Pattern electroretinography in patients with delayed pattern visual evoked potentials due to distal anterior visual pathway dysfunction.

Between March 1983 and January 1988 delayed pattern visual evoked potentials (PVEP) were observed in 67 patients with distal visual pathway dysfunction. Many of these patients had been referred for neurophysiological examination because of possible optic nerve dysfunction. These patients also had pattern electroretinography (PERG) performed which in all cases showed an abnormality of the main positive P50 component. None of these patients had an abnormality confined to the negative N95 component, the type of abnormality usually found if the PERG is abnormal in optic nerve disease. It is suggested that PERG recording should now be a routine adjunct to the PVEP in the assessment of anterior visual pathway dysfunction.     

Pattern visual evoked potentials in malingering.

OBJECTIVES: We previously developed a new method for estimating objective visual acuity by means of pattern visual evoked potentials (PVEP). In this study, this method was applied to the diagnosis of malingering. MATERIALS AND METHODS: Six patients ranging in age from 40 to 54 years (mean 47 years) with suspected malingering were evaluated by means of the visual evoked potential test, optokinetic nystagmus (OKN) inhibition test, and the visual field test. In the PVEP study, the stimulus consisted of black and white checkerboards (39', 26', 15', and 9') with a visual angle of 8 degrees, contrast level of 15%, and a frequency of 0.7 Hz. One hundred PVEP responses were averaged per session. RESULTS: Routine ophthalmic examinations were normal in all patients. Five patients had a tubularly constricted visual field, and the remaining patient had a normal visual field. The objective visual acuities of the six patients estimated from PVEP were better than their subjective visual acuities estimated with Landolt rings. CONCLUSIONS: Among a variety of psychophysical and electrophysiologic ancillary tests, we consider our PVEP method a useful method for objectively determining visual acuity in a patient with signs of ocular malingering.     

Simultaneous recording of pattern electroretinography and visual evoked potentials in multiple sclerosis. A method to separate demyelination from axonal damage to the optic nerve

Pattern electroretinograms (P-ERGs) and visual evoked potentials (VEPs) were recorded in 35 patients with multiple sclerosis and in 35 age-matched normal subjects. Four patterns of abnormalities were noted in the group with multiple sclerosis. The most frequent abnormality consisted of the following: normal P-ERG, delayed P100, and prolonged interpeak interval between the b-wave of the P-ERG and P100 (retinocortical time). This pattern indicates demyelination of the optic nerve. A second pattern consisted of absent P-ERG and absent VEP. This pattern was associated with optic atrophy and/or central scotoma, indicating severe optic nerve axonopathy with retrograde degeneration of ganglion cells. A third pattern consisted of normal P-ERG and absent VEP, suggesting a total block of transmission at the optic nerve. A fourth pattern consisted of present but low-amplitude P-ERG, delayed VEP, and prolonged retinocortical time, indicating a demyelinating process with partial axonal involvement. The concomitant use of P-ERG and VEP results in a better classification of the type and severity of dysfunction affecting the optic nerve. The prognostic value of the four patterns for recovery of visual function is discussed.     

Electro-oculography, electroretinography, visual evoked potentials, and multifocal electroretinography in patients with vigabatrin-attributed visual field constriction

PURPOSE: Symptomatic visual field constriction thought to be associated with vigabatrin has been reported. The current study investigated the visual fields and visual electrophysiology of eight patients with known vigabatrin-attributed visual field loss, three of whom were reported previously. Six of the patients were no longer receiving vigabatrin. METHODS: The central and peripheral fields were examined with the Humphrey Visual Field Analyzer. Full visual electrophysiology, including flash electroretinography (ERG), pattern electroretinography, multifocal ERG using the VERIS system, electro-oculography, and flash and pattern visual evoked potentials, was undertaken. RESULTS: Seven patients showed marked visual field constriction with some sparing of the temporal visual field. The eighth exhibited concentric constriction. Most electrophysiological responses were usually just within normal limits; two patients had subnormal Arden electro-oculography indices; and one patient showed an abnormally delayed photopic b wave. However, five patients showed delayed 30-Hz flicker b waves, and seven patients showed delayed oscillatory potentials. Multifocal ERG showed abnormalities that sometimes correlated with the visual field appearance and confirmed that the deficit occurs at the retinal level. CONCLUSION: Marked visual field constriction appears to be associated with vigabatrin therapy. The field defects and some electrophysiological abnormalities persist when vigabatrin therapy is withdrawn.     

Evolution of visual evoked potentials in optic neuritis

The visual evoked potential (VEP) latency was either abnormally prolonged or absent in the involved eye of 47 patients with optic neuritis. Twenty-two of these patients with known multiple sclerosis (MS), had similar abnormalities to 25 patients with no clinical evidence of MS. Follow-up clinical assessment and VEP were done 10 to 42 (mean 22) months later in 34 patients. In 15 of 34 patients with no VEP from the involved eye during initial examination, 6 returned to normal, 8 had prolonged latencies and 1 still had no response at follow up. Of 19 patients who initially had prolonged latencies in the involved eye, 6 returned to normal, 11 had prolonged latencies and 2 had no response at follow up. The VEP is helpful in confirming the diagnosis of ON. The examination must be performed when the patient is symptomatic or soon thereafter as 35% of our patients with an abnormal initial VEP had a normal VEP at follow up. This normalization was not related to the severity of the initial VEP abnormality.     

Visually evoked potentials and electroretinography in neurologic evaluation.

Electrophysiologic testing of the visual system requires primarily the ERG and the VEP. The flash electroretinogram provides information about the outer retina only. The pattern electroretinogram is derived from both the outer retina and the innermost retinal layers including the ganglion cell layer. The VEP is based on electrical information recorded from the visual cortex in response to stimulation of the retina. Thus, the integrity of the entire visual pathway can be tested. Localizing ability of the VEP is limited. Since the visual cortex is heavily weighted by representation of the central retina, peripheral lesions, including those producing peripheral visual field abnormalities that do not impinge upon central fixation, may produce relatively little disturbance of the VEP. As with most tests, electrophysiologic studies of the visual system must be placed in context of the entire examination, including the patient's history and neurologic and especially neuro-ophthalmologic evaluation. Electrophysiologic testing has three main uses in neurology. Pattern-reversal VEPs may be useful in detecting hidden visual loss in multiple sclerosis; VEPs and ERGs can distinguish function from organic visual loss; and VEPs and ERGs can be useful in the diagnosis of visual loss in nonverbal patients, especially in children.     

Pattern reversal visual evoked potentials in awake rats

A method for recording pattern reversal evoked potentials (PREPs) from awake restrained rats has been developed. The procedure of Onofrj et al. [26] was modified to eliminate the need for anesthetic, thereby avoiding possible interactions of the anesthetic with other manipulations of interest. Rats were restrained in a harness and placed in front of a pattern generating TV screen displaying a black and white alternating square wave grating. Using various stimulation and recording parameters, normative data are presented from 141 adult male Long-Evans hooded and 11 adult male Sprague-Dawley albino rats. Reliable waveforms were recorded with five identifiable peaks. The labels and mean latencies of these peaks in hooded rats were: N1, 47.3 msec; P1, 65.7 msec; N2, 83.3 msec; P2, 94.4 msec: and N3, 129.8 msec. Spatial acuity functions generated with PREPs gave acuity estimates which corresponded closely to values determined behaviorally for hooded and albino rats [4,11].     

Do visual evoked potentials give relevant information to the neuro-ophthalmological examination in optic nerve lesions?

The visual evoked potentials (VEPs) and neuro-ophthalmological examinations of 134 patients were compared. The VEPs were abnormal in 95 % of the eyes with optic neuritis. Defective color vision was found in 99 %, visual field defects in 88 %, decreased vision in 66 % and an afferent pupillary defect in 55 %. 29 patients with optic neuritis were followed up with repeated tests. VEPs and color vision recovered more slowly than visual acuity and visual field.
Abnormal VEPs were observed in 68 % of 50 MS patients. An analysis of symptomatic and asymptomatic eyes showed that testing of color vision, visual field and red-free ophthalmoscopy were equally as useful diagnostic tools as VEPs. 4 (8 %) of the MS patients had abnormal VEPs despite a normal neuro-ophthalmological examination; 94 % of MS patients with symptoms and 47 % of MS patients without visual symptoms had abnormal VEPs.
VEPs were pathological in 59 % of 24 patients with traumatic or compressive optic nerve diseases or optic atrophies of unknown etiology. The neuro-ophthalmological examination was more sensitive than VEPs in the diagnosis of these disorders. A neuro-ophthalmological examination is in most cases sufficient to diagnose optic nerve lesions. VEPs are of diagnostic aid especially in mild optic nerve lesions.     

Pattern reversal visual evoked potentials in Japanese patients with multiple sclerosis.

Forty-seven Japanese patients with multiple sclerosis, 29 probable (clinically definite) and 18 possible, were studied by black-and-white checkerboard pattern reversal visual evoked potential and were compared with a control group of 20 healthy young adults. The major positive peak (P100) was found to be abnormal in 70% of all cases, 90% of probable cases and 39% of possible cases. P100 was delayed in 38% of all cases and was absent in 23% of all cases. None of the eyes showing a flat pattern response was in the acute stage of optic neuritis. The percentage of cases with no response (23% of all cases) was greater than any of the previously reported series from Western countries, substantiating the previously reported clinical features of oriental multiple sclerosis. The pattern response was absent only when testing eyes with severe visual impairment, whereas delayed latency of P100 was seen regardless of the severity of visual impairment, suggesting the usefulness of P100 latency for detecting subclinical optic nerve lesions.     

Midbrain evoked potentials and oculomotor activity produced by optic nerve stimulation

Evoked potentials, produced by electrical stimulation of the optic nerve, were recorded from the superior colliculus and the midbrain close to the oculomotor nucleus. The experiments were done on 23 adult cats immobilized by gallamine triethiodide after hemidecerebration by prethalamic transection. The evoked potentials consisted of three components, namely, an early wave, a series of recurrent waves with latency of about 6 msec, and a late slow wave with latency of about 16–23 msec. It was concluded that the early wave was due to the arrival of optic nerve impulses, the recurrent wave to repetitive firing of collicular neurons, and the late slow wave to the collicular outflow onto the oculomotor nucleus. In the majority of instances in which the late slow wave was observed, the preceding optic nerve volley revealed facilitation of the antidromic spike of oculomotor nucleus. In a few instances in which the late slow wave was not obtained, either facilitation or inhibition of spontaneous oculomotor discharge was observed, indicating the dual facilitatory and inhibitory components of colliculofugal outflow to the oculomotor nucleus.     

Visual pathway abnormalities Wilson's disease: an electrophysiological study using electroretinography and visual evoked potentials

The pathogenesis of the pattern reversal visual evoked potential (PRVEP) abnormalities in patients with Wilson's disease (WD) has not been investigated earlier. Since electroretinography (ERG) assesses the functional integrity of the retina, it was used along with PRVEP to localize the abnormalities in PRVEP in Wilson's patients. Ten newly diagnosed Wilson's disease patients underwent PRVEP and flash ERG soon after the diagnosis was established. The PRVEP latencies were prolonged in comparison with the controls (P<0.001). Photopic and scotopic A waves and oscillatory potentials were prolonged (P<0. 02) with reduction in amplitudes of photopic A and B waves (P<0.001). Six of these patients were subjected to repeat PRVEP and flash ERG after the clinical improvement with specific therapy. Comparison of the pre and post-treatment visual electrophysiological studies revealed significant reduction in latencies of PRVEP and flash ERG A wave (P<0.05) with increase in amplitudes of P100 of PRVEP (P<0.05), A and B waves of flash ERG (P<0.01). These findings confirm the reported PRVEP changes in WD and in addition demonstrate the reversibility of the retinal dysfunction which partially contributes to the PRVEP abnormalities. To the best of our knowledge this is the first study of ERG in patients with Wilson's disease in the literature. Further, there have been no earlier reports in the literature evaluating the effect of specific treatment on the PRVEP and ERG in Wilson's disease.     

Delayed and pseudodelayed visual evoked potentials in optic neuritis compared with long time echo-short tau inversion recovery magnetic resonance imaging of optic nerve

Twenty patients affected by optic neuritis (ON) underwent serial visual evoked potential (VEP) recordings, performed with multiple electrode arrays, and with stimuli of 1 and 3 cycles per degree (cpd) for 1 year. VEP findings were correlated with long time echo-short tau inversion recovery (LTE-STIR) magnetic resonance imaging (MRI) of optic nerves and with visual field tests. MRI showed lesions in 95.2% of acute ON and in 66.6% of the 1 year follow-up. VEPs were classified into really 'delayed' VEPs and 'pseudodelayed' VEPs, based on their scalp distribution. Furthermore, VEPs to 1 or 3 cpd could be 'delayed' or 'pseudodelayed' in the same patient. Real delays could be recorded at onset or shortly after ON, and indicated the possibility of recovery of visual functions and good functional prognosis. Pseudodelays, to 3 cpd, corresponded to prominent central scotomata and indicated poor prognosis for the recovery of visual function, unless a breakthrough of normal or delayed components appeared in the first 4 months following acute ON. Pseudodelayed VEPs clustered in patients with longer demyelinating lesions, as shown by LTE-STIR MRI. There was no correlation between latency of VEPs and length of plaques. Our study addresses some reconsiderations of the pathophysiology of conduction delay in acute optic neuritis.     

Multifocal visual evoked potentials in optic neuritis and multiple sclerosis: A review

Multifocal visual evoked potential (mf-VEP) represents a new approach to the classical full field (ff-)VEP with separate responses from up to 60 sectors of the visual field. A thorough literature survey of the use of mf-VEP in optic neuritis (ON) and multiple sclerosis (MS) is presented (38 published studies were retrieved). Mf-VEP provides direct topographical information of specific lesions and facilitates investigations on structural-functional correlations thus providing new methods for exploring the interplay between demyelination, atrophy and remyelination in MS. Good correlation was shown between mf-VEP and OCT, ff-VEP, MRI (MTR, DTI), 30-2 standard automated perimetry and low-contrast-visual acuity. All but one study showed superior sensitivity and specificity compared to ff-VEP, especially with regards to small, peripheral lesions or lesions of the upper visual field. Mf-VEP has shown superior sensitivity and specificity than established methods in diagnosing optic nerve lesions and tracking functional recovery following lesions. Abnormal mf-VEP responses in the fellow, non-ON afflicted eye may predict MS risk in ON patients. No standardization currently exists and no direct comparisons in ON and MS between at least 5 different commercially available mf-VEP systems have so far been published. Despite these limitations, mf-VEP is a promising new tool of diagnostic and prognostic value of mf-VEP in ON and MS.     

Pattern visual evoked potentials and flash electroretinogram in clinically definite multiple sclerosis

The authors have studied, by means of pattern visual evoked potential (VEP) and flash electroretinogram (ERG) recordings, a group of 15 patients affected by definite multiple sclerosis. All of the subjects examined presented a clinical history indicating involvement of the visual pathways; VEPs were altered in a high percentage of eyes examined (93.3%), while a lower percentage of abnormal ERGs was seen (20% of eyes examined). The only type of ERG alteration found consisted of a pathologic b wave voltage increase, observed mainly with red flash stimuli. This finding could be attributed to an involvement of centrifugal optic nerve fibers having inhibitory functions on retinal cells.     

Reduced visual evoked responses in multiple sclerosis patients with optic neuritis: comparison of functional magnetic resonance imaging and visual evoked potentials.

The limited application of functional magnetic resonance imaging (fMRI) for investigations of multiple sclerosis (MS) patients has already shown that deficits of the motor, cognitive and visual systems may be identified by differences in the patterns of activation in response to a suitable stimulus. In MS patients with unilateral optic neuritis, the area of activation in the primary visual cortex, measured by fMRI techniques, is dramatically reduced in response to stimulation of the affected eye. The latency of the major positive component of the visual evoked potential (VEP) recorded upon stimulation of the affected eye is significantly increased in these patients, as compared to the unaffected eye and normal volunteers. We have found a correlation between the neural response measured using fMRI and the latency of the VEP. fMRI signal responses have the potential to provide more detailed topographic information relating to functional deficits in MS.     

The incidence of abnormal pattern electroretinography in optic nerve demyelination.

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.