Function and organization in dysgenic cortex. Case report

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...

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Published inJournal of neurosurgery Vol. 87; no. 1; p. 113
Main Authors Preul, M C, Leblanc, R, Cendes, F, Dubeau, F, Reutens, D, Spreafico, R, Battaglia, G, Avoli, M, Langevin, P, Arnold, D L, Villemure, J G
Format Journal Article
LanguageEnglish
Published United States 01.07.1997
Subjects
Adolescent
Brain - abnormalities
Brain - physiopathology
Brain - surgery
Brain Diseases - complications
Brain Diseases - diagnosis
Brain Mapping
Choristoma - complications
Choristoma - diagnosis
Electroencephalography
Epilepsy - diagnosis
Epilepsy - etiology
Evoked Potentials, Somatosensory
Humans
Immunohistochemistry
Intraoperative Period
Magnetic Resonance Spectroscopy
Male
Neurosurgery
Periaqueductal Gray
Tomography, Emission-Computed
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Summary: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.
ISSN:0022-3085
DOI:10.3171/jns.1997.87.1.0113