West Syndrome: Clinical Characteristics, Therapeutics, Outcomes and Prognosis

摘要: Introduction: West syndrome (WS) is the most severe, devastating and/or catastrophic epileptic encephalopathy during lactation period. However, until now, there are few comprehensive clinical studies in this regard Cuba. Objective: To identify characteristics of patients with WS, related etiology, therapeutics and prognostic factors. Methods: An observational, descriptive retrospective study 39 WS from Centro Habana Teaching Pediatric Hospital, Havana, Cuba, comprising period between January 2005 December 2016 was carried out. Clinical data were recorded for each patient by review history. Statistical analysis performed use Package Social Sciences. Results: The genetic, structural/metabolic etiology predominant. Hypoxic ischemic (25.6%) neurocutaneous syndromes (17.9%) cause more frequent WS. combined treatment adrenocorticotropic hormone (ACTH) vigabatrin (VGB) used 25 (64.1%). In 18 them (72%) control infantile spasms or reduction ≥ 50%, while 14 (56%) hypsarrhythmia disappeared first 6 months (p<0.05). All that ACTH had transient hypertension as an adverse effect drug. 71.8% moderate to severe delay psychomotor development, 35.9% diagnosed Lennox-Gastaut 43.6% other epilepsies. There unfavorable evolution 74.3% patients. significant factors male gender, symptomatic abnormality neurological examination, seizures, previous paroxysmal electroencephalogram, poor response treatment, persistence hypsarrhythmia, combination than three 68.9% (p <0.05). Conclusions: Our findings showed VGB may decrease time disappearance Moreover, these factors.