Paradoxical robust visual evoked potentials in young patients with cortical blindness.

The objective of this study was to review retrospectively cases of clinically blind children in whom robust pattern visual evoked potentials (VEPs) were recorded. VEP records from a 10-year period (1990-2000) were reviewed. We searched for charts of children who were clinically cortically blind, but in whom assessment of visual acuity, using visual evoked potentials (VEPs), was normal or close to normal.

It is not always amblyopia.

A child is referred because of reduced visual acuity in one or both eyes. This scenario is equally familiar to the ophthalmologist, orthoptist, electrophysiologist, and imaging specialist. Each specialty is looking for markers for disease and / or dysfunction to aid in diagnosis.

Dynamic fusional vergence eye movements in congenital esotropia.

Purpose: To evaluate whether a selected group of 9 children with history of congenital esotropia is capable of producing vergence eye responses to fusional disparity stimuli.

Methods: Nine children with history of congenital esotropia and 5 age-matched children with normal binocular vision were examined. Using a full-field target, vergence responses to base out 3 prism diopters placed in front of both eyes were recorded.

Ocular phenotypes of three genetic variants of Bardet-Biedl syndrome.

Bardet-Biedl syndrome is a genetically heterogeneous multisystem disorder that causes severe visual impairment. Retinitis pigmentosa (RP), hypogonadism, digit and renal anomalies, obesity, and a variable degree of mental retardation characterize the disorder. Eight different loci have been identified on 2q31(BBS5), 3p13 (BBS3), 4q27 (BBS7), 11q13 (BBS1), 14q32 (BBS8), 15q22.

Characteristic retinal atrophy with secondary "inverse" optic atrophy identifies vigabatrin toxicity in children.

Objective: To describe the clinical pattern of retinal atrophy in children caused by the anticonvulsant vigabatrin.

Design: An interventional case series report.

Participants: One hundred thirty-eight patients, mainly infants, were evaluated regularly for evidence of possible vigabatrin toxicity in the Eye and Neurology clinics at the Hospital for Sick Children, Toronto.

Changes in the electroretinogram resulting from discontinuation of vigabatrin in children.

Electroretinograms (ERGs) have been recorded longitudinally in children before and during treatment with the antiepileptic drug vigabatrin for the past 3.5 years. Vigabatrin induced changes in ERG responses occur in children; the most dramatic changes occur in the oscillatory potentials.

Longitudinal changes in photopic OPs occurring with vigabatrin treatment.

Purpose: Vigabatrin (gamma-vinyl-GABA) is an antiepileptic drug successful in the management of infantile spasms. Photopic ERGs were tested in children followed longitudinally before and during vigabatrin treatment.

Methods: Subjects were 26 infants (age range 1.

The Hospital for Sick Children, Toronto, Longitudinal ERG study of children on vigabatrin.

The purpose of this longitudinal study was to identify changes in ERG responses associated with vigabatrin treatment. We accomplished this by recording longitudinally ERGs in children before and during vigabatrin treatment and comparing results between children on vigabatrin monotherapy and those taking additional anticonvulsive medications. Thirty-three children on vigabatrin therapy were tested; the duration between visits was approximately 6 months.