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Purpose: The photopic negative response (PhNR) is an electrophysiological method that provides retinal ganglion cell function assessment using full-field stimulation that does not require clear optics or refractive correction. The purpose of this study was to assess ganglion cell function by PhNR in affected and asymptomatic carriers from Brazilian families with LHON. Methods: Individuals either under suspicion or previously diagnosed with LHON and their family members were invited to participate in this cross-sectional study. Screening for the most frequent LHON mtDNA mutations was performed. Visual acuity, color discrimination, visual fields, pattern-reversal visual evoked potentials (PRVEP), full-field electroretinography and PhNR were tested. A control group of healthy subjects was included. Full-field ERG PhNR were recorded using red (640 nm) flashes at 1 cd.s/m2, on blue (470 nm) rod saturating background. PhNR amplitude (μV) was measured using baseline-to-trough (BT). Optical coherence tomography scans of both the retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) were measured. PhNR amplitudes among affected, carriers and controls were compared by Kruskal-Wallis test followed by post-hoc Dunn test. The associations between PhNR amplitude and OCT parameters were analyzed by Spearman rank correlation. Results: Participants were 24 LHON affected patients (23 males, mean age=30.5 ± 11.4 yrs) from 19 families with the following genotype: m.11778G>A [ N = 15 (62%), 14 males]; m.14484T>C [ N = 5 (21%), all males] and m.3460G>A [ N = 4 (17%), all males] and 14 carriers [13 females, mean age: 43.2 ± 13.3 yrs; m.11778G>A (N = 11); m.3460G>A (N = 2) and m.14484T>C (N = 1)]. Controls were eight females and seven males (mean age: 32.6 ± 11.5 yrs). PhNR amplitudes were significantly reduced (p = 0.0001) in LHON affected (−5.96 ± 3.37 μV) compared to carriers (−16.53 ± 3.40 μV) and controls (−23.91 ± 4.83; p < 0.0001) and in carriers compared to controls (p = 0.01). A significant negative correlation was found between PhNR amplitude and total macular ganglion cell thickness (r = −0.62, p < 0.05). Severe abnormalities in color discrimination, visual fields and PRVEPs were found in affected and subclinical abnormalities in carriers. Conclusions: In this cohort of Brazilian families with LHON the photopic negative response was severely reduced in affected patients and mildly reduced in asymptomatic carriers suggesting possible subclinical abnormalities in the latter. These findings were similar among pathogenic mutations. [ABSTRACT FROM AUTHOR]

Copyright of Arquivos Brasileiros de Oftalmologia is the property of Arquivos Brasileiros de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Arquivos Brasileiros de Oftalmologia is the property of Arquivos Brasileiros de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Arquivos Brasileiros de Oftalmologia is the property of Arquivos Brasileiros de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Arquivos Brasileiros de Oftalmologia is the property of Arquivos Brasileiros de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Arquivos Brasileiros de Oftalmologia is the property of Arquivos Brasileiros de Oftalmologia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purpose: To determine visual impairment due to optic pathway tumors in children unable to perform recognition acuity tests., Methods: Grating visual acuity scores, in logMAR, were obtained by sweep visually evoked potentials (SVEP) in children with optic pathway tumors. The binocular grating visual acuity deficit was calculated by comparison with age-based norms and then assigned to categories of visual impairment as mild (from 0.10 to 0.39 logMAR), moderate (from 0.40 to 0.79 logMAR), or severe (≥0.80 logMAR). Interocular differences were calculated by subtraction and considered increased if >0.10 logMAR., Results: The participants were 25 children (13 boys; mean ± SD age, 35.1 ± 25.9 months; median age, 32.0 months) with optic pathway tumors (24 gliomas and 1 embryonal tumor), mostly located at the hypothalamic-chiasmatic transition (n=21; 84.0%) with visual abnormalities reported by parents (n=17; 68.0%). The mean grating acuity deficit was 0.60 ± 0.36 logMAR (median, 0.56 logMAR). Visual impairment was detected in all cases and was classified as mild in 10 (40.0%), moderate in 8 (32.0%), and severe in 7 (28.0%) children, along with increased interocular differences (>0.1 logMAR) (n=16; 64.0%). The remarkable ophthalmological abnormalities were nystagmus (n=17; 68.0%), optic disc cupping and/or pallor (n=13; 52.0%), strabismus (n=12; 48.0%), and poor visual behavior (n=9; 36.0%)., Conclusion: In children with optic pathway tumors who were unable to perform recognition acuity tests, it was possible to quantify visual impairment by sweep-visually evoked potentials and to evaluate interocular differences in acuity. The severity of age-based grating visual acuity deficit and interocular differences was in accordance with ophthalmological abnormalities and neuroimaging results. Grating visual acuity deficit is useful for characterizing visual status in children with optic pathway tumors and for supporting neuro-oncologic management.

Purpose:: To analyze the clinical features, visual acuity, and full-field electroretinogram (ERG) findings of 15 patients with the neuronal ceroid lipofuscinosis (NCL) phenotype and to establish the role of ERG testing in NCL diagnosis., Methods:: The medical records of five patients with infantile NCL, five with Jansky-Bielschowsky disease, and five with juvenile NCL who underwent full-field ERG testing were retrospectively analyzed., Results:: Progressive vision loss was the initial symptom in 66.7% of patients and was isolated or associated with ataxia, epilepsy, and neurodevelopmental involution. Epilepsy was present in 93.3% of patients, of whom 86.6% presented with neurodevelopmental involution. Fundus findings ranged from normal to pigmentary/atrophic abnormalities. Cone-rod, rod-cone, and both types of dysfunction were observed in six, one, and eight patients, respectively., Conclusion:: In our study, all patients with the NCL phenotype had abnormal ERG findings, and the majority exhibited both cone-rod and rod-cone dysfunction. We conclude that ERG is a valuable tool for the characterization of visual dysfunction in patients with the NCL phenotype and is useful for diagnosis.

Purpose: The photopic negative response (PhNR) is a slow negative component of a flash photopic full-field ERG that has been shown to be specific for retinal ganglion cell (RGC) activity. Direct evaluation of RGC function is desirable in patients with Leber's hereditary optic neuropathy (LHON) in which the loss of central acuity can make it difficult to monitor patients with standard metrics. The purpose of this study was to evaluate the use of PhNR as an objective noninvasive clinical metric in LHON., Methods: Full-field photopic ERG recordings were collected in subjects with the mt.11778G>A/ND4 LHON mutation using a red on blue stimulus. The PhNR was identified using a computer-based automated detection system, and data were manually examined to remove movement artifacts., Results: The PhNR amplitude was compared between controls (n = 13), carriers (n = 17), and affected (n = 6). Mean PhNR amplitude decreased significantly across groups (P < 0.0001). Post hoc Tukey's test revealed a significant decrease in PhNR amplitude between carriers and controls (P < 0.05) and between carriers and affected (P < 0.01)., Conclusions: We are able to demonstrate that the PhNR amplitude is significantly decreased in patients affected by LHON compared to carriers in a well-described pedigree. Surprisingly, there was also a decrease in PhNR in carriers, suggesting potential subclinical RGC dysfunction in some carriers. This is important in patients affected with LHON who typically have a dense central scotoma. The PhNR may be a useful objective outcome measure for future clinical trials.

Purpose:: To investigate the contributions of transient pattern-reversal visual evoked potentials in the diagnosis of ocular malingering at a Brazilian university hospital., Methods:: Adult patients with suspected malingering in one or both eyes were referred for visual evoked potential testing. Data from patients' medical records were reviewed and analyzed retrospectively. Data analysis included the distance optotype visual acuity based on a ETDRS retro-illuminated chart and the transient pattern-reversal visual evoked potential parameters of latency (milliseconds) and amplitude (microvolts) for the P100 component, using checkerboards with visual subtenses of 15' and 60'. Motivations for malingering were noted., Results:: The 20 subjects included 11 (55%) women. Patient ages ranged from 21 to 61 years (mean= 45.05 ± 11.76 years; median= 49 years). In 8 patients (6 women), both eyes exhibited reduced visual acuity with normal pattern-reversal visually evoked potential parameters (pure malingerers). The remaining 12 patients (7 men) exhibited reduced vision in only 1 eye, with simulated reduced vision in the contralateral eye (exaggerators). Financial motivation was noted in 18 patients (9 men)., Conclusion:: Normal pattern-reversal visually evoked potential parameters with suspected ocular malingering were observed in a 20 patient cohort. This electrophysiological technique appeared to be useful as a measure of visual pathway integrity in this specific population.

Purpose: To determine normative values for pattern-reversal electroretinogram (PERG) in healthy adult volunteers according to the standard protocol recommended by the International Society for Clinical Electrophysiology of Vision-ISCEV., Methods: Participants were 30 healthy volunteers (15 males) with ages from 18 to 61 years (mean= 30.8 +/- 8.7 yrs.; median= 29.5 yrs). Inclusion criteria were: visual acuity of 0.0 logMAR (20/20 Snellen) in each eye, absence of visual complaints, absence of media opacities, negative history for ocular or neurological diseases, negative family history for ocular and informed consent. PERG was recorded from each eye in a darkened room at 1 m from a high resolution display monitor. Stimuli were monochromatic checkerboards subtending 60, 15 e 7.5 minutes of visual angle reversing at 1.9 Hz. Responses were obtained from modified disposable fiber electrodes developed at UNIFESP. Latency (ms) for N35, P50 and N95 components as well as peak-to-peak amplitudes (microV) for N35-P50 and P50-N95 were determined. For normative values only one randomly chosen eye was included. Normal limits were calculated as 97.5% percentiles for latency and 2.5% percentile for amplitudes for each stimulus size., Results: Normal limits for N35, P50 and N95 latencies for 60', 15' and 7.5' stimuli were respectively: N35 - 40.1; 39.9 and 41.3 ms; P50 - 60.5; 64.4 and 65.6 ms and N95 - 103.4; 104.6 and 104.6 ms. For amplitude the normative values for N35-P50 and P50-N95 for 60', 15' and 7.5' were respectively: N35-P50 - 1.7; 1.6 and 0.9 microV; P50-N95 - 3.8; 2.8 and 1.5 microV. No gender differences were found either for latency or for amplitude in the three stimulus sizes. There was no correlation between PERG latency and amplitude with age, except for P50 amplitude for stimulus 15'(r=0.39; P=0.035)., Conclusions: Normative values were determined for PERG parameters of amplitude and latency for three stimulus sizes. These parameters are important for evaluating the normal functioning of retinal ganglion cells and the macula. The normative values obtained in this study are comparable to previous studies in the literature.

Purpose: To evaluate eye care services from the user's perspective in a low income population from the east zone of the city of São Paulo--Brazil., Methods: A household survey was performed using cluster sampling in three low income districts of the city of São Paulo--Brazil. From July/2004 to January/2005, 1055 interviews with an adult household representative were carried and an eye care system responsiveness questionnaire was administered through individual interview., Results: 71.56% of the participants were women. Respondents' age ranged from 18 to 92 years (41.42 +/- 15.67 years). Regarding schooling, 525 (49.77%) had four years or less; 489 (46.35%) between five and eleven years, 40 (3.79%) had eleven or more years of study. Eye care services need was reported as 712 (67.49%) declaring themselves or someone else of the household needing and obtaining services and 135 (12.80%) had never needed eye care. The most frequently cited barriers to obtain the eye care service by respondents was cost (77.29%), followed by unsuccessful attempt to obtain eye care (42.21%). General satisfaction for the criteria contained in the questionnaire was 63.37%. In the 36.63% dissatisfied respondents, the most frequently cited claim was the amount of time waited before consultation., Conclusion: The main barriers to obtain eye care services were cost of medical appointment and lack of access to the services. 63.37% of the individuals in need who had received eye care in last 12 months were satisfied with the service provided.

Purpose: To evaluate macular function by focal electroretinography and fluorescein angiography (FA) in patients with neovascular age-related macular degeneration submitted to verteporfin photodynamic therapy (VPT)., Methods: Prospective study involving 22 patients with age-related macular degeneration and predominantly classic subfoveal neovascular membrane, in non consecutive series, treated with VPT and followed for 12 months. They had their best corrected visual acuity measured by ETDRS chart, changes of lesion measured by fluorescein angiography and cone function assessed by focal electroretinography at baseline and each 3-month follow-up., Results: All 22 patients completed the scheduled follow-up. After a mean of 3.5 sessions of treatment per patient, the mean visual acuity variation was not significant at the end of study. Eleven patients showed variation > or =1 line. 86% of patients achieved stabilization of lesion leakage at the end of the study. Focal electroretinography showed a mean of 194.88 nV in amplitude and 29.19 ms in latency and did not present a significant variation during treatment., Conclusions: There were no significant differences in focal electroretinography amplitudes and latencies after a 9-month period. Visual acuity did not show important variations during the 12 months. The decrease of lesion size showed a significant difference at 12 months with negative correlation between the amplitude of focal electroretinography and best corrected visual acuity.

Purpose: To determine age norms for grating visual acuity and interocular acuity differences measured by the sweep-visually evoked potentials (VEP) technique in the first three years of life., Methods: Monocular grating visual acuity was measured using the sweep-VEP in 67 healthy normal infants and children in the first 36 months of life., Results: Sweep-VEP grating acuity ranged from 0.80 logMAR (20/125 Snellen equivalent) in the first month of life to 0.06 logMAR (20/20 Snellen equivalent) at 36 months of age. Lower normal limits (95th percentile limit) ranged from 0.95 logMAR (20/180) to 0.12 logMAR (20/25) with a progression of approximately 3 octaves in the first 36 months of age. The largest acceptable interocular acuity difference for clinical purposes was 0.10 logMAR., Conclusions: Age norms for grating acuity along with interocular acuity differences were determined using the sweep-VEP technique. These norms should be incorporated in clinical practice for precise diagnosis of visual status in infants and preverbal children.

Introduction: Multiple sclerosis is an idiopathic demyelinating disease that may affect the optic nerve leading to acute unilateral visual loss, which could be observed by means of evoked visual potential (VEP). This exam is much valued for studying prechiasmatic visual paths in multiple sclerosis., Purpose: To analyze the findings of pattern reversal VEP in patients with prior diagnosis of multiple sclerosis and to compare them to visual acuity., Methods: Twenty-four patients with a definite diagnosis of multiple sclerosis were submitted to pattern reversal VEP from October 2001 to March 2007., Results: In P100 component response, at 15' and 1 degree stimuli, 19 eyes presented latency response up to 115 ms in both stimuli, which coincided in 6 eyes with latency response between 116 and 135 ms; 11 eyes had a latency response higher than 135 ms, and four eyes did not respond to stimuli. Correlating visual acuity with P100 latency for 15', in Pearson r correlation, r=0.85 with p=0.000000123, and for 1 degree, r = 0.87 with p=0.0000000338., Conclusion: Approximately 60.4% of patients presented abnormalities. However, the correlation between the P100 latency (15' and 1 degree) and visual acuity was statistically significant; therefore the better the visual acuity, the better the response to stimuli of the pattern-reversal VEP.

Purpose: To investigate clinical, anatomic and electroretinographic changes in eyes that suffered blunt ocular trauma with commotio retinae., Methods: Patients who presented commotio retinae after unilateral blunt ocular trauma less then 72 hours before were submitted to visual acuity testing, biomicroscopy, binocular indirect ophthalmoscopy, fluorescein angiography, optical coherence tomography and full-field electroretinography. Full-field ERG was repeated after 15 and 30 days., Results: Sixteen patients were included in the study. On the first examination there was a statistically significant difference between affected and fellow eye in all response amplitudes, without b/a ratio alteration, and a delay in single-flash cone response and 30-Hz flicker implicit time. On the second examination, the difference between the eyes remained for oscillatory potentials, but disappeared on the last examination. In fluorescein angiography, all patients presented mottled hyperfluorescence and hypofluorescent areas, due to alterations in the pigment barrier. On optical coherence tomography, we found optically empty spaces at the site of the lesion., Conclusion: Found changes suggested photoreceptor and ganglion cells, but not Müller cell functional alterations, as well as pigment mobilization. These changes disappeared 30 days after the trauma.

Purpose: Usher's syndrome (US) is a group of genetically distinct autosomal conditions, characterized by sensorineural hearing loss accompanied by a retinal dystrophy indistinguishable from retinitis pigmentosa (RP). The purpose of this study was to analyze full-field electroretinography (ERG) and visual acuity (VA) among patients with type I and II Usher's syndrome., Methods: Electroretinography responses and visual acuity were studied in 22 patients (mean age at test = 26.8+/-16.8 years). Seventeen patients had SU type I and 5 patients were diagnosed as Usher's syndrome type II., Results: Mean visual acuity was 0.9 logMAR (20/160, Snellen equivalent) for patients with Usher's syndrome type I and 0.4 logMAR (20/50, Snellen equivalent) for patients with Usher's syndrome type II. Scotopic rod and maximal responses were non-detectable in both groups. Mean amplitude for oscillatory potentials was 14.5 microV+/-6.1 in Usher's syndrome type I and 12.6 microV+/-5.2 in Usher's syndrome type II. Cone responses were non-detectable in 95% of the patients with Usher's syndrome I and in 100% of patients with Usher's syndrome II. Mean amplitude for 30 Hz flicker photopic cone response was 3.1 microV+/-4.1 for Usher's syndrome type I and 1.0 microV+/-0.6 for type II with mean implicit time of 34.0 ms+/-6.2 (US I) and 35.8 ms+/-3.1 (type II)., Conclusions: Visual acuity was relatively preserved in both groups, however Usher's syndrome II group showed better visual acuity results. Electroretinography findings were severely reduced in both groups, with most patients showing non-detectable rod and cone responses.