Focal cortical dysplasia: prevalence, clinical presentation and epilepsy in children and adults

Acta Neurologica ScandinavicaVolume 113, Issue 2 p. 72-81 Focal cortical dysplasia: prevalence, clinical presentation and epilepsy in children and adults T. Bast, T. Bast Department of Paediatric Neurology, University of Heidelberg, Heidelberg, GermanySearch for more papers by this authorG. Ramantani, G. Ramantani Department of Paediatric Neurology, University of Heidelberg, Heidelberg, GermanySearch for more papers by this authorA. Seitz, A. Seitz Department of Neuroradiology, University of Heidelberg, Heidelberg, GermanySearch for more papers by this authorD. Rating, D. Rating Department of Paediatric Neurology, University of Heidelberg, Heidelberg, GermanySearch for more papers by this author T. Bast, T. Bast Department of Paediatric Neurology, University of Heidelberg, Heidelberg, GermanySearch for more papers by this authorG. Ramantani, G. Ramantani Department of Paediatric Neurology, University of Heidelberg, Heidelberg, GermanySearch for more papers by this authorA. Seitz, A. Seitz Department of Neuroradiology, University of Heidelberg, Heidelberg, GermanySearch for more papers by this authorD. Rating, D. Rating Department of Paediatric Neurology, University of Heidelberg, Heidelberg, GermanySearch for more papers by this author First published: 11 January 2006 https://doi.org/10.1111/j.1600-0404.2005.00555.xCitations: 82 Thomas Bast, Department of Paediatric Neurology, University Hospital of Heidelberg, INF 150, D 69120 Heidelberg, Germany Tel.: (+49) 6221 568228 Fax: (+49) 06221 565744 e-mail: thomas.bast@med.uni-heidelberg.de Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Abstract Focal cortical dysplasias (FCD) are defined as circumscribed malformations of cortical development. They result from an impairment of neuronal proliferation, migration and differentiation. In the diagnosis of focal epilepsy FCD prevalence ranges between 5% and 25%, depending on patient collective and imaging techniques. Several 'cryptogenic' epilepsies may be caused by FCD but have not been diagnosed because of the lack of high-quality magnetic resonance imaging assessment. Retrospective analysis of patients who have undergone epilepsy surgery can be biased because of the fact that they represent a mere subset of potential FCD diagnoses. Epilepsy typically manifests within the first years of life, but has been documented up to the age of 60 years. Cognitive impairment commonly accompanies early onset. Epilepsy is often refractory to antiepileptic drug (AED) treatment. Clinical observations and pathophysiological findings illustrate intrinsic epileptogenicity. Upregulation of drug transporter proteins has been found in FCD tissue. There is no specific drug treatment in FCD, as any AED used in focal epilepsy could prove effective. A sequential AED therapy should be designed individually and take side effects as well as developmental progresses into consideration. Fifty to sixty-five percent of FCD patients are rendered seizure-free after surgery. Presurgical evaluation should be initiated after two unsuccessful AED trials. Both risks and potential benefits regarding seizure control and developmental impairment need to be considered on an individual basis when deciding between surgical intervention and conservative treatment. Current knowledge on epilepsy course and psychomotor development in FCD is limited in the absence of qualified long-term studies combining imaging with cognitive evaluation. Citing Literature Volume113, Issue2February 2006Pages 72-81 RelatedInformation