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Sweep visually evoked potentials and visual findings in children with West syndrome
Patrícia de Freitas Dotto1, Nívea Nunes Cavascan1, Adriana Berezovsky1
1Departamento de Oftalmologia, Universidade Federal de São Paulo - UNIFESP, São Paulo, SP, Brazil.
Insights
Grating visual acuity thresholds (GVA) measured by sweep visually evoked potentials (sweep-VEP) reliably assess visual cortex function in West syndrome (WS) children. This method helps classify visual impairment, correlating with observed poor visual behavior in affected infants.
Area of Science:
- Ophthalmology
- Neuroscience
- Pediatric Neurology
Background:
- West syndrome (WS) is a severe form of early childhood epilepsy.
- WS is characterized by progressive neurological deterioration, significantly impacting visual development.
- Accurate assessment of visual function is crucial for managing WS patients.
Purpose of the Study:
- To establish the clinical utility of grating visual acuity thresholds (GVA) measurement using sweep visually evoked potentials (sweep-VEP).
- To demonstrate sweep-VEP as a reliable method for evaluating visual cortex status in children with West syndrome.
- To correlate GVA findings with other ophthalmological features in WS.
Main Methods:
- Retrospective analysis of best-corrected binocular GVA and ophthalmological data from 30 WS children (1998-2012).
- GVA was measured using the sweep visually evoked potentials (sweep-VEP) technique.
- GVA deficit was calculated relative to age norms and classified as mild, moderate, or severe.
Main Results:
- The majority of WS children exhibited severe GVA deficits (mean logMAR = 0.82 ± 0.32).
- Associated findings included poor visual behavior, high prevalence of strabismus, and variable ocular positioning.
- GVA deficit severity did not significantly correlate with gender, WS type, birth age, perinatal factors, or early visual stimulation.
Conclusions:
- The sweep-VEP technique is a dependable tool for assessing visual system impairment in West syndrome.
- Sweep-VEP findings align with the observed poor visual behavior in WS children.
- This method aids in classifying the extent of visual dysfunction in this pediatric epilepsy population.
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 n° 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.
