Clinical and Radiological Manifestations of Dyke-Davidoff-Masson Syndrome

PURPOSE. This study aims to determine the clinical-radiological features of Dyke-Davidoff-Masson syndrome (DDMS) in children and adults. MATERIALS AND METHODS. A total of 11 patients (7 males, 4 females) aged 2-46 years with a diagnosis of cerebral hemiatrophy at our institution between 2012 and 2018 were retrospectively reviewed. The 11 patients were classified into two groups: children (n = 4) and adults (n = 7). Clinical symptoms, etiologies, and imaging characteristics found in computed tomography and magnetic resonance imaging were compared and analyzed. RESULTS. Among the 11 patients, left hemisphere involvement was observed in 5 patients, and right hemisphere involvement was noted in 6 patients. Etiologic investigation found a congenital cause in 1 patient and acquired causes in 9 patients; the etiology for 1 patient was not identified. Seizure was observed in all patients, contralateral hemiparesis was found in 9 patients (3 children and 6 adults), and mental retardation was noted in 10 patients (4 children and 6 adults). Imaging characteristics including ipsilateral sulcal enlargement, lateral ventricular dilatation, and ipsilateral calvarial thickening were observed in all patients. Porencephalic cyst and Wallerian degeneration of ipsilateral midbrain and pons were found in 7 patients (2 children and 5 adults). Midline structural shift, elevation of orbital roof, and petrous ridge were seen in 8 patients (2 children and 6 adults). Hyperpneumatization of a paranasal sinus and mastoid were found in 6 patients (5 adults and 1 child). All differences in imaging characteristics between these two groups did not reach statistical significance. CONCLUSION. Clinical presentations of seizure, contralateral hemiparesis, and mental retardation might appear in a patient with DDMS regardless of age group. Subtle differences between children and adults were identified in radiological manifestations of DDMS. Identifications of porencephalic cyst, Wallerian degeneration of ipsilateral midbrains and pons, midline structural shift, paranasal sinus, and mastoid hyperpneumatization were more likely among adults. Nonetheless, larger samples are required to establish distinct features of the different age groups.