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

Abstract

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.

Dyke-Davidoff-Masson Syndrome as a Rare Cause of Cerebral Hemiatrophy: Insights From a Case Series

“…Seizures can be focal or generalized. A complex partial seizure with secondary generalization had also been reported [ 12 ].…”

Dyke-Davidoff-Masson syndrome with Coarctation of Aorta: Case Report of A Novel Association

“…It is characterized by asymmetrical growth of cerebral hemispheric with atrophy of one side and midline shift, ipsilateral osseous hypertrophy with hyper pneumatisation of sinuses mainly frontal and mastoid air cells with contralateral paresis 3 . Clinical presentations include variable degree of facial asymmetry, seizures (focal ,complex partial, generalized), contralateral hemiparesis, mental retardation, learning disabilities, impaired speech, etc 4 .Both sexes and any of the hemisphere may be affected, but male gender and left side involvement are more common 5 .Two forms of the disease exist: a congenital form manifesting in the perinatal or infancy period, likely due to vascular occlusion in the gestational or neonatal phase and an acquired form, likely due to a later trauma, infection, ischaemia, or haemorrhage. The acquired form is likely to have a variable time period of presentation, appearing up to and into adolescence.…”

Dyke-Davidoff-Masson syndrome

“…Complex partial seizure with secondary generalization also had been reported. [10] Both sexes and any of the hemisphere may be affected, but male gender and left side involvement are more common. [11] The human brain reaches half of its adult size during the first year of life and three fourth of the adult size is attained by the end of 3 years.…”