Objective:

The objective of this study was to describe the cognitive functioning and psychiatric comorbidities of patients with epilepsy with eyelid myoclonia (EEM).

Background:

EEM is a rare childhood-onset epilepsy syndrome characterized by eyelid myoclonia, photosensitivity, and eyelid closure-induced seizures or EEG paroxysms. While normal cognition is reported in many patients, there is limited data about neurocognitive abilities and psychiatric comorbidities in EEM.  

Design/Methods:

A database of 134 patients with EEM was reviewed for patients who underwent neuropsychometric testing. Demographics, types of seizures, antiseizure medications, and psychiatric comorbidities were identified. Psychometric testing included standardized intelligence tests, including the Wechsler Adult Intelligence Scale (4th Ed., WAIS-IV), Wechsler Intelligence Scale for Children (5th Ed., WISC-V)) or Differential Abilities Scale (2nd Ed. DASI-II). Index scores were utilized for descriptive analysis, including the intelligence quotient (IQ), Verbal Comprehension Index (VCI), Perceptual Reasoning Index (PRI) or Visual Spatial Index (VSI), Working Memory Index (WMI), and Processing Speed Index (PSI). Scores were documented as standard scores (SS) with a mean of 100 and standard deviation of 15. IQ scores were categorized as low average and above (SS = 80-130) and below average (SS<80).

Results:

Fifteen patients (13 females, 86.7%) underwent neuropsychometric testing. Median age of epilepsy onset was 4 (range 1-14), and median age at neuropsychometric testing was 17 (range 7-22).   Median IQ was 84 (range 56-110); 7 patients had below average IQ. Other median results of neuropsychometric measures were: VCI 93 (range 69-116), PRI/VSI 82 (range 67-115), WMI 78.5 (range 54-100), and PSI 84 (range 70-100). The most common psychiatric comorbidities were anxiety (N=10), depression (N=7), ADHD (N=6), and autism (N=2).  

Conclusions: