Temporo-spatial analyses define epileptogenic and functional zones in a case of Dyke–Davidoff–Masson syndrome

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.