Abstracts

Rationale: To outpoint prevalence, electro-clinical characteristics and long term outcome of infantile and early childhood onset epilepsies, according to the new proposal for Epilepsy Classification (ILAE, 2010). Methods: We reviewed clinical records of patients referred to Epilepsy Center, San Paolo Hospital, Milan, Italy since 1992 to 2012, searching for infantile onset epilepsies (< 3 years) and collected demographical, clinical, neurophysiological and developmental data. Results: From 3096 screened records, we selected 266 patients (144 M), mean age at onset was 17.5 months (range < 1 month -36 month; 16 had onset < 1 month, 147 <1 year). Mean age at follow up was 29.4 years (range 1-63), mean follow-up 8.7 years (range 1-21). Most of the patients (100 patients) presented with non syndromic epilepsy (57 structural abnormalities, 19 genetic, 7 metabolic, 7 neurocutaneous syndrome, 7 cortical development abnormalities, 3 tumors ); 90 presented an electro-clinical syndrome (more common: 34 WS, 17 LGS, 14 CAE, 5 DS); 76 presented distinctive constellation (58 focal seizure, 11 MTLE HS, 6 generalized epilepsies). On the basis of etiology 114 epilepsy were of unknown origin; 31 genetic , 12 metabolic , 23 cortical development abnormalities , 73 structural , 3 tumoral, 10 neurocutaneous syndrome . At follow-up 117 patients (66.5%) had intellectual disability (29.7% severe). Neurological examination was abnormal in 137 patients. 173 (65%) had drug resistant epilepsy according to ILAE criteria and 113 patients were seizure free since at least 2 years at last follow-up visit. Conclusions: In our population prevalence of drug-resistant epilepsy and severe neurocognitive delay is higher than observed by other groups, this is probably due to the selection of a population of very early onset epilepsy. Worse outcome in terms of seizure control and intellectual disability was associated with earlier onset (particularly in the first month of life) and the presence of a recognizable cause of the disease.