Macular Telangiectasia type 2: multimodal assessment of retinal function and microstructure.

PURPOSE: To assess the impact of neurodegenerative morphologic alterations due to macular telangiectasia type 2 (MacTel) on microperimetry (MP) and multifocal electroretinography (mfERG). METHODS: Thirty-five eyes of 18 patients with MacTel were examined using spectral domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), mfERG and MP. Software was used to match SD-OCT B-scans with the corresponding retinal sensitivity map and multifocal electroretinograms (mfERGs), thus enabling direct structure/function correlation. RESULTS: Loss of the ellipsoid zone (EZ) had the strongest negative association with retinal sensitivity (16.77 dB versus 4.58 dB, adj. p < 0.001) of all parameters examined, and a limited negative effect on mfERGs (0.32 SD versus -1.97 SD adj. p = 0.121). Ellipsoid zone (EZ) irregularity was associated with reduced MP values but preserved mfERGs. There was a significant association between areas of inner retinal hyporeflectivity and loss of MP sensitivity (adj. p < 0.001) but the reduction in sensitivity was less than in locations with EZ loss. Areas of mfERG abnormality showed similar sensitivity loss with either inner retinal hyporeflectivity or EZ loss (adj. p = 0.063). In areas with EZ loss alone, preservation of the external limiting membrane (ELM) was associated with higher MP values than in areas with additional ELM loss; the integrity of the ELM alone was not associated with changes either in MP or mfERG. Increased FAF was observed in 51% of eyes, mixed/reduced FAF in 40%, and no abnormality was detected in 9% of eyes. CONCLUSION: The data suggest both MP and mfERG to be useful non-invasive modalities for detecting localised macular dysfunction in MacTel. The findings suggest a different sensitivity of the two modalities to inner and outer retinal changes in macular function and are therefore complementary.

Applying a New Automated Perimetry Pattern Based on the Stimulus Distribution of the Multifocal ERG to Improve Structure-Function Investigation in Glaucoma.

PURPOSE: To validate a new automated perimetry pattern (mf103 pattern) for the investigation of retinal structure-function relationships in glaucoma in comparison to the standard G2 pattern and to relate either field's performance to optical coherence tomography (OCT). METHODS: Automated perimetry data from the mfERG103 pattern were compared with the standard G2 pattern in glaucoma patients (18) and controls (15). The results of both (mean defect (MD) and mean sensitivity (MS)) were compared with optical coherence tomography (OCT): retinal nerve fiber layer (RNFL) thickness, macular thickness (mT), and ganglion cell analysis (GCIPL). Nine patients were followed up after one year. RESULTS: G2 pattern and mf103 pattern did not differ significantly in MD or MS. The mf103 pattern associated significantly with more RNFL sectors in both MD and MS (p < 0.01 and p < 0.05, resp.). GCIPL thickness was not significantly associated with either SAP protocols. Both protocols remained comparable after one-year follow-up. CONCLUSIONS: G2 and mf103 pattern can both differentiate patients from controls with no significant difference in performance. RNFL thickness defects correlated better with mf103 than G2 with POAG. The mfERG-103 perimetry pattern can be used to establish structure-function correlations in glaucoma and may enable a more direct comparison with objective electrophysiological data.

Ganglion cell layer segmentation and the two-flash multifocal electroretinogram improve structure function analysis in early glaucoma.

PURPOSE: To improve structure-function analysis in primary open-angle glaucoma (POAG) by including the two-global flash multifocal electroretinogram (2F-mfERG) and macular ganglion cell layer segmentation. METHODS: Twenty-five glaucoma patients (six pre-perimetric (PPG), 19 POAG) and 16 controls underwent 2F-mfERG, optical coherence tomography (OCT), and standard automated perimetry (SAP). For 2F-mfERG, the root mean square was calculated for the focal flash response at 15-45 ms (DC) and the global flash responses at 45-75 ms (IC1) and 75-105 ms (IC2). For OCT, macular total thickness (mT) and ganglion cell-inner plexiform layer (GCIPL) thickness were analysed. Values from the central 10° and 15° of 2F-mfERG were compared to the corresponding areas from OCT and visual field. RESULTS: Both PPG and POAG had significantly lower mfERG responses in the central 10° and 15° than the control group. Of the glaucoma patients, 30.7% (three PPG, five POAG) showed central mfERG and GCIPL reduction without a SAP defect in the central 15 degrees. Four patients had a central SAP defect associated with a reduced GCIPL without any detectable dysfunction on mfERG. MfERG DC and IC2 were larger with increased mT (p ≤ 0.02), but GCIPL only related positively to IC2 (p = 0.027). SAP sensitivity also increased with thicker mT but not with GCIPL (p < 0.03 and p = 0.35). DC, IC2, and GCIPL could best differentiate glaucoma from control (AUC values: 0.897, 0.903, and 0.905). CONCLUSIONS: Structure function analysis in glaucoma can be improved when the GCIPL thickness as well as the 2F-mfERG is included as these measures complement information obtained by SAP.

Wavelet decomposition analysis in the two-flash multifocal ERG in early glaucoma: a comparison to ganglion cell analysis and visual field.

PURPOSE: To further improve analysis of the two-flash multifocal electroretinogram (2F-mfERG) in glaucoma in regard to structure-function analysis, using discrete wavelet transform (DWT) analysis. METHODS: Sixty subjects [35 controls and 25 primary open-angle glaucoma (POAG)] underwent 2F-mfERG. Responses were analyzed with the DWT. The DWT level that could best separate POAG from controls was compared to the root-mean-square (RMS) calculations previously used in the analysis of the 2F-mfERG. In a subgroup analysis, structure-function correlation was assessed between DWT, optical coherence tomography and automated perimetry (mf103 customized pattern) for the central 15°. RESULTS: Frequency level 4 of the wavelet variance analysis (144 Hz, WVA-144) was most sensitive (p < 0.003). It correlated positively with RMS but had a better AUC. Positive relations were found between visual field, WVA-144 and GCIPL thickness. The highest predictive factor for glaucoma diagnostic was seen in the GCIPL, but this improved further by adding the mean sensitivity and WVA-144. CONCLUSIONS: mfERG using WVA analysis improves glaucoma diagnosis, especially when combined with GCIPL and MS.

Comparing DTL microfiber and Neuroline skin electrode in the Mini Ganzfeld ERG.

BACKGROUND: In infant ERG recordings skin electrodes frequently result in a better compliance. In order to assess the quality of such recordings, we compared the recording characteristics of DTL microfiber and Neuroline surface electrodes using a modified ISCEV protocol in the Mini Ganzfeld ERG. METHODS: A prospective cohort study on healthy adult subjects was conducted at the Department of Ophthalmology, University of Basel, Switzerland. Thirty healthy volunteers were tested. The microfiber electrode (DTL Plus Electrode) was placed across the cornea, above the lower eyelid. The Neuroline skin electrode was placed on the surface of the lower lid on the opposite eye. The eye on which each electrode type was placed was randomised. Amplitudes of the rod, standard combined, standard flash cone, light-adapted 3.0 Hz flicker and red cone responses were analysed, as well as their respective implicit times. RESULTS: Both electrode recordings showed the same waveform characteristics. Responses with the Neuroline electrode were significantly weaker than those from the DTL electrode. Amplitudes of the rod, standard combined, standard flash cone, light-adapted 3.0 Hz flicker and red cone responses were up to four times larger when recorded with the DTL electrode (p < 0.005, ANOVA). Implicit times of the red cone ERGs were slightly faster for the Neuroline skin electrode recordings (p ≤ 0.039). CONCLUSIONS: Comparison of full-field ERG recordings with microfiber DTL and Neuroline skin electrodes showed that DTL electrodes produce larger ERGs. Hence, we provide evidence that both electrode types allow successful full-field ERG recording, although separate normative data for both electrodes are necessary.

Comparison of Two Different OCT Systems: Retina Layer Segmentation and Impact on Structure-Function Analysis in Glaucoma.

Purpose. To compare two different spectral-domain optical coherence tomography (OCT) systems in regard to full macular thickness (MT) and ganglion cell layer-inner plexiform layer (GCIPL) measures and in regard to structure-function correlation when compared to standard automated perimetry (SAP). Methods. Seventeen primary open angle glaucoma patients and 16 controls (one eye per subject) were enrolled. MT and GCIPL thicknesses were measured by Cirrus and Spectralis OCTs. Octopus Perimeter 101 (G2 protocol) reports sensitivity in mean defect (dB). Differences between measurements were assessed with Student's t-test and Bland Altman. Diagnostic performance was also compared between each parameter calculating the areas under the operator receiver (ROC). Linear models were used to investigate structure-function association between OCT and SAP. Results. Disagreement between OCTs in both MT and GCIPL values was significant. Spectralis values were thicker than Cirrus. Average difference between OCTs was 21.64 µm (SD 4.5) for MT and 9.8 µm (SD 5.4) for GCIPL (p < 0.001). Patients differed significantly from controls in both OCTs, in both measurements. MT and GCIPL were negatively associated with MD (p < 0.001). Conclusions. Although OCT values were not interchangeable, both machines differentiated patients from controls with statistical significance. Structure-function analysis results were comparable, when either OCT was compared to SAP.

A possible early sign of hydroxychloroquine macular toxicity.

PURPOSE: Hydroxychloroquine (HCQ) has a low risk of retinal toxicity which increases dramatically with a cumulative dose of >1000 g. Here we report a case of HCQ macular toxicity presentation in a young patient with a cumulative dose of 438 g. METHODS: A 15-year-old female started attending annual consultations for retinal toxicity screening in our clinic after 3 years of HCQ treatment for juvenile idiopathic dermatomyositis. She had been diagnosed at age 12 and had been on hydroxychloroquine 200 mg/day, cyclosporin 150 mg/day and vitamin D3 since. Screening consultations included: complete ophthalmologic examination, automated perimetry (AP, M Standard, Octopus 101, Haag-Streit), multifocal electroretinogram (VERIS 6.06™, FMSIII), optical coherence tomography (OCT, fast macular protocol, Cirrus SD-OCT, Carl Zeiss), fundus autofluorescence imaging (Spectralis OCT, Heidelberg Engineering Inc.) and color testing (Farnsworth-Panel-D-15). RESULTS: After 5 years of treatment, AP demonstrated reduced sensibility in only one extra-foveal point in each eye (p < 0.2). Even though other exams showed no alteration and the cumulative dose was only around 353 g, consultations were increased to every 6 months. After 2-year follow-up, that is, 7 years of HCQ, a bilateral paracentral macula thinning was evident on OCT, suggestive of bull's eye maculopathy. However, the retinal pigmented epithelium appeared intact and AP was completely normal in both eyes. Further evaluation with ganglion cell analysis (GCA = ganglion cell + inner plexiform layer, Cirrus SD-OCT, Carl Zeiss) showed a concentric thinning of this layer in the same area. Although daily and cumulative doses were still under the high toxicity risk parameters, HCQ was suspended. At a follow-up 1 year later, visual acuity was 20/16 without any further changes in OCT or on any other exam. CONCLUSIONS: This may be the first case report of insidious bull's eye maculopathy exclusively identified using OCT thickness analysis, in a patient in whom both cumulative and daily dosages were under the high-risk parameters for screening and the averages reported in studies. As ganglion cell analysis has only recently become available, further studies are needed to understand toxicity mechanisms and maybe review screening recommendations.

Impact of a Digital Power Line Filter in the 2-Global-Flash Multifocal Electroretinogram of Glaucoma Patients Compared to Controls.

PURPOSE: To assess the effect of a 50 Hz power line digital filter on the response to a 2-global-flash multifocal electroretinogram (mfERG) in primary open angle glaucoma (POAG) compared to control. MATERIALS AND METHODS: A 2-global-flash mfERG (VERIS™) was recorded (23 control, 34 POAG). Eight recordings were noise contaminated: 4 control, 4 POAG. Response averages from the central 10° and 7 surrounding groups were analyzed with and without a 50 Hz digital filter for the following mfERG response epochs: direct component (15-45 ms), 1st (45-75 ms) and 2nd (75-105 ms) induced components. RESULTS: A digital 50 Hz filter had little effect on uncontaminated with noise waveforms but, in noisy recordings, changed the waveform dramatically to resemble uncontaminated waveforms. In controls' 50 Hz-filtered uncontaminated with noise mfERGs differed significantly from unfiltered responses in induced components. Uncontaminated with noise recordings from glaucoma patients did not differ with or without the notch filter (p > 0.1 for all three epochs of mfERG). The mfERG response in the central 10°in glaucoma patients differed significantly from controls, whether the notch filter was used or not (p < 0.001). CONCLUSIONS: A 50 Hz notch filter allows grossly contaminated waveforms to be analyzed in a meaningful manner. With a 50 Hz filter, glaucoma patients still differed significantly from normal.

Structural and functional changes in glaucoma: comparing the two-flash multifocal electroretinogram to optical coherence tomography and visual fields.

PURPOSE: To correlate multifocal electroretinogram (mfERG) findings in the macular area of glaucoma patients with automated perimetry (visual fields) and with optical coherence tomography (OCT). METHODS: A two-global flash mfERG (VERIS™) was recorded in 20 eyes with primary open-angle glaucoma. The root mean square was calculated, and three response epochs were analysed: the direct component (15-45 ms) and two induced components (IC-1 at 45-75 ms and IC-2 at 75-105 ms). The central 10° of the mfERG was compared to the central 10° of the OCT and of the visual field. Responses grouped in a superior and in an inferior semicircle, extending between 10° and 20°, were also compared to the corresponding areas of the OCT and of the visual fields. In addition, the area of the papillomacular bundle was also analysed separately. RESULTS: In glaucoma patients, mfERG responses showed a significant positive association with retinal thickness in the central 10° for IC2 (p = 0.001) and a trend for IC1 (p = 0.066). A significant association was found between the central IC1 and IC2 of the mfERG and corresponding perimetric sensitivities expressed in linear units (p < 0.01). The OCT showed a positive association with visual field sensitivities (p < 0.05) in all areas examined (p < 0.05). Separation of the papillomacular bundle area did not improve structure-function association further. CONCLUSIONS: In our study, mfERG showed a statistically significant correlation with perimetric sensitivity measured in linear units and with structural macular changes detected with time-domain OCT.

Anterior segment dysgenesis associated with Williams-Beuren syndrome: a case report and review of the literature.

BACKGROUND: Williams-Beuren syndrome is characterized by mild mental retardation, specific neurocognitive profile, hypercalcemia during infancy, distinctive facial features and cardiovascular diseases. We report on complete ophthalmologic, sonographic and genetic evaluation of a girl with a clinical phenotype of Williams-Beuren syndrome, associated with unilateral anterior segment dysgenesis and bilateral cleft of the soft and hard palate. These phenotypic features have not been linked to the haploinsufficiency of genes involved in the microdeletion. CASE PRESENTATION: A term born girl presented at the initial examination with clouding of the right cornea. On ultrasound biomicroscopy the anterior chamber structures were difficult to differentiate, showing severe adhesions from the opacified cornea to the iris with a kerato-irido-lenticular contact to the remnant lens, a finding consistent with Peters' anomaly. Genetic analyses including FISH confirmed a loss of the critical region 7q11.23, usually associated with the typical Williams-Beuren syndrome. Microsatellite analysis showed a loss of about 2.36 Mb. CONCLUSIONS: A diagnosis of Williams-Beuren syndrome was made based on the microdeletion of 7q11.23. The unique features, including unilateral microphthalmia and anterior segment dysgenesis, were unlikely to be caused by the microdeletion. Arguments in favor of the latter are unilateral manifestation, as well as the fact that numerous patients with deletions of comparable or microscopically visible size have not shown similar manifestations.

The effect of filtering on the two-global-flash mfERG: identifying the optimal range of frequency for detecting glaucomatous retinal dysfunction.

PURPOSE: To study the effects of filtering bandwidth on the two-global-flash multifocal electroretinogram (mfERG) responses in primary open-angle glaucoma (POAG) compared with control subjects. METHODS: A two-global-flash mfERG (VERIS 6.06™, FMS III) was recorded in 20 healthy subjects and 22 POAG patients with a band-pass filter (BPF) of 1-300 Hz (103 Hexagons, M-sequence stimulus: Lmax 100 cd/m(2), Lmin < 1 cd/m(2), global flash: 200 cd/m(2)). The root-mean-square average of the central 10° was calculated. Three response epochs were analysed: the response to the focal flash, at 15-45 ms (DC), and the following two components induced by the effects of the preceding focal flash on the response to the global flashes at 45-75 ms (IC1) and at 75-105 ms (IC2). The following BPF settings were analysed: 1-300 Hz, 3-300 Hz, 10-300 Hz, 100-300 Hz, 200-300 Hz, 1-10 Hz, 1-100 Hz and 1-200 Hz. RESULTS: Filtering at 1-300 Hz showed significantly lower responses in POAG than in control subjects (p < 0.001) for all epochs analysed. At 1-100 Hz, this also held true even though the difference between the groups became smaller. At 1-10 Hz, responses were extremely small and did not differ between POAG and control (p > 0.5). This would suggest a filter setting of 10-300 Hz for mfERG recordings in POAG. However, when a filter setting of 10-300 Hz was compared to 1-300 Hz, with a filter setting of 10-300 Hz, the DC in POAG differed more (p < 0.0001) from normal than with 1-300 Hz (p = 0.0002). For IC1 and IC2, the stronger difference between POAG and control was found with 1-300 Hz (p < 0.0001) rather than with 10-300 Hz (p < 0.0001 and p = 0.0005, respectively). For the 'oscillatory potentials' at 100-300 Hz, POAG and control differed significantly in IC1 and IC2 (p < 0.05), but not in DC (p = 0.8). However, filtering at 200-300 Hz did not show a difference between POAG and control (p > 0.5). Thus, we applied a filter setting of 1-200 Hz, which seemed to be most sensitive in detecting glaucomatous retinal dysfunction (p < 0.0001). CONCLUSIONS: A filter setting of 1-200 Hz appears most sensitive to detect glaucomatous damage if using a two-global-flash mfERG: using a band-pass filter a with lower low-frequency cut-off, containing the 10 Hz component, may be especially important in the small induced components that show glaucomatous damage most sensitively. High frequencies of 100-300 Hz also contain information that differentiates glaucoma from normal and thus should be included in the analysis.

Function and morphology in macular retinoschisis associated with optic disc pit in a child before and after its spontaneous resolution.

Optic disc pit (ODP) is a rare congenital defect within the optic nerve head. Macula elevation associated with ODP develops in 75-93% of the adult patients. Macular involvement in children with optic disc pit is rare, and only a few cases have been published to date. In the present case, we have observed morphology and function of the central retina in a child with ODP-associated macular detachment and following its spontaneous resolution. An 8-year-old white boy diagnosed with a macular detachment in an eye with an ODP. Optical coherent tomography (OCT) and multifocal electroretinography (mfERG), as well as visual acuity and visual field, were performed in the follow-up of the unilateral schisis-like retinal detachment. A large retinoschisis associated with ODP in a child showed a tendency to spontaneously resolve at 3 months, which was confirmed on OCT. At this time, an mfERG revealed markedly reduced responses. Despite morphologic reattachment at follow-up and improvement in visual acuity, increased mfERG responses were still not the same as in the fellow healthy eye. In contrast to the OCT which is very helpful to assess the extent of the neurosensory detachment, the mfERG offers an additional tool for follow-up of retinal function in this disorder. The good visual outcome in our patient shows that in the presence of residual retinal function on mfERG and in the absence of further lesions on OCT, follow-up is a valid option in children with an ODP-associated macular detachment.

ISCEV standard for clinical multifocal electroretinography (mfERG) (2011 edition).

The clinical multifocal electroretinogram (mfERG) is an electrophysiological test of local retinal function. With this technique, many local ERG responses are recorded quasi-simultaneously from the cone-driven retina under light-adapted conditions. This document, from the International Society for Clinical Electrophysiology of Vision (ISCEV: www.iscev.org ), replaces the ISCEV guidelines for the mfERG published in 2007. Standards for performance of the basic clinical mfERG test with a stimulus array of 61 or 103 hexagons, as well as for reporting the results, are specified.

MfERG responses to long-duration white stimuli in glaucoma patients.

The intent of our study was to evaluate whether the response to a long-duration white stimulus in the multifocal electroretinogram (mfERG) is sufficiently sensitive to detect early retinal dysfunction in glaucoma. On-off mfERGs were recorded from 15 NTG and 15 HTG patients and compared with 14 control subjects. Recording parameters were the following: LED stimulus screen (RETIscan™), 100-ms stimulus duration, 200-ms stimulus interval, 11-min total recording time, stimulus matrix of 61 elements, frame rate: 70 Hz, Lmax: 180 cd/m(2), Lmin: 0 cd/m(2), and filter setting: 1-200 Hz. The second negative response following stimulus onset (N2-on), as well as following stimulus offset (N2-off), was analyzed as an overall response and in quadrants, as well as in 4 small central and four adjoining peripheral areas per quadrant. The latency of the N2-on was significantly delayed in HTG in all response averages tested, while in NTG this was only seen in the overall response and in the small central response averages (P < 0.05). The most sensitive measure in HTG was the latency of the N2-on of the small peripheral response average of the superior temporal quadrant with an area under the ROC curve of 0.881. For NTG, the most representative measure was the latency of the N2-on of the small central response average of the inferior nasal quadrant with an area under the ROC curve of 0.793. Our results showed that in stimulation with long-duration flashes, the second negative response following the on response, representative of the early PhNR, is affected in glaucoma where N2-on showed a latency delay in POAG patients. The latency delay of the N2-on was more prominent for HTG than for NTG.

Globe restriction in a severely myopic patient visualized through oculodynamic magnetic resonance imaging (od-MRI).

Different mechanisms have been hypothesized as contributing to abduction deficit in high myopia: the size of the eye within the orbit, tightness of the medial rectus muscles, decompensation of longstanding esotropia, and inferior displacement of the lateral rectus muscle. Using oculodynamic magnetic resonance imaging, enhanced by computer-aided visualization, we demonstrate globe restriction by the medial orbital wall on abduction in a patient with high myopia.

ISCEV guidelines for clinical multifocal electroretinography (2007 edition).

The clinical multifocal electroretinogram (mfERG) is an electrophysiological test of local retinal function. With this technique, many local ERG responses, typically 61 or 103, are recorded from the cone-driven retina under light-adapted conditions. This document specifies guidelines for performance of the test. It also provides detailed guidance on technical and practical issues, as well as on reporting test results. The main objective of the guidelines is to promote consistent quality of mfERG testing and reporting within and among centers. These 2007 guidelines, from the International Society for Clinical Electrophysiology of Vision (ISCEV: http://www.iscev.org ), replace the ISCEV guidelines for the mfERG published in 2003.

The 'two global flash' mfERG in high and normal tension primary open-angle glaucoma.

PURPOSE: To analyse the sensitivity of the '2 global flash' multifocal electroretinogram (mfERG) to detect glaucomatous dysfunction in normal tension (NTG) and high tension primary open angle glaucoma (POAG) patients. METHODS: MfERGs were recorded from 20 NTG and 20 POAG patients and compared to those of 20 controls. The mfERG array consisted of 103 hexagons. Each m-sequence step started with a focal flash that could be either dark or light (m-sequence: 2--13, L(max): 200 cd/m(2), L(min): 1 cd/m(2)), followed by two global flashes (L(max): 200 cd/m(2)) at an interval of approximately 26 ms. Focal scalar products (SP) were calculated using focal templates derived from the control recordings (VERIS 4.8). We analyzed 5 response averages (central 7.5 degrees and 4 adjoining quadrants) of the response to the focal flash, the direct component at 10-40 ms (DC) and the following two components induced by the effects of the preceding focal flash on the response to the global flashes at 40-70 ms (IC-1) and at 70-100 ms (IC-2). RESULTS: Both NTG and POAG patients differed from controls in the IC-1 response to the superior quadrants, and POAG patients also differed from controls in the centre. The most sensitive parameter was the IC-1 of the superior temporal quadrant with an area under the ROC curve of 0.82 for POAG and 0.79 for NTG. The DC and the IC-2 did not differ significantly between the groups. When all five response averages of the IC-1 were taken into consideration 90% of the NTG patients and 85% of the POAG patients were correctly classified as abnormal while 80% of the control subjects were correctly classified as normal. CONCLUSIONS: This stimulus sequence holds promise for the diagnosis of early functional changes in POAG. A new finding is that both NTG, as well as POAG can be differentiated from control subjects.

Antiphospholipid antibodies in ocular arterial and venous occlusive disease.

PURPOSE: It was the aim of this study to evaluate antiphospholipid antibodies (APA), i.e. lupus anticoagulants (LA) and anticardiolipin (ACA) IgG and IgM, in ophthalmic occlusive disease. METHODS: Over a 3.5-year period, APA were evaluated in 368 patients. RESULTS: Eighty-six patients (23.4%), compared to 5% in the general population, tested positive for APA. APA did not differ significantly between patients with venous (20.6%) or arterial (25.5%) occlusive disease. This included 93 patients with central retinal vein occlusion (18% APA positive), 67 with retinal branch vein occlusion (24% APA positive), 41 with central retinal artery occlusion (22% APA positive), 53 with retinal branch artery occlusion (32% APA positive), 71 with anterior ischemic optic neuropathy (23% APA positive), 12 with posterior ischemic optic neuropathy (33% APA positive) and 31 patients with amaurosis fugax (23% APA positive). Excluding patients with accepted main risk factors, APA were positive in 15.3% of 85 patients. CONCLUSION: The high APA prevalence confirms its relevance in ocular occlusive disorders.