Lennox–Gastaut Syndrome

Abstract

A 28-year-old mentally retarded male resident of a group home had a documented medical history indicating that he had “shakes” when he was 12 months old that required “shots.” His charted information identified a past medical history of frequent spells during his childhood that responded to “steroids” and eventually ceased. His development had been normal after the “spells”; however, he had a notable loss of cognitive function and was severely delayed in his development. He was diagnosed with epilepsy after developing episodes where he would “become rigid” and abruptly fall to the ground, which often resulted in injury. Sometimes there would be a sudden jerk prompting him to drop to the floor. “Grand mal” seizures became prominent. He also developed daily episodes of staring. He was brought for an opinion regarding treatment by his group home managers and was diagnosed with epilepsy that quickly became resistant to multiple AEDs. The ketogenic diet was instituted, but he was unable to maintain compliance with the food restrictions. Seizures and “pseudoseizures” were reported based upon bizarre “behaviors” and jerking that would occur despite being fully conscious. This prompted his group home to introduce behavioral modification techniques with “time-outs” and food deprivation. MRI demonstrated mild atrophy. A routine EEG (Fig. 5.1) revealed the following: