Dyke-Davidoff-Masson syndrome (cerebral hemiatrophy): Radiological findings

Abstract

The aim of this study was to present crosssectional cranial imaging findings of cases with Dyke-Davidoff-Masson syndrome (DDMS). Methods: The findings of 16 cases in whom unilateral cerebral hemispheric atrophy was detected at computed tomography and/or magnetic resonance imaging were retrospectively evaluated. The cases consisted of 8 females and 8 males, the ages ranged between 5 and 53 (mean:24). Six of the cases were children and 10 were males. Five of the patients had CT and 13 had MRI scan. The images were retrospectively evaluated and cerebral parenchymal findings and compensatory cranial findings were noted. Results: All cases had unilateral cerebral hemispheric atrophy, ipsilateral cortical sulcal and lateral ventricular dilatation. Together with hemispheric atrophy ipsilateral atrophy of corpus callosum in 6 cases (37.5%), ipsilateral thalamic atrophy in 13 cases (81%), ipsilateral parahippocampal atrophy in 8 cases (50%), ipsilateral cerebral pedincular atrophy in 7 cases (44%) and ipsilateral pontine atrophy in 3 cases (19%) were detected. Gliotic signal changes were observed in 13 cases (81%). Of compensatory findings, unilateral calvarial thickening was focal in 4 cases (25%), and diffuse in 12 cases (75%). There was expansion in ipsilateral half of frontal sinus in 15 cases (94%) and expansion in temporal bone aeration in 5 cases (31%). Conclusion: DDMS is a syndrome presenting with findings of cerebral hemiatrophy and calvarial hypertrophy. Cross-sectional radiological imaging findings may be variable among cases.