Research Article
Volume 10 Issue 9 - 2018
West Syndrome: Clinical Characteristics, Therapeutic, Evolution and Prognostic Factors
Ernesto Portuondo Barbarrosa1* and Iraida de la Caridad Pérez Ferrer2
1Assistant Professor of Pediatrics, Neuropediatric Service, Pediatric Teaching Hospital Centro Habana, Havana, Cuba
2Neurophysiology Service, Pediatric Hospital Teaching Center Havana, Havana, Cuba
*Corresponding Author: Ernesto Portuondo Barbarrosa, Assistant Professor of Pediatrics, Neuropediatric Service, Pediatric Teaching Hospital Centro Habana, Havana, Cuba.
Received: May 21, 2018; Published: August 03, 2018
Citation: Ernesto Portuondo Barbarrosa and Iraida de la Caridad Pérez Ferrer. "West Syndrome: Clinical Characteristics, Therapeutic, Evolution and Prognostic Factors”. EC Neurology 10.9 (2018): 820-834.
Abstract
Introduction: West syndrome (SW) is the most severe, devastating and/or catastrophic epileptic encephalopathy of breastfeeding.
Objective: To identify clinical features of West syndrome, etiology, therapeutics and prognostic factors.
Methods: An observational study was performed based on review of clinical files. Inclusion criteria were defined. We included 39 patients admitted from January 2010 to December 2016. Statistical analysis was applied.
Results: The genetic, structural/metabolic etiology was predominant. Hypoxic-ischemic encephalopathy (25.6%) and neurocutaneous syndromes (17.9%) were more frequent. In 25 (64.1%) the combined treatment of adrenocorticotropic hormone (ACTH) and Vigabatrin was used, in 18 (72%) control of the IE or reduction was achieved ≥ 50% and in 14 (56%) hypsarrhythmia disappeared in the first 6 months (p ˂ 0.05). 100% had transient hypertension as an adverse effect to ACTH. 71.8% developed moderate to severe psychomotor retardation, 35.9% to Lennox-Gastaut syndrome and 43.6% to other epilepsies. There was an unfavorable evolution in 74.3%. The most significant prognostic factors for unfavorable evolution were male gender, symptomatic etiology, psychomotor retardation and/or abnormality on neurological examination, epileptic seizures and previous pathologic electroencephalogram, poor response to treatment and persistence of hypsarrhythmia and Combination of factors by more than 65% (p ˂ 0.05).
Conclusions: Combined use of Vigabatrin and ACTH may reduce the length of spasms and the EEG Hypsarrhythmic pattern. Poor outcomes are related to a combination of prognostic factors.
Keywords: Infantile Spasms; Hypsarrhythmia; Etiology and Prognostic Factors
Copyright: © 2018 Ernesto Portuondo Barbarrosa and Iraida de la Caridad Pérez Ferrer. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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