is the most debilitating and poor prognostic indicator of this disease. Neuroimaging plays pivotal role in establishing the diagnosis of DDMS syndrome. CT/MR imaging shows unilateral cerebral parenchymal atrophy, prominent sulcal spaces, ipsilateral ventriculomegaly, cerebellar atrophy, falcine and superior Sagittal sinus shift to affected side. Bone windowing in CT scan shows decreased skull volume on affected side calvarial thickening, elevation of sphenoid wing and petrous temporal bone, expanded sinuses and mastoids. Here we describe 34 years old female who had hemiparesis, seizures and facial asymmetry since childhood and imaging evaluation established diagnosis of congenital type of DDMS.

Keywords: Dyke-Davidoff-Masson syndrome, seizures, hemiatrophy and ventriculomegaly

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