Sweep visually evoked potentials and visual findings in children with West syndrome

Background: West syndrome (WS) is a type of early childhood epilepsy characterized by progressive neurological development deterioration that includes vision.Aim: To demonstrate the clinical importance of grating visual acuity thresholds (GVA) measurement by sweep visually evoked potentials technique (sweep-VEP) as a reliable tool for evaluation of the visual cortex status in WS children.Methods: This is a retrospective study of the best-corrected binocular GVA and ophthalmological features of WS children referred for the Laboratory of Clinical Electrophysiology of Vision of UNIFESP from 1998 to 2012 (Committee on Ethics in Research of UNIFESP no 0349/08). the GVA deficit was calculated by subtracting binocular GVA score (logMAR units) of each patient from the median values of age norms from our own lab and classified as mild (0.1-0.39 logMAR), moderate (0.40-0.80 logMAR) or severe (>0.81 logMAR). Associated ophthalmological features were also described.Results: Data from 30 WS children (age from 6 to 108 months, median = 14.5 months, mean +/- SD = 22.0 +/- 22.1 months; 19 male) were analyzed. the majority presented severe GVA deficit (0.15-1.44 logMAR; mean +/- SD = 0.82 +/- 0.32 logMAR; median = 0.82 logMAR), poor visual behavior, high prevalence of strabismus and great variability in ocular positioning. the GVA deficit did not vary according to gender (P = .8022), WS type (P = .908), birth age (P = .2881), perinatal oxygenation (P = .7692), visual behavior (P = .8789), ocular motility (P = .1821), nystagmus (P = .2868), risk of drug-induced retinopathy (P = .4632) and participation in early visual stimulation therapy (P = .9010).Conclusions: the sweep-VEP technique is a reliable tool to classify visual system impairment in WS children, in agreement with the poor visual behavior exhibited by them. (C) 2013 European Paediatric Neurology Society. Published by Elsevier B.V. All rights reserved.Universidade Federal de São Paulo UNIFESP, Dept Oftalmol, BR-04023062 São Paulo, BrazilUniversidade Federal de São Paulo UNIFESP, Dept Oftalmol, BR-04023062 São Paulo, BrazilWeb of Scienc