title: Function and organization in dysgenic cortex. Case report.
abstract: Cerebral dysgenesis is a subject of interest because of its relationship to cerebral development and dysfunction and to epilepsy. The authors present a detailed study of a 16-year-old boy who underwent surgery for a severe seizure disorder. This patient had dysgenesis of the right hemisphere, which was composed of a giant central frontoparietal nodular gray matter heterotopia with overlying large islands of cortical dysplasia around a displaced central fissure. Exceptional insight into the function, biochemistry, electrophysiology, and histological structure of this lesion was obtained from neurological studies that revealed complementary information: magnetic resonance (MR) imaging, [18]fluoro-2-deoxy-D-glucose positron emission tomography (PET), functional PET scanning, proton MR spectroscopic (1H-MRS) imaging, intraoperative cortical mapping and electrocorticography, in vitro electrophysiology, and immunocytochemistry. These studies demonstrated compensatory cortical reorganization and showed that large areas of heterotopia and cortical dysplasia in the central area may retain normal motor and sensory function despite strikingly altered cytoarchitectonic organization and neuronal metabolism. Such lesions necessitate appropriate functional imaging studies prior to surgery and cortical mapping to avoid creating neurological deficits. Integrated studies, such as PET, 1H-MRS imaging, cortical mapping, immunocytochemistry, and electrophysiology may provide information on the function of developmental disorders of cerebral organization.
authors.affiliation: McConnell Brain Imaging Centre, Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University, Quebec, Canada.
authors.forename: M C
authors.initials: MC
authors.lastname: Preul
authors.forename: R
authors.initials: R
authors.lastname: Leblanc
authors.forename: F
authors.initials: F
authors.lastname: Cendes
authors.forename: F
authors.initials: F
authors.lastname: Dubeau
authors.forename: D
authors.initials: D
authors.lastname: Reutens
authors.forename: R
authors.initials: R
authors.lastname: Spreafico
authors.forename: G
authors.initials: G
authors.lastname: Battaglia
authors.forename: M
authors.initials: M
authors.lastname: Avoli
authors.forename: P
authors.initials: P
authors.lastname: Langevin
authors.forename: D L
authors.initials: DL
authors.lastname: Arnold
authors.forename: J G
authors.initials: JG
authors.lastname: Villemure
mesh_terms: D000293:Adolescent; D001921:Brain / Q000002:abnormalities* / Q000503:physiopathology* / Q000601:surgery; D001927:Brain Diseases / Q000150:complications / Q000175:diagnosis; D001931:Brain Mapping; D002828:Choristoma / Q000150:complications / Q000175:diagnosis; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis / Q000209:etiology; D005073:Evoked Potentials, Somatosensory; D006801:Humans; D007150:Immunohistochemistry; D007432:Intraoperative Period; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D009493:Neurosurgery; D010487:Periaqueductal Gray; D014055:Tomography, Emission-Computed