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.