Search

To investigate the diagnostic contribution of grating visual acuity (GVA) measured by sweep pattern-reversal visually evoked potentials (SPRVEP) in unexplained visual loss (UVL).This case-control study included adult patients under suspicion of UVL referred to SPRVEP and transient pattern-reversal visually evoked potentials (TPRVEP) testing. Optotype visual acuity (OVA) was measured by ETDRS 4-meter chart and GVA by SPRVEP. UVL patients were assigned into three distinctive categories, according to the presence of ocular disease, motivation, and electrophysiological evaluation, as follows: exaggerators, malingerers, and psychogenic. Healthy controls and patients with organic visual loss were also tested. Receiver operating characteristic (ROC) curve was constructed to evaluate the diagnostic performance of GVA and TPRVEP parameters.A total of 76 patients with UVL were analyzed: 60 (79.0%) exaggerators, 11 (14.4%) malingerers, and 5 (6.6%) psychogenic. Controls were 49 subjects evaluated for TPRVEP and 28 subjects for SPRVEP. There were 13 patients with organic visual loss enrolled. Mean difference between OVA and GVA was 1.19±0.67 (median=0.84; 95% CI: 1.04 to 1.34) in UVL and 0.14 ±0.09 (median= 0.14; 95% CI: 0.08 to 0.20) in organic visual loss. The area under the ROC curve (AUC) of GVA to distinguish UVL from healthy controls was 0.998 with a cutoff of 0.09 logMAR showing specificity of 100% and sensitivity of 96.0%.GVA measured by SPRVEP had good diagnostic validity to discriminate patients with unexplained visual loss from healthy controls and patients with organic visual loss, demonstrating its contribution to the diagnosis of this condition.

Background/AimsWe analysed the ability of B-scan ultrasound, ocular electrophysiology testing and videoendoscopic examination for predicting visual prognosis in Boston Type 1 keratoprosthesis (KPro-1) candidates. Indirect anatomical and electrophysiological findings and results from direct endoscopic evaluations were correlated with postoperative functional data.MethodsIn this prospective and interventional study, we included 13 individuals who had previously been indicated for Kpro-1 surgery. All subjects underwent preoperative screening, including ophthalmic evaluation, B-scan ultrasound, electrophysiological testing, and perioperative intraocular videoendoscopic evaluation (VE). B-scan ultrasound, electrophysiological testing, and VE evaluation results were categorised as favourable or unfavourable predictors of postoperative functional results according to predefined criteria. The predictability values of B-scan ultrasound, electrophysiological testing, and VE prognostication were calculated based on the visual acuity level achieved.ResultsAll surgeries and perioperative VEs were uneventful. Preoperative best-corrected visual acuity (BCVA) ranged from light perception to counting fingers. The 1-year postoperative BCVA was better than 20/200 (satisfactory visual acuity result) in 10 eyes (76.9%) and 20/40 or better in 5 eyes (38.5%). B-scan ultrasound presented a positive predictive value (PPV) of 85.7% for satisfactory postoperative visual acuity, electroretinography showed a PPV of 66.7%, and visual evoked potential presented a PPV of 66.7%. The perioperative VE PPV of a negative finding for satisfactory visual acuity was 100%.ConclusionsFundoscopic visualisation by intraocular VE is a minimally invasive procedure that can be used to predict functional outcomes in keratoprosthesis candidates. This technique demonstrated better prognostication in keratoprosthesis candidates than B-scan ultrasound and electrophysiological testing.

To determine normative values, intra- and inter-session variability for a range of parameters derived from the photopic negative response (PhNR) using a handheld mini-Ganzfeld stimulator in healthy normal adults. Light-adapted flash full-field electroretinograms (ERGs) were recorded from healthy individuals with no visual complaints, visual acuity equal to or better than 0.0 logMAR (20/20 Snellen), and negative family history for visual diseases. ERGs were recorded from both eyes using a DTL® type fiber electrode after dilation of the pupils with instillation of 1 drop of tropicamide eye drops (1%). The full-field PhNR stimulus conditions were produced by a LED-based ColorBurst™ (Diagnosys LLC, Lowell, MA, USA) handheld stimulator. Red flashes of 1, 5 and 7 cd.s/m2 on a blue background of 10 cd/m2 were presented. A-wave, b-wave and PhNR amplitude (determined by both baseline to trough-BT and peak to trough-PT) and peak times were analyzed. Normal limits were determined as 5% percentile for amplitudes and 95% percentile for latencies. Intra- and inter-session variability were assessed with Wilcoxon signed-rank test, intraclass correlation coefficient (ICC) and the coefficient of variability (COV). Normative limits for PhNR amplitude (µV) using 1, 5 and 7 cd.s./m2 stimuli were, respectively: 20.81; 18.06 and 19.60 for BT and 69.11; 77.98; 76.51 for PT. Peak times (ms) normative limits for 1, 5 and 7 cd.s/m2 intensities were, respectively, 65.98; 78.20 and 77.96. Overall, intra-session variability assessed by coefficients of variation ranged from 1.35 to 10.28%. Inter-session variability disclosed significant intraclass correlation values for all PhNR parameters only for 1 cd.s/m2 stimuli. The normative values provided by this study are clinically helpful in the diagnosis of inner retinal disorders, especially those affecting retinal ganglion cells such as glaucoma and other optic neuropathies. Further studies, including a larger sample with variable age range would extend the validity of the current results.

Purpose: The purpose of this study was to assess visual function by visually evoked potentials in adults with orbital and other primary brain tumors affecting the optic pathway. Methods: In this retrospective case–control series, patients with orbital (intraconal and extraconal) or midline/chiasmatic tumors were included. Visually evoked potentials using pattern-reversal visually evoked potential and flash visually evoked potential stimuli were performed according to the international standards. Outcome measures were visually evoked potential parameters of amplitude (µV) and peak times (ms) measured both for the P100 component (pattern-reversal visually evoked potentials) and the N2P2 complex (flash visually evoked potential). Individual results were also compared with gender-based normative values. Results: A group of 21 adult patients (17 females) and age- and sex-matched controls were evaluated. Tumor location was intraconal (6 meningiomas, 3 hemangiomas, 1 glioma), extraconal (6 meningiomas), and midline (3 pituitary adenomas, 2 hypothalamic/chiasmatic low-grade gliomas). Abnormal fundus (76%), abnormal pupillary reflexes (71%), reduced visual acuity (62%), strabismus (48%), and proptosis (38%) were present. Visually evoked potential abnormalities were found in at least one eye of all cases. Affected eyes had significantly reduced amplitudes and prolonged peak times for pattern-reversal visually evoked potentials ( p Conclusion: Visually evoked potential abnormalities were found in all adult patients with orbital and other intracranial primary tumors, even in eyes with normal exam and good visual acuity. Visually evoked potential can be used as a non-invasive ancillary test to characterize and monitor visual function in subjects with these neoplastic lesions.

Objectives:To determine whether optic disc hemorrhages (ODH) and cotton wool spots (CWS) at presentation are associated with worse visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH).Methods:Retrospective institutional review of 100 eyes of 50 consecutive pediatric IIH patients (aged 16 years or less) who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardized manner. Visual function was assessed using visual acuity (VA) and visual field grade (VFG).Results:At least one ODH was found in 41% of eyes, at least one CWS was found in 27% of eyes, and 20% of eyes had both ODH and CWS. At presentation, Frisén grade was associated with the presence of CWS (p = 0.013) and showed no association with ODH (p = 0.060). When controlling for Frisén grade, ODH and CWS were not associated with worse VA or VFG at final follow-up. Severe ODH were associated with worse VA and VFG at presentation (p < 0.03), but not at final follow-up. Severe CWS at presentation was strongly associated with a worse Humphrey mean deviation of 5.0 dB (95% confidence interval 1.6–8.3) at final follow-up (p = 0.002).Conclusion:When controlling for the severity of papilledema, ODH do not provide any additional prognostic value in pediatric IIH patients. Frisén grade and severe CWS at presentation were independently associated with worse visual outcomes at the final follow-up.

Purpose To measure the central corneal thickness (CCT) using anterior segment optical coherence tomography (AS-OCT) in older adults with and without pterygium from the Brazilian Amazon Region Eye Survey (BARES). Methods BARES is a population-based epidemiological cross-sectional study conducted in Parintins city. Participants were residents ≥45 years of age identified through a door-to-door interview. Eligible participants were invited for a comprehensive eye exam. Pterygium occurrence and severity were assessed by ophthalmologists through slit-lamp examination considering its location (nasal or/and temporal) and severity (lesion with extension Results A total of 671 subjects, 533 (79.4%) with pterygium in at least one eye and 138 (20.6%) without pterygium in either eye, were examined. The mean CCT evaluated by multiple linear regression and adjusted for demographic variables and pterygium severity was 521 ± 34 μm (median = 521; range = 304–665). Decreased CCT was significantly associated with age and pterygium severity. Individuals aged 65–74 years had CCT 7 μm thinner than those aged 45–54 years ( p = 0.044), individuals aged 75 years and older had CCT 15 μm thinner than those aged 45–54 years ( p = 0.001), and eyes with severe pterygium had CCT 33 μm thinner than eyes without pterygium ( p Conclusions The CCT analysis in this population-based sample shows that a thinner cornea is associated with pterygium severity and older age.

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.

To report a case of a child with strabismus and delayed discovery of a metallic intraocular foreign body with good recovery of visual acuity and stereopsis with 36-month follow-up. A 4-year-old girl was evaluated due to exotropia of right eye initiated 9 months before with progressive worsening. Visual acuity was 1.00 logMAR (20/200) in the right eye and 0.00 logMAR (20/20) in the left eye. Anterior segment evidenced a small paracentral corneal leukoma, posterior synechia and mild lens opacity in the temporal quadrant only in the right eye. Fundus examination in the right eye identified the presence of an intraocular foreign body, with appearance of metallic components surrounded by retinal pigmented endothelial cells atrophy. Full-field electroretinography (ERG) showed reduced amplitudes and delayed implicit times for both rods and cones in the affected eye. All tests were normal in the fellow eye. Pars plana vitrectomy was promptly performed in the right eye, followed by phacoemulsification with intraocular lens implantation 4 months later due to worsening of the lens opacification. The full-field ERG was repeated after the surgical procedures. The ERG showed mild worsening of all responses in the right eye. After 36 months of follow-up, visual acuity was 0.20 logMAR (20/32) with improvement of the ocular misalignment and with 60 s of arc stereopsis with ERG responses unchanged. In this young girl perforating ocular trauma with metallic material was lately diagnosed with strabismus as a sign of alert. Prompt surgical intervention and proper management were essential to provide reasonable visual function including some degree of stereopsis, even though retinal dysfunction characterized by ERG was persistent.

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. RESUMO Objetivo: Determinar o grau de deficiência visual em crianças com tumores da via óptica incapazes de informar a acuidade visual de reconhecimento. Método: A acuidade visual de grades, em logMAR, foi estimada por potenciais visuais evocados de varredura em crianças com tumores das vias ópticas. O déficit da acuidade visual de grades binocular foi calculado em relação ao valor mediano normativo esperado para a idade e a deficiência visual, classificada como leve (0,10 a 0,39 logMAR), moderada (0,40 a 0,79 logMAR) ou grave (≥0,80 logMAR). Diferenças inter-oculares foram calculadas por subtração e consideradas aumentadas se >0,10 logMAR. Resultados: Foram avaliadas 25 crianças (13 meninos; média de idade ± DP=35,1± 25,9 meses; mediana=32,0 meses) com tumores da via óptica (24 gliomas e 1 tumor embrionário) localizados particularmente na transição hipotalâmico-quiasmática (n=21; 84,0%) e com anormalidades visuais detectadas pelos pais (n=17; 68,0%). A média do déficit da acuidade de grades foi 0,60 ± 0,36 logMAR (mediana=0,56 logMAR). Observou-se deficiência visual leve em 10 (40,0%), moderada em 8 (32,0%) e grave em 7 (28,0%), além de aumento da diferença interocular da acuidade visual (n=16; 64,0%). As principais alterações oftalmológicas encontradas foram: nistagmo (n=17; 68,0%), aumento da escavação do disco óptico e/ou palidez (n=13; 52,0%), estrabismo (n=12; 48,0%) e comportamento visual pobre (n=9; 36,0%). Conclusão: Em crianças com tumor da via óptica e incapazes de responder aos testes de acuidade visual de reconhecimento, foi possível quantificar deficiência visual por meio dos potenciais visuais evocados de varredura e avaliar a diferença interocular da acuidade visual de grades. A gravidade do déficit da acuidade visual de grades relacionado à idade e a diferença interocular da acuidade visual de grades foram congruentes com alterações oftalmológicas e neuroimagem. O déficit da acuidade visual de grades foi útil à caracterização do estado visual em crianças com tumores da via óptica e ao embasamento da assistência neuro-oncológica.

Purpose: The Bayley Scales of Infant and Toddler Development-Third Edition (Bayley-III) is a tool for measuring the developmental status of children, including cognitive and motor functioning, in the first three years of life. This study aims to evaluate the correlation between grating visual acuity and visual functionality in healthy children using the Bayley-III. Methods: Binocular grating visual acuity was measured using Teller Acuity Cards followed by the Bayley-III in healthy children aged 1-42 months. Visual acuity (logMAR) and Bayley-III scores for both cognitive and motor (gross and fine) skills were compared. Results: Forty children (20 boys) aged 1.2-42.1 months were tested. Their mean visual acuity was 0.39 ± 0.27 logMAR, which was within the normal age limits for all children. There was a strong and significant negative correlation between visual acuity and age (r=-0.83, p

Background/aimsIt remains unclear whether the presence of optic disc haemorrhages (ODH) or cotton wool spots (CWS) at presentation in patients with papilloedema from idiopathic intracranial hypertension (IIH) has prognostic value. The aim of this study was to determine if optic disc appearance at presentation predicts visual outcome in patients with IIH.MethodsRetrospective study of 708 eyes of 360 consecutive patients with IIH who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardised manner. Visual function was assessed using Snellen converted to logMAR visual acuity, Humphrey mean deviation and visual field grade.ResultsAt least one ODH was found in 201 (28.4%) eyes, at least one CWS was found in 101 (14.3%) eyes and 88 eyes had both ODH and CWS (12.4%). At presentation, Frisén grade was associated with the presence and severity of ODH and CWS (pConclusions and relevanceODH and CWS at baseline are not independent predictors of final visual function in IIH when controlling for the severity of papilloedema.

Purpose To investigate the prevalence and causes of near vision impairment (NVI) in a population of older adults from the Brazilian Amazon Region. Design Population-based cross-sectional study. Methods Cluster sampling was used in randomly selecting subjects 45 years of age and older from urban and rural areas of Parintins city, Brazil. Participants underwent ophthalmic examination, including uncorrected (UCNVA), presenting (PNVA), and best-corrected near visual acuity (BCNVA) from each eye; biomicroscopy; funduscopy; and subjective refraction, including testing with additional lenses for near vision optical correction. A principal cause for NVI was assigned by the ophthalmologist and presbyopia was defined as UCNVA ≤ 20/40 changing to > 20/40 with BCNVA. Free-of-charge glasses were provided for those in need. Results A total of 2384 subjects were enumerated and 2025 had reliable NVA measurements from both eyes. The prevalence of NVI in the better-seeing eye was 96.5% with UCNVA, decreasing to 81.1% with PNVA and to 20.5% with BCNVA. Presbyopia was the principal cause of NVI in 71.8%, followed by cataract (16.5%) and pterygium (2.5%), and was associated with younger age and high schooling. Glasses for near vision were prescribed and provided to 1414 (69.8%) participants. Conclusions A high prevalence of NVI was detected even in those wearing glasses for near. Prescription and provision of low-cost reading glasses should be considered by Brazilian health authorities to address this easily and promptly correctable form of vision impairment.

Purpose To illustrate the natural history of Leber's hereditary optic neuropathy (LHON). Design Prospective observational case series. Participants The Soave-Brazil pedigree of m.11778G>A/ND4 mitochondrial DNA LHON mutation. Methods A prospectively acquired database of the Soave-Brazil pedigree was reviewed. Data from 285 individuals were included in the database over a 15-year period. The pedigree was reviewed for unaffected mutation carriers who converted to affected status, 6 patients with LHON were identified. The medical records were reviewed 1 year preconversion to 1 year postconversion for visual acuity (logarithm of the minimum angle of resolution [logMAR]), Humphrey Visual Field (HVF) mean deviation (MD), and retinal nerve fiber layer (RNFL) thickness, as measured by Cirrus (Carl Zeiss, Oberkochen, Germany) optic coherence tomography (OCT). The RNFL thickness values were normalized for age. Visual acuity, HVF, and processed RNFL data from each of the 12 eyes were then sorted into 2-month time periods relative to the date of conversion, within which they were averaged. Main Outcome Measures The main outcome measures were visual acuity, HVF MD, and RNFL thickness. Results Decreased visual acuity preceded conversion by up to 2 months and then declined up to 8 months postconversion. Decrease in HVF MD occurred at least 4 months preceding conversion, after which values decreased until plateau at 6 to 8 months. Average RNFL thickness was above normal baseline thickness in all 4 quadrants as measured by OCT at the time of conversion. Increase in RNFL thickness preceded conversion as early as 4 to 6 months, peaked at conversion, and decreased until individual plateaus. The temporal quadrant was first to be involved, then the inferior and superior quadrants, and the nasal quadrant showed the latest and least changes. Conclusions Subclinical changes preceded the date of conversion and may reflect the complicated nature of identifying the date of conversion in LHON. Early increases in RNFL preceding conversion suggest that structural changes precede clinically significant vision loss. Asynchronous quadrant involvement supports a previously published mathematical model. The natural history of LHON is not a subacute process, as previously believed, but progresses more slowly, taking up to 8 months to plateau.

To describe the study design, operational strategies, procedures, and baseline characteristics of the Brazilian Amazon Region Eye Survey (BARES), a population-based survey of the prevalence and causes of distance and near visual impairment and blindness in older adults residing in the city of Parintins.Cluster sampling, based on geographically defined census sectors, was used for cross-sectional random sampling of persons 45 years and older from urban and rural areas. Subjects were enumerated through a door-to-door survey and invited for measurement of uncorrected, presenting and best-corrected visual acuity and an ocular examination.Of 9931 residents (5878 urban and 4053 rural), 2384 individuals (1410 urban and 974 rural) were eligible and 2041 (1180 urban and 861 rural) had a clinical examination (response rate 85.6%). The majority of participants were female (1041, 51.0%); the average age was 59.9 ± 11.1 years (60.2 ± 11.2 years for urban and 59.4 ± 11.1 years for rural); 1360 (66.6%) had primary schooling or less (58.1% in urban and 78.4% in rural) and 57.8% were resident in urban areas. The age distribution between sexes was similar (p = 0.178). Both sex and age distributions of the sample were comparable to that of the Brazilian Amazon Region population.The BARES cohort will provide information about the prevalence and causes of near and distance vision in this underprivileged and remote population in Brazil.

PURPOSE To investigate cataract surgery prevalence, complications, visual outcomes, and coverage in a population of older adults from the Brazilian Amazon region. DESIGN Population-based cross-sectional study. METHODS Individuals ≥45 years of age from urban and rural areas of Parintins City, Brazil, were identified by cluster random sampling. Participants underwent ophthalmic examination, including uncorrected visual acuity, acuity with presenting correction (APC), and best-corrected visual acuity (BCVA) from each eye. In those with previous cataract surgery, surgical technique and complications including its contribution to vision impairment were noted. The association of surgical complications and surgical coverage with sociodemographic variables was assessed by multiple logistic regression. RESULTS A total of 2,384 persons were determined to be eligible, and 2,041 (85.6%) were examined. Overall, prevalence of cataract surgery was 8.6%, with 11.3% urban and 5.0% rural. Surgical complications were associated with males, older age, and urban residency and were found in 60.4%, with posterior capsule opacification in 29.6% and posterior capsule rupture in 16.3%. Among the 270 eyes having cataract surgery, APC ≥20/32 was found in 44.4%, APC 20/40 to 20/63 in 20.8%, APC

Purpose: To determine the prevalence and causes of visual impairment and blindness in adults aged 45 years and older from Parintins, Brazilian Amazon Region. Methods: A random cluster sampling method was used to identify subjects 45 years of age and older from urban and rural census sectors of Parintins city, Amazonas State, from March 2014 to May 2015. Participants underwent a detailed ocular examination, including presenting (PVA) and best corrected visual acuity (BCVA). The main cause of PVA

AimsTo determine prevalence of pterygium, its role as main cause of unilateral and bilateral visual impairment and blindness and its impact on refractive errors from adults living in a high ultraviolet exposure area in the Brazilian Amazon Region.MethodsCluster sampling was used in randomly selecting subjects ≥45 years of age from urban and rural areas of Parintins city. Eligible subjects were enumerated through a door-to-door household survey and invited for an eye exam including refraction. Pterygium was assessed considering location (nasal, temporal or both) and size (ResultsA total of 2384 persons were enumerated and 2041 (85.6%) were examined. Prevalence of pterygium was 58.8% (95% CI 53.8% to 63.7%) and associated with male gender (OR=1.63; 95% CI 1.37 to 1.94; p=0.001), while higher education was a protective factor (OR=0.63; 95% CI 0.44 to 0.92; p=0.018). Older age and rural residence were associated with pterygium ≥3 mm reaching or not pupillary margin, while higher education was a protective factor for pterygium ≥3 mm reaching pupillary margin. Prevalence of pterygium as cause of visual impairment and blindness was 14.3% and 3.9%, respectively. Significantly higher hyperopic refractive errors were found in eyes with pterygium ≥3 mm reaching or not pupillary margin.ConclusionsPterygium was highly prevalent and the second cause of visual impairment and blindness after provision of refractive correction. Risk factors for pterygium were male gender, advanced age, lower education and rural residency. Strategies to provide pterygium early detection and proper management should be considered by healthcare authorities in this population.

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 illustrate the natural history of Leber's hereditary optic neuropathy (LHON). Design: Prospective observational case series. Participants: The Soave-Brazil pedigree of m.11778G>A/ND4 mitochondrial DNA LHON mutation. Methods: A prospectively acquired database of the Soave-Brazil pedigree was reviewed. Data from 285 individuals were included in the database over a 15-year period. The pedigree was reviewed for unaffected mutation carriers who converted to affected status, 6 patients with LHON were identified. The medical records were reviewed 1 year preconversion to 1 year postconversion for visual acuity (logarithm of the minimum angle of resolution [logMAR]), Humphrey Visual Field (HVF) mean deviation (MD), and retinal nerve fiber layer (RNFL) thickness, as measured by Cirrus (Carl Zeiss, Oberkochen, Germany) optic coherence tomography (OCT). The RNFL thickness values were normalized for age. Visual acuity, HVF, and processed RNFL data from each of the 12 eyes were then sorted into 2-month time periods relative to the date of conversion, within which they were averaged. Main Outcome Measures: The main outcome measures were visual acuity, HVF MD, and RNFL thickness. Results: Decreased visual acuity preceded conversion by up to 2 months and then declined up to 8 months postconversion. Decrease in HVF MD occurred at least 4 months preceding conversion, after which values decreased until plateau at 6 to 8 months. Average RNFL thickness was above normal baseline thickness in all 4 quadrants as measured by OCT at the time of conversion. Increase in RNFL thickness preceded conversion as early as 4 to 6 months, peaked at conversion, and decreased until individual plateaus. The temporal quadrant was first to be involved, then the inferior and superior quadrants, and the nasal quadrant showed the latest and least changes. Conclusions: Subclinical changes preceded the date of conversion and may reflect the complicated nature of identifying the date of conversion in LHON. Early increases in RNFL preceding conversion suggest that structural changes precede clinically significant vision loss. Asynchronous quadrant involvement supports a previously published mathematical model. The natural history of LHON is not a subacute process, as previously believed, but progresses more slowly, taking up to 8 months to plateau.

To investigate the contribution of full-field transient pattern-reversal visually evoked potentials (PRVEP) on cross-sectional evaluations of visual function in patients with and without neurofibromatosis type 1 (NF1) affected by optic pathway low-grade gliomas (OPLGG). Participants were children and adolescents referred for visual function evaluation and receiving treatment for OPLGG, linked (NF1-OPLGG) or not to NF1 (Non-NF1-OPLGG). An age-adjusted control group was included for comparison. Monocular full-field PRVEPs were recorded from each eye in accordance with ISCEV standards. Parameters of peak-to-peak P100 amplitude (µV) and P100 peak time (ms) were measured. Cutoff normative values obtained from controls for 15′ and 60′ check sizes were ≥ 9.0 µV for N75-P100 amplitude and ≤ 103.0 ms for P100 peak time. The association of age, gender, tumor resection and NF1 with P100 amplitude reduction and P100 peak time delay was explored by Firth logistic regression modeling. Participants were 30 patients (15 males, 60% Non-NF1) with ages from 3.6 to 19.9 years (mean ± SD = 9.2 ± 3.8 years; median = 8.4 years) and 19 controls (12 males) with ages from 3.7 to 19.9 years (mean ± SD = 10.4 ± 4.9 years; median = 9.5 years). Overall, 68% of tested eyes presented reduced P100 amplitudes for both check sizes (46% in the NF-1 and 83% in the Non-NF1) and delayed P100 for both check sizes (38% in NF1 and 89% in Non-NF1). Absence of NF1 adjusted for age, gender and tumor resection was significantly associated with marginally reduced P100 amplitude for 15′ checks [odds ratio (OR): 6.26; 95% confidence interval (CI) = 0.96–40.94; p = 0.055]. Full-field PRVEP on cross-sectional evaluations contributed to detect visual dysfunction in two-thirds of patients with OPLGG by highlighting subclinical evidence of visual loss. Abnormalities were more frequent and more severe in OPLGG not linked to NF1 than in NF1-OPLGG; however, there was a difference in surgical management between these groups. PRVEP parameters may provide reliable evidence of visual pathway involvement in OPLGG, helping to hasten treatment before optic atrophy is detected.

To investigate pattern-reversal visual evoked potentials (PRVEP) in asymptomatic maternally and non-maternally related members from a large Brazilian 11778/ND4 LHON pedigree. Transient PRVEP for check sizes 15' and 60' were recorded from asymptomatic mutation carriers and non-mutant descendants of affected/non-affected males, all with best-corrected visual acuity of 20/20. A control group of spouses (off-pedigree) was also included. Parameters of N75, P100 and N135 latencies (ms) and N75-P100 peak-to-peak amplitude (μV) as well as temporal dispersion (latency of N135-latency of N75) were determined. Longitudinal testing was obtained in a subseries of carriers in three annual consecutive visits. We tested 48 asymptomatic mutation carriers, 19 descendants of affected males, 9 descendants of non-affected males and 27 off-pedigrees, all of the latter being non-mutant. All non-mutant male descendants did not differ from off-pedigree controls. Statistically prolonged P100 latencies were found in mutation carriers (P = 0.0143) when compared with off-pedigrees for check sizes 15', as well as significantly larger temporal dispersions for both check size 15' (P = 0.0012) and check size 60' (P = 0.0271). Serial testing in 15 mutation carriers disclosed prolonged P100 latencies and larger temporal dispersion that did not change over time. Subclinical PRVEP abnormalities were detected in this large group of asymptomatic carriers of the 11778/ND4 LHON mutation from the same family, confirming and extending previous psychophysical and structural findings of a selective involvement of the parvocellular pathway. PRVEP is a useful test to characterize and monitor visual dysfunction in this devastating disease.

Purpose To report the ophthalmologic characteristics of a newly identified seven-generation pedigree of 11778/Haplogroup J Leber hereditary optic neuropathy consisting of 328 living individuals, 111 of whom are maternally related. Design Observational population cohort study. Methods This prospective study of a large Brazilian Leber hereditary optic neuropathy pedigree was carried out as a field investigation in Brazil. We describe the ophthalmologic findings of 192 eyes from 96 maternally related individuals of this pedigree. Spouses were used as control subjects. We conducted comprehensive neuro-ophthalmologic examinations with psychophysical tests, Humphrey visual fields, and fundus photographs. We also correlated the ophthalmologic findings with the previously published epidemiologic assessment of risk factors. Results We examined 76 carriers and 20 affected individuals. The affected individuals showed severe disease with a mean visual acuity of 2.04 logarithm of the minimal angle of resolution and without evidence of recovery. All the affected individuals showed diffuse optic atrophy with a cup-to-disk ratio greater than 0.5 in 55% of cases. Moreover, among Affected individuals, smokers had a poorer visual acuity ( P = .002). Among carriers there were several subclinical abnormalities, including microangiopathy, swelling of nerve fibers, and visual field abnormalities that did not correlate with tobacco or alcohol consumption. Conclusions Our results demonstrate a significant influence of environmental risk factors, particularly smoking, for developing Leber hereditary optic neuropathy and for the severity of its clinical expression. However, smoking did not correlate with the subclinical abnormalities detected in carriers. Moreover, subclinical abnormalities were equally distributed between gender.

To investigate contributing factors to visual evoked potential (VEP) grating acuity deficit (GAD) and inter-ocular acuity difference (IAD) measured by sweep-VEPs in children with cerebral visual impairment (CVI). VEP GAD was calculated for the better acuity eye by subtracting acuity thresholds from mean normal VEP grating acuity according to norms from our own laboratory. Deficits were categorized as mild (0.17 ≤ deficit

Purpose: To compare perceptions of the quality of ophthalmological services offered to outpatients from the public healthcare system to those from the private healthcare system, and to determine which measures are seen as necessary and a priority for improving the quality of care. Methods: This was a prospective observational study on 200 patients, 101 and 99 of whom were from the public and private healthcare systems, respectively. All patients underwent an ophthalmological examination at an ophthalmology hospital in Belo Horizonte, Minas Gerais, Brazil. Personal interviews were conducted using two structured questionnaires adapted from the modified SERVQUAL scale. Results: Overall, patients from the private healthcare system were significantly more dissatisfied than those from the public healthcare system. In both systems, reliability was considered to be the most important determinant of quality, and it presented the highest level of dissatisfaction. Satisfaction with the public healthcare system was significantly greater than that with the private healthcare system in terms of the tangibles, reliability, responsiveness, and assurance determinants of the SERVQUAL scale. Conclusions: Institutions must plan, execute, evaluate, and monitor measures that seek to improve the overall patient satisfaction with the quality of services provided, particularly in the private healthcare system, and special attention must be paid to reliability in both healthcare systems. The identification and monitoring of the quality of healthcare services through the periodic use of the SERVQUAL scale may provide healthcare managers with information so that they can identify, plan, and monitor necessary and priority measures. This could be a key strategy for improving the quality of outpatient health services in the public and private systems. RESUMO Objetivo: Comparar a percepção da qualidade dos serviços oftalmológicos prestado aos pacientes ambulatoriais do sistema público com a do sistema privado e detectar quais ações são percebidas como necessárias e prioritárias para melhorar a qualidade do atendimento. Métodos: Foi realizado estudo prospectivo observacional de 200 pacientes sendo 101 do sistema público de saúde e 99 do sistema privado submetidos a exame oftalmológico em Hospital Especializado em Oftalmologia (HEO) - Belo Horizonte - MG - Brasil. Realizaram-se entrevistas pessoais, mediante a aplicação de dois questionários estruturados adaptados da escala SERVQUAL modificada. Resultados: No geral, detectou-se que pacientes do sistema de saúde privado, estão significativamente mais insatisfeitos que aqueles do sistema público de saúde. Em ambos os sistemas a confiabilidade foi considerada o determinante de qualidade mais importante e o que apresentou o maior índice de insatisfação. No sistema público a satisfação foi significativamente superior à do sistema privado a nível dos determinantes da escala SERVQUAL: tangibilidade, confiabilidade, atendimento e segurança. Conclusões: A instituição deve planejar, executar, avaliar e monitorar ações que busquem melhorar a satisfação geral dos pacientes com a qualidade do serviço recebido, principalmente do sistema privado, com atenção especial à confiabilidade nos dois sistemas. A identificação e monitorização da qualidade dos serviços de saúde, empregando periodicamente a escala SERVQUAL, poderá fornecer informações à administração dos serviços de saúde para que possam detectar, planejar e monitorizar as ações necessárias e prioritárias, podendo funcionar como chave estratégica para o aprimoramento da qualidade dos serviços de saúde ambulatoriais públicos e privados.

To determine gender-based normative values for pattern-reversal (PR) and flash (F) visually evoked potentials (VEP) under binocular and monocular stimulation in healthy adults. Healthy adults (age ≥18 years) were recruited among university employees and students. Inclusion criteria were absence of abnormalities in fundoscopy, tracking ability, stereopsis and pupillary reflexes; best-corrected visual acuity ≤.00 logMAR; and refractive error (spherical equivalent) from −6.00 to +6.00. Exclusion criteria were previous intraocular surgery, systemic and/or neurological disorders. Binocular and monocular tests were performed according to International Society for Clinical Electrophysiology of Vision standards for PRVEP (reversal rate = 1.9 Hz, checkerboard stimuli 15′ and 60′ at 100% contrast) and FVEP (3 cd s/m2, rate = 1 Hz). VEP parameters of amplitude (µV) and peak times (ms) were measured. Inter-ocular differences, inter-peak intervals (N135–N75) and binocular summation were determined. Fifty-four subjects (28 females; mean age = 40.4 ± 13.7 years; median = 40.0 years) were included. Mean P100 latencies for 15′ and 60′ stimuli were, respectively, 94.6 ± 4.7 ms and 96.1 ± 4.2 for women. Mean values of P100 latency for men were 97.4 ± 4.9 for 15′ and 97.7 ± 4.2 for 60′ stimuli. Larger mean P100 for 15′ checks was observed in women (12.8 ± 5.7 µV) than men (8.6 ± 2.5 µV) in PRVEP. Similar results were found for FVEP N2–P2 amplitudes (mean = 14.6 ± 4.9 for women and 9.8 ± 4.0 for men). Gender-based normative values for PRVEP and FVEP were determined, with women disclosing higher responses than men for smaller stimuli in the visual pathway. The use of gender-based normative values in the analysis of clinical VEP data for diagnostic and therapeutic purposes is recommendable. Additional analysis including inter-peak intervals and binocular summation ratio might improve the diagnostic power of VEP.

Tamoxifen, an effective treatment of breast cancer, has been shown to cause ocular toxic effects. The purpose of this study was to determine retinal toxicity by full-field and focal electroretinograms (ERGs) in patients treated with tamoxifen. Fullfield and focal ERGs were obtained from three groups: Tamoxifen-14 females (47-72 years, mean 58.3 + or - 9.1) with normal fundus, treated with tamoxifen from 2 to 37 months; No Treatment-10 females (39-65 years, mean 50.1 + or - 8.7) with previous breast cancer diagnosis and before tamoxifen treatment; Control-13 normal female volunteers (41-81 years, mean 52.7 + or - 12.1). Peak-to-peak amplitude and b-wave implicit time were measured and statistically analyzed.Mean peak-to-peak amplitudes and implicit time from full-field and focal ERGs were comparable for the three different groups. Low-dosage tamoxifen showed no retinotoxic effect assessed by full-field and focal ERG in this small group of women with breast cancer.

OBJETIVO: A proposta deste estudo é avaliar a eficácia e eficiência em uma série de casos do protótipo de um equipamento nacional de magnificação para leitura. MÉTODOS: Participaram deste estudo 30 pacientes na faixa etária entre 9 e 80 anos (17 do sexo masculino). Foi desenvolvido um aparelho portátil, patenteado pela UNIFESP (PI#020050145260), com um sistema de captura de imagens acoplado a um monitor de 5,6 polegadas proporcionando um aumento de 15 X. Foram analisadas a eficácia da acuidade visual e a eficiência de leitura após a utilização do protótipo proposto. RESULTADOS: Seis pacientes (20%) apresentaram AV 8M, 12 pacientes (40%) apresentaram AV 6M, 7 pacientes (23,3%) apresentaram 5 M, 5 pacientes (16,7%) apresentaram 4M. A média de acuidade visual antes da utilização do SLP medida pela tabela LHNV-1 logMAR foi de 5,75M e após a utilização 100% dos pacientes atingiram a eficácia de AV J1. CONCLUSÃO: O protótipo do SLP mostrou-se um recurso alternativo no processo de inclusão social das pessoas com baixa visão com diferentes níveis de resíduo visual. Também pode proporcionar incentivo psicológico, permitir conforto, mobilidade e independência àqueles que necessitam de uma leitura mais prolongada e maior distância de trabalho. PURPOSE: The proposal of this study is to test and to validate as the effectiveness and efficiency in a series of cases the prototype of a national equipment of magnification to reading. METHODS: A recently developed of equipment of magnification (patent pending Brazilian Institutte of Industrial Property # 020050145260) was tested in a group of 30 patients (age range 9 to 80 years, 17 males). A portable apparatus was developed with a system of capture of images coupled with a 5,6 inch monitor, providing an increase of 15 X. The effectiveness of the visual acuity and the reading efficiency were analyzed after the use of the proposed prototype. RESULTS: Six patients (20%) presented AV 8M, 12 patients (40%) presented AV 6,0M, 7 patients (23.3%) presented AV 5M, 5 patients (16.7%) presented 4M. The average of visual acuity before the utilization of SPR measured by LHNV-1 logMAR chart was 5,75M. After the use of LHNV-1 logMAR chart, 100% (one hundred percent) of the patients reached the efficacy of AV J1. CONCLUSION: The prototype of SPR is an alternative resource in the social inclusion process of low vision patients with different levels of visual residue. It demonstrates psychological incentive, allows comfort, mobility and independence to those who need a more extended lecture and more distance of work.

OBJETIVO: Avaliar a qualidade de vida relacionada à visão em crianças com catarata congênita bilateral, utilizando o Questionário de Função Visual Infantil (QFVI). MÉTODOS: O QFVI possui duas diferentes apresentações: crianças menores e maiores de 3 anos, sendo dividido em seis domínios: saúde geral, saúde geral da visão, competência, personalidade, impacto familiar e tratamento. O QFVI foi aplicado aos pais/cuidadores/responsáveis de crianças com catarata congênita bilateral do Instituto da Visão da Universidade Federal de São Paulo. As notas encontradas nos domínios foram comparadas entre os grupos controle - crianças com visão normal (n=32, média da idade=38 meses; dp=17,3) e experimental - crianças com catarata congênita (menores de 3 anos, n=19, idade média=14,6 meses; dp=10,7 e maiores de 3 anos, n=18, idade média=4,4 anos; dp=1,5, alocados segundo o grau de comprometimento visual em n=16 - sem deficiência visual; n=10 - deficiência visual leve; n=11 - deficiência visual grave). Para a análise estatística foram utilizados os testes t e análise de variância (ANOVA). RESULTADOS: O grupo experimental apresentou redução estatisticamente significativa em todos os domínios do questionário, quando comparado ao grupo controle. O domínio competência apresentou-se com baixa nota havendo redução estatisticamente significante de acordo com a gravidade da deficiência visual (ANOVA F 5,1, p=0,01; teste de Tukey p

Purpose: Alternative recording methods have been tested to allow the electroretinogram (ERG) recording in uncooperative patients and/or patients with palpebral alterations, including recordings with skin electrodes. The purpose of this study was to compare ERG recorded with skin electrodes and well-established microfiber electrodes and to determine normative values of ERG parameters for recording with skin electrodes. Methods: Fifty healthy volunteers (17-26 years; mean 20.63 ± 2.01 years) participated in the study. A gold disk skin electrode was placed on the lower orbital rim of a randomly chosen eye. On the contralateral eye, a microfiber electrode was positioned in the lower conjunctival sac. Gold disc electrodes were positioned at the ipsilateral outer canthus of both eyes acting as reference electrodes for the creation of a potential difference. Two ground electrodes were placed on the lobe of each ear. ERGs were recorded according to the International Society of Clinical Electrophysiology Visual (ISCEV) protocol using the VERIS 5.1.9 system for data acquisition and analysis. Results: Both types of electrodes showed similar wave response morphologies. The implicit time of responses between the two electrodes was comparable. On peak-to-peak amplitude, skin electrode recordings showed an amplitude reduction of 61.4% for rod responses, 61.5% for maximal responses, 46.2% for oscillatory potentials, 57.4% for cone responses, and 54.4% for 30Hz-flicker responses, when compared with microfiber electrode recordings. Based on these findings, normative values for peak-to-peak amplitude and implicit time to be used as a reference for ERGs recorded with skin electrodes were determined. Conclusions: ERGs recorded with skin electrodes presented lower peak-to-peak amplitude compared with microfiber electrodes. However, using appropriate normative values, skin electrodes may be useful for specific target populations such as uncooperative infants and/or patients with ocular surface alterations. RESUMO Objetivo: O eletrorretinograma de campo total (ERG) em pacientes não colaborativos e/ou com alterações palpebrais pode ser registrado com diferentes métodos de captação de respostas, dentre eles os eletrodos de pele. O objetivo deste estudo foi comparar o eletroretinograma obtido com eletrodos de pele e eletrodos de microfibra, determinando valores normativos para os parâmetros registrados com eletrodos de pele. Métodos: Cinquenta voluntários saudáveis (17-26 anos; média 20,63 ± 2,01) participaram deste estudo. Um eletrodo de cúpula de ouro foi aderido à pele na margem orbital inferior de um dos olhos escolhido ao acaso. No olho contralateral, um eletrodo de microfibra foi posicionado no saco conjuntival inferior. Eletrodos de cúpula de ouro foram posicionados na região lateral de cada olho para a formação da diferença de potencial com o eletrodo ativo. Dois eletrodos terra foram posicionados no lobo de cada orelha. O eletrorretinograma foi registrado de acordo com o protocolo da ISCEV (Sociedade Internacional de Eletrofisiologia Visual), com o sistema VERIS 5.1.9 para aquisição e análise dos registros. Resultados: Os dois tipos de eletrodos apresentaram morfologias de onda similares. O tempo implícito das respostas foi comparável entre os dois tipos de eletrodo. Os registros feitos com eletrodo de pele quando comparados aos registros com eletrodos de microfibra mostraram reduções na amplitude das ondas, de 61,4% para resposta de bastonetes, 61,5% para resposta máxima, 46,2% para potenciais oscilatórios, 57,4% para resposta de cones, e 54,4% para flicker 30Hz. Baseado nestes resultados, foram determinados valores normativos para amplitude e tempo de implícito para ERGs obtidos com eletrodos de pele. Conclusões: ERGs registrados com eletrodos de pele apresentam respostas com amplitudes menores quando comparados aos registrados com eletrodos de microfibra. No entanto, usando valores normativos apropriados, os eletrodos de pele podem ser uma alternativa útil para populações especificas como pacientes não colaborativos e/ou com alterações palpebrais.

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. Univ Fed Sao Paulo UNIFESP, Dept Oftalmol & Ciencias Visuais, Lab Eletrofisiol Visual Clin, EPM, Sao Paulo, SP, Brazil Univ Fed Sao Paulo UNIFESP, Dept Oftalmol & Ciencias Visuais, Lab Eletrofisiol Visual Clin, EPM, Sao Paulo, SP, Brazil Web of Science

Purpose: To evaluate visual acuity and transient pattern reversal (PR) visual evoked potentials (VEPs) in the fellow eyes of children with strabismic and/or anisometropic amblyopia. Methods: Children diagnosed with strabismic and/or anisometropic amblyopia were recruited for electrophysiological assessment by VEPs. Monocular grating and optotype acuity were measured using sweep-VEPs and an Early Treatment Diabetic Retinopathy Study chart, respectively. During the same visit, transient PR-VEPs of each eye were recorded using stimuli subtending with a visual angle of 60', 15', and 7.5'. Parameters of amplitude (in μV) and latency (in ms) were determined from VEP recordings. Results: A group of 40 strabismic and/or anisometropic amblyopic children (22 females: 55%, mean age= 8.7 ± 2.2 years, median= 8 years) was examined. A control group of 19 healthy children (13 females: 68.4%, mean age= 8.2 ± 2.6 years, median= 8 years) was also included. The fellow eyes of all amblyopes had significantly worse optotype acuity (p=0.021) than the control group, regardless of whether they were strabismic (p=0.040) or anisometropic (p=0.048). Overall, grating acuity was significantly worse in the fellow eyes of amblyopes (p=0.016) than in healthy controls. Statistically prolonged latency for visual angles of 15' and 7.5' (p=0.018 and 0.002, respectively) was found in the strabismic group when compared with the control group. For the smaller visual stimulus (7.5'), statistically prolonged latency was found among all fellow eyes of amblyopic children (p

PURPOSE To investigate the pupillary light reflex (PLR) of patients with severe loss of vision due to Leber's Hereditary Optic Neuropathy (LHON) in the context of a proposed preservation of melanopsin-expressing retinal ganglion cells (mRGCs). METHODS Ten LHON patients (7 males; 51.6 ± 14.1 years), with visual acuities ranging from 20/400 to hand motion perception and severe visual field losses, were tested and compared with 16 healthy subjects (7 males; 42.15 ± 15.4 years) tested as controls. PLR was measured with an eye tracker and the stimuli were controlled with a Ganzfeld system. Pupil responses were measured monocularly, to 1 second of blue (470 nm) and red (640 nm) flashes with 1, 10, 100, and 250 cd/m² luminances. The normalized amplitude of peak of the transient PLR and the amplitude of the sustained PLR at 6 seconds after the flash offset were measured. In addition, optical coherence topography (OCT) scans of the peripapillary retinal nerve fiber layer were obtained. RESULTS The patient's peak PLR responses were on average 15% smaller than controls (P < 0.05), but 5 out of 10 patients had amplitudes within the range of controls. The patients' sustained PLRs were comparable with controls at lower flash intensities, but on average, 27% smaller to the 250 cd/m² blue light, although there was considerable overlap with the PLR amplitudes of control. All patients had severe visual field losses and the retinal nerve fiber layer thickness was reduced to a minimum around the optic disc in 8 of the 10 patients. CONCLUSIONS The PLR is maintained overall in LHON patients despite the severity of optic atrophy. These results are consistent with previous evidence of selective preservation of mRGCs.

The aim of this study was to investigate possible functional and structural ocular changes caused by chronic sildenafil therapy to treat pulmonary arterial hypertension (PAH). Case–control study included patients with pulmonary arterial hypertension: chronically using sildenafil and without sildenafil treatment. A comprehensive ophthalmologic exam including ectoscopy, extrinsic ocular motility, logMAR visual acuity measurement, contrast sensitivity test, color test, anterior segment biomicroscopy, Schirmer test 1, intraocular pressure, fundus exam under pupil dilation, fundus pictures, time domain and spectral domain optical coherence tomography, ocular Doppler ultrasound were performed. Full-field electroretinography (ERG) was tested for each eye in a subgroup of sildenafil-treated patients. Twenty patients from each group were tested. Bilateral severe keratitis was found in seven (35 %) patients under sildenafil therapy. Lacrimal film break-up time (BUT) was significantly reduced (p = 0.006 respectively) and Doppler ultrasound showed a reduced resistance index of the central retinal artery in the group of sildenafil users (p = 0.019). No diffuse retinal functional abnormalities were found in ERG in treated patients. Visual acuity, contrast sensitivity and color discrimination were normal in both groups. No abnormalities were found in both time-domain and spectral-domain OCT for retinal parameters. One-third of the treated PAH group showed severe bilateral keratitis. This finding could be related to connective tissue abnormalities usually present in patients with this condition that might be exacerbated with the sildenafil usage. The resistance index of the central retinal artery was diminished in the chronic users group and it could be associated to the vasodilation caused by the medication in the choroidal vessels. An ophthalmic assessment for these patients is recommended to diagnose and treat possible ocular surface and choroidal blood flow abnormalities caused by sildenafil.

OBJETIVO: A prevalência do astigmatismo refracional e sua relação com a acuidade visual de resolução foram avaliados em amostra de crianças pré-verbais. MÉTODOS: Foram submetidas a exame oftalmológico 482 crianças normais com idades variando entre 2 e 36 meses. Após exclusão da amostra de 14 crianças com evidências de doenças oculares que pudessem alterar a acuidade visual, permaneceram 468 crianças (936 olhos), sendo 230 (49%) do sexo masculino e 238 (51%) do feminino. Todas foram submetidas a exame de acuidade visual binocular e monocular pelo procedimento do olhar preferencial através do método dos cartões de acuidade de Teller. Foi realizado em todas as crianças exame de refração sob cicloplegia e fundoscopia com oftalmoscópio indireto. RESULTADOS: O astigmatismo foi encontrado em 222 (47,43%), crianças da amostra, com predomínio do tipo hipermetrópico composto e da forma a favor da regra em todas as faixas etárias. Em relação à magnitude, esta ametropia foi maior ou igual a 1,00 dioptria cilíndrica em 24,35% dos sujeitos, maior ou igual a 1,00 dioptria cilíndrica em 24,35% dos sujeitos, maior ou igual a 2,00 dioptrias cilíndricas em 5,55%, menor que 1,00 dioptria cilíndrica em 26,92% e maior ou igual a 1,00 dioptria cilíndrica e menor que 2,00 dioptrias cilíndricas em 18,73%. A acuidade visual esteve dentro da faixa de normalidade em 219 crianças da amostra, independentemente da magnitude, tipo e orientação do astigmatismo. CONCLUSÃO: A acuidade visual de resolução medida pelo método dos cartões de acuidade de Teller não foi influenciada pelo astigmatismo. PURPOSE: To evaluate refractional astigmatism prevalence and its relationship with grating acuity in a cohort of non-verbal children. METHODS: 482 normal children, aged from 2 to 36 months, were submitted to ophthalmological examination. Fourteen subjects were excluded due to ocular disease and the sample remained with 468 subjects (936 eyes); 230 (49%) males e 238 (51%) females. Grating acuity was assessed binocularly and monocularly with Teller acuity cards. All children underwent eye examination including cycloplegic retinoscopy and fundus by indirect ophthalmoscopy. RESULTS: Astigmatism was found in 222 (47.43%) of the children, with the hyperopic and with the rule types most frequently found in all ages. Concerning magnitude, this condition was equal or greater than 1.00 cylindric diopter in 24.35% of the children; equal or greater than 2.00 cylindric diopter in 5.55%; lower than 1.00 in 26.92% and between 1.00 and 2.00 in 18.73%. Grating acuity was normal in 219 of the subjects, despite magnitude, type and orientation of astigmatism. CONCLUSION: Visual acuity assessed by the acuity card procedure was not influenced by astigmatism.

OBJETIVOS: Identificar a percepção da qualidade dos serviços oftalmológicos prestados aos pacientes ambulatoriais do Sistema Único de Saúde - SUS - e detectar quais ações são percebidas como necessárias e prioritárias para melhorar a sua qualidade. MÉTODOS: Foi realizado estudo descritivo quantitativo de 100 pacientes ambulatoriais do SUS, submetidos a exame oftalmológico na Fundação Hilton Rocha, em Belo Horizonte - MG, no período de 1 de junho a 30 de julho de 2004. Realizaram-se entrevistas pessoais, mediante a aplicação de dois questionários estruturados adaptados da escala SERVQUAL modificada. Essa escala foi adaptada à realidade da instituição estudada. RESULTADOS: A escala SERVQUAL adaptada foi submetida à validação estatística apresentando adequado índice de consistência interna. Em termos gerais, detectou-se ligeira insatisfação geral com a qualidade do atendimento oftalmológico. Os entrevistados deram maior importância à segurança e à confiabilidade. Detectou-se o maior grau de insatisfação na confiabilidade, principalmente em relação ao cumprimento das atividades nos horários marcados e em relação à execução dos serviços no prazo prometido. CONCLUSÕES: A instituição deve planejar e executar ações que levem a melhora geral da satisfação de seus pacientes com a qualidade do serviço recebido, principalmente no aspecto confiabilidade. A monitorização da qualidade do serviço pelo emprego periódico da escala SERVQUAL permitiria não só planejar estratégias precisas de intervenção de alta efetividade neste e em outros serviços de saúde, como também permitiria monitorizar a resposta a essas ações, contribuindo, dessa forma, para a melhora da qualidade do serviço no sistema como um todo.PURPOSE: To identify the perception of the ophthalmic service quality provided for outpatients of the public healthcare system as well as to detect which actions should be considered necessary and priority in order to improve its quality. METHODS: A quantitative descriptive study was carried out on 100 outpatients of the public healthcare system which were submitted to ophthalmic tests at Fundação Hilton Rocha - Belo Horizonte - MG, from July 1st - July 30th 2004. Individual interviews were carried out by giving the interviewees two structured questionnaires adapted from the modified SERVQUAL. This scale is in agreement with the reality of the studied institute. RESULTS: The adapted SERVQUAL scale was submitted to statistical validation and it showed a suitable internal consistency index. In general terms, a slight general dissatisfaction was detected regarding ophthalmological service quality. The interviewees cared more about safety and reliability. A higher degree of dissatisfaction was detected mainly concerning fulfillment of procedures at scheduled appointments related to the execution of services within due timelimits. CONCLUSIONS: The institute is supposed to plan as well as carry out actions which lead to a general improvement in the patient's satisfaction regarding service quality and mainly reliability. Service quality monitoring through periodic use of the SERVQUAL scale will not only make it possible to plan highly precise and effective intervention strategies in these and in other healthcare services but it will also allow monitoring the responses to these actions. All these actions will contribute to the improvement of the service in the system as a whole.

PURPOSE: To determine normative values for focal electroretinography in normal young adults, according to the standard protocol recommended by the International Society for Clinical Electrophysiology of Vision - IsCEV. METHODS: 33 normal volunteers aged from 14 to 38 years (mean 22.96±5.73) were included in this study. Focal electroretinography was recorded by corneal electrode after white flicker light stimulation in the macula at high temporal frequencies -42Hz and 31.25Hz. Focal electroretinography parameters as amplitude (nanovolts) and latency (milliseconds) were extracted and analyzed after discrete Fourier transformation. Median, values below 95% and above 95%; maximum; minimum and percentiles were calculated for both parameters; a t test was calculated between amplitudes and latencies for both frequencies. RESULTS: For 42Hz. Amplitude: median 614nV; below 95% 529nV; above 95% 650nV; minimum 370 nV; maximum 794 nV; 2.5th percentile 378.8nV; 97.5th percentile 779.6nV. Latency: median 26.4ms; below 95% 25.4ms; above 95% 26.8ms; minimum 23.4ms; maximum 28.2ms; 2.5th percentile 23.8ms; 97.5th percentile 27.88ms. For 31.25Hz. Amplitude: median 632nV; below 95% 429nV; above 95% 750nV; minimum 364nV; maximum 1036nV; 2.5th percentile 364.8nV; 97.5th percentile 1018.4nV. Latency: median 30.1ms; below 95% 29.3ms; above 95% 31.2ms; minimum 25.9ms; maximum 33.9ms; 2.5th percentile 27.18ms; 97.5th percentile 33.34ms. CONCLUSIONS: Normal limits for focal electroretinography according to the International Society for Clinical Electrophysiology of Vision, guidelines were established in a cohort of young adults. Comparable amplitude values were found for both 42 Hz and 31.25 Hz temporal frequencies. However, latencies was significantly faster for 42 Hz stimuli.

OBJETIVO: O tamoxifeno é um antiestrógeno utilizado como agente terapêutico eficaz no câncer de mama. A droga pode causar efeitos tóxicos oculares em dosagens altas. O objetivo deste estudo foi determinar possíveis efeitos retino-tóxicos centrais usando o eletrorretinograma multifocal (mfERG) em grupo de pacientes tratadas com baixa dosagem de tamoxifeno (20 mg/dia) após cirurgia do câncer de mama. MÉTODOS: O eletrorretinograma multifocal (mfERG) de 30 graus centrais da retina foi obtido em 3 diferentes grupos: Grupo experimental - 15 mulheres (41-59 anos, média 48,6 ± 4,5) com fundo de olho normal, tratadas com tamoxifeno em baixa dosagem por 1 a 55 meses. Grupo controle - 6 mulheres (30 -76 anos, média 49,9±18,8) com diagnóstico prévio de câncer de mama que não receberam tratamento com tamoxifeno até o teste do eletrorretinograma multifocal. Grupo controle normal - 15 voluntárias normais (30-71 anos, média 47,7 ± 12,9). A amplitude e a latência de eletrorretinograma multifocal para N1-P1 foram analisados estatisticamente (análise de variância de uma via). RESULTADOS: As amplitudes médias N1-P1 (nV/grau²) foram comparáveis para respostas de diferentes excentricidades (0 a 25 graus) nos três grupos. Não houve diferença estatisticamente significativa para a latência de N1 e P1 (ms) do eletrorretinograma multifocal entre os 3 grupos. CONCLUSÕES: O tratamento com baixa dosagem de tamoxifeno não mostrou efeitos retino-tóxicos em pequeno grupo de mulheres após cirurgia do câncer de mama. Investigação seriada proporcionará melhor compreensão desses efeitos. PURPOSE: Tamoxifen, an antiestrogen, has been used as an effective therapeutic agent in the treatment of breast cancer. The drug has been shown to cause ocular toxic effects. The purpose of this study was to determine possible central retinal toxicity by multifocal electroretinograms (mfERGs) in a cohort of patients treated with low-dose tamoxifen (20 mg/day) for breast cancer. METHODS: Multifocal electroretinograms of the central 30 degrees of the retina were obtained of 3 different groups: Experimental group - 15 females (41-59 years, mean 48.6 ± 4.5) with normal fundus, treated with low-dose tamoxifen for 1 to 55 months. Control group - 6 females (30-76 years, mean 49.9 ± 18.8) with previous breast cancer diagnosis who had not received tamoxifen as treatment until ERG testing. Normal control group - 15 normal female volunteers (30-71 years, mean 47.7 ± 12.9). The multifocal amplitude and latency for N1-P1 were measured and statistically analyzed (one-way ANOVA). RESULTS: Mean amplitudes N1-P1 (nV/deg²) were comparable for responses of different eccentricity (0 to 25 degrees). There was no statistically significant difference for implicit time of N1 and P1 (ms) in multifocal electroretinograms between the 3 groups. CONCLUSIONS: Low-dose tamoxifen showed no retinotoxic effect in this small group of women with breast cancer. Follow-up investigation could provide a better understanding of these effects.

We evaluated the color vision of mercury-contaminated patients and investigated possible retinal origins of losses using electroretinography. Participants were retired workers from a fluorescent lamp industry diagnosed with mercury contamination (n= 43) and age-matched controls (n= 21). Color discrimination was assessed with the Cambridge Colour Test (CCT). Retinal function was evaluated by using the ISCEV protocol for full-field electroretinography (full-field ERG), as well as by means of multifocal electroretinography (mfERG). Color-vision losses assessed by the CCT consisted of higher color-discrimination thresholds along the protan, deutan, and tritan axes and significantly larger discrimination ellipses in mercury-exposed patients compared to controls. Full-field ERG amplitudes from patients were smaller than those of the controls for the scotopic responseb-wave, maximum response, sum of oscillatory potentials (OPs), 30-Hz flicker response, and light-adapted cone response. OP amplitudes measured in patients were smaller than those of controls for O2 and O3. Multifocal ERGs recorded from ten randomly selected patients showed smaller N1–P1 amplitudes and longer latencies throughout the 25-deg central field. Full-field ERGs showed that scotopic, photopic, peripheral, and midperipheral retinal functions were affected, and the mfERGs indicated that central retinal function was also significantly depressed. To our knowledge, this is the first demonstration of retinal involvement in visual losses caused by mercury toxicity.

OBJETIVO: Estabelecer valores normativos para o eletro-oculografia (EOG), em grupo de voluntários normais, segundo o protocolo recomendado pela Sociedade Internacional de Eletrofisiologia Visual Clínica (ISCEV). MÉTODO: Participaram 34 sujeitos com idades de 18 a 55 anos (média 26,75 ± 10,50), 9 homens e 24 mulheres. O EOG foi realizado utilizando eletrodos e estímulos luminosos. A tarefa do sujeito foi fixar estímulos luminosos (LEDs) em frente ou realizando movimentos sacádicos horizontais. O exame constou de três fases: 1- Pré-adaptação; 2- Escotópica e 3- Fotópica. O índice de Arden foi determinado como sendo a proporção entre o pico máximo da amplitude na fase fotópica, dividido pelo vale máximo da amplitude na fase escotópica. RESULTADO: Na fase escotópica, o vale máximo ocorreu entre 14 e 21 minutos após o início do exame (média 17,82 ± 2,11 minutos) e a amplitude variou de 206 a 635 µV (média 365,73 ± 122,52 µV). Na fase fotópica, o pico máximo ocorreu entre 27 a 34 minutos (média 30,06 ± 1,80 minuto) e a amplitude variou de 646 a 1249,50µV (média 950,70 ± 179,16). O índice de Arden variou de 1,85 a 4,02 (média 2,79±0.63) sendo o limite inferior de 1,92 e o superior de 4,00. CONCLUSÃO: Os valores para o índice de Arden estão de acordo com valores descritos anteriormente na literatura, sendo o limite inferior de normalidade quando este for maior ou igual a 1,92 e serão úteis para avaliação de pacientes com distúrbios retinianos. A inclusão de outras faixas etárias fornecerá dados normativos mais abrangentes. PURPOSE: To determine normative values for EOG in a group of healthy adult volunteers, according to the standard protocol recommended by the International Society for Clinical Eletrophisiology of Vision - ISCEV. METHODS: 33 volunteers aging from 18 to 55 years (mean=26.75±10.50) were included in the study. EOG was recorded by skin electrodes positioned in the external canthi of the eyes, with LED stimulus presented in a Ganzfeld dome. Subject's task was to fix the LED stimulus either straight ahead or with saccadic horizontal movements. The potential difference between the cornea and posterior pole of the eye was recorded each minute. The examination was done in 3 phases: 1- pre-adaptation; 2- dark (scotopic) and 3- light (photopic). The Arden ratio was calculated as the proportion between the maximum peak amplitude in the light phase divided by the minimum peak amplitude in the dark. RESULTS: At the scotopic phase, the minimum peak amplitude occurred between 14 and 21 minutes after the beginning of the stimulation (mean=17.82±2.11 min) and the amplitude ranged from 206 to 635 µV (mean 365.73±122.52 µV). At the photopic phase the maximun peak amplitude occurred between 27 and 34 minutes (mean=30.06±1.80 min); the amplitude ranged from 646 to 1250 µV (mean= 950.70±179.16 µV). Arden ratio ranged from 1.85 to 4.02 with a lower normal limit of 1.92 and the upper limit of 4.00. CONCLUSION: The results obtained in this study are comparable to those previously reported in the literature, with a lower normal limit of 1.92 for Arden ratio. These values will be helpful to evaluate patients with several retinal disorders. Inclusion of other age groups will provide extended normative data.

Invasive sinonasal mucormycosis is a rare fungal infection that usually occurs in immunocompromised or diabetic patients, and it is often fatal. The authors present a case of a woman patient suffering from systemic lupus erythematosus and diabetes mellitus treated with prednisone, presenting with a rapidly progressive rhino-orbital-cerebral mucormycosis. She was successfully treated with combined intravenous antifungal therapy and radical debridement followed by complex defect reconstruction with a free vertical rectus abdominis myocutaneous flap, tissue expander, and ophthalmic prosthesis.

To conduct systematic epidemiologic, neuro-ophthalmologic, psychophysical, and mitochondrial DNA (mtDNA) genetic examinations on a newly identified pedigree with Leber hereditary optic neuropathy (LHON).Observational population cohort study.A prospective investigation of an entire Brazilian LHON family.A field investigation by an international team conducted in a remote part of Brazil.We evaluated 265 (both eyes) of the 328 living family members of this LHON pedigree. Only members of this pedigree were studied. Those entering the pedigree as spouses were used as controls.We conducted epidemiologic interviews emphasizing possible environmental risk factors, comprehensive neuro-ophthalmologic examinations, psychophysical tests, Humphrey visual field studies, fundus photography, and blood testing for mitochondrial genetic analysis.We reconstructed a seven-generation maternal lineage descended from a common ancestor dating to the 1870s. All maternally related family members were invariably homoplasmic 11778 with a haplogroup J mtDNA, 33 being affected, of which 22 are still living. With each subsequent generation, there was a progressive decrease of penetrance, and only males were affected in the last two generations. A significant exposure (greater than 95% confidence intervals) to a variety of environmental risk factors characterized the affected individuals, with smoking as the most common (P.01). Both affected and carriers (95% confidence intervals) presented with a significantly lower incidence of hypertension and high cholesterol compared with the control group (P.05).Almost 95% of a 328-living-member pedigree with LHON 11778/J haplogroup was comprehensively studied. Our initial results indicate the strong influence of environmental risk factors. The remarkably reduced incidence of cardiovascular risk in the maternal lineage is discussed. Further genetic analysis may reveal a role for the nuclear genome.

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) OBJETIVO: Determinar a freqüência de diferentes categorias diagnósticas em um laboratório de eletrofisiologia visual clínica de um hospital universitário. MÉTODOS: Os pacientes participaram de um estudo transversal sendo submetidos a testes eletrodiagnósticos visuais entre outubro de 1998 e outubro de 2000 (N=570). Os exames de eletrorretinografia (ERG) foram realizados em 385 pacientes (68%) e de potenciais visuais evocados (PVE) por reversão de padrão e/ou flashes e varredura em 185 pacientes (32%). RESULTADOS: Após avaliação da eletrorretinografia, as categorias diagnósticas mais freqüentes foram identificadas na seguinte ordem: retinose pigmentária, doença de Stargardt, distrofia de cones, degeneração do tipo cone-bastonete, retinotoxicidade devido a vários agentes (cloroquina, tamoxifeno, etc) e trauma ocular, além de perdas visuais de causas idiopáticas. Em 23% dos casos, não foi possível obter o diagnóstico final ao primeiro exame. O grupo mais freqüente referido para o eletrorretinografia foi para documentar ou excluir as degenerações tapeto-retinianas (42%). Neste grupo, 110 casos de retinose pigmentária foram identificados: 91 casos isolados e 19 sindrômicos. Para o exame de potenciais visuais evocados, as categorias diagnósticas mais freqüentes foram: doenças que afetam somente o nervo óptico (atrofia de nervo óptico, neurites ópticas, edema de disco óptico, etc.); condições neurológicas afetando a via visual (esclerose múltipla, paralisia cerebral, trauma crânio-encefálico, tumores, etc.), condições neurológicas e/ou doenças oculares pediátricas (catarata congênita, deficiência visual cortical, glaucoma congênito, etc.), e opacidade de meios (leucoma, trauma ocular, etc.). CONCLUSÕES: A análise deste amplo grupo de pacientes, mostra as indicações mais freqüentes e significativas para registros eletrofisiológicos visuais e decisões diagnósticas. Vários diagnósticos podem ser de difícil conclusão sem os testes eletrofisiológicos visuais. Dentre essas doenças incluem-se estágios iniciais da retinose pigmentária, distrofia progressiva de cones, retinopatia tóxica sem alterações fundoscópicas, função de nervo óptico ou retiniana em opacidades de meios, envolvimento de nervo óptico em esclerose múltipla e avaliação da acuidade visual em doenças oculares infantis com ou sem alterações neurológicas. PURPOSE: To determine the frequency of diagnostic categories obtained in a clinical vision electrophysiology laboratory at a university hospital. METHODS: Patients who underwent visual electrodiagnostic testing from October 1998 to October 2000 (N=570) were cross-sectionally studied. Electroretinogram (ERG) was performed in 385 (68%) patients and pattern and/or flash visually evoked potentials were performed in 185 (32%) patients. RESULTS: After electroretinogram assessment, the most frequently identified diagnostic categories were (in this order): retinitis pigmentosa, Stargardt's disease, cone dystrophy, cone-rod degeneration, retinotoxic effects due to several agents (chloroquine, tamoxifen, methanol, etc) and ocular trauma, besides unclear visual loss. In 23% of the cases, it was impossible to obtain a final diagnosis on the first examination. The largest group referred to electroretinogram testing was for documentation or exclusion of tapetoretinal degenerations (42%). In this group, 110 cases of RP were identified: 91 isolated and 19 syndromic cases. Visually evoked potential testing helped to identify the following diagnostic categories: diseases affecting only the optic nerve (optic nerve atrophy, optic neuritis, optic disk edema, etc); neurological conditions affecting the visual pathway (multiple sclerosis, cerebral palsy, blunt head trauma, tumors, etc): pediatric eye and/or neurological conditions (congenital cataract, cortical visual impairment, congenital glaucoma, etc) and opaque media (leucoma, ocular trauma, etc). CONCLUSIONS: Frequent and meaningful indications for visual electrophysiologic recording and diagnostic decisions arise from this relatively large group of studied patients. A number of diagnoses can hardly, if not all, be established without visual electrophysiologic testing. These include early stages of retinitis pigmentosa, progressive cone dystrophy, toxic retinopathy without fundus changes, optic nerve or retinal dysfunction in opaque media, optic nerve involvement in multiple sclerosis and pediatric eye disease with or without neurological damage. Universidade Federal de São Paulo (UNIFESP) Laboratório de Eletrofisiologia Visual UNIFESP, Laboratório de Eletrofisiologia Visual SciELO

To investigate by optical coherence tomography (OCT) the topographic pattern and temporal sequence of fiber loss in the peripapillary retinal nerve fiber layer (RNFL) of patients with Leber's hereditary optic neuropathy (LHON) in a longitudinal follow-up.Cohort study.Six eyes of 4 patients with molecularly defined LHON were enrolled before the subacute period of visual loss.Subjects were studied by StratusOCT (Carl Zeiss Meditec, Inc., Dublin, CA) during a 9-month follow-up starting from the presymptomatic stage of the disease. Examinations were carried out at 4 different time points: presymptomatic stage, time of visual loss, and 3 and 9 months later.Peripapillary RNFL thickness for each quadrant of the optic nerve. Statistical comparisons were performed by ordinary analysis of variance with Dunnett's post-test.A significant increase of RNFL thickness was detected in the temporal and inferior quadrants between the presymptomatic stage and the disease onset (P0.05). The 360-degree average and the superior and nasal quadrants showed a nonstatistically significant increase of thickness at this time. In the 360-degree average (P0.01), superior (P0.01), nasal (P0.05), and inferior (P0.01) quadrants, RNFL thickening showed statistically significant changes between the presymptomatic stage and the 3-month follow-up. At 3 months, a nonsignificant reduction of RNFL thickness was detected in the temporal quadrant. A significant reduction of RNFL was detected in all but the nasal quadrants between the presymptomatic stage and the 9-month Follow-up.The RNFL thickness increase first appeared at the temporal and inferior quadrants. Conversely, at 3 months the thickening fibers were more evident in the superior and nasal quadrants. These findings are consistent with the established preferential early involvement of the papillomacular bundle in LHON. We also demonstrated the previously unrecognized simultaneous early involvement of the inferior quadrant. The late involvement of both superior and nasal quadrants suggests a dynamic evolution of the acute stage that continues for 3 months and may represent a therapeutic window of opportunity.

To determine uptake, barriers and outcomes in the follow-up of patients referred for free-of-charge, expedited cataract surgery in the Sao Paulo Eye Study (SPES).SPES was a population-based study of urban, low-middle income residents aged ≥50 years. Presenting visual acuity (PVA), best-corrected visual acuity, refraction, and slit-lamp examination were performed in 3677 participants. For subjects with cataract as a principal cause of best-corrected visual acuity ≤20/40, surgery was offered free of charge. Two years after the baseline study, surgery uptake, barriers to surgery, and visual outcomes were analyzed.Among 210 (5.71%) participants who had a cataract surgery indication at baseline, 164 (78.1%) were successfully contacted and 55 (33.5%) reported being operated on for cataract, with 51 agreeing to be re-examined. In a multiple logistic regression model, age, sex, schooling, previous cataract surgery, and PVA at baseline were not significantly associated with surgery uptake. Co-existing health conditions (20.4%), fear of surgery (12.2%) and fear of losing eyesight (11.6%) were the most frequent barriers to cataract surgery adherence. Among the 69 eyes operated on in the interval between baseline and follow-up, PVA ≥20/63 was observed in 50 (72.6%, 95% confidence interval, CI, 62.2-82.3%), PVA20/63-20/200 in 11 (15.8%, 95% CI 8.9-22.9%) and PVA20/200 in 8 (11.6%, 95% CI 5.3-17.9%).Quality of surgery is an increasing determinant of uptake rates. Although free-of-charge and expedited cataract surgery was offered, surgical outcomes might have influenced the low uptake. Aside from cataract surgery campaigns, improvement of surgeon skills, accurate biometry, treatment of ocular comorbidities, postoperative follow-up and eye-care education are needed.

Leber Hereditary Optic Neuropathy (LHON) is a maternally inherited blinding disease caused by missense mutations in the mitochondrial DNA (mtDNA). However, incomplete penetrance and a predominance of male patients presenting with vision loss suggest that modifying factors play an important role in the development of the disease. Evidence from several studies suggests that both nuclear modifier genes and environmental factors may be necessary to trigger the optic neuropathy in individuals harboring an LHON-causing mtDNA mutation. Recently, an optic neuropathy susceptibility locus at Xp21-Xq21 has been reported. In this study, we performed X-chromosomal linkage analysis in a large Brazilian family harboring a homoplasmic G11778A mtDNA mutation on a haplogroup J background. We report the identification of a novel LHON susceptibility locus on chromosome Xq25-27.2, with multipoint non-parametric linkage scores of5.00 (P = 0.005) and a maximum two-point non-parametric linkage score of 10.12, (P = 0.003) for marker DXS984 (Xq27.1). These results suggest genetic heterogeneity for X-linked modifiers of LHON.