Contribution of objectively measured grating acuity by sweep visually evoked potentials to the diagnosis of unexplained visual loss.

PURPOSE: To investigate the diagnostic contribution of grating visual acuity (GVA) measured by sweep pattern-reversal visually evoked potentials (SPRVEP) in unexplained visual loss (UVL). METHODS: 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. RESULTS: 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%. CONCLUSIONS: 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.

Grating visual acuity impairment assessed by sweep visually evoked potentials in children with optic pathway tumors unable to perform optotype acuity tests / Deficiência na acuidade visual de grades avaliada pelos potenciais visuais evocados de varredura em crianças com tumores da via óptica incapazes de informar a acuidade de optotipos

ABSTRACT 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.(AU)

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.(AU)

Grating visual acuity impairment assessed by sweep visually evoked potentials in children with optic pathway tumors unable to perform optotype acuity tests.

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.

Visual function assessed by visually evoked potentials in adults with orbital and other primary intracranial tumors.

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 < .001) and significantly reduced amplitudes for flash visually evoked potential (p < .001). In unilateral orbital tumors, abnormally prolonged pattern-reversal visually evoked potential peak times were also detected in some contralateral eyes (n = 6/16). 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.

Impaired Ganglion Cell Function Objectively Assessed by the Photopic Negative Response in Affected and Asymptomatic Members From Brazilian Families With Leber's Hereditary Optic Neuropathy.

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.

Visual function assessed by visually evoked potentials in optic pathway low-grade gliomas with and without neurofibromatosis type 1.

PURPOSE: 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). METHODS: 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. RESULTS: 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]. CONCLUSIONS: 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.

Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype.

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.

Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype / Estudo da função retiniana em pacientes com o fenótipo da lipofuscinose ceróide neuronal

ABSTRACT 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.

RESUMO Objetivo: Analisar o quadro clínico, a acuidade visual e o eletrorretinograma de campo total (ERG) de 15 pacientes com o fenótipo da lipofuscinose ceróide neuronal (LCN), estabelecendo o papel do eletrorretinograma no seu diagnóstico. Métodos: Eletrorretinograma foi realizado em 5 pacientes com lipofuscinose ceróide neuronal infantil, 5 com doença de Jansky-Bielschowsky e 5 com lipofuscinose ceróide neuronal juvenil sendo feita uma análise retrospectiva dos registros médicos. Resultados: A perda progressiva da acuidade visual foi o sintoma inicial em 66,7%; isolada ou associada à ataxia, epilepsia e involução do desenvolvimento neuropsico motor. Epilepsia foi o sintoma inicial em 93,3% e 86,6% apresentaram involução do desenvolvimento neuropsicomotor. Achados fundoscópicos variaram de normal a alterações pigmentares/atróficas. Disfunção de cone-bastonete foi constatada em 6 pacientes, bastonete-cone em 1 e em 8 pacientes observou-se disfunção proporcional de ambos os sistemas. Conclusão: O eletrorretinograma foi alterado em todos os pacientes, e o achado mais frequente foi o comprometimento de cones e bastonetes. O eletrorretinograma constitui, portanto, uma ferramenta valiosa para caracterizar a disfunção visual em pacientes com o fenótipo da lipofuscinose ceróide neuronal, contribuindo para seu diagnóstico.

Gender-based normative values for pattern-reversal and flash visually evoked potentials under binocular and monocular stimulation in healthy adults.

PURPOSE: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Pattern-reversal visual evoked potentials as a diagnostic tool for ocular malingering.

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.

Dysfunction in the fellow eyes of strabismic and anisometropic amblyopic children assessed by visually evoked potentials.

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<0.001). CONCLUSIONS:: The fellow eyes of amblyopic children showed worse optotype and grating acuity, with subtle abnormalities in the PR-VEP detected as prolonged latencies for smaller size stimuli when compared with eyes of healthy children. These findings show the deleterious effects of amblyopia in several distinct visual functions, mainly those related to spatial vision.

Dysfunction in the fellow eyes of strabismic and anisometropic amblyopic children assessed by visually evoked potentials / Alterações dos potenciais visuais evocados nos olhos contralaterais de crianças com ambliopia estrabísmica e anisometrópica

ABSTRACT 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<0.001). Conclusions: The fellow eyes of amblyopic children showed worse optotype and grating acuity, with subtle abnormalities in the PR-VEP detected as prolonged latencies for smaller size stimuli when compared with eyes of healthy children. These findings show the deleterious effects of amblyopia in several distinct visual functions, mainly those related to spatial vision.

RESUMO Objetivo: Avaliar a acuidade visual e os potenciais visuais evocados transientes por reversão de padrões no olho contralateral de crianças com ambliopia estrabísmica e/ou anisometrópica. Métodos: Foram avaliados os potenciais visuais evocados de crianças com ambliopia estrabísmica e/ou anisometrópica. As acuidades visuais monoculares de grades e de optotipos foram mensuradas utilizando o PVE de varredura e a tabela EDTRS, respectivamente. Na mesma visita, foram registrados os PVERP transients de cada olho usando estímulos de ângulo visual de 60'; 15' e 7,5'. Parâmetros de amplitude (em microvolts) e latência (em milissegundos) foram determinados para os registros dos potenciais visuais evocados. Resultados: Um grupo de 40 crianças amblíopes estrábicas e/ou anisometrópicas (22 meninas - 55%, media idade= 8,7 ± 2,2, mediana= 8) foi examinado. Um grupo de 19 crianças saudáveis (13 meninas 68,4%, media idade= 8,2 ± 2,6, mediana= 8) de controle também foi incluído. A acuidade visual por optotipos foi significativamente pior (p=0,021) nos olhos contralaterais de todos os amblíopes, quando comparado com o grupo controle, independentemente se estrábico (p=0,040) ou anisometrópico (p=0,048). No geral, a acuidade visual por grades foi significativamente pior nos olhos contralaterais dos amblíopes (p=0,016), quando comparados com o grupo controle. Foi encontrada latência estatisticamente prolongada para ângulos visuais de 15' (p=0,018) e 7,5' (p=0,002) no grupo estrábico, quando comparado com o grupo controle. Para o menor estímulo visual (7,5') foi encontrada latência estatisticamente prolongada nos olhos contralaterais de todas crianças amblíopes (p<0,001). Conclusões: Os olhos contralaterais de crianças amblíopes mostraram pior acuidade visual de optotipo e de resolução de grades, com alterações sutis nos PVERP, detectadas pelas latências prolongadas para estímulos de menor tamanho, quando comparados com os olhos de crianças saudáveis. Estes resultados mostram os efeitos deletérios da ambliopia em várias funções visuais distintas, principalmente relacionadas à visão espacial.

Pattern-reversal visual evoked potentials as a diagnostic tool for ocular malingering / Potenciais visuais evocados por padrões reversos como uma ferramenta diagnóstica de simulação

ABSTRACT 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.

RESUMO Objetivo: Investigar a contribuição dos potenciais visuais evocados por padrões reversos no diagnóstico de simulação de baixa de visão em um hospital universitário do Brasil. Métodos: Um grupo de pacientes adultos com suspeita de simulação de baixa de visão em um ou ambos os olhos foi avaliado e os dados analisados retrospectivamente. Foram medidos: acuidade visual de optotipos informada para longe utilizando a tabela ETDRS, parâmetros dos potenciais visuais evocados por padrões reversos de latência (milissegundos) e amplitude (microvolts) para o componente P100 com estímulos de ângulos visuais de 15' e 60'. A motivação do paciente para a simulação foi anotada. Resultados: Os participantes foram 20 indivíduos com 11 (55%) do sexo feminino. A idade variou de 21 a 61 anos (média= 45,05 ± 11,76 anos; mediana= 49 anos). Em 8 pacientes (6 mulheres) ambos os olhos tinham acuidade visual reduzida com parâmetros dos potenciais visuais evocados por padrão reverso normais para ambos os olhos (simuladores puros). Uma subsérie separada de 12 pacientes (7 homens) tinha visão reduzida em apenas um olho e estavam simulando redução da visão no outro olho (exacerbadores). A motivação financeira foi observada em 18 pacientes (9 homens). Conclusões: Parâmetros dos potenciais visuais evocados por padrões reversos normais foram encontrados neste grupo de 20 pacientes com suspeita de simulação. Esta técnica eletrofisiológica pode ser útil como uma medida da integridade do sistema visual nesta população de doentes.

Sweep visually evoked potentials and visual findings in children with West syndrome.

BACKGROUND: West syndrome (WS) is a type of early childhood epilepsy characterized by progressive neurological development deterioration that includes vision. AIM: To demonstrate the clinical importance of grating visual acuity thresholds (GVA) measurement by sweep visually evoked potentials technique (sweep-VEP) as a reliable tool for evaluation of the visual cortex status in WS children. METHODS: This is a retrospective study of the best-corrected binocular GVA and ophthalmological features of WS children referred for the Laboratory of Clinical Electrophysiology of Vision of UNIFESP from 1998 to 2012 (Committee on Ethics in Research of UNIFESP n° 0349/08). The GVA deficit was calculated by subtracting binocular GVA score (logMAR units) of each patient from the median values of age norms from our own lab and classified as mild (0.1-0.39 logMAR), moderate (0.40-0.80 logMAR) or severe (>0.81 logMAR). Associated ophthalmological features were also described. RESULTS: Data from 30 WS children (age from 6 to 108 months, median = 14.5 months, mean ± SD = 22.0 ± 22.1 months; 19 male) were analyzed. The majority presented severe GVA deficit (0.15-1.44 logMAR; mean ± SD = 0.82 ± 0.32 logMAR; median = 0.82 logMAR), poor visual behavior, high prevalence of strabismus and great variability in ocular positioning. The GVA deficit did not vary according to gender (P = .8022), WS type (P = .908), birth age (P = .2881), perinatal oxygenation (P = .7692), visual behavior (P = .8789), ocular motility (P = .1821), nystagmus (P = .2868), risk of drug-induced retinopathy (P = .4632) and participation in early visual stimulation therapy (P = .9010). CONCLUSIONS: The sweep-VEP technique is a reliable tool to classify visual system impairment in WS children, in agreement with the poor visual behavior exhibited by them.

Contributing factors to VEP grating acuity deficit and inter-ocular acuity difference in children with cerebral visual impairment.

PURPOSE: 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). METHODS: 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 < 0.40 log units), moderate (0.40 ≤ deficit < 0.70 log units) or severe (deficit ≥0.70 log units). Maximum acceptable IAD was 0.10 log units. RESULTS: A group of 115 children (66 males-57 %) with ages ranging from 1.2 to 166.5 months (median = 17.7) was examined. VEP GAD ranged from 0.17 to 1.28 log units (mean = 0.68 ± 0.27; median = 0.71), and it was mild in 23 (20 %) children, moderate in 32 (28 %) and severe in 60 (52 %). Severe deficit was significantly associated with older age and anti-seizure drug therapy. IAD ranged from 0 to 0.49 log units (mean = 0.06 ± 0.08; median = 0.04) and was acceptable in 96 (83 %) children. Children with strabismus and nystagmus had IAD significantly larger compared to children with orthoposition. CONCLUSION: In a large cohort of children with CVI, variable severity of VEP GAD was found, with more than half of the children with severe deficits. Older children and those under anti-seizure therapy were at higher risk for larger deficits. Strabismus and nystagmus provided larger IADs. These results should be taken into account on the clinical management of children with this leading cause of bilateral visual impairment.

[Evaluation of anterior segment foreign bodies with ultrasound biomicroscopy]. / Avaliação de corpos estranhos no segmento anterior com ultrassonografia biomicroscópica.

INTRODUCTION: Ocular trauma is considered a health care problem because is an important cause for visual impairment. Intraocular foreign bodies are related to activities involving usage of metallic objects, but other materials can be present in the eye and have to be diagnosed and localized. Ultrasound biomicroscopy is an adequate subsidiary tool to evaluate an anterior chamber intraocular foreign body. PURPOSE: To characterize nature, localization, dimensions of foreign bodies and associated lesions to the anterior segment using ultrasound biomicroscopy. METHODS: Retrospective clinical study of 7,182 patient's charts submitted to ultrasound biomicroscopy examination between 1999 and 2008, totalizing 59 eyes suspected of anterior segment foreign body. RESULTS: Five cases presented two foreign bodies, fragments from the same nature, in different locations or not; and three cases had uncountable fragments in the anterior segment. Concerning localization: cornea, 11 (26%); conjunctiva, 10 (23%); iris, 10 (23%); lens, 9 (21%); sclera, 5 (12%); corneal angle, 5 (12%); ciliary body, 3 (7%). Among the associated lesions, it was identified: corneal perforation in 12 cases, corneal laceration in 2 cases, anterior synechia in 6 cases, traumatic aniridia 1 case, traumatic iridotomy in 3 cases, anterior chamber reaction in 10 cases and rupture of the anterior lens capsule in 4 cases. Considering composition: metallic, 21 (50%); non metallic, 20 were glass (48%) and 1 was vegetal (2%). The size of foreign bodies varied from 0.09 to 2.45 mm (average: 0.84 mm). CONCLUSIONS: Ultrasound biomicroscopy is useful to localize foreign bodies in the anterior segment. This imaging method can give orientation about composition, path, and localization of foreign bodies and associated lesions, thus facilitating therapeutic planning prior to intervention, avoiding additional lesions to the eye.

Avaliação de corpos estranhos no segmento anterior com ultrassonografia biomicroscópica / Evaluation of anterior segment foreign bodies with ultrasound biomicroscopy

INTRODUÇÃO: O traumatismo ocular é a causa mais importante de perda visual no mundo, sendo um problema de saúde pública. Os corpos estranhos intraoculares são relacionados a atividades que envolvem manuseio de objetos metálicos, porém outros materiais podem estar presentes, e devem ser diagnosticados e localizados no olho. A ultrassonografia biomicroscópica é uma ferramenta complementar adequada para avaliar um corpo estranho do segmento anterior do olho. OBJETIVO: Caracterizar a natureza, localização, dimensões de corpos estranhos e lesões associadas ao segmento anterior, utilizando-se ultrassonografia biomicroscópica. MÉTODO: Estudo clínico retrospectivo, por meio da análise de 7.182 prontuários dos anos de 1999 a 2008, com 59 exames de pacientes com suspeita de corpo estranho no segmento anterior ocular. RESULTADOS: Dentre os 59 exames, confirmou-se pela ultrassonografia biomicroscópica a presença de corpo estranho no segmento anterior em 42 (71,19%) olhos de 42 pacientes. A idade dos pacientes variou entre 9 e 76 anos (média= 37,4 anos). Houve predominância do sexo masculino (85,71%). Cinco casos apresentaram dois corpos estranhos, fragmentos da mesma natureza, em diversas localizações ou não; e três casos continham incontáveis fragmentos dispostos no segmento anterior. Considerando sua localização: córnea, 11 (26%); conjuntiva, 10 (23%); íris, 10 (23%); cristalino, 9 (21%); esclera, 5 (12%); seio camerular, 5 (12%); corpo ciliar, 3 (7%). Dentre as lesões associadas, identificou-se: perfuração corneana em 12 casos, laceração corneana em 2 casos, sinéquia anterior em 6 casos, aniridia traumática em 1 caso, iridotomia traumática em 3 casos, reação de câmara anterior em 10 casos e rotura de cápsula anterior do cristalino em 4 casos. Considerando a composição: metálicos, 21 (50%); não-metálicos, 20 de vidro (48%) e 1 de origem vegetal (2%). O tamanho dos corpos estranhos variou de 0,09 a 2,45 mm (média: 0,84 mm). CONCLUSÕES: A ultrassonografia biomicroscópica é útil para localizar corpos estranhos no segmento anterior. Este método de imagem pode proporcionar orientação sobre a natureza, o trajeto, a localização dos corpos estranhos e lesões associadas, facilitando o planejamento cirúrgico prévio à intervenção, evitando lesões adicionais ao olho.

INTRODUCTION: Ocular trauma is considered a health care problem because is an important cause for visual impairment. Intraocular foreign bodies are related to activities involving usage of metallic objects, but other materials can be present in the eye and have to be diagnosed and localized. Ultrasound biomicroscopy is an adequate subsidiary tool to evaluate an anterior chamber intraocular foreign body. PURPOSE: To characterize nature, localization, dimensions of foreign bodies and associated lesions to the anterior segment using ultrasound biomicroscopy. METHODS: Retrospective clinical study of 7,182 patient's charts submitted to ultrasound biomicroscopy examination between 1999 and 2008, totalizing 59 eyes suspected of anterior segment foreign body. RESULTS: Five cases presented two foreign bodies, fragments from the same nature, in different locations or not; and three cases had uncountable fragments in the anterior segment. Concerning localization: cornea, 11 (26%); conjunctiva, 10 (23%); iris, 10 (23%); lens, 9 (21%); sclera, 5 (12%); corneal angle, 5 (12%); ciliary body, 3 (7%). Among the associated lesions, it was identified: corneal perforation in 12 cases, corneal laceration in 2 cases, anterior synechia in 6 cases, traumatic aniridia 1 case, traumatic iridotomy in 3 cases, anterior chamber reaction in 10 cases and rupture of the anterior lens capsule in 4 cases. Considering composition: metallic, 21 (50%); non metallic, 20 were glass (48%) and 1 was vegetal (2%). The size of foreign bodies varied from 0.09 to 2.45 mm (average: 0.84 mm). CONCLUSIONS: Ultrasound biomicroscopy is useful to localize foreign bodies in the anterior segment. This imaging method can give orientation about composition, path, and localization of foreign bodies and associated lesions, thus facilitating therapeutic planning prior to intervention, avoiding additional lesions to the eye.

Visual acuity and retinal function in patients with Bardet-Biedl syndrome.

OBJECTIVE: Bardet-Biedl syndrome is a genetic, multisystem disorder that causes severe visual impairment. This condition is characterized by retinal dystrophy, obesity, digit anomalies, renal disease, and hypogonadism. The purpose of this study was to analyze visual acuity and full-field electroretinogram findings in patients with the Bardet-Biedl syndrome phenotype. METHODS: The visual acuity of a group of 23 patients (15 males) with ages ranging from 6-36 years (mean = 15.8 ± 6.4; median = 14.7) was assessed. Retinal function was evaluated by full-field electroretinography, and dark-adapted thresholds were assessed. RESULTS: Visual acuity in the better-seeing eye was 20/40 or better in 5 patients (21.7%), 20/50-20/150 in 13 (56.5%) patients, 20/200-20/400 in 2 (8.7%) patients and worse than 20/400 in one (4.3%) patient. The mean acuity in the better-seeing eye was 0.7 ± 0.6 logMAR (20/100, Snellen equivalent). Scotopic rod and maximal responses were nondetectable in 21 (91.3%) patients, and cone responses were non-detectable in 15 (65.2%) patients. Elevated dark-adapted visual thresholds were observed in all 19 patients who were able to be assessed, with 10 (52.6%) patients having thresholds greater than 30 dB. CONCLUSIONS: In a relatively young cohort of patients with Bardet-Biedl syndrome, only 21% had 20/40 or better vision. ERG scotopic responses were absent in the majority of cases, with cone responses being observed in less than half of cases. These findings showed the early deleterious effects in retinal function and visual acuity caused by this condition.

Visual acuity and retinal function in patients with Bardet-Biedl syndrome

OBJECTIVE: Bardet-Biedl syndrome is a genetic, multisystem disorder that causes severe visual impairment. This condition is characterized by retinal dystrophy, obesity, digit anomalies, renal disease, and hypogonadism. The purpose of this study was to analyze visual acuity and full-field electroretinogram findings in patients with the Bardet-Biedl syndrome phenotype. METHODS: The visual acuity of a group of 23 patients (15 males) with ages ranging from 6-36 years (mean = 15.8±6.4; median = 14.7) was assessed. Retinal function was evaluated by full-field electroretinography, and dark-adapted thresholds were assessed. RESULTS: Visual acuity in the better-seeing eye was 20/40 or better in 5 patients (21.7 percent), 20/50-20/150 in 13 (56.5 percent) patients, 20/200-20/400 in 2 (8.7 percent) patients and worse than 20/400 in one (4.3 percent) patient. The mean acuity in the better-seeing eye was 0.7±0.6 logMAR (20/100, Snellen equivalent). Scotopic rod and maximal responses were nondetectable in 21 (91.3 percent) patients, and cone responses were non-detectable in 15 (65.2 percent) patients. Elevated darkadapted visual thresholds were observed in all 19 patients who were able to be assessed, with 10 (52.6 percent) patients having thresholds greater than 30 dB. CONCLUSIONS: In a relatively young cohort of patients with Bardet-Biedl syndrome, only 21 percent had 20/40 or better vision. ERG scotopic responses were absent in the majority of cases, with cone responses being observed in less than half of cases. These findings showed the early deleterious effects in retinal function and visual acuity caused by this condition.