title: Animal models of focal cortical dysplasia and tuberous sclerosis complex: recent progress toward clinical applications.
abstract: Focal cortical dysplasia (FCD) and related malformations of cortical development (MCDs) represent an increasingly recognized cause of medically intractable epilepsy. However, the underlying mechanisms of epileptogenesis are poorly understood, and treatments for epilepsy due to various cortical malformations are often limited or ineffective. Animal models offer a number of advantages for investigating cellular and molecular mechanisms of epileptogenesis and developing novel, rational therapies for MCD-related epilepsy. This review highlights specific examples of how animal models have been useful in addressing several clinically relevant issues about epilepsy due to FCDs and related cortical malformations, including the pathologic and clinical features, etiologic factors, localization of the epileptogenic zone, neuronal and astrocytic contributions to epileptogenesis, and the development of antiepileptogenic therapies.
authors.affiliation: Department of Neurology and the Hope Center for Neurological Disorders, Washington University School of Medicine, St Louis, Missouri 63110, USA. wong_m@wustl.edu
authors.forename: Michael
authors.initials: M
authors.lastname: Wong
mesh_terms: D000818:Animals; D000927:Anticonvulsants / Q000627:therapeutic use; D002540:Cerebral Cortex / Q000473:pathology / Q000503:physiopathology*; D004195:Disease Models, Animal; D015195:Drug Design; D004827:Epilepsy / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D054220:Malformations of Cortical Development / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D000076222:Mechanistic Target of Rapamycin Complex 1; D051379:Mice; D018345:Mice, Knockout; D046912:Multiprotein Complexes; D011506:Proteins; D051381:Rats; D020123:Sirolimus / Q000627:therapeutic use; D058570:TOR Serine-Threonine Kinases; D014157:Transcription Factors / Q000235:genetics; D014402:Tuberous Sclerosis / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*
chemical_list: D000927:Anticonvulsants; D046912:Multiprotein Complexes; D011506:Proteins; D014157:Transcription Factors; D000076222:Mechanistic Target of Rapamycin Complex 1; D058570:TOR Serine-Threonine Kinases; D020123:Sirolimus