Abstract
BACKGROUND: Epilepsy with Eyelid Myoclonia (EEM) is a rare childhood-onset epilepsy syndrome. There is limited data about cognitive function and psychiatric comorbidities in patients with EEM. DESIGN/METHODS: A database of 134 patients with EEM was reviewed for patients who underwent neuropsychological testing. Psychiatric comorbidities and psychometric test scores were identified. Group comparison was performed between those who underwent neuropsychological testing and those who did not. In addition, we evaluated whether clinical factors were associated with IQ score. RESULTS: Fourteen patients underwent neuropsychological testing (12 females, 85.7%), with a median age at testing of 17 (range 7-22). Median IQ was 79 (range 56-110); 7 patients had below average IQ. Other median results of neuropsychometric measures were: Verbal Comprehension Index 85.5 (range 66-116), Perceptual Reasoning Index or Visual Spatial Index 81.5 (range 67-100), Working Memory Index 77 (range 54-100), Processing Speed Index 84 (range 53-94), and Reading Standardized Scores 84 (range 64-126). Common psychiatric comorbidities were anxiety (n = 10), depression (n = 7), ADHD (n = 6), and autism (n = 2). Those who underwent neuropsychological testing had a younger age of epilepsy onset, longer follow-up at our institution, and were more likely to have myoclonic seizures or psychosis than those who did not undergo neuropsychological testing. No clinical factors were statistically associated with IQ score. CONCLUSIONS: EEM is associated with a wide range of cognitive abilities, with half of our patients having a below average IQ. Psychiatric comorbidities were common. Identifying cognitive impairment and psychiatric comorbidities is crucial to implement appropriate management strategies.