Simultaneous Unilateral Abducens Nerve Palsy and Contralateral Anterior Ischaemic Optic Neuropathy as the Presenting Signs of Giant Cell Arteritis.

Ischaemic optic neuropathy is the most common, feared, and recognised ocular manifestation of giant cell arteritis (GCA), while extraocular muscle palsy rarely occurs in the disease. Overlooking the diagnosis of GCA in aged patients with acquired diplopia and strabismus is not only sight- but also life-threatening. Here, we present, for the first time, a case of unilateral abducens nerve palsy and contralateral anterior ischaemic optic neuropathy as the presenting signs of GCA in a 98-year-old woman. Prompt diagnosis and treatment prevented further visual loss and systemic complications and allowed for rapid resolution of the abducens nerve palsy. We also aim to discuss the possible pathophysiological mechanisms of diplopia in GCA and to emphasise that acquired cranial nerve palsy must raise suspicion of this severe disease in elderly patients, particularly in association with ischaemic optic neuropathy.

Retinitis pigmentosa and nanophthalmos in a patient with attenuated Hunter's syndrome.

PURPOSE: To describe a case of retinitis pigmentosa and nanophthalmos in a patient with attenuated Hunter's syndrome. METHODS: Fundus photography, total field electroretinogram, ultrasound, computerized visual field examination, biochemical examination and genetic testing were obtained. RESULTS: The fundus exam showed diffuse arteriolar attenuation, optic disc with regular contours, and pigment agglomerates like "bone spicules" in the middle periphery. Ultrasound examination revealed scleral thickening and short axial diameter in both eyes. The total field electroretinogram exam showed a subnormal result with greater impairment of the scotopic phase of the exam. Computerized visual field examination demonstrated a diffuse reduction in retinal sensitivity in the periphery. Biochemical examination showed increased urine glycosaminoglycan excretion and iduronate-2-sulphatase activity (IDS) deficiency in leukocytes, confirming the type II mucopolysaccharidosis. Molecular analysis revealed a novel missense mutation (p.A77D) in the IDS gene. CONCLUSION: The case report is about a patient presented an attenuated form of the syndrome, with no cognitive impairment. Ophthalmologic follow-up is still an important part of multidisciplinary treatment for Hunter's syndrome.

Quality of Life (QoL) in Non-Acute Vogt-Koyanagi-Harada Disease (VKHD) at Two Time Points 24 Months Apart.

AIMS: To investigate the changes in quality-of-life (QoL) metrics at a 24-month interval in non-acute VKHD patients and their association with inflammation, treatment, and visual function. METHODS: SF-36 and VFQ-25 questionnaires were administered at two 24-month-apart moments to 22 non-acute VKHD patients followed for ≥12 months since acute disease onset. "Improvement," "unchanged," or "worsening" in questionnaires scores (difference >5-point) between M1 and M2 and their associations were sought. RESULTS: Absence of systemic treatment or optic disc hyperfluorescence was associated with improved general health (SF-36). Improvement in binocular contrast sensitivity resulted in better ocular pain score; absence of anterior uveitis relapse, stable fundus findings, no use of cyclosporine or no intravitreal injections resulted in unchanged/better dependency score; no intravitreal injections resulted in unchanged/better mental health score (VFQ-25). CONCLUSION: Stability/improvement in QoL scores was associated with controlled inflammation, better visual function, and no need for treatment. Subclinical inflammatory signs did not impact QoL scores.

Electroretinography reveals retinal dysfunction in Parkinson's disease despite normal high-resolution optical coherence tomography findings.

INTRODUCTION: Parkinson's disease (PD)-associated inner retinal abnormalities, particularly the retinal ganglion cells (RGC) layer, on optical coherence tomography (OCT) have recently gained importance as a biomarker of non-motor involvement of the disease but functional RGC evaluation using photopic negative response (PhNR) has not yet been determined. This study aims to compare structural and functional findings of the retina and optic nerve in PD with healthy controls (CT) including PhNR and OCT. METHODS: Forty-one eyes of 21 PD patients and 38 eyes of 19 CT underwent ophthalmic examination including visual contrast sensitivity test (CS), OCT, light-adapted full-field electroretinography (ffERG), and PhNR. OCT was used to measure the peripapillary retinal nerve fiber layer, the segmented macular layers, and the choroid. For functional parameters, CS, ffERG (oscillatory potentials, photopic response, 30 Hz-flicker), and PhNR waves were used. Measurements were compared using generalized estimating equation and significance was set at P ≤ 0.05. RESULTS: The PD group presented a significantly lower mono- and binocular CS, oscillatory potentials amplitude, b-wave amplitude on ffERG (152.3[45.4] vs 187.1[32.7]µV; P = 0.002), and PhNR amplitude (135.0[35.0] vs 156.3[34.1]µV; P = 0.025). There was no statistically significant difference in OCT measurements between groups. No correlation was found between statistically significant measurements and clinical data. CONCLUSIONS: Functional abnormalities on CS, ffERG, and PhNR can be detected in PD even when structural damages are not observed on OCT. PhNR represents a new potential biomarker in PD. Our findings indicate dysfunction of bipolar, amacrine, and retinal ganglion cells in PD, probably with a cellular dysfunction overcoming morphological damage.

Fingerprint sign in Vogt-Koyanagi-Harada disease: a case series.

BACKGROUND: The tomographic finding, which has been called the "fingerprint sign" in en face reconstructions, seems to be the result of a variety of processes that cause distension of the outer plexiform layer (OPL) and the Henle fiber layer (HFL). The aim of this paper is to describe the appearance of concentric rings at the OPL/HFL interface visualized using en face reconstructions of cross-sectional optical coherence tomography images of patients with Vogt-Koyanagi-Harada disease. METHODS: Retrospective analysis of images of six eyes of three patients obtained by cross-sectional OCT imaging and en face reconstruction at the level of the OPL/HFL interface. RESULTS: All eyes presented with a dentate or saw-tooth pattern of the OPL/HFL interface on cross-sectional OCT with corresponding concentric rings on en face OCT reconstruction, consistent with the recently published "fingerprint sign". Initial OPL/HFL interface changes were observed between the first and fourth months after treatment and resolution of VKHD associated serous retinal detachments. These OPL/HFL interface changes have persisted for many years following the resolution of the active inflammation. CONCLUSIONS: Changes in the OPL/HFL interface can be identified following successful treatment of VKHD. These included both a dentate or saw-tooth pattern on cross-sectional imaging and concentric rings or the "fingerprint sign" on en face reconstructions. These changes persisted for many years despite disease quiescence.

Impact of Inflammation and Treatment on Self-reported Quality of Life in Patients with Non-acute Vogt-Koyanagi-Harada Disease (VKHD).

Aims: To evaluate associations between vision-related (VR-) and health-related (HR-) QoL metrics and inflammation and treatment in non-acute VKHD patients.Methods: Cross-sectional study in a tertiary center in Sao Paulo, Brazil with 22 patients with non-acute VKHD followed prospectively for ≥12 months since acute disease onset, with systematic evaluation and predefined treatment protocols. VR- and HR-QoL aspects were assessed by VFQ-25 and SF-36 questionnaires, respectively. Associations between the questionnaire's subscale item scores with inflammation and systemic medical therapies were assessed.Results: After generalized linear model analysis, worse VA, severe fundus changes, fluctuation of VA and fluctuation of anterior chamber cells impacted negatively on VR-QoL items. Higher cumulative total dose of corticosteroids and use of immunosuppressive therapy impacted negatively on both questionnaires.Conclusion: Worse VA, clinical inflammation and systemic treatment have a significant impact on VR- and HR-QoL questionnaires. Subclinical choroidal inflammation did not seem to impact QoL.

Associations between functional and structural measurements in non-acute Vogt-Koyanagi-Harada disease.

PURPOSE: To evaluate associations between functional and structural measurements in patients with non-acute VKHD. METHODS: In this cross-sectional study, 16 non-acute VKHD patients (32 eyes; 14 female) were evaluated with multifocal electroretinogram (mfERG), standardized automated perimetry (SAP) and optical coherence tomography (OCT)examinations. All included patients had a minimum 12 months of follow-up from acute onset and were participants of an ongoing prospective study since acute phase with systematic clinical imaging evaluations and electroretinogram examinations within a predefined treatment. Age- and gender-matched controls were included. Main outcomes were functional and structural abnormalities and their correlation; secondary outcome was correlation of these findings with clinical characteristics, including fundus abnormalities. RESULTS: SAP and mfERG parameters were significantly worse in patients than in controls. Fourteen eyes (43.7%) had disrupted ellipsoid zone (EZ); visual acuity (VA) was similar between eyes with intact or disrupted EZ. Eyes with intact and disrupted EZ differed significantly concerning N1 and P1 amplitudes and N1 peak time values on mfERG and mean sensitivity (MS), central sensitivity (CS), foveal threshold, visual field index, mean deviation (MD) and pattern standard deviation values on SAP. The area under the curve on receiver operating curves for P1 amplitude was 0.81 (cut-off value = 34.7 nV/deg2 ) and for MD value was 0.84 (cut-off value = -5.2 dB). Central retinal thickness (CRT) significantly correlated with N1 and P1 amplitudes and P1 peak time values on mfERG (r = 0.354, r = 0.442 and r = -0.405, respectively) and MD, MS, CS and fovea threshold (log values) on SAP (r = 0.372, r = 0.406, r = 0.431 and r = 0.414, respectively). Statistically significant associations were found with the presence of peripapillary atrophy and recurrent anterior uveitis with a worse MD value (p = 0.004 and p < 0.001, respectively). CONCLUSION: In non-acute VKHD, disrupted EZ and reduced CRT were correlated with impaired mfERG and SAP parameters, even in patients with good VA.

Self-Reported Quality of Life in Patients with Long-Standing Vogt-Koyanagi-Harada Disease.

Purpose: To assess health-related (HR-) and vision-related (VR-) quality of life (QoL) in patients with long-standing Vogt-Koyanagi-Harada disease (VKHD).Methods: Cross-sectional study of 49 patients with disease duration ≥12 months followed at Uveitis Service, Universidade de São Paulo, BR, for at least 12 months. HR- and VR-QoL were evaluated using SF-36 and NEI VFQ-25 questionnaires, respectively. Demographic, clinical and visual function data were compared with questionnaire scores.Results: After generalized linear models, lower mensal household income was associated with lower scores in both questionnaires while unemployment was associated with SF-36 questionnaire only. Treatment with peri-/intraocular medications and ocular surgery were associated with higher scores on SF-36 questionnaire. Worse visual acuity (VA), ocular complications and no ocular surgery were related to lower scores on NEI VFQ-25 questionnaire.Conclusions: On HR- and VR-QoL questionnaires difficulties perceived by patients with long-standing VKHD were mainly associated with socio-economic aspects, VA, local treatment and ocular complications.

Phacoemulsification and 1% atropine eye drops for treatment of antimetropic congenital microcoria associated with cataracts / Facoemulsificação e colírio de atropina a 1% para tratamento de microcoria congênita antimetrópica associada a catarata

ABSTRACT A rare case of bilateral congenital microcoria associated with antimetropia in a 47-year-old man is here described. The patient presented with a chief complaint of progressive vision loss in his right eye over the past five years. A slit-lamp examination and ultrasound biomicroscopy confirmed congenital microcoria and cataracts. Phacoemulsification was performed using an iris expansion device and the anterior capsule was stained using the "trypan down under" technique. Preoperative considerations, the surgical approach, and postoperative management are discussed.

RESUMO Um caso raro de microcoria congênita bilateral associada à antimetropia em um homem de 47 anos de idade é descrito aqui. O paciente queixava-se de perda visual progressiva em seu olho direito nos últimos 5 anos. Um exame com lâmpada de fenda e biomicroscopia ultrassônica confirmaram microcoria congênita e catarata. A facoemulsificação foi realizada usando dispositivo de expansão iriana, e a cápsula anterior foi corada através da técnica "trypan down under". Considerações pré-operatórias, abordagem cirúrgica e manejo pós-operatório são discutidos.

Phacoemulsification and 1% atropine eye drops for treatment of antimetropic congenital microcoria associated with cataracts.

A rare case of bilateral congenital microcoria associated with antimetropia in a 47-year-old man is here described. The patient presented with a chief complaint of progressive vision loss in his right eye over the past five years. A slit-lamp examination and ultrasound biomicroscopy confirmed congenital microcoria and cataracts. Phacoemulsification was performed using an iris expansion device and the anterior capsule was stained using the "trypan down under" technique. Preoperative considerations, the surgical approach, and postoperative management are discussed.

Full-field electroretinogram behavior in Vogt-Koyanagi-Harada disease: a 24-month longitudinal study in patients from acute onset evaluated with multimodal analysis.

PURPOSE: To prospectively evaluate the dynamic changes of the full-field electroretinogram (ff-ERG) and its association with inflammatory signs in patients with Vogt-Koyanagi-Harada disease (VKHD) followed up after acute onset. METHODS: Twelve acute VKHD patients, who were followed up for at least 24 months, were enrolled at a tertiary center from June 2011 to January 2017. Treatment consisted of intravenous methylprednisolone followed by 1 mg/kg/day of oral prednisone with a slow tapering associated with late non-steroidal immunosuppressive therapy in previously defined cases. Inflammation was systematically evaluated with clinical and posterior segment imaging (PSI) exams (fluorescein angiography, FA, indocyanine green angiography, ICGA, enhanced depth imaging optical coherence tomography, EDI-OCT). A ff-ERG was performed upon enrollment as well as at predefined intervals. Scotopic ff-ERG parameters changes between the 12th and 24th months defined the ERG-stable or ERG-worsening groups. "Flare" was defined as an appearance or worsening of inflammatory signs (after the initial 6 months following disease onset) under the predefined treatment protocol. RESULTS: ff-ERG parameters initially improved in all eyes; in the evaluation between the 12th and 24th months, ff-ERG results were stable in 17 eyes (71 %) and worsened in 7 eyes (29 %). Subnormal ff-ERG results were observed in 15 eyes (62 %) at the 24th month. On the other hand, the flare was observed in 8 eyes (33 %) as cells in the anterior chamber and in 24 eyes (100 %) as any PSI inflammatory sign. The ERG-worsening group presented thicker subfoveal choroid at the first month (p = 0.001) and fluctuations in choroidal thickness more often during follow-up when compared to the ERG-stable group (p = 0.02). CONCLUSIONS: Scotopic ff-ERG parameters worsened between the 12th and 24th months in a quarter of the patients. Subclinical inflammation detected as an increase in CT seems to be related to worsening in visual function measured with ffERG.

Prospective evaluation of intravitreal bevacizumab for ischemic central retinal vein occlusion.

BACKGROUND: Although previous studies have evaluated the effect of anti-VEGF therapies for central retinal vein occlusion (CRVO) patients, the majority of previous studies have excluded or included a very small number of patients with ischemic CRVO (iCRVO). The aim of our study is to examine the effects of bevacizumab on macular edema secondary to ischemic central retinal vein occlusion, as well as the effects on central choroidal thickness and best-corrected visual acuity. METHODS: In this prospective, interventional case series, iCRVO was defined by the presence of ≥ 10 or more disc diameter areas of retinal nonperfusion by fluorescein angiography (FA) and by the presence of a b/a ratio less than 1.5 by full-field electroretinogram (ffERG). Nine eyes with iCRVO received monthly bevacizumab 0.5 mg injections at baseline and months 1 to 5 for a maximum of six injections. Main outcome measures were visual acuity (Snellen), central foveal thickness, and central choroidal thickness as measured by Spectral-Domain Optical Coherence Tomography (SD-OCT) at baseline and at 6 month following initial intravitreal bevacizumab injection. Pairwise t-tests and the Wilcoxon signed-rank test were conducted to compare the outcome measures. RESULTS: After intravitreal administration of bevacizumab, there was a significant reduction of central foveal thickness from 858 ± 311 µm at baseline to 243 ± 106 µm at the 6-month follow-up, as well as a significant reduction of central choroidal thickness from 282 ± 38 µm at baseline to 227 ± 56 µm at the 6-month follow-up (p = 0.0006, p = 0.0003 respectively). The visual acuity worsened from a median of 1.3 to 1.7 (p = 0.02). CONCLUSION: In patients with iCRVO, intravitreal bevacizumab led to a reduction of central macular edema and central choroidal thickness, but a worsening of visual acuity. Intravitreal bevacizumab reduces macular edema but is not able to overcome the poor prognosis of iCRVO.

Multimodal image analysis of the retina in Hunter syndrome (mucopolysaccharidosis type II): Case report.

INTRODUCTION: We report a case of retinal and posterior ocular findings in a 33-year-old man diagnosed with Hunter syndrome (Mucopolysaccharidosis type II) in a multimodal imaging way. CASE PRESENTATION: Our patient was complaining of blurred night vision for the past 3 years. He had not received any systemic treatment for Hunter syndrome. Vision acuity was 20/20 in both eyes and corneas were clear. Fundus examination revealed bilateral crowded and hyperemic optic nerve heads (elevated in the ocular ultrasound) and areas of subretinal hypopigmentation. There was hyperautofluorescence at the central fovea and perifovea, and a diffuse bilateral choroidal fluorescence in angiography. Macular SD-OCT showed a thinning of the external retina at the perifovea in both eyes. Visual field testing showed a bilateral ring scotoma. The full field ERG was subnormal, with a negative response in the scotopic phase. Visual Evoked Potencial test and cranial MRI were normal. CONCLUSION: Our multimodal analysis reported here attempted to contribute to the knowledge of the natural history of GAG deposition in the eye, focusing on the retina and retinal pigment epithelium. Defining this natural history is essential for a proper comparison with Hunter patients receiving systemic treatment, thus determining if it can or cannot improve retinal function in humans with this disorder.

Ophthalmologic manifestations of celiac disease.

Celiac disease is an autoimmune disorder that affects the small intestine of genetically predisposed individuals. Ophthalmic manifestations are within the extra-intestinal manifestations, and can be divided into those of autoimmune disorders or those due to absorptive disabilities. This article reviewed the ophthalmologic manifestation of celiac disease. Ophthalmic symptoms are rare, but should be investigated in patients with celiac disease and taken into consideration as the first systemic manifestation.

The role of pattern-reversal electroretinography in the diagnosis of glaucoma / Importância do eletroretinograma de padrão reverso no diagnóstico do glaucoma

Pattern electroretinography is used to assess the function of the inner retinal layers, particularly the retinal ganglion cell layer, using a reversing checkerboard or grating pattern that maintains a constant overall mean luminance over time. A normal transient response comprises a positive component of the wave (P50) followed by a longer negative component of the wave (N95). Glaucomatous optic neuropathy causes progressive loss of retinal ganglion cells, potentially detectable as abnormalities on examination, particularly in the N95 component. Therefore, pattern electroretinography may be useful in the diagnosis and evaluation of glaucoma. The present article is an updated review of published data regarding the use of pattern electroretinography for the detection of glaucoma-induced retinal changes.

O eletroretinograma de padrão reverso é utilizado para avaliar a função das camadas internas da retina, particularmente a camada de células ganglionares retinianas, utilizando um estímulo em xadrez ou barras alternantes, mantendo constante o nível de contraste total. A resposta transiente normal é constituída por uma onda positiva (P50) seguida de uma onda maior negativa (N95). A neuropatia óptica glaucomatosa causa perda progressiva das células ganglionares da retina, detectável como anormalidades no exame, especialmente na onda N95. Por isso, o eletroretinograma de padrão reverso pode ser útil no diagnóstico e seguimento de pacientes glaucomatosos. Este artigo é uma revisão atualizada dos dados publicados a respeito da capacidade do eletroretinograma de padrão reverso em detectar alterações retinianas induzidas pelo glaucoma.

Progressão atípica de perda visual em paciente com glaucoma primário de ângulo aberto / Atypical progression of visual loss in a patient with primary open-angle glaucoma

A dolicoectasia da artéria carótida interna (ACI) é uma condição rara que pode ser acompanhada de manifestações neuro-oftalmológicas, como perda da acuidade e alteração do campo visual decorrente da compressão do nervo óptico (NO). O objetivo é relatar um caso de paciente do sexo masculino, 67 anos, portador de glaucoma primário de ângulo aberto (GPAA) com evolução atípica, assimetria de escavação, palidez da rima do NO à esquerda, devido à neuropatia óptica compressiva à esquerda, por segmento dolicoectásico da ACI. O diagnóstico foi baseado na história clínica, aspecto do NO e exames de neuroimagem.

Dolichoectasia of the internal carotid artery (ICA) is a rare condition that may be associated with neuro-ophthalmic manifestations, such as loss of visual acuity and visual field resulting from compression of the optic nerve (ON). The aim is to report a 67-year-old male patient with primary open-angle glaucoma (POAG) with atypical evolution, asymmetry of cupping and increased pallor of the rim of the left ON, due to compressive optic neuropathy by the dolichoectatic segment. The diagnosis was based on clinical history, appearance of the ON and neuroimaging.

The role of pattern-reversal electroretinography in the diagnosis of glaucoma.

Pattern electroretinography is used to assess the function of the inner retinal layers, particularly the retinal ganglion cell layer, using a reversing checkerboard or grating pattern that maintains a constant overall mean luminance over time. A normal transient response comprises a positive component of the wave (P50) followed by a longer negative component of the wave (N95). Glaucomatous optic neuropathy causes progressive loss of retinal ganglion cells, potentially detectable as abnormalities on examination, particularly in the N95 component. Therefore, pattern electroretinography may be useful in the diagnosis and evaluation of glaucoma. The present article is an updated review of published data regarding the use of pattern electroretinography for the detection of glaucoma-induced retinal changes.

Multifocal pattern electroretinography for the detection of neural loss in eyes with permanent temporal hemianopia or quadrantanopia from chiasmal compression.

AIMS: To evaluate the ability of multifocal transient pattern electroretinography (mfPERG) to detect neural loss and assess the relationship between mfPERG and visual-field (VF) loss in eyes with chiasmal compression. METHODS: 23 eyes from 23 patients with temporal VF defects and band atrophy of the optic nerve and 21 controls underwent standard automated perimetry and mfPERG using a stimulus pattern of 19 rectangles, each consisting of 12 squares. The response was determined for the central rectangle, for the nasal and temporal hemifields (eight rectangles each) and for each quadrant (three rectangles) in both patients and controls. Comparisons were made using variance analysis. Correlations between VF and mfPERG measurements were verified by linear regression analysis. RESULTS: Mean ± SD mfPERG amplitudes from the temporal hemifield (0.50 ± 0.17 and 0.62 ± 0.32) and temporal quadrants (superior 0.42 ± 0.21 and 0.52 ± 0.35, inferior 0.51 ± 0.23 and 0.74 ± 0.40) were significantly lower in eyes with band atrophy than in controls (0.78 ± 0.24, 0.89 ± 0.28, 0.73 ± 0.26, 0.96 ± 0.36, 0.79 ± 0.26 and 0.91 ± 0.31, respectively). No significant difference was observed in nasal hemifield measurements. Significant correlations (0.36-0.73) were found between VF relative sensitivity and mfPERG amplitude in different VF sectors. CONCLUSIONS: mfPERG amplitude measurements clearly differentiate eyes with temporal VF defect from controls. The good correlation between mfPERG amplitudes and the severity of VF defect suggests that mfPERG may be used as an indicator of ganglion cell dysfunction.

Pattern-reversal electroretinograms for the diagnosis and management of disorders of the anterior visual pathway.

The pattern electroretinogram is an electrophysiological test that assesses the function of inner retinal layers, particularly the ganglion cells layer of retina, using a reversing checkerboard or grating pattern that produces no change in average luminance over time. The normal pattern electroretinogram is composed of a proeminent positive component (P50) and a large later negative component (N95). Since structural damage that compromises the retinal ganglion cell layer can lead to pattern electroretinogram changes, particularly in the N95 amplitude, the test can be useful in the treatment of a number of anterior visual pathway diseases. In this article, we review the methods for recording pattern electroretinogram and its usefulness in the diagnosis and management of diseases including inflammatory, hereditary, ischemic and compressive lesions of the anterior visual pathway.