West Syndrome Case of Unknown Cause of Difficult Treatment

West syndrome is an age-dependent epileptic encephalopathy, is a kind of catastrophic epilepsy in the first year of life, characterized by the electroclinical triad of epileptic spasms, delayed psychomotor development, and hypsarrhythmia on the electroencephalogram, although one of these elements may be absent. Its pathophysiology remains unknown and presents a wide range of etiologies, under which can be classified into: genetic, structural/metabolic and unknown cause (cryptogenic). The prognosis depends on: (a) the cause, which gives origin to the attacks (the complex malformation forms being more severe); (b) the EEG pattern(s); (c) the appearance of seizures prior to the spasms; and (d) the rapid response to treatment. Currently, the first-line treatment includes the adrenocorticotropic hormone ACTH and vigabatrin. In the near future the gold standard could be the development of new therapies that target specific pathways of pathogenesis. Due to its high mortality rate and refractoriness to antiepileptic drugs Conventional, is considered a type of "catastrophic epilepsy".