Abstracts

The 19Y boy presented with motor tics, facial diplegia and learning disability, and was found to have BPP. His seizures consisted of independent right and left upper extremity paraesthesias associated with anxiety. His first seizure evolved to a generalized tonic-clonic seizure followed by Todd’s paresis. His scalp EEGs repeatedly failed to show EEG correlates for his seizures. On his 3rd vEEG admission subtle focal rhythmic slowing with superimposed sharps was captured over bilateral centroparietal regions independently. PET showed hypometabolic foci in the bilateral perisylvian areas. sEEG further localized the seizure onset zones (SOZ) to the sensory cortical regions corresponding to the bilateral upper extremities, confirmed with an fMRI. We confirmed normal cortical activity in a significant portion of the abnormal appearing cortex. RNS depth electrodes were placed in the bilateral SOZs due to the presence of overlapping eloquent cortex. Greater than 90% reduction in seizures was noted at 6 months follow up. The 6Y boy presented with profound developmental delay, multiple monthly nocturnal convulsions, frequent convulsive status epilepticus and spike wave activation in sleep (SWAS) associated with extensive BPP plus frontoparietal MCD. vEEG detected abundant multifocal epileptiform discharges over the left centrotemporal and central regions. MEG confirmed a tight cluster of dipoles in the left hemisphere along the sylvian fissure/superior temporal gyrus. While scalp EEG poorly localized SOZ, sEEG localized 6 seizures to the left Supplementary Motor Area (SMA) with early spread captured in the left centromedian nucleus of the thalamus (LCM). sEEG also confirmed the overwhelming burden of interictal discharges (abundant periodic spike and wave discharges) that became nearly continuous in sleep. RNS depth electrodes in the SMA and LCM are capturing both focal seizures and SWAS.