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.
Highlights
Dyke-Davidoff-Masson syndrome (DDMS) is cerebral hemiatrophy occurring following insult resulting from infarct, trauma or infection in utero or soon after birth. [1,2,3,4] Clinical features of this syndrome include variable degree of contralateral hemiparesis, facial asymmetry seizures and mental retardation. [3,5] Imaging of brain will reveal diffuse volume loss of brain on the affected side, prominent sulcal spaces and dilated ventricle on the atrophic side and sometimes gliosis [2,3,4,5]
DDMS was described by Dyke-Davidoff and Masson in their study of skull radiographs and pneumoencephalographic data in 9 patients whose characteristics included hemiparesis, seizures, facial-asymmetry, and mental retardation in 1933
(2) Hangmen etal in their study of 19 cases showed that males are more often affected than females by DDMS and left sided hemiatrophy is more common than right sided hemiatrophy [6]
Summary
Dyke-Davidoff-Masson syndrome (DDMS) is cerebral hemiatrophy occurring following insult resulting from infarct, trauma or infection in utero or soon after birth. [1,2,3,4] Clinical features of this syndrome include variable degree of contralateral hemiparesis, facial asymmetry seizures and mental retardation. [3,5] Imaging of brain will reveal diffuse volume loss of brain on the affected side, prominent sulcal spaces and dilated ventricle on the atrophic side and sometimes gliosis [2,3,4,5]. 34 years young lady came with complaints of recurrent seizure episodes since 4 years of her age Her seizures were left focal getting secondarily generalised. Neurological examination revealed mental retardation of MMSE score 15 She had dystonic involuntary movement is her left hand & left foot. She had spastic weakness of left upper and lower limb 4/5, deep tendon jerks were normal right side and brisk on the left side, she had facial asymmetry. Her plantars response was down going on both side. First monthly follow up was done but there were no signs decrease frequency of seizures attacks
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