The incidence of abnormal pattern electroretinography in optic nerve demyelination

doi:10.1016/0013-4694(91)90014-U

Electroencephalography and Clinical Neurophysiology

Volume 78, Issue 1, January 1991, Pages 18-26

https://doi.org/10.1016/0013-4694(91)90014-U Get rights and content

Abstract

This report describes the pattern electroretinogram (PERG) findings in 141 patients with optic nerve demyelination in one or both eyes. The overall incidence of PERG abnormality in the 199 eyes with abnormally delayed pattern visual evoked potential (PVEP) P100 component was 39.2%, with 84.6% of these PERG abnormalities being confined to the N95 component. The incidence of abnormal PERG was greater (53.3%) in those eyes with a history of retrobulbar neuritis than in those with sub-clinical demyelination (22.8%). The importance of stimulus parameters is noted. The value of the PERG in the improved interpretation of an abnormal PVEP is discussed.

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Ischemic optic neuropathy often results in PERG N95 reduction (Atilla et al., 2006; Parisi et al., 2008) associated with loss of the retinal ganglion cells (Almarcegui et al., 2001). The PERG P50 component is more frequently affected in ischemic optic neuropathy than in optic neuritis (Holder, 1991a,b; Almarcegui et al., 2001), reflecting the severity of retinal ganglion cell involvement. Compressive lesions of the anterior visual pathway cause insidious and progressive visual impairment with visual field defects that reflect the location of the lesion in the visual pathway.
This chapter reviews common applications of visual electrophysiology relevant to neuro-ophthalmology practice. The use of standard tests and extended protocols are described including the cortical visual evoked potential and pattern and full-field electroretinogram (PERG; ERG) methods, the latter including the photopic negative response. Abnormalities of these recordings are rarely specific but provide valuable diagnostic guidance and an objective measure of visual pathway function, difficult or impossible to infer by other methods. The electrophysiological phenotypes associated with Leber hereditary optic neuropathy, OPA1- and SSBP1-associated dominant optic atrophy, and WFS1-related syndromes are described. Typical changes in retinal and optic nerve function tests associated with acquired disease are highlighted, including those related to demyelination, ischemic, compressive, nutritional and toxic, and nonorganic etiologies. The importance of complementary testing using different electrophysiological techniques is emphasized, for the purposes of differential diagnosis and in disorders that may masquerade as optic nerve pathology.
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In the absence of visual symptoms, and in the presence of normal visual acuity, the presence of a delayed PR-VEP can be taken as evidence of optic nerve disease; there is no need under such circumstances to confirm normal macular function. PERG has a normal P50 component in the majority of patients with optic nerve demyelination; approximately 40% will have PERG N95 component abnormalities (Holder, 1991). The VEP can also be abnormal in a variety of degenerative diseases of the nervous system such as Friedreich ataxia, other hereditary ataxias, familial spastic paraplegia, adrenoleukodystrophy (ALD), or hereditary motor and sensory neuropathies.
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The aim of this study was to evaluate the correlation between functional eye examinations (visual evoked potentials: VEPs; pattern electroretinogram: PERG) and structural measurements of the optic nerve (optical coherence tomography: OCT; scanning laser polarimetry: GDx) in patients with multiple sclerosis (MS).
Patients with definite MS and disease-free controls were enrolled in the study.
VEPs and PERG were recorded in all subjetcs. Ophthalmologic examination, including visual acuity, visual field determination, OCT and GDx were performed.
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Des patients présentant une sclérose en plaques et des sujets sains appariés en âge et sexe ont été inclus dans l’étude. Les PEVD et ERGD ont été enregistrés chez tous les sujets. Un examen ophtalmologique a également été réalisé chez tous les sujets; il incluait: la mesure de l’acuité visuelle, la détermination du champ visuel, la polarimétrie à balayage laser (GDx) et la tomographie en cohérence optique (OCT).
Dix-neuf patients avec sclérose en plaques et 19 sujets contrôle ont été inclus dans l’étude. Des différences significatives entre les deux groupes ont été observées pour les PEVD (amplitude et latence de la composante P100), l’ERGP (amplitude de la composante N95 et rapport entre les amplitudes N95/P50) et les paramètres de l’OCT (moyenne, volume temporal et maculaire). Les paramètres PEVD ou ERGD étaient significativement corrélés aux résultats de l’OCT ou du GDx.
Chez les patients ayant une sclérose en plaques, l’atteinte axonale des cellules ganglionnaires peut être détectée avec l’OCT et le GDx. L’ERGD est un examen complémentaire très utile pour identifier ces lésions.
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This chapter discusses the most common cause of optic neuritis disorder, a primary demyelinating disease in which optic neuritis may develop as the first manifestation or during the course of multiple sclerosis (MS). The clinical parameters that predict a good or poor recovery are not clearly understood, but patients with a good outcome have been observed to recover more quickly in the early stages than those with a poor outcome. Blood tests are in general normal, and a modest spinal fluid pleocytosis is observed in about 50%. Oligoclonal bands are seen in 70%. MRI scans of the optic nerve show a high signal lesion on T2-weighted imaging, which enhances in almost every case on T1-weighted imaging. The nerve is swollen and subsequently reduces in size, implying that atrophy of the nerve occurs. There is a clear relationship between the presence and severity of residual visual symptoms and signs and the presence of optic nerve atrophy and retinal nerve fiber layer defects measured on optic coherence tomography. Sixteen percent to 42% of patients undergo a recurrence of optic neuritis either in the same or the other eye. This is associated with the subsequent development of MS in some but not all patients. The incidence of MS in the clinical studies of optic neuritis rises progressively from about 40% at 5 years to 60% at 40 years. The presence of four or more lesions on MRI scans of the brain is associated with a substantially increased risk of the subsequent development of MS, around 90% over 10 years.
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The incidence of abnormal pattern electroretinography in optic nerve demyelination

Abstract

Am. J. Ophthalmol.

Electroenceph. clin. Neurophysiol.

Electroenceph. clin. Neurophysiol.

Electroenceph. clin. Neurophysiol.

Lancet

Electroenceph. clin. Neurophysiol.

Electroenceph. clin. Neurophysiol.

Ophthalmology

Electroenceph. clin. Neurophysiol.

Electroenceph. clin. Neurophysiol.

Clinical and experimental evidence that the pattern electroretinogram (PERG) is generated in more proximal retinal layers than the focal electroretinogram (FERG)

Ann. NY Acad. Sci.

Visual evoked potentials in macular disease

Invest. Ophthalmol. Vis. Sci.

The pattern electroretinogram and its contamination

Clin. Vis. Sci.

The pattern electroretinogram

Eye

Spatial tuning of the pattern ERG across temporal frequency

Doc. Ophthalmol.

Visual field defects and pathoological alterations in topography: factors complicating the estimation of visual evoked response “delay” in multiple sclerosis

Correlations among clinical data, pattern electroretinogram, visual evoked potential and retinal fibre layer findings in multiple sclerosis

Pattern ERGs and visual evoked potentials in maculopathies and optic nerve disease

Invest. Ophthalmol. Vis. Sci.

Simultaneous recording of pattern electroretinography and visual evoked potentials in multiple sclerosis

Arch. Neurol.

Flash ERG abnormalities in patients with clinically definite multiple sclerosis

Can. J. Neurol. Sci.

Variations in PERG amplitude and nuclear layer thickness with retinal eccentricity

Electroenceph. clin. Neurophysiol.

Changes in the electroretinogram in patients with optic nerve lesions

Doc. Ophthalmol.

The ERG in response to alternating gratings in patients with diseases of the peripheral visual pathway

Invest. Ophthalmol. Vis. Sci.

Electroretinographic abnormalities in advanced multiple sclerosis

Invest. Ophthalmol. Vis. Sci.