Neuropsychology and behavior in juvenile myoclonic epilepsy

Following decades of neglect, there has been an increasing interest in the behavioral aspects of juvenile myoclonic epilepsy (JME) in the recent literature. A number of authors have investigated psychiatric comorbidity, cognitive profiles, and related behavioral features associated with JME. Although these findings are not entirely uniform, most studies suggest an increased incidence in psychiatric comorbidity and specific cognitive deficits that explain some of the clinical observations of poor compliance and other unhealthy behaviors in people suffering from JME. Neuropsychological profiles in JME are suggestive of subtle frontal dysfunctions, and some of the observations have been linked with sophisticated structural and functional imaging findings. Taken together, there is evidence that JME is associated with dysfunctions in networks linking motor and cognitive neuronal centers. Interestingly, there is evidence from family studies that the behavioral abnormalities in JME are genetically determined, suggesting an underlying developmental disorder. This article is part of a supplemental special issue entitled Juvenile Myoclonic Epilepsy: What is it Really? (C) 2013 Elsevier Inc. All rights reserved.