title: Hemispheric malformations of cortical development.
abstract: The term hemispheric malformations of cortical development (MCDs) is used in this article to represent congenital malformations that predominantly or exclusively involve complete or substantial portion of one cerebral hemisphere. Hemispheric MCDs usually present during early childhood with intractable epilepsy, cognitive delay, and contralateral hemiparesis. Early identification and selection of children who may be candidates for epilepsy surgery are important during diagnostic evaluation of hemispheric MCD. Surgical intervention at an appropriate time, whenever possible, offers the best chance for seizure freedom and improved cognitive outcome. In addition, making an accurate genetic diagnosis is imperative to offer genetic testing and counseling to the family. In this article, the authors discuss the developmental and genetic mechanisms, pathologic features, diagnosis, and treatment of hemispheric MCD that are substrates of catastrophic childhood epilepsy amenable to surgical treatment.
authors.affiliation: Department of Neurology, Cleveland Clinic Foundation, Cleveland, OH 44195, USA. guptaa1@ccf.org
authors.forename: Ajay
authors.initials: A
authors.lastname: Gupta
authors.forename: Mar
authors.initials: M
authors.lastname: Carreño
authors.forename: Elaine
authors.initials: E
authors.lastname: Wyllie
authors.forename: William E
authors.initials: WE
authors.lastname: Bingaman
mesh_terms: D001927:Brain Diseases / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000254:growth & development / Q000473:pathology*; D002648:Child; D003072:Cognition Disorders / Q000209:etiology; D004827:Epilepsy / Q000209:etiology; D007839:Functional Laterality; D006801:Humans; D008279:Magnetic Resonance Imaging; D009421:Nervous System Malformations / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D010291:Paresis / Q000209:etiology