Abstracts

Rationale: Juvenile myoclonic epilepsy (JME) represents 10% of all epilepsies with a peak age of onset between 12-18 years. Historically, JME was considered a “benign” epilepsy since seizures are often well controlled with medication and individuals typically have average intellectual function. Recent long-term studies have revealed cognitive and behavioral difficulties that negatively impact life outcomes. The purpose of this study was to comprehensively characterize the status of cognition, academic problems, psychiatric, and behavioral problems at or near the onset of JME. Methods: This study included children aged 8-18 years that included 41 youth with JME diagnosed within the past 12 months with a mean age at diagnosis of 14.3 years along with 70 age matched controls.  Children completed a cognitive assessment and confirmatory factor analysis identified 5 cognitive constructs (Verbal, Perceptual, Attention, Speed, Executive). Parents were interviewed regarding academic services and also completed behavioral questionnaires (CBCL and BRIEF). Additionally, children and their parents participated in a semi-structured psychiatric interview using the Kiddie Schedule for Affective Disorders-Present Lifetime (KSADS-PL). Rates of academic services and psychiatric diagnoses were examined using a Pearson’s Chi-square analyses. MANOVAs were computed to assess group (control vs. JME) differences on cognitive factor scores and behavioral measures. Results: There was a significant main effect of group across the cognitive factor scores (F(1,105) =3.85, p<0.01) with significantly lower cognitive scores in the JME group compared to the control group. Specifically, Attention, Speed, and Verbal factors were  lower at p=0.05 and Executive and Perceptual factors were  lower at p=0.001. Academic service rates were higher in children with JME compared to healthy controls (47% vs. 19%, p=0.002) (Figure 2).  Prevalence rates of any Axis I psychiatric disorder were higher in children with JME compared to controls (54% vs. 23%, p=0.001). In particular, ADHD and depression were significantly higher for children with JME compare to controls (27% vs. 7%, p=0.01; 27% vs. 1%, p=0.001). The BRIEF Metacognition and Behavioral Regulation scales were found to have a significant overall main effect with significant differences found in both scales (p=0.001). On the CBCL significant main and group differences were found in Total Competence, Internalizing Problems and Externalizing Problems (p=0.001). Conclusions: Youth with JME demonstrate a diverse range of problems in cognition, academics, psychiatric diagnoses, behavioral and executive complications compared to healthy age matched controls. These issues have been documented in chronic JME, but here we present evidence that these issues are apparent very early in the course of the disorder and require intervention to improve life course. Funding: NINDS 3RO1 44351