[P1-49] Evolution of West Syndrome in Infants with Tuberous Sclerosis Complex
Seven patients who visited Okayama University Hospital between 2011 and 2015 were diagnosed with Tuberous sclerosis complex (TSC) during infancy. We retrospectively investigated the evolution of epilepsy including West syndrome (WS) in these patients, particularly focusing on the chronological and topographical changes of epileptic discharge in electroencephalogram (EEG) before the onset of WS. These results were compared to our previous findings on EEG changes prior to the onset of symptomatic WS in high-risk premature infants. All of the patients developed WS. Two (one with epileptic spasms and the other with only partial seizures) showed hypsarrhythmia in EEG at the first visit at 6 months of age. In the remaining five patients, EEG was recorded prior to the onset of WS at ages ranging from 2 to 6 months (median 2 months). Their EEGs showed multifocal spikes in various areas with a tendency toward predominance of one hemisphere. Their EEG features then developed into hypsarrhythmia at around 6 months of age. Three of these five patients had partial seizures before the development of hypsarrhythmia and/or spasms. As a conclusion, a high proportion of infantile patients with partial seizures associated with TSC were found to subsequently develop WS. The initial EEG findings prior to the development of WS differ between the infants with TSC and the premature infants. The former initially exhibited multifocal epileptic discharges in EEG, while the latter exhibited discharges in the occipital area at a median age of 4 months.