A 28 year-old mentally retarded male resident of a group home had "shakes" when he was 18 months old that required "shots". He was diagnosed with epilepsy after developing episodes where he would suddenly fall to the ground often resulting in injury. "Grand mal" seizures were prominent and refractory to multiple AEDs and the ketogenic diet. Daily episodes of staring occurred and he was brought for an opinion regarding treatment by his group home managers. Seizures and "pseudoseizures" were reported based upon bizarre "behaviors" and jerking that would occur despite being fully conscious prompting behavioral time-out" and food restriction. MRI demonstrated mild atrophy. A routine EEG revealed the following without clinical signs noted.
Figure: Interictal EEG demonstrating a 5 Hz posterior dominant rhythm with a 3 second burst of SSW-GPFA prior to an electrographic seizure (arrow). The recording parameters include a longitudinal bipolar montage with a sensitivity 7 uv and filter settings of 1-70 Hz.
The Lennox-Gastaut syndrome (LGS) is the prototypic encephalopathic generalized epilepsy with an electroclinical profile that includes frequent uncontrolled mixed seizures, mental retardation, and slow-spike-and-wave (SSW) on EEG. Epileptic spasm (treated with ACTH) may precede LGS and some seizures may appear bizarre and falsely mimic "pseudoseizures". Tonic seizures are characteristic and maybe subclinical with video-EEG disclosing variable degrees of posturing during bursts of low-voltage ictal fast activity evolving (above) or as bursts of GPFA. Tonic-clonic, atypical absence, and myoclonic seizures may also co-exist and occur as status epilepticus though commonly resulting in injury. In this case vEEG demonstrated that all the events were epileptic and were not appropriately treated with continuing behavioral modification. VNS was effective in reducing generalized seizures. Corpus callosotomy was offered with potentially greater efficacy but associated with a higher risk of complications1.
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