Dyke-Davidoff-Masson syndrome (DDMS) is a rare epilepsy syndrome that is 
characterized by cerebral hemiatrophy, homolateral skull hyperplasia, 
hyperpneumatization of the paranasal sinuses, seizures with or without mental 
retardation, and contralateral hemiparesis. We describe a case of DDMS in a 
40-year-old female who had complex partial seizures with occasional secondary 
generalization since the age of 4 years. Her seizure frequency was 10-20 
seizures/month even though she took four antiepileptic drugs. We applied 
magnetic resonance imaging (MRI), positron emission tomography (PET), functional 
MRI, and invasive electroencephalography (EEG) to define her epileptogenic and 
functional zones. Brain MRI showed prominent atrophy in the left frontal dorsal 
and lateral regions and mild atrophy of the left superior temporal gyrus and 
left parietal gyri. Interictal PET revealed decreased glucose metabolism in the 
atrophic regions. Functional MRI demonstrated that the inferior frontal and 
inferior parieto-occipital regions of the right hemisphere were activated by 
language testing. Invasive EEG revealed that the left lateral temporal lobe was 
the sole source of her seizures. Our results imply that the "metabolic border 
zone" rather than the atrophic region plays an important role in seizure 
activity, and that reorganization of functional zones occur after cerebral 
damage early in life.