VEP characteristics in children with achiasmia, in comparison to albino and healthy children

Brecelj, Jelka; Šuštar, Maja; Pečarič-Meglič, Nuška; Škrbec, Miha; Stirn-Kranjc, Branka

doi:10.1007/s10633-012-9315-4

Cited by 13 publications

(14 citation statements)

References 51 publications

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“…A marked loss of the left visual field is unexpected, given that monocular visual fields are full in patients with human albinism [17,28]. However, bi-temporal hemianopia has been reported in a few, but not all, cases of human achiasmia [14,29].…”

Section: Discussionmentioning

confidence: 99%

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Case report: Unilateral optic nerve aplasia and developmental hemi-chiasmal dysplasia with VEP misrouting

et al. 2020

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Purpose To describe the trans-occipital asymmetries of pattern and flash visual evoked potentials (VEPs), in an infant with MRI findings of unilateral optic nerve aplasia and hemi-chiasm dysplasia. Methods A child with suspected left cystic microphthalmia, left microcornea, left unilateral optic nerve aplasia, and hemi-chiasm underwent a multi-channel VEP assessment with pattern reversal, pattern onset, and flash stimulation at the age of 16 weeks. Results There was no VEP evidence of any postretinal visual pathway activation from left eye with optic nerve aplasia. The VEP trans-occipital distribution from the functional right eye was skewed markedly across the midline, in keeping with significant misrouting of optic nerve fibres at the chiasm. This was supported by the anatomical trajectory of the optic chiasm and tracts seen on MRI. Conclusion This infant has chiasmal misrouting in association with unilateral optic nerve aplasia and unilateral microphthalmos. Chiasmal misrouting has not been found in patients with microphthalmos or anophthalmos, but has been reported after early eye loss in animal models. Our findings contribute to our understanding of the discrepancy between the visual pathway physiology of human unilateral microphthalmia and animal models.

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Section: Discussionmentioning

confidence: 99%

“…Pattern onset VEPs positive peaks, in contrast, are maximal over the functional ipsilateral hemisphere [11,12]. These contrasting VEP distributions have been used to investigate developmental chiasmal malformations in humans including achiasmia and albinism [13][14][15].…”

Section: Introductionmentioning

confidence: 99%

Case report: Unilateral optic nerve aplasia and developmental hemi-chiasmal dysplasia with VEP misrouting

et al. 2020

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“…Visual evoked potentials (VEPs) are a validated technique for identifying chiasmal dysfunction or disproportional decussation in the clinical environment [8,9,10]. In order to detect chiasmal disproportion, monocular VEPs must be recorded employing a trans-occipital electrode array (clinically three to five electrodes are suggested) [11].…”

Section: Introductionmentioning

confidence: 99%

“…If a trans-occipital asymmetry of the VEP from one eye is mirrored across the midline by the other eye it is termed a “crossed asymmetry” [12]. This can be observed if too many fibers cross as in albinism but also in the other extreme in achiasmia where there is no crossing [8,13,14,15].…”

Section: Introductionmentioning

confidence: 99%

Occipital Petalia and Albinism: A Study of Interhemispheric VEP Asymmetries in Albinism with No Nystagmus

Liasis

Handley

Nischal

2019

JCM

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The purpose of this study was to assess chiasmal misrouting in a cohort of children with albinism with no nystagmus using hemifield visual evoked potentials (VEP) measures. Methods: Monocular VEPs were recorded and analyzed from three electrodes (O1, Oz, and O2 referred to Fz) from 16 children with albinism without nystagmus. Pattern reversal (full field and hemifield stimulation), full field pattern appearance and flash stimuli were used to evoke VEPs for each eye. Results: The amplitude of the pattern reversal VEPs to stimulation of the hemifield corresponding to the crossing pathways were as expected significantly larger than those to the non-crossing in each eye ((right eye p = 0.000004), (left eye p = 0.001)). Pattern reversal VEPs recorded from the left hemisphere were also larger than those from the right and most evident when comparing the crossing pathways of each eye (p = 0.004). Conclusions: This study has demonstrated electrophysiological differences in visual pathway function of the left and right hemisphere in subjects with albinism like that previously described in controls. Nasal field stimulation activated crossing and non-crossing pathways in patients with albinism and as a result, nasal hemifield VEPs in albinism are less lateralized compared to what is found in normal subjects.

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“…1,2 In our previous studies of children with INS, electroretinogram (ERG) and/or visually evoked potential (VEP) characteristics in association to clinical findings were shown, to differentiate a number of retinal or visual pathway dysfunctions. [3][4][5][6][7][8][9] Nystagmus is idiopathic when ocular or systemic abnormalities are excluded. 1 The ERG is normal in IIN, 4,[10][11][12][13][14] but possible nystagmus impact on ERG was indicated in one study.…”

Section: Introductionmentioning

confidence: 99%

Electroretinograms in idiopathic infantile nystagmus, optic nerve hypoplasia and albinism

Kurent

Brecelj

Stirn-Kranjc

2018

European Journal of Ophthalmology

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Purpose: To study electroretinograms in infantile nystagmus syndrome associated with idiopathic infantile nystagmus, optic nerve hypoplasia, and albinism. Methods: A total of 30 children with idiopathic infantile nystagmus, 18 with optic nerve hypoplasia, and 18 with albinism were studied. Three electroretinogram protocols were applied according to child’s age: 58 (mean: 2.0 years) were recorded with skin electrode to Great Ormond Street Hospital protocol, 11 (mean: 5.3 years) with skin electrode to International Society for Clinical Electrophysiology of Vision protocol, and 7 children (mean: 12.2 years) with HK electrode to International Society for Clinical Electrophysiology of Vision protocol. The electroretinograms were compared to those of age-matched controls. Results: Electroretinogram waveforms in idiopathic infantile nystagmus, optic nerve hypoplasia, and albinism were comparable to controls in all protocols. Electroretinogram amplitudes in idiopathic infantile nystagmus group showed increased white scotopic and photopic electroretinograms in 26 children (skin electrode to Great Ormond Street Hospital protocol), no difference to the controls in 3 children (skin electrode to International Society for Clinical Electrophysiology of Vision protocol), and increased rod electroretinogram in 3 children (HK electrode to International Society for Clinical Electrophysiology of Vision protocol). Optic nerve hypoplasia group showed increased white scotopic, photopic, and blue electroretinograms in 15 children (skin electrode to Great Ormond Street Hospital protocol); increased 30-Hz electroretinogram in 3 children (HK electrode to International Society for Clinical Electrophysiology of Vision protocol); and reduced combined rod-cone, cone, and 30-Hz electroretinograms in 3 children (skin electrode to International Society for Clinical Electrophysiology of Vision protocol). Albinism group showed increased white scotopic, photopic, and 30-Hz electroretinograms in 17 children (skin electrode to Great Ormond Street Hospital protocol), while it showed reduced cone and 30-Hz electroretinograms in 5 children (skin electrode to International Society for Clinical Electrophysiology of Vision protocol). Implicit times were shorter in albinism. Conclusion: Electroretinogram waveforms in idiopathic infantile nystagmus, optic nerve hypoplasia, and albinism were normal with mostly increased electroretinograms, while reduced electroretinograms did not show a specific pattern as in early-onset retinal dystrophies.

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VEP characteristics in children with achiasmia, in comparison to albino and healthy children

Cited by 13 publications

References 51 publications

Case report: Unilateral optic nerve aplasia and developmental hemi-chiasmal dysplasia with VEP misrouting

Case report: Unilateral optic nerve aplasia and developmental hemi-chiasmal dysplasia with VEP misrouting

Occipital Petalia and Albinism: A Study of Interhemispheric VEP Asymmetries in Albinism with No Nystagmus

Electroretinograms in idiopathic infantile nystagmus, optic nerve hypoplasia and albinism

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