Sweep visually evoked potentials and visual findings in children with West syndrome. 2014

Patrícia de Freitas Dotto, and Nívea Nunes Cavascan, and Adriana Berezovsky, and Paula Yuri Sacai, and Daniel Martins Rocha, and Josenilson Martins Pereira, and Solange Rios Salomão
Departamento de Oftalmologia, Universidade Federal de São Paulo - UNIFESP, São Paulo, SP, Brazil.

BACKGROUND West syndrome (WS) is a type of early childhood epilepsy characterized by progressive neurological development deterioration that includes vision. OBJECTIVE 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.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005074 Evoked Potentials, Visual The electric response evoked in the cerebral cortex by visual stimulation or stimulation of the visual pathways. Visual Evoked Response,Evoked Potential, Visual,Evoked Response, Visual,Evoked Responses, Visual,Potential, Visual Evoked,Potentials, Visual Evoked,Response, Visual Evoked,Responses, Visual Evoked,Visual Evoked Potential,Visual Evoked Potentials,Visual Evoked Responses
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective
D013036 Spasms, Infantile An epileptic syndrome characterized by the triad of infantile spasms, hypsarrhythmia, and arrest of psychomotor development at seizure onset. The majority present between 3-12 months of age, with spasms consisting of combinations of brief flexor or extensor movements of the head, trunk, and limbs. The condition is divided into two forms: cryptogenic (idiopathic) and symptomatic (secondary to a known disease process such as intrauterine infections; nervous system abnormalities; BRAIN DISEASES, METABOLIC, INBORN; prematurity; perinatal asphyxia; TUBEROUS SCLEROSIS; etc.). (From Menkes, Textbook of Child Neurology, 5th ed, pp744-8) Cryptogenic Infantile Spasms,Hypsarrhythmia,Infantile Spasms,Jackknife Seizures,Nodding Spasm,Salaam Seizures,Spasmus Nutans,Symptomatic Infantile Spasms,West Syndrome,Cryptogenic West Syndrome,Lightning Attacks,Salaam Attacks,Symptomatic West Syndrome,Attack, Lightning,Attacks, Lightning,Attacks, Salaam,Cryptogenic Infantile Spasm,Hypsarrhythmias,Infantile Spasm,Infantile Spasm, Cryptogenic,Infantile Spasm, Symptomatic,Infantile Spasms, Cryptogenic,Infantile Spasms, Symptomatic,Jackknife Seizure,Lightning Attack,Nodding Spasms,Seizure, Jackknife,Seizures, Jackknife,Seizures, Salaam,Spasm, Cryptogenic Infantile,Spasm, Nodding,Spasm, Symptomatic Infantile,Spasms, Cryptogenic Infantile,Spasms, Nodding,Spasms, Symptomatic Infantile,Symptomatic Infantile Spasm,Syndrome, Cryptogenic West,Syndrome, Symptomatic West,Syndrome, West,West Syndrome, Cryptogenic,West Syndrome, Symptomatic
D014793 Visual Cortex Area of the OCCIPITAL LOBE concerned with the processing of visual information relayed via VISUAL PATHWAYS. Area V2,Area V3,Area V4,Area V5,Associative Visual Cortex,Brodmann Area 18,Brodmann Area 19,Brodmann's Area 18,Brodmann's Area 19,Cortical Area V2,Cortical Area V3,Cortical Area V4,Cortical Area V5,Secondary Visual Cortex,Visual Cortex Secondary,Visual Cortex V2,Visual Cortex V3,Visual Cortex V3, V4, V5,Visual Cortex V4,Visual Cortex V5,Visual Cortex, Associative,Visual Motion Area,Extrastriate Cortex,Area 18, Brodmann,Area 18, Brodmann's,Area 19, Brodmann,Area 19, Brodmann's,Area V2, Cortical,Area V3, Cortical,Area V4, Cortical,Area V5, Cortical,Area, Visual Motion,Associative Visual Cortices,Brodmanns Area 18,Brodmanns Area 19,Cortex Secondary, Visual,Cortex V2, Visual,Cortex V3, Visual,Cortex, Associative Visual,Cortex, Extrastriate,Cortex, Secondary Visual,Cortex, Visual,Cortical Area V3s,Extrastriate Cortices,Secondary Visual Cortices,V3, Cortical Area,V3, Visual Cortex,V4, Area,V4, Cortical Area,V5, Area,V5, Cortical Area,V5, Visual Cortex,Visual Cortex Secondaries,Visual Cortex, Secondary,Visual Motion Areas

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