West Syndrome Infantile Spasms Research Articles

Dental Considerations and Treatment Approaches in West Syndrome—Infantile Spasms: A Case Report

Dental Considerations and Treatment Approaches in West Syndrome—Infantile Spasms: A Case Report

Evolution and course of early life developmental encephalopathic epilepsies: Focus on Lennox-Gastaut syndrome.

Developmental encephalopathic epilepsies (DEEs) are characterized by refractory seizures, disability, and early death. Opportunities to improve care and outcomes focus on West syndrome/infantile spasms (WS/IS). Lennox-Gastaut syndrome (LGS) is almost as common but receives little attention. We examined initial presentations of DEEs and their evolution over time to identify risk and indicators of developing LGS. Data are from the Connecticut Study of Epilepsy, a prospective, longitudinal study of 613 children with newly diagnosed epilepsy recruited in 1993-1997. Central review of medical records permitted classification of epilepsy syndromes at diagnosis and at reclassification 2, 5, and 9years later. DEEs were compared to other epilepsies for seizure and cognitive outcomes and mortality. Analyses examined the evolution of DEE syndromes after initial presentation, with specific comparisons made between WS/IS and LGS. Statistical analyses were performed with t tests and chi-square tests. Fifty-eight children (9.4%) had DEEs, median onset age=1.1years (interquartile range ([IQR]0.3-1.3) in DEEs and 6.0years (IQR3.0-9.0) in other epilepsies (P<0.001). DEEs vs other epilepsies had more pharmacoresistance (71% vs 18%), intellectual disability (84% vs 11%), and mortality (21% vs <1%; all P<0.001). During follow-up, the form of epilepsy evolved in 33 children. WS/IS was the most common initial diagnosis (N=23) and in 5 children WS/IS evolved later. LGS was diagnosed initially in 4 children (1 later revised) and in 22 by the end of follow-up, including 7 evolving from WS/IS and 12 from nonsyndromic generalized, focal, or undetermined epilepsies. Evolution to LGS took a median of 1.9years. LGS developed in 13% of infants, including 9% of those who did not present initially with WS/IS. DEEs account for disproportionate amounts of pharmacoresistance, disability, and early mortality. LGS often has a window between initial presentation and full expression. LGS should become targeted for early detection and prevention.

Pesquisa em epilepsia na infância

Research in epilepsy in the childhood This report describes current pediatric epilepsy research carried out in Brazilian academic centers, presented at the National Epilepsy Forum, during the XX Brazilian Clinical Neurophysiology Congress. It illustrates the different groups’ efforts to promote research and increase awareness of epilepsy in the pediatric population. Main current research lines include epidemiology, electroencephalography, genetic and cognitive issues in pediatric epilepsy, controlled clinical trials with antiepileptic drugs in different childhood epileptic syndromes, ketogenic diet, single and febrile seizures, temporal and frontal lobe epilepsy, West syndrome-infantile spasms, status epilepticus, malformations of cortical development, sleep and epilepsy, epilepsy surgery in childhood, psychiatric co-morbities and neuroimaging in childhood epilepsy. Collaborative studies, an important tool in fostering research in pediatric epilepsy in our country, are being carried out by some academic centers.