[PS20-3D-2] An axial twisting epileptic spasms generated from the unilateral cerebral hemisphere in a case of Aicardi syndrome
The aim of this report is to show the characteristic ictal/interictal features recorded on VTR-EEG in a four-month-old female infant with Aicardi syndrome. She was referred to our hospital at 3 months for focal tonic seizure as well as subsequently developed epileptic spasms. Neurological examination showed incomplete hemifacial palsy and poor eye contact as well as chorioretinal lacunae. X-ray study presented hemi-vertebrae associated with severe scoliosis. MRI showed agenesis of the corpus callosum as well as periventricular heterotopic gray matter and polymicrogyria in the frontal lobe. Interictal EEG showed suppression-burst limited on the right side. The VTR-EEG studies exhibited two types of seizures. The first type was composed of ictal manifestations starting with several times of twitch movements of limbs, then being associated with tonic components, lastly forming as generalized clonic seizures. Simultaneous EEG showed irregular 2.5 Hz high voltage slow waves started in the left frontal region, forming finally irregular incomplete 4Hz spike-wave discharge on EEG. The second type of seizure comprised of axial body twisting epileptic spasms, during which time EEG showed positive high-voltage slow waves in the right parietal-occipital area, which were followed by slow wave discharge in the contralateral side. From these VTR-EEG observations, we speculate that axial twisting epileptic spasms are not provoked with the epileptic activation from the brainstem, but from the right cerebral hemisphere. This is contrary to the hypothesis that the brainstem play a primary role as a generator of epileptic spasms.