Síndrome de Lennox-Gastaut, una revisión actualizada.

The Lennox-Gastaut Syndrome is a severe form of chronic epileptic encephalopathy, characterized clinically by the triad: slow spike-wave pattern of the EEG, multiple types of seizures and cognitive decline. Corresponds to 3-6% of children with epilepsy, with male predominance and has a peak of onset between 3 and 5 years. The LGS is associated with a wide variety of etiologic factors, although up to 40% of the causes are unknown, and its pathophysiological substrate remains uncertain. Treatment is lifelong and aims to improve the quality of life of patients by decreasing the frequency of seizures, since there is no optimal therapy for complete remission. Drugs that have proven useful are Valproic Acid (irst line AED at Chile), Lamotrigine, Topiramate, Felbamate and Ruinamide. Other treatment options for refractory cases are the ketogenic diet, vagus nerve stimulation and surgery. The LGS has a poor long-term prognosis, with a mortality of 10% before 11 years of age. RESUMEN