Dyke-Davidoff-Masson Syndrome: Cases of Two Brothers and Literature Review

Dyke-Davidoff-Masson syndrome (DDMS) has cerebral hemiatrophy and compensatory ipsilateral skull thickening, and is manifested by recurrent seizures and hemiparesis. We present one case with typical DDMS, who had a brother suffering from epilepsy with mild imaging abnormality relevant to DDMS and similar seizure semiology. A 26-year-old man had a history of developmental delay, mental retardation, hemiparesis and recurrent seizures. His brother, 23-year-old man had also experienced recurrent seizures, but he had no neurological deficits. Older brother experienced focal motor seizures with/without secondary generalization. Sometimes, he noted an auditory aura. MRI demonstrated the hemispheric atrophy with the adjacent bony hypertrophy. The seizures of younger brother were mainly of the auditory type and the MRI showed mild hemispheric atrophy with hippocampal sclerosis without any bony change. Our sibling cases might have a familial predisposition and support the idea that clinical courses and radiological findings of DDMS are varied even within one family.