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Typical absence seizures in childhood absence epilepsy with aberrant electroclinical features (2)

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A 3.8-year-old girl who, for the past 6 months, had episodes of staring with eyes up, head back, and some eyebrow jerking. Occasionally she would lose her balance. She was given phenobarbitone by her pediatrician. A few weeks before she was seen, she was having 100 episodes daily. Background EEG on sleep-awake EEG showed slow delta waves in the occipital region during eye closure that attenuated on eye opening. Generalized spike-wave discharges of around 3 Hz and lasting from 1 to 16 seconds, some of which were fragmented, were recorded during sleep and awake EEG. During intermittent photic stimulation from 7 to 35 Hz, she had episodes of staring with retropulsive head movement, eyebrow jerking, occasional myoclonic jerk, and unresponsive behavior concomitant with generalized spike-wave discharges. She regained consciousness immediately after the abrupt termination of the discharges. She had periods of complete clinical control while on valproate and ethosuximide. Due to family problems, she had many relapses attributed to noncompliance. She is presently 15 years old. (Contributed by Dr. Athanasios Covanis.)