The value of electrophysiology results in patients with epilepsy and vigabatrin associated visual field loss

PURPOSE: To determine the value of electrophysiological findings in patients with temporal lobe epilepsy and to relate these findings to the amount of concentric contraction of the visual field and the use of vigabatrin. METHODS: Electro-retinograms and electro-oculograms were done on 30 patients, operated for temporal lobe epilepsy. The patients were divided into three groups: (A) concentric contraction of the visual field associated with a history of vigabatrin medication (15 patients), (B) normal visual field with vigabatrin use (11 patients) and (C) normal visual field without vigabatrin medication (4 patients). RESULTS: Electrophysiological abnormalities were found in 50% of the patients in group A. The Arden ratio of the EOG was lowered in 57%. Abnormalities in the ERG were found: b-wave implicit time photopic F was prolonged (50%), b-wave amplitudes scotopic B (53%), C (73%) and G (50%) and photopic H (50%) were diminished. The amount of visual field loss and the total dose of vigabatrin used, showed only slight correlation with the ERG and EOG. The use of vigabatrin during the ERG and EOG recording in group A, gave a higher b-wave amplitude scotopic G in 64% of cases. The a-wave implicit times scotopic G (73%) and photopic G (59%) and H (73%) were shortened in group B. CONCLUSION: EOG was abnormal in 57% in group A. ERG abnormalities could only be found in 50% of group A, mainly in the inner retina. Since also the total dose of vigabatrin and the amount of visual field loss did not really show a correlation with the electrophysiological findings and results of literature are not unanimous, electrophysiology does not appear at present to be a good method to detect patients with, or at risk of, vigabatrin associated visual field loss. Regularly performed visual field examination remains the cornerstone in screening.     

Visual electrophysiological effect of a GABA transaminase blocker

Vigabatrin is an antiepileptic drug for the treatment of partial seizures. The anticonvulsant effect is achieved by irreversible inhibition of the enzyme GABA-transaminase which catalyses the inactivation of GABA. Vigabatrin has been associated with visual field loss and electrophysiological abnormalities. The purpose of the study was to determine any alterations in normal volunteers of the visual field and the visual electrophysiology resulting from a short exposure to vigabatrin. A three-way, double-blind study of placebo, carbamazepine and vigabatrin was undertaken at baseline and on days two, four and nine. Seven subjects completed all three cycles and 14 subjects (six females and eight males; mean age 27.3 years SD 6.7) completed at least one cycle. Static threshold automated perimetry comprised Humphrey Visual Field Analyzer Programs 30-2 and 30/60-2. Electro-oculography and electroretinograms were performed with undilated pupils using the Medelec Ganzfeld stimulator GS2000. The visual field was unaffected by placebo, carbamazepine or vigabatrin. The group mean amplitudes and latencies for the scotopic ERG, 30Hz flicker ERG and the oscillatory potentials remained unchanged for any cycle. The group mean photopic ERG b-wave latency increased from baseline (p < 0.05); no significant change occurred with carbamazepine or placebo. The group mean Arden Index for vigabatrin decreased from baseline to day 9 (p <; 0.01); no significant differences were present for carbamazepine or placebo. Vigabatrin has a rapid effect on both the photopic ERG and the EOG; however, the changes merely reflect alterations in retinal GABA levels secondary to concomitant blocking of GABA transaminase by existing vigabatrin therapy. This revised version was published online in July 2006 with corrections to the Cover Date.     

Visual field and electrophysiological abnormalities due to vigabatrin

The purpose of this study was to determine the electrophysiological changes in patients using the anti epileptic drug vigabatrin and to correlate these findings with the previously reported risk for visual field loss in these patients. In 1998 the neurologists of both involved hospitals referred all patients on vigabatrin medication for ophthalmological examination to the outpatients clinics. Of the 33 patients whom were referred to our outpatient clinics, four had to be dropped from the study because of disability to perform the examinations the remaining 29 patients were included in the study. Standard ophthalmological investigations were carried out, and contrast sensitivity, visual field (Humphrey 30-2 and Esterman or Octopus 32), colour vision (panel D15), ERG and EOG according to ISCEV standards were tested. 18 patients continued the medication and 11 stopped taking the drug during the study. Nine of the patients who stopped the drug were followed during at least half a year afterwards, this group will be described in the combined article `Electro ophthalmic recovery after withdrawal from vigabatrin' (Graniewski and Van der Torren, this issue). The electro-ophthalmological findings in the group of 29 patients were correlated with the visual fields and the daily and cumulative dosages of vigabatrin. Of the patients, 32% showed no visual field constriction at all; from these patients 64% had EOG and/or ERG changes. Of the patients with slight to marked visual field constriction, 90% presented EOG and/or ERG changes. Significant correlation between daily dosages of vigabatrin and visual field defects was shown as well as between visual field defects and rod and cone b wave amplitude reductions. Cumulative vigabatrin dosages presented a significant correlation with EOG ratio and ERG rod b-wave amplitude. Conclusively EOG and ERG testing were found to be even an more accurate way to monitor the direct vigabatrin effect on the outer retina and is possible different from the visual field testing.     

Predominant loss of the photopic negative response in central retinal artery occlusion

PURPOSE: To determine how the photopic negative response (PhNR) is affected in central retinal artery occlusion (CRAO). DESIGN: Observational case series. METHODS: Seven patients with unilateral CRAO were included. Full-field scotopic and photopic electroretinograms (ERGs) including the PhNR were recorded. Each ERG amplitude in the affected eye was expressed as a percentage of amplitude of the corresponding wave in the unaffected eye. RESULTS: Mean of the PhNR amplitude was reduced to 12.3 +/- 11.7% of that of unaffected eyes whereas the cone b-wave amplitude was attenuated to only 73.4 +/- 30.4%. This reduction of the PhNR amplitude was more significant than that of other waves including the rod b-wave, maximum a-wave and b-wave, cone a-wave and b-wave, and 30 Hz flicker ERG (P <.005). CONCLUSIONS: The PhNR was severely affected in CRAO despite relative preservation of the cone b-wave, implicating massive loss of ganglion cells and their axons.     

Electroretinographic (ERG) responses in pediatric patients using vigabatrin

The antiepileptic drug vigabatrin is known to cause retinal and visual dysfunction, particularly visual field defects, in some patients. Electroretinography (ERG) is used in an attempt to identify adverse effects of vigabatrin (VGB) in patients who are not candidates for conventional perimetry. We report data from 114 pediatric patients taking VGB referred for clinical evaluation; median age at test was 22.9 (2.4 to 266.1) months, and median duration of VGB use was 9.7 (0.3 to 140.7) months. Twenty-seven of them were tested longitudinally (3 to 12 ERG tests). ERG responses to full-field stimuli were recorded in scotopic and photopic conditions, and results were compared to responses from healthy control subjects. We found that abnormalities of photoreceptor and post-receptor ERG responses are frequent in these young patients. The most frequently abnormal scotopic parameter was post-receptor sensitivity, log σ, derived from the b-wave stimulus-response function; the most frequently abnormal photopic parameter was the implicit time of the OFF response (d-wave) to a long (150 ms) flash. Abnormal 30-Hz flicker response amplitude, previously reported to be a predictor of visual field loss, occurred infrequently. For the group as a whole, none of the ERG parameters changed significantly with increasing duration of VGB use. Four of the 27 patients tested longitudinally showed systematic worsening of log σ with duration of VGB use. In a subset of patients who underwent perimetry (N = 39), there was no significant association of any ERG parameter with visual field defects. We cannot determine whether the ERG abnormalities we found were due solely to the effects of VGB. We caution against over-reliance on the ERG to monitor pediatric patients for VGB toxicity and recommend further development of a reliable test of peripheral vision to supplant ERG testing.     

Electro-ophthalmological recovery after withdrawal from vigabatrin

This study describes the effects of stopping of the anti-epileptic drug vigabatrin on the visual field and electrophysiological changes in one third of the group of patients which is described in the paper `Visual field and electrophysiological abnormalities due to vigabatrin' (Van der Torren and Graniewski, 2002). In 1997 several reports described the possible oculotoxic effect of vigabatrin followed by a reconsideration of this medication in epileptic patients. Vigabatrin was discontinued in one-third of the patients on chronic medication (mean duration 4.8 years). The visual field and electrophysiological examinations were repeated every 3 months if possible, otherwise at 6-month intervals. The EOG Arden index and the ERG rod b-wave showed a significant improvement when vigabatrin was discontinued. Repeated examinations of visual fields and electrophysiology shortly after discontinuation of the drug (between 1 and 3 months) and later on after 6 months and 1 year showed a recovery of the EOG Arden index and the ERG rod b-wave during this period. The visual fields did not change in either direction. Conclusion: the recovery effect is a strong argument for the hypothesis that the reduction in EOG and ERG b-wave is an oculotoxic effect. The electrophysiological improvement during 6 months or longer after discontinuation and the unchanged visual fields are an argument for the hypothesis that the visual field represents the irreversible intoxicating effect on the retina, whereas the electrophysiology represents a more direct effect on the retinal glial cells level.     

影响视网膜脱离患者视网膜功能的因素

目的:评价视网膜脱离患者年龄、病程、视网膜脱离面积、黄斑脱离情况等因素对视网膜功能的影响。方法:将孔源性视网膜脱离(retinal detachment,RD)188例189眼的患者年龄、病程、视网膜脱离面积、黄斑脱离情况、视力与视网膜脱离眼的fERG和mfERG各指标进行相关分析。结果:影响RD眼fERG和mfERG的主要因素是脱离面积、黄斑脱离和视力。以|γ|>0.4,且P≤0.05判定为有相关性。脱离面积与明视和暗适应最大反应ERG a、b波幅值,30Hz闪烁光幅值,OPs波数和幅值负相关;与mfERG象限野P1波幅值密度、幅值负相关。相关系数最高者为fERG暗适应最大反应b波幅值(γ=-0.704)。黄斑脱离与明视和暗适应最大反应ERG b波幅值,30Hz闪烁光ERG幅值负相关;与mfERG环形野环1的P1波幅值密度、幅值负相关,与mfERG象限野P1波幅值密度负相关,相关系数最高者为mfERG环形野环1的P1波幅值(γ=-0.584)。视力与明视和暗适应最大反应a、b波幅值,30Hz闪烁光幅值,OPs子波数和幅值正相关;与mfERG环形野环1的P1波幅值密度、幅值正相关,相关系数最高者为30Hz闪烁光ERG幅值(γ=0.597)。RD眼的fERG异常率最高者为暗适应最大反应ERG b波幅值,异常率为71.3%,视网膜脱离象限野mfERG异常率最高者为P1波幅值,异常率为85.3%。结论:影响RD患者视网膜功能的重要因素是脱离面积和黄斑脱离情况。RD眼对视网膜功能异常反应的mfERG对视网膜功能异常反应的敏感性高于fERG。     

Electrophysiological findings in familial exudative vitreoretinopathy

Three patients in a family with familial exudative vitreoretinopathy (FEVR) were studied especially electrophysiologically. Two cases showed abnormal ERG, namely reduction of amplitude of oscillatory potentials, a and b wave of bright white flash ERG, scotopic and photopic b waves. One case showed reduced Light peak/Dark trough ratio of EOG. Next, the electrophysiological findings of cases with FEVR reported in the literature were analyzed. The result was that FEVR has electrophysiological varieties but a statistical significant relationship between electrophysiological findings and visual acuity. That is, cases with abnormal ERG or EOG have more impaired visual acuity than normal ones. Furthermore, there was a tendency that eyes with abnormal ERG or EOG showed more disturbed color vision and advanced fundus findings than normal ones. These results seemed to be understandable by anatomically characteristic fundus feature of FEVR.presented at the 6th Meeting of the International Society of Genetic Eye Disease and 3rd International Symposium on Retinoblastoma, Amsterdam, the Netherlands, May 1–3, 1986.     

Analysis of photoreceptor function and inner retinal activity in juvenile X-linked retinoschisis

Thirteen retinoschisis males with genotyped XLRS1 gene mutations were examined by electroretinogram (ERG) techniques to determine photoreceptor involvement and ON-pathway and OFF-pathway sites of dysfunction. Parameters R(max) and logS determined by fitting the mathematical model of the activation phase of phototransduction to the scotopic and photopic a-wave responses, were not significantly different from normal. However, the XLRS photopic a-wave amplitudes were significantly lower than normal across all intensities, consistent with defective signaling in the OFF pathway. Long flash (150 ms) ON-OFF photopic responses showed reduced b-wave amplitude but normal d-wave amplitude, giving a reduced b/d ratio of <1.32 Hz photopic flicker ERG fundamental frequency responses showed reduced amplitude and delayed phase, consistent with abnormal signaling by both the ON- and OFF-pathway components. These results indicate that the XLRS1 protein appears not to affect photoreceptor function directly for most XLRS males, and that ERG signaling abnormalities occur in both the ON- and OFF-pathway components that originate in the proximal retina.     

Clinical and electrophysiological findings in three patients with toluene dependency

We recorded the electrophysiological findings of three patients with toluene dependency who developed visual disturbance. In five of six eyes the peak latency of the pattern visual evoked cortical potential was prolonged as compared with that of normal subjects. The amplitude and the latency of the a-wave of the electroretinogram were decreased in five of the six eyes and prolonged in four. Those of the b-wave in patients were less affected than the a-wave: in one of six eyes the amplitude of the b-wave was decreased and in two the b-wave latency was prolonged. The amplitudes of the oscillatory potentials were decreased in three of six eyes. The electroretinogram was investigated in one patient and showed little increase in amplitude during light stimulation, so that a lowered light peak to dark trough ratio was obtained.Our findings suggest that any part of the visual pathway, including the distal part of the retina and retinal pigment epithelium, might be impaired by the chronic inhalation of toluene vapors.Abbreviations EOG Electrooculogram - ERG Electroretinogram - L/D light peak to dark trough ratio - OP Oscillatory potential - VECP Visual evoked cortical potential     

Electrophysiological similarities between two eyes with X-linked recessive retinoschisis

Electroretinograms (ERG) and electro-oculograms (EOG) were studied in 88 eyes of 44 male patients with X-linked recessive retinoschisis. Differences of fundus appearance, ERG, and EOG between the eyes of each patient were analyzed. Fundus abnormalities were symmetrical in 77.3% of the cases. The amplitude of the ERG a-wave was normal in 26.1% and was abnormally low in 73.9%. The amplitude of the b-wave was below normal in all eyes; thus a small b-wave/a-wave ratio, which is characteristic of X-linked recessive retinoschisis, was observed in every case. The light peak to dark trough (LP/DT) ratio of the EOG was normal in 90.8% of the cases. The relative electrophysiological differences between the two eyes were calculated and showed that a-wave amplitude was not different between eyes in 75.0% of the cases; b-wave amplitude was not different in 77.3% of the cases; b-wave/a-wave ratio was symmetrical in 93.2% of the cases; and the LP/DT ratio was consistent between eyes in 86.8% of the cases. These results suggest that in most cases of X-linked recessive retinoschisis the fundus appearance, ERG, and EOG are similarly affected in both eyes of the patient.     

Vigabatrin; its effect on the electrophysiology of vision

Vigabatrin is known to induce visual field defects in approximately one third of patients treated with the drug. It is apparent from electrophysiological studies that the cause of this defect is at retinal level probably as a result of the build up of GABA. Studies of electrophysiological retinal parameters such as the EOG and photopic, scotopic and 30-Hz flicker ERG have revealed changes in Arden Index, photopic a and b wave latency and amplitude, changes in oscillatory potentials, and changes in latency and amplitude of the 30Hz response. However, many of these changes such as the Arden Index, oscillatory potentials, latency and amplitude of photopic b wave appear to be related to current anti-epileptic drug treatment rather than visual field defects. Certain parameters, particularly the amplitude of the 30-Hz flicker response, do appear to correlate with the severity of the field defect. Paediatric patients treated with the drug at age 9 years or below cannot reliably perform visual field perimetry. To identify these patients a special VEP H-Stimulus has been developed to produce separate responses from central and peripheral field stimulation by alternating at slightly separate rates. Forty-five healthy children between ages 3 and 10 years have been used to develop a normal database. This technique has a sensitivity of 75% and a specificity of 87.5% in identifying the field defect and may be used in children with epilepsy from age 3 upwards.     

A comparison of photopic and scotopic electroretinographic changes in early diabetic retinopathy.

Previous studies of early diabetic retinopathy have shown that oscillatory potential (OP) amplitudes are reduced in many diabetic patients. OP amplitude is believed to be a more sensitive indicator of the development of future retinopathy than b-wave amplitude of the scotopic electroretinogram (ERG). Because OPs measured to a bright white flash reflect both rod and cone system activity, it is important to compare OP amplitudes to photopic ERG measures as well as scotopic measures in early diabetic retinopathy. In this study, OPs and ERG responses were measured under photopic and scotopic conditions in a group of diabetic patients. Although OPs were reduced in amplitude in the diabetic group, several other parameters of the scotopic and photopic b-waves were impaired. The results indicate that b-wave activity may indicate retinal changes in early diabetic retinopathy in the same manner as the OPs.     

Correlation of electroretinographic and fluorescein angiographic findings in unilateral central retinal vein obstruction

Background: In central retinal vein obstruction (CRVO), electroretinogram (ERG) abnormalities and extensive retinal capillary dropout (CD) in the fluorescein angiogram (FA) are good indicators of retinal ischemia. We retrospectively studied patients with unilateral CRVO and compared the ERG and FA results Methods: Single white flash ERG, photopic ERG, scotopic ERG and flicker ERG were recordered in 30 cases of unilateral CRVO. We analyzed the correlation between the ERG results and the presence/absence of extensive CD Results: The ERG b/a-wave amplitude ratios, photopic and scotopic b-wave amplitudes, and flicker amplitudes were significantly smaller (P<0.05) in eyes with extensive CD (n=12, 40%), than in eyes without (n=18, 60%). When the photopic or scotopic b-wave amplitudes were normal or supernormal, extensive CD on FA was absent in all eyes. When the b/a-wave ratios were 1.0 or when the b-wave amplitudes with white flash or flicker amplitudes were normal or supernormal, extensive CD was present in less than 32% of eyes Conclusion: These results suggest that the ERG results, especially the b/awave amplitude ratio, are significantly correlated with the presence/absence of CD on FA in CRVO.     

The 52nd ISCEV International Symposium Abstract Issue

Purpose

We have monitored retinal function in patients treated for retinoblastoma (primarily, but not exclusively by intra-arterial chemotherapy infusion) by electroretinography (ERG) recordings for the past 7 years. We here present data from 599 ERG studies of 108 patients, in which a complete ERG protocol including both photopic and scotopic recordings was performed, in justification of our frequent practice of reporting primarily 30-Hz photopic flicker amplitude data.

Methods

Patients referred for treatment of retinoblastoma underwent ERG recordings during examination under anesthesia whenever possible: at baseline and following most treatment sessions. Correlations were calculated for the complete datasets between the four primary amplitude response parameters: photopic single flash b-wave, photopic 30-Hz flicker peak-to-trough, scotopic rod-isolating b-wave, and scotopic maximal flash b-wave.

Results

Using our adaptation of the International Society for Clinical Electrophysiology of Vision-recommended standard ERG protocol, ERG responses of eyes of patients with untreated retinoblastoma or following traditional or intra-arterial treatment for retinoblastoma show very high correlations between 30-Hz flicker amplitude responses and three other standard photopic and scotopic ERG response amplitudes. Reductions in ERG amplitudes seen in these eyes following treatment show no significant difference between retinal dysfunction estimated using rod- or cone-dominated responses.

Conclusion

These observations support the use of photopic response amplitudes (especially in response to 30-Hz flicker) as the primary ERG outcome measure in studies of treated and untreated eyes with retinoblastoma when more complete ERG protocols may be impractical.     

The effects of vigabatrin on electrophysiology and visual fields in epileptics: a controlled study with a discussion of possible mechanisms

Purpose: To compare the visual electrophysiology and visual fields of patients taking vigabatrin to those of a control group of epileptics on other anti-epileptic drugs (AEDs). Methods: Fourteen epileptics treated with vigabatrin and 10 control patients treated with other AEDs underwent ERG and EOG. Goldmann visual fields were performed and analysed using standard software to measure areas contained within I4e isopters. Results: The cone and rod b-waves of the ERG, the oscillatory potential amplitudes and Arden indices were reduced in vigabatrin-treated subjects and the oscillatory potentials delayed. The Arden indices were reduced due to an increased dark trough. The areas contained within the I4e isopter of vigabatrin treated subjects were reduced compared to the control group and these areas correlated well with oscillatory potential amplitudes and b-wave amplitudes in the vigabatrin group only. Conclusions:The use of vigabatrin is associated with a reduction of the ERG cone b-wave amplitude and oscillatory potentials which correlates with visual field loss. The Arden ratio is reduced in subjects taking vigabatrin but may recover after cessation. However, visual loss may persist in the presence of a recovered EOG. These findings suggest further effects of the drug than those mediated by GABA receptors, and support the contention that the cause of the field loss may be at least in part due to retinal effects. Possible mechanisms are discussed.     

Electrophysiological changes after 360 degrees retinotomy and macular translocation for subfoveal choroidal neovascularisation in age related macular degeneration

AIM: To evaluate electrophysiological changes after 360 degrees retinotomy and macular translocation for subfoveal choroidal neovascularisation in patients with age related macular degeneration (AMD). METHODS: A consecutive series of 32 patients suffering from subfoveal choroidal neovascularisation secondary to AMD underwent 360 degrees retinotomy and macular translocation. The ERG served as the main parameter of the study and was recorded 1 day before the translocation surgery and no earlier than 4 weeks after the silicone oil removal. RESULTS: The scotopic ERG amplitudes were significantly reduced after translocation surgery. Depending on the applied flash luminance the mean b-wave amplitude reduction of the scotopic ERG varied between 67% (0.2 cd.s/m2) and 74% (0.03 cd.s/m2). The a-waves and b-waves of the saturating light response decreased significantly by 46% and 59%, respectively. The photopic a-wave and b-wave amplitudes were significantly lower after the translocation surgery resulting in a mean reduction of 27% and 43%, respectively. CONCLUSIONS: Although macular translocation may provide the potential of preserving and even restoring vision in patients with subfoveal choroidal neovascular membranes secondary to AMD the present study indicates that a significant electrophysiological decrease is caused by surgical procedures associated with this technique. Further research is necessary to clarify if certain modifications of the surgical procedure are able to substantially reduce the neuroretinal trauma.     

Abnormalities of the photoreceptor-bipolar cell synapse in a substrain of C57BL/10 mice

PURPOSE: A preferential loss of ERG b-wave was detected in a substrain of C57BL/10 mice. Electroretinographic and histologic techniques were used to investigate this hitherto unknown retinopathy. METHODS: ERGs were obtained from normal and affected C57BL/10. C57BL/6 mice served also as controls. a-Wave and c-wave analyses were performed. Microscopic investigations were done at two different ages. RESULTS: In the scotopic ERG, a severe reduction of the b-wave amplitude could be observed, whereas the a-wave was only moderately attenuated ("negative ERG"). With age, the a-wave amplitude further decreased, but the rate of reduction was comparable to normals. Oscillatory potentials were severely altered, and the photopic ERG was absent. The ERG c-wave was comparable to normal. ERG a-wave analysis also revealed a reduced maximum amplitude, but no significant difference of receptor sensitivity. Light microscopy revealed a thinning of all retinal layers but mostly of the outer plexiform layer. The number of photoreceptor nuclei was reduced by one third. Electron microscopy revealed a profound loss of ribbon-shaped synapses between rod and rod-bipolar cells and severely abnormal ribbons in the case of cones. CONCLUSIONS: The so-called negative ERG was associated with alterations in the synaptic layer between rods and rod bipolars. The absent cone ERG may be due to the altered cone-on-bipolar synapses. The overall thinning of the retina as well as the moderately reduced scotopic a-wave amplitude remain unexplained.     

The function of eye and vision system in children and youth treated with vigabatrin--our own experiences

PURPOSE: The aim of this study is to find the relationship between visual function changes and vigabatrin treatment continuation or discontinuation. MATERIAL AND METHODS: 19 patients (11 males, 8 females) from 8 to 20 years old, treated with vigabatrin because of partial epilepsy, were examined. Best corrected visual acuity, contrast sensitivity and static visual field were done. The examinations were repeated after 1-2 months and after 1-2 years. The longest follow-up was about three years (one girl). In some children from this group additional elctrophysiologic tests (ERG, EOG according to ISCEV standards), were done. RESULTS: Corrected visual acuity was 5/5 in all eyes. Contrast sensitivity was normal and it was not changed in follow-up period. Abnormal visual field was found in more than half of examined patients. The improvement of visual field occurred after vigabatrin discontinuation, but in one patient the improvement occurred although vigabatrin treatment was continued. Most of the patients refused control ERG and EOG, but in first examination decreased or border b-wave amplitude after "flicker 30Hz" was found. CONCLUSIONS: In some patients treated with vigabatrin reversible visual field changes occurred. Multicentre study should be performed, to solve the problem of relationship between visual field changes, vigabatrin and other antiepileptic treatment. All tests in group of epileptic patients are difficult because of poor cooperation.     

Evidence supportive of a functional discrimination between photopic oscillatory potentials as revealed with cone and rod mediated retinopathies

We report on a family where four of the eleven children presented with reduced visual acuities, a red-green deficit at the Farnsworth-Munsel FM 100-hue test, normal appearing fundi and unexpected electroretinographic findings. Light- (photopic) and dark- (scotopic) adapted electroretinograms (ERG) and oscillatory potentials (OPs) were obtained following an accepted standard protocol. The b-wave of their photopic ERG was significantly more attenuated than the a-wave due to the specific abolition of OP4, while the amplitudes of OP2 and OP3 were within the normal range, giving to the b-wave a truncated appearance reminiscent of that seen in congenital stationary night blindness (CSNB) with myopia. Interestingly in the latter condition, which is believed to result from an ON-retinal pathway anomaly, it is OP2 and OP3 which are specifically abolished while OP4 is of normal amplitude thus resulting in an OP response pattern which complements that seen with our patients. Also of interest is the fact that, in our patients, the amplitude of the dark-adapted OP2 was, on average, 240% larger than that measured in light-adaptation while, in normal, a non-significant 14% increase is noted; a finding which is in keeping with other studies reporting supernormal scotopic ERGs in some forms of cone dystrophies. Based on the photopic OP response pattern, our patients represent the electrophysiological complement of patients affected with CSNB. Interestingly their symptoms are also complementary, a finding which could support a functional discrimination between the photopic OPs. This revised version was published online in July 2006 with corrections to the Cover Date.