West Syndrome. Clinical features, therapeutics, outcome and prognosis

Abstract

OBJECTIVE: To identify clinical features of West syndrome, etiology, and electroencephalography patterns (EEG), therapeutics, side effects and prognostic factors.

METHODS: An observational study was performed based on review of clinical files. Inclusion criteria were defined. We included 45 patients admitted from January 2010 to December 2015. Statistical analysis was applied.

RESULTS: Genetic, metabolic/structural etiology was predominant. There was a high frequency of hypoxic–ischemic encephalopathy (33.3 %) and neurocutaneous syndromes (15.5 %). On 66.6 % of patient’s muscular spasms were absent by four weeks of treatment with combined use of vigabatrine and ACTH; p< 0.05. All patients developed transient hypertension as a side effect. 88.8 % of patients developed moderate to severe developmental retardation, 33.3 % Lennox–Gastaut syndrome and 44.4 % focal epilepsy. Prognostic factors related to poor outcome were prenatal and perinatal antecedents, symptomatic etiology, neurodevelopmental retardation, epileptic crisis, previous positive EEG and age under 4 months (40 %) and combinations of factors, p< 0.05.

CONCLUSIONS: Combined use of vigabatrine and ACTH may reduce the length of spasms and the EEG hypsarhythmic pattern. Poor outcomes are related to a combination of prognostic factors.