Electrophysiological Evidences of Visual Field Alterations in Children Exposed to Vigabatrin Early in Life

Hébert-Lalonde, Noémie; Carmant, Lionel; Major, Philippe; Roy, Mélissa; Lassonde, Maryse; Saint-Amour, Dave

doi:10.1016/j.pediatrneurol.2016.03.001

Cited by 7 publications

(3 citation statements)

References 43 publications

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“…The field-specific VEP had a sensitivity of 75% and a specificity of 87.5% [ 55 ]. This technique was further developed by Hébert-Lalonde et al [ 56 , 57 ] that used steady-state VEP and pattern ERG to field-specific radial checkerboards. They showed significantly lower responses to peripheral stimulations in the vigabatrin-exposed group, that were negatively correlated to vigabatrin exposure duration.…”

Section: Peripheral Visual-field Defectmentioning

confidence: 99%

What can visual electrophysiology tell about possible visual-field defects in paediatric patients

Handley

Šuštar

Pompe

2021

Eye

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Recognising a potential visual-field (VF) defect in paediatric patients might be challenging, especially in children before the age of 5 years and those with developmental delay or intellectual disability. Visual electrophysiological testing is an objective and non-invasive technique for evaluation of visual function in paediatric patients, which can characterise the location of dysfunction and differentiate between disorders of the retina, optic nerve and visual pathway. The recording of electroretinography (ERG) and visual-evoked potentials (VEP) is possible from early days of life and requires no subjective input from the patient. As the origins of ERG and VEP tests are known, the pattern of electrophysiological changes can provide information about the VF of a child unable to perform accurate perimetry. This review summarises previously published electrophysiological findings in several common types of VF defects that can be found in paediatric patients (generalised VF defect, peripheral VF loss, central scotoma, bi-temporal hemianopia, altitudinal VF defect, quadrantanopia and homonymous hemianopia). It also shares experience on using electrophysiological testing as additional functional evidence to other tests in the clinical challenge of diagnosing or excluding VF defects in complex paediatric patients. Each type of VF defect is illustrated with one or two clinical cases.

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Section: Peripheral Visual-field Defectmentioning

confidence: 99%

What can visual electrophysiology tell about possible visual-field defects in paediatric patients

Handley

Šuštar

Pompe

2021

Eye

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“…Retinal toxicity has been associated with the drug vigabatrin, which is often used as first-choice therapy in the treatment of refractory seizures typical of TSC [ 87 ]. Retinal toxicity is shown with visual field loss that initiates as a bilateral nasal defect and evolves to concentric vision loss [ 88 ].…”

Section: Tuberous Sclerosis Complexmentioning

confidence: 99%

“…OCT of the peripapillary RNFL has shown that the alterations predominantly involve the nasal, superior, and inferior sectors [ 89 ]. Electroretinogram examination has been held as the optimal method to evaluate vigabatrin toxicity in children under nine years of age where perimetric examination is not reliable [ 87 ], but this frequently requires general anesthesia as some children cannot collaborate due to their young age or neurological condition. Thus, OCT has been proposed as a method to monitor therapy.…”

Section: Tuberous Sclerosis Complexmentioning

confidence: 99%

An Update on the Ophthalmologic Features in the Phakomatoses

Abdolrahimzadeh

Plateroti

Recupero³

et al. 2016

Journal of Ophthalmology

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Neurofibromatosis type 1, tuberous sclerosis complex, and Von Hippel-Lindau disease, historically classified as the phakomatoses, are hereditary multisystem disorders characterized by the presence of hamartoma, which carry the risk of malignant transformation. The alteration of tumor suppressor genes seems to be at the basis of their pathophysiogenetic mechanism. Lisch and choroidal nodules in neurofibromatosis type 1, retinal astrocytomas in tuberous sclerosis complex, and retinal capillary hemangioma in Von Hippel-Lindau disease are the principal ophthalmic hamartomatous manifestations. The advent of novel imaging techniques such as near infrared reflectance and optical coherence tomography has provided unprecedented insight on the choroidal and retinal features of these diseases. These methods have improved early diagnosis and the ongoing surveillance in these conditions. Among an array of treatment modalities, antivascular endothelial growth factor therapy has been used in the management of retinal hamartomas but results have been varied. This review is an update on the pathophysiogenetic mechanisms, ophthalmic manifestations, and novel treatment strategies in the phakomatoses with emphasis on the role of imaging techniques.

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