Mark is an 8-year-old boy with a history of intractable epilepsy. Mark's seizures started when he was five years old, lasting less than a minute, with 7-10 episodes occurring in succession. Daytime seizures were described by his parents a “staring events where he does not respond, he will pick at clothes and speak gibberish.” He was often disorientated for the remainder of the day. Nighttime seizures were described as “sitting up straight in bed, staring at the ceiling, and being unresponsive.” An increase in his seizure frequency after multiple anticonvulsant medications prompted a surgical evaluation. A magnetic resonance (MR) brain scan indicated mild encephalomacia in the left hemisphere. A video electroencephalogram (EEG) demonstrated that the seizures initiated from the left hemisphere in association with multiple subclinical seizures. A PET scan showed decreased uptake in the left frontal lobe compared to the right. At 7 years of age Mark underwent a left frontal temporal-parietal resection. He had a post-surgical infection, but no other medical sequelae. After surgery, there was a significant decrease in seizures with only one seizure in the 2 month post operative period.
Mark had neuropsychological testing prior to and following surgery. Pre-surgical results indicated that his IQ was within the low-average range. Visual-perceptual abilities, motor tasks and attention domains indicated difficulties. Post-surgical neuropsychological evaluation revealed a positive outcome. IQ remained in the low average range and there was a mild improvement in visual-perceptual/visual-constructional areas. Academic skills were unchanged with the exception of a slight decline in reading ability. Attention scores improved although redirection was required to sustain his attention during tasks. An increase in non-compliant behavior and emotional liability was noted by his parents.
At the time of referral, when Mark was 8-years 3-months old, parental concerns included inattention, anger and emotional lability. The referral question posed was: “Does Mark's inattention represent an attention deficit/hyperactivity disorder, anxiety, or other psychological problems and what is the relationship of his current behaviors to his epilepsy?”
Carin Cunningham Ph.D., Department of Pediatrics, Western Reserve School of Medicine, Cleveland, Ohio.
Ingrid Tuxhorn MD, Prakash Kotagal MD, William Bingaman MD, Epilepsy Center, Neurological Institute, Cleveland Clinic, Cleveland, Ohio.
Martin T. Stein, MD, University of California San Diego, Rady Children's Hospital San Diego.
The case summary for the Challenging Case was posted on the Developmental & Behavioral Pediatrics web site http://dbpeds.org.list. Comments were solicited.