Radiological imaging findings of Dyke-Davidoff-Masson syndrome

Abstract

Radiological findings of Dyke-Davidoff-Masson syndrome (DDMS) in patients with different etiologies are presented in our study. The study included 12 patients (seven females, five males) for whom radiological examinations were requested due to reasons such as epilepsy, mental retardation, and/or hemiplegia. CT was performed in 12, MRI in 6, MRA in 1, and DSA in 1 patient. Following imaging findings were evaluated: cerebral and cerebellar involvement (laterality, encephalomalacia), affected territories, ventricular enlargement, sulcal enlargement, calvarial thickening, and paranasal sinus enlargement hyperaeration. Age range of the patients was 5-62 (mean 34.1 ± 21.7). Left hemicrania was affected in eight patients, right hemicrania in four. Ipsilateral calvarial thickening and lateral ventricular dilatation were observed in all patients. 11 patients had ipsilateral frontal sinus hyperaeration, sulcal enlargement and encephalomalacia. Wallerian degeneration of the mesencephalon and middle fossa hypoplasia was seen in ten patients, mastoid hyperaeration, third ventricular enlargement and thalamic involvement in nine, and corpus callosum, basal ganglion injury, and sphenoid sinus hyperaeration in eight. MCA, ACA, and PCA territories were involved in six patients. Only MCA territory involvement was seen in four patients. Cerebellar atrophy was contralateral in two patients. Symmetric bilateral atrophy was observed in one patient. DDMS can be encountered with different radiological findings based on cerebral damage formation process and the extent of damage. Patients may have different levels of cerebral hemiatrophy, ipsilateral carvarial thickening, and lateral ventricular dilatation.

Keywords: Calvarial thickening; Cerebral hemiatrophy; Computed tomography; Dyke–Davidoff–Masson syndrome; Magnetic resonance imaging.