CROQ Vol 1 Number 3 by MediConcept

CROQ

Clinical Refractive & Optometry Quebec EDITION

VOLUME 1, NUMBER 3, 2016

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All articles are accredited by the OOQ for Category A UFC credits

MacuScope and QuantifEye Macular Pigment Densitometers What an Optometrist Needs to Know About Lupus Erythematosus Blind Spot Mapping: A Case Report Homonymous Hemianopia in a Young Adult: A Case Report

Clinical

&Refractive Optometry Quebec

Editorial Board • Volume 1, Number 3, 2016 Editor-in-Chief

Associate Editor

Yvon Rhéaume, OD Montreal, Quebec

Richard Maharaj, OD, FAAO Toronto, Ontario

Leonid Skorin, Jr., OD, DO, MS Albert Lea, Minnesota

Contributing Editors Brad Almond, OD Calgary, Alberta

Danielle DeGuise, OD Montreal, Quebec

Langis Michaud, OD Montreal, Quebec

Jean Bélanger, OD Montreal, Quebec

Pierre Forcier, OD Montreal, Quebec

Rodger Pace, OD Waterloo, Ontario

Scott D. Brisbin, OD Edmonton, Alberta

John Jantzi, OD Vancouver, British Columbia

Maynard Pohl, OD Bellevue, Washington

Lorance Bumgarner, OD Pinehurst, North Carolina

Gerald Komarnicky, OD Vancouver, British Columbia

Barbara Robinson, OD Waterloo, Ontario

Barbara Caffery, OD Toronto, Ontario

Bart McRoberts, OD Vancouver, British Columbia

Jacob Sivak, OD, PhD Waterloo, Ontario

Louis Catania, OD Philadelphia, Pennsylvania

Ron Melton, OD Charlotte, North Carolina

Randall Thomas, OD Concord, North Carolina

Publication Staff Publisher Lawrence Goldstein

Managing Editor Mary Di Lemme

Senior Medical Editor Evra Taylor

Layout Editor Colin MacPherson

Graphics & Design Mediconcept Inc.

Our Mission Statement Clinical & Refractive Optometry Quebec is a peer-reviewed, optometric journal produced in both print and online editions. The journal is dedicated to disseminating clinical and scientific COPE approved articles which have been accredited by the Order of Optometrists of Quebec (OOQ) and offered as Category A, UFC-credit courses. The contents of this publication are composed of articles that will be of particular use and interest to practicing eye care professionals in Quebec. Test participants who answer the UFC test questionnaires contained in this journal, and who score 50% or more, will receive a presonalized UFC-credit certificate by return email.

Why is this Journal published in English? The regulations governing continuing education credits in Quebec have been amended so that CE-credit journal articles, which have been COPE approved by the American Regulatory Board of Optometry (ARBO) can now be offered in print and online to Optometrists in Quebec for Category A, UFC-ce-credit. In this regard, it’s important to note that all of these articles were originally written, approved, and accredited in English and cannot be translated or reproduced into any other language. For this reason, all the Catagory A, UFC-accredited courses which are offered in this journal are presented in English with the approval of the Order of Optometrists of Quebec (OOQ).

Clinical

&Refractive Optometry Quebec

Contents • Volume 1, Number 3, 2016

UFC CREDIT ARTICLES 86 A Comparison of the MacuScope and QuantifEye Macular Pigment Densitometers in Two Distinct Population Types Robert J. Donati, PhD; Elizabeth Wyles, OD ABSTRACT: At the time of this study there were two compact commercially available heterochromatic flicker photometry instruments that measure macular pigment optical density in the USA. Previous studies revealed significant variability between instruments. Our aim was to determine if the variability was instrument driven, and we wanted to determine if the same variability would be found in a young, healthy, population compared to an older population for which these instruments have more significance.

What an Optometrist Needs to Know About Lupus Erythematosus Mark L. Landig, OD; Pauline F. Ilsen, OD ABSTRACT: Lupus erythematosus (LE) is a chronic autoimmune disease that elicits a type III hyper-sensitivity reaction in which antibody-immune complexes precipitate and causes further immune response. There are three types of LE: discoid, systemic, and drug-induced. Systemic lupus erythematosus (SLE) is among the most common and concerning of the three. SLE harms mostly the heart, joints, skin, blood vessels, liver, kidneys, and nervous system. In addition to systemic issues, LE uniquely manifests itself in and around the eye. Secondary Sjögren’s syndrome and severe dry eyes are common among LE patients.

106

Blind Spot Mapping: A Case Report Stacey Chong, OD; Patricia K. Hrynchak, OD; Michelle J. Steenbakkers, OD; Derek Y. Ho, MD; Natalie Hutchings, PhD; Nadine M. Furtado, OD ABSTRACT: The blind spot is an essential element in static perimetry as the absolute scotoma produced is used to ensure that the patient is fixating steadily throughout the test. If the optic nerve is anomalous, the scotoma might not be in the predicted location. This case outlines a few potential pitfalls in perimetry in a patient with an anomalous optic nerve head.

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Clinical & Refractive Optometry Quebec is published 4 times per year by Mediconcept. The Journal is made available to all practicing optometrists in Quebec on www.crojournal.com. Advertising insertion orders and copy must be received before the first day of the preceding month for which the advertising is scheduled. While the editorial staff of Clinical & Refractive Optometry Quebec exercises great care to ensure accuracy, we suggest that the reader consult the manufacturer’s instructions before using products mentioned in this publication. The views contained in the Journal are those of the respective authors and not of the Publisher. Please direct all correspondence to: Mediconcept Editorial & Sales Office 3484 Sources Blvd., Suite 518 Dollard-des-Ormeaux, Quebec Canada H9B 1Z9 Tel.: (514) 245-9717 E-mail: info@mediconcept.ca Printed in Canada. All rights reserved. Copyright © 2016 Mediconcept. The contents of the publication may not be mechanically or electronically reproduced in whole or in part without the written permission of the publisher. All drug advertisements have been cleared by the Pharmaceutical Advertising Advisory Board.

Homonymous Hemianopia in a Young Adult: A Case Report Kathryn Dailey, BS; Leonid Skorin Jr., OD, DO ABSTRACT: When a patient presents with acute visual field loss correlating the pattern of loss with the area of visual pathway damaged is critical to proper patient management. Homonymous hemianopia field loss can occur with damage to any area of the retrochiasmal visual pathway and is often devastating for patients. In older adults the most common cause of homonymous hemianopia is ischemic infarction of the occipital lobe. In younger adults causes of homonymous hemianopia are more heterogeneous and multi-factorial. This often presents the clinician with a diagnostic challenge and indicates the need for a full systemic health evaluation.

ISSN: 2369-498X; Date of Issue: May 2016

Cover Image: Discoid lesion Courtesy of: Dr. Pauline Ilsen

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Clinical & Refractive Optometry Quebec is pleased to present this continuing education (CE) article by Dr. Robert Donati and Dr. Elizabeth Wyles, Illinois College of Optometry, Chicago, IL. This article has been approved for 1 Category A, UFC credit in Ocular Health by the Ordre des Optométristes du Québec. In order to obtain your credit, please refer to page 91 for complete instructions.

A Comparison of the MacuScope and QuantifEye Macular Pigment Densitometers in Two Distinct Population Types Robert J. Donati, PhD; Elizabeth Wyles, OD

ABSTRACT Background: At the time of this study there were two compact commercially available heterochromatic flicker photometry (HFP) instruments that measure macular pigment optical density (MPOD) in the USA. Previous studies revealed significant variability between instruments. Our aim was to determine if the variability was instrument driven, and we wanted to determine if the same variability would be found in a young, healthy, population compared to an older population for which these instruments have more significance. Methods: Young healthy adults and older adults with and without early signs of AMD were recruited for the study. Macular pigment optical density was measured using the MacuScope™ and QuantifEye®. Results: The primary result demonstrated that the mean standard deviation of each subject’s MPOD readings was significantly different between the MacuScope and the QuantifEye within each age group and in the combined age groups. Conclusion: If MPOD is monitored with the possibility of altering treatment, the need for reliable measurements is imperative. From this limited study, both instruments appear to demonstrate reliability, however, when critically looking at each subject’s data, there is significant variability between the instruments.

R.J. Donati; E. Wyles — Illinois College of Optometry, Chicago, IL Correspondence to: Dr. Robert Donati, Illinois College of Optometry, 3241 S. Michigan Avenue, Chicago, IL USA 60616; E-mail: rdonati@ico.edu Funding provided by the Illinois College of Optometry Research Resource Committee. The authors have no financial or proprietary interest in any of the instruments used in this study. This article has been peer reviewed.

Clinical & Refractive Optometry Quebec 1:3, 2016

INTRODUCTION Age-related macular degeneration (AMD) is a leading cause of blindness among individuals 65 years of age and older.1 It has been suggested that the development of AMD may be associated with having reduced macular pigment.2-4 It is well known that Caucasians are at higher risk for progression to advanced AMD as compared to African-Americans and there is some evidence that the differences in macular pigment (MP) between the races could be a factor in this progression.5-7 Studies have suggested that reduced levels of MP in conjunction with other risk factors like, increasing age, tobacco use, and family history, may increase the risk for developing AMD.3 Macular pigment is composed primarily of two carotenoids, lutein and zeaxanthin.8 A third carotenoid, meso-zeaxanthin is a rare isomer of zeaxanthin which is also present in macular pigment, however not normally consumed in the diet.9 Several studies have demonstrated that the presence of these compounds and others (vitamin C, beta carotene) may lower the risk of developing AMD due to their anti-oxidative properties.10-12 Since the levels of these macular pigments can be augmented with oral supplementation, measuring MP levels in patients at risk for AMD is gaining popularity in patient care. Heterochromatic flicker photometry (HFP) is currently the standard for measuring macular pigment optical density (MPOD) in clinic.13 HFP compares the luminance of two light sources with different wavelengths. A number of studies have evaluated and compared different HFP instruments.15-21 Early evidence suggested when using a standardized protocol, HFP could estimate MPOD in older subjects reliably.14 More recent evidence is suggesting that there exists greater variability in measurements than initially anticipated.15-17 At the time of this study, there were two compact commercially available HFP instruments that measured MPOD in the USA, the MacuScope and the QuantifEye. Our aim was to re-evaluate variability of these instruments. Our primary goal was to determine if the variability was instrument driven, and if so, which instrument was less variable. Our secondary goal was to determine if the same variability would be found in a young, healthy, population compared to an older population for which these instruments have more significance suggesting that the variability is population driven.

METHODS

RESULTS

Twenty-eight young healthy optometry students (21 to 31 years old) and twenty-eight older adults (50 to 70 years old) with and without early signs of AMD were recruited from the Illinois Eye Institute patient base. The visual acuity of all eyes used for MPOD determination was 6/9 (20/30) or better on the ETDRS chart and were free of all other macular disease. This study was conducted with the approval of the Illinois College of Optometry Institutional Review Board and informed consent was obtained by all research subjects.

The mean age of the older adult group was 59.0 ± 4.86 (15 females and 13 males) while the mean age for the younger adult group was 24.3 ± 1.85 (21 females and 7 males). The disparity in the number of males and females from the younger adult group could be due to the fact that the subjects were recruited from the Illinois College of Optometry student body which is about twothirds female. Initial experiments looked to compare the mean MPOD readings between the two instruments from each age group and the combined age groups (Fig. 1). There was no significant difference between the mean MPOD readings from the two instruments for the 50 to 70 age group. The mean reading from the 46 eyes was 0.317 ± 0.191 for the QuantifEye and 0.341 ± 0.149 for the MacuScope (Fig. 1A). However, there was a significant difference between the mean MPOD readings from the two instruments for the 21 to 31 age group. The mean MPOD reading of these 53 eyes was 0.3997 ± 0.174 for the QuantifEye and 0.338 ± 0.146 for the MacuScope (p=0.0362) (Fig. 1B). Combining the age groups yielded no significant difference in the means, 0.361 ± 0.186 for the QuantifEye and 0.340 ± 0.146 for the MacuScope (Fig. 1C). In order to look at the precision and systematic error of the instruments, a Bland-Altman analysis was performed using the 99 valid paired data points collected. The differences in the MPOD measurements between the two instruments were plotted against the average MPOD reading of the two techniques (Fig. 2). The mean of the differences (bias) was -0.0193 from the 50 to 70 age group (Fig. 2A), 0.0554 from the 21 to 31 age group (Fig. 2B), and 0.0230 from the combined groups (Fig. 2C) which reflects the systematic error. The upper and lower limits of agreement (± 1.96 SD) were 0.4975 and -0.5361 for the 50 to 70 group, 0.5349 and -0.4241 for the 21 to 31 group, and 0.5300 and -0.4840 for the combined groups. These represent the precision of the instruments. In order to examine the difference in intra-instrument variability when considering individual subjects, we compared the mean standard deviations derived from each subject’s standard deviation on each instrument (Fig. 3). Data from adults aged 50 to 70 resulted in a mean SD of MPOD measurements of 0.0577 ± 0.0544 from the QuantifEye and 0.1074 ± 0.0798 from the MacuScope. A paired student’s t-test was performed and the p-value of 0.0026 indicates a significant difference when looking at the mean SD of MPOD measurements between the two instruments (Fig. 3A). A similar significant difference was observed in the young adult population, adults aged 21 to 31, with mean SD measurements of 0.0487 ± 0.0553 from the QuantifEye and 0.0826 ± 0.0831 from the MacuScope resulting in a p-value of 0.0207 (Fig. 3B).

MPOD Measurements The MacuScope (MacuChek, LLC, USA) and the QuantifEye (Zeavision, LLC, USA) are heterochromatic flicker photometers and were used in this study to determine MPOD. Data was collected for each patient in one session. A single, but different trained operator collected data for each of the patient populations. Whether a subject was first tested on the MacuScope or QuantifEye was randomly determined. A demonstration was conducted immediately prior to testing for each instrument to ensure the subjects understood the testing protocol. Pupil dilation is not required for HFP and therefore was not performed. Prior to the start of testing on each of the instruments, the subject was dark adapted by occluding the eye to be tested with an eye patch for 5 minutes. After the demonstration and dark adaptation, the eye patch was moved to the eye not being tested. MPOD measurements were taken on each instrument according to the specific manufactures operating manual instructions. A stimulus was displayed which alternated between two wavelengths. The test wavelength is absorbed by macular pigment (blue, 460 nm) and the reference wavelength is not absorbed (green, 540 nm). The subject viewing the stimulus observed flicker between the two wavelengths and was asked to adjust the intensity of the test wavelength so that the flicker was no longer noticeable. The subject was asked to minimize flicker while viewing the stimulus centrally, then peripherally, the difference between these two measurements yielded the MPOD.10 Two measurements per eye were taken on each instrument. If the difference was greater than 0.04 absorbance units between these two measurements on a single instrument,13 a third measurement was taken. Invalid or off-scale readings (0 or > 1.0) were excluded. Of the 103 eyes that provided valid readings, 99 eyes provided valid data on both instruments for comparison of the mean MPOD readings, while 103 (MacuScope) and 102 (QuantifEye) individual eyes provided data for comparison of the individual subjects standard deviations. Statistical Analysis Paired t-tests using GraphPad Prism 5.0 software were done to compare the statistical significance of the results from both instruments and age groups. Bland-Altman plots using MedCalc® (v 12.2.1.0) were done for an added comparison.

MacuScope and QuantifEye Macular Pigment Densitometers — Donati, Wyles

Fig. 1 Comparison of the mean MPOD level in the eyes of subjects using the QuantifEye and MacuScope. (A) The mean MPOD reading for adults aged 50 to 70 (n=46 eyes). (B) The mean MPOD reading for the adults aged 21 to 31 (n= 53 eyes). (C) The mean MPOD for the combined age groups (n=99 eyes). A paired studentâ&#x20AC;&#x2122;s t-test was performed using GraphPad Prism 5.0 software and there was no significant difference found between the two machines in the 50 to 70 and combined groups, but there was a significant difference in the 21 to 31 group, p=0.0362.

Clinical & Refractive Optometry Quebec 1:3, 2016

Fig. 2 Repeated measures Bland-Altman plot comparison of the QuantifEye and the MacuScope. (A) 46 eyes from adults aged 50 to 70 years old with at least 1 and no more than 3 MPOD readings per eye were used in the comparison of the instruments. (B) 53 eyes from adults aged 21 to 31 years old with at least 1 and no more than 4 MPOD readings per eye were used in the comparison of the instruments. (C) 99 eyes from the combined age groups with at least 1 and no more than 4 MPOD readings per eye were used in the comparison of the instruments. The differences in the MPOD measurements between the two techniques are plotted against the average MPOD reading of the two techniques.

The combined group data demonstrated a higher degree of significance with mean values of 0.0528 ± 0.0548 from the QuantifEye and 0.0946 ± 0.0821 from the MacuScope and a p-value of 0.0002 (Fig. 3C).

DISCUSSION

Fig. 3 Comparison of the mean standard deviations (SD) of repeated MPOD measurements from individual eyes on the QuantifEye and MacuScope. (A) Eyes were measured 2 to 3 times with the QuantifEye (n=46) and with the MacuScope (n=50) from adults aged 50 to 70. A paired student’s t-test was performed using GraphPad Prism 5.0 software. The p=0.0026 indicates a significant difference when looking at the individual subject variability between the two instruments. (B) Eyes were measured 2 to 3 times with the QuantifEye (n=56) and with the MacuScope (n=53) from adults aged 21 to 31. The p=0.0207 indicates significant difference when looking at the individual subject variability between the instruments. (C) Eyes were measured 2 to 3 times with the QuantifEye (n=102) and with the MacuScope (n=103) from the combined groups. The p=0.0002 indicates a significant difference when looking at the individual subject variability between the instruments.

The MacuScope and the QuantifEye are two commercially available HFP systems used to measure MPOD in patients with the end goal of helping to assess risk and monitoring the progression of AMD as well as treatment efficacy. These instruments use the same unit of measurement on a scale of 0 to 1.0. A person considered low risk for AMD would have a measurement of approximately 1.0 to 0.5. A patient considered high risk would yield a measurement of approximately 0.25 or less. Since these instruments are being used in clinical practice,21 it is important for clinicians to carefully consider variability and reliability before using MPOD results as an influential factor in patient care. Anyone who has performed an MPOD examination using HFP understands the level of difficulty and anxiety that comes with trying to accurately determine the endpoint during the testing procedure. Thus, we wanted to consider patient variability on each instrument and if one instrument yielded less patient variability as compared to the other. When we compare the two instruments by simply looking at the mean MPOD of each group (Fig. 1), we are led to believe that the instruments perform similarly since the only group that demonstrated a mildly significant difference in means is the younger group of subjects. Similarly, the Bland-Altman analysis in Figure 2 demonstrates that there is really no difference between the two machines and that only a few subjects fall outside the limits of agreement. However, the range covering the limits of agreement is very high and the spread of the paired observations between the limits of agreement demonstrates a lack of precision within the instruments. One has to consider that the limits of agreement are determined by two times the standard deviation, which in this case is 0.5 and -0.54. This means that someone who potentially achieved an acceptable MPOD reading on one instrument and a high risk reading on the other can fall within the limits of agreement. In fact, 82% of the older subjects and 85% of the younger subjects involved in this study yielded a measurement in at least one eye that put them in two different AMD risk categories on the different instruments. Is this instrument variability acceptable in optometric practice? Additionally, this makes it difficult to compare results from one instrument to the other despite using the same MPOD unit of measurement. When considering the results presented in Figure 3 we see variability in patient responses on both instruments, however the QuantifEye was significantly less variable. This result held true for all three subject groups.

MacuScope and QuantifEye Macular Pigment Densitometers — Donati, Wyles

This suggests that the QuantifEye testing method may be more patient-friendly and easier to perform. However, the variability demonstrated by the broad range of standard deviations in the individual subject readings seen in Figure 3 appears to be unacceptable for optometric practice. Ideally, the standard deviation from a single patient, on a single instrument, would be as close to zero as possible. The data does not come close to reflecting this ideal, so we are left with the question of: “How much variability is acceptable for the instrument to be used in patient care?” One must consider the populations studied as a source of variability. The two age groups were extremely different when comparing backgrounds and educational level which might lead one to believe that one group would have a better understanding of the tests leading to reduced variability. Surprisingly, when examining the results in Figure 3A and 3B we observe that age and background do not play a role in variability. The mean of the standard deviations is not significantly different on instruments between age groups. Thus, this study questions the accuracy and reliability of these HFP systems, and demonstrates significant variability in the measurements of MPOD by HFP in both young and older subjects on both instruments. In summary, the clinician should take instrument variability into account when deciding to use MPOD as an indicator for AMD risk and/or clinical care assessment. Granted, the variability found in this study may be due to the small sample size; but, larger studies conducted with these two systems have produced similar results.15,16 There are a number of other ways to measure MPOD and some of these methods are more reliable and accurate than HFP,23,24 however, some of these instruments are far too cumbersome for the average clinical optometric practice. Recent publications suggests that more reliable and patient-friendly instrumentation for the measurement of MPOD is on the horizon for the clinical practitioner.25 If MPOD is going to be monitored clinically to assess risk of AMD with the possibility of causing altered treatment regimens, the need for reliable data measurements is imperative. ❏ Acknowledgements: The authors are grateful for the contributions of the Illinois College of Optometry students that helped with data collection over the course of the study. We would also like to thank Dr. Susan Kelly for her help with the statistical analysis. The MacuScope was loaned to the Illinois College of Optometry by MacuChek, LLC. The QuantifEye was loaned to the Illinois College of Optometry by Zeavision, LLC.

REFERENCES 1.

Klaver CCW, Wolfs RCW, Vingerling JR, Hofman A, De Jong PTVM. Age-specific prevalence and causes of blindness and visual impairment in an older population – The Rotterdam Study. Arch Ophthalmol 1998; 116(5): 653-658. Ciulla TA, Hammond BR. Macular pigment density and aging, assessed in the normal elderly and those with cataracts and agerelated macular degeneration. Am J Ophthalmology 2004; 138: 582-587.

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Nolan JM, Stack J, O' Donovan O, Loane E, Beatty S. Risk factors for age-related maculopathy are associated with a relative lack of macular pigment. Exp Eye Res. 2007; 84: 61-74. Gale CR, Hall NF, Phillips DI, Martyn CN. Lutein and zeaxanthin status and risk of age-related macular degeneration. Invest Ophthalmol Vis Sci. 2003; 44(6): 2461-2465. Congdon N, O’Colmain B, Klaver CC, et al. Causes and prevalence of visual impairment among adults in the United States. Arch Ophthalmol 2004; 122:477-485. Wolf-Schnurrbusch UE, Roosli N, Weyermann E, Heldner MR, et al. Ethnic differences in macular pigment density and distribution. Invest Ophthalmol Vis Sci 2007; 48(8): 3783-3787. Bressler SB, Muñoz B, Solomon SD, West SK, the Salisbury Eye Evaluation (SEE) Study Team. Racial differences in the prevalence of age-related macular degeneration: The Salisbury Eye Evaluation Project. Arch Ophthalmol 2008; 126(2): 241-245. Delcourt C. Application of nutrigenomics in eye health. Forum Nur 2007; 60: 168-175. Bone RA, Landrum JT, Cao Y, Howard AN, et al. Macular pigment response to a supplement containing meso-zeaxanthin, lutein and zeaxanthin. Nutrition and Metabolism 2007; 4: 12. Curran-Celentano J, Burke JD, Hammond BR. In vivo assessment of retinal carotenoids: macular pigment detection techniques and their impact on monitoring pigment status. J Nutrition 2002; 132(suppl): 535S-539S. Kassoff A, et al. A randomized, placebo-controlled, clinical trial of high-dose supplementation with vitamins C and E, beta carotene, and zinc for age-related macular degeneration and vision loss. AREDS Report No. 8. Arch Ophthalmol 2001; 119: 1417-1436. Loskutova E, Nolan J, Howard A, Beatty S. Macular pigment and its contribution to vision. Nutrients 2013; 5: 1962-1969. Bone RA, Landrum JT. Heterochromatic flicker photo-metry. Arch Biochem Biophys 2004; 430(2): 137-142. Snodderly DM, Mares JA, Wotten BF, Oxton L, et al. Macular pigment measurement by heterochromatic flicker photometry in older subjects: the carotenoids and age-related eye disease study. Invest Ophthalmol Vis Sci 2004; 45: 531-538. Barlett H, Acton J, Eperjesi F. Clinical evaluation of MacuScope macular densitometer. Br J Opthalmol 2010; 94: 328-331. Barlett H, Stainer L, Singh S, Eperjesi F, et al. Clinical Evaluation of the MPS 9000 Macular Pigment Screener Br J Opthalmol 2010; 94: 753-756. Howells O, Eperjesi F, Barlett H. Improving the repeatability of heterochromatic flicker photometry for the measurement of macular pigment optical density. Graefes Arch Clin Exp Ophthalmol 2013; 251: 871-880. de Kinkelder R, van der Veen RL, Verbaak FD, Faber DJ, et al. Macular pigment optical density measurements: evaluation of a device using heterochromatic flicker photometry. Eye 2011; 25: 105-112. Hagen S, Krebs I, Glittenberg C, Binder S. Repeated measures of macular pigment optical density to test reproducibility of heterochromatic flicker photometry. Acta Ophthalmologica 2010; 88: 207-211. van der Veen RL, Berendschot TT, Hendrikse F, Carden D, et al A new desktop instrument for measuring macular pigment optical density based on a novel technique for setting flicker thresholds. Ophthalmic Physiol Opt 2009; 29: 127-137. Loane E, Stack J, Beatty S, Nolan JM. Measurement of macular pigment optical density using two different heterochromatic flicker photometers. Curr Eye Res 2007; 32: 555-564. Murray IJ, Hassanali B, Carden D. Macular pigment in ophthalmic practice; a survey. Graefes Arch Clin Exp Ophthalmol 2013; 251: 2355-2362. Howells O, Eperjesi F, Bartlett H. Measuring macular pigment optical density in vivo: a review of techniques. Graefes Arch Clin Exp Ophthalmol 2011; 249: 315-347. Dennison JL, Stack J, Beatty S, Nolan JM. Concordance of macular pigment measurements obtained using customized heterochromatic flicker photometry, dual-wavelength autofluorescence, and single-wavelength reflectance. Exp Eye Res 2013; 116: 190-198. Bone RA, Mukherjee A. Innovative Troxler-free measurement of macular pigment and lens density with correction of the former for the aging lens. J Biomedical Optics 2013; 18(10): 107003.

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QUESTIONNAIRE A Comparison of the MacuScope and QuantifEye Macular Pigment Densitometers in Two Distinct Population Types Robert J. Donati, PhD; Elizabeth Wyles, OD 1. ❑ ❑ ❑ ❑

2. ❑ ❑ ❑ ❑

All of the following statements about the study results cited in the paper are true, EXCEPT: Caucasians are at higher risk for progression to advanced AMD than African-Americans There was a significant difference between the macular pigment optical density (MPOD) readings from the two instruments for the 21 to 31-year-old age group According to the Bland-Altman analysis, there is really no difference between the QuantifEye and MacuScope equipment There was a significant difference between the MPOD readings from the two instruments for the 50 to 70-year-old age group Reduced levels of macular pigment may increase the risk for developing AMD in conjunction with all of the risk factors below, EXCEPT: Increasing age Alcohol abuse Tobacco use Family history

MacuScope and QuantifEye Macular Pigment Densitometers — Donati, Wyles

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CATEGORY A UFC C REDIT A PPLICATION F ORM 92

3. ❑ ❑ ❑ ❑

All of the following compounds may lower the risk of developing AMD, EXCEPT: Vitamin D Beta carotene Carotenoids Vitamin C

4. ❑ ❑ ❑ ❑

The visual acuity for all eyes used for MPOD determination was: 6/4.8 (20/16) 6/7.5 (20/25) 6/9 (20/30) 6/12 (20/40)

5. ❑ ❑ ❑ ❑

A patient considered high risk would yield an MPOD measurement of which of the following? 0.18 or less 0.20 or less 0.25 or less 0.28 or less

In the study cited in the paper, what percentage of the older subjects yielded a measurement in at least one eye that put them in two different AMD risk categories on the different instruments? 50% 65% 73% 82%

❑ ❑ ❑ ❑ 7. ❑ ❑ ❑ ❑

In the study cited in the paper, what percentage of the younger subjects yielded a measurement in at least one eye that put them in two different AMD risk categories on the different instruments? 62% 75% 85% 93%

8. ❑ ❑ ❑ ❑

All of the following statements regarding the study cited in the paper are true, EXCEPT: Subject background was not seen to play a role in variability The QuantifEye was significantly less variable than the MacuScope The education level of the subjects was not a factor leading to reduced variability Age did not play a role in variability

9. ❑ ❑ ❑ ❑

What would be the approximate MPOD measurement for a person considered low risk for AMD? 1.0-0.2 1.0-0.3 1.0-0.4 1.0-0.5

10. ❑ ❑ ❑ ❑

All of the following statements about AMD are false, EXCEPT: Asians are at higher risk for progression to advanced AMD than African-Americans Females are more susceptible to developing AMD than males AMD is a leading cause of blindness among individuals 65 years of age and older The average age for onset of AMD is 50 years old

Clinical & Refractive Optometry Quebec 1:3, 2016

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Les gélules sont la forme posologique au 1er rang des préférences des patients1 Seule la marque PreserVisionMD AREDS 2 offre à la fois : • Des gélules petites, lisses et faciles à avaler • Une correspondance exacte à la formule AREDS 2 éprouvée en clinique ayant démontré pouvoir réduire le risque de progression chez les patients atteints de DMLA modérée à avancée2 DMLA = dégénérescence maculaire liée à l'âge; AREDS 2 = Age-Related d Eye Disease Study 2 (seconde étude sur les maladies oculaires liées à l’âge) * Rapport d’analyse indépendante basé sur le déplacement volumétrique de mesures de laboratoire en vue de comparer les gélules PreserVisionMD AREDS 2 et les comprimés VitaluxMD Advanced. Références : 1. Jones WJ, Fracis JJ. Softgels: Consumer perceptions and market impact relative to other dosage forms. Adv Ther 2000;17(5):213–21. 2. Age-Related Eye Disease Study 2 (AREDS2) Research Group. Lutein + zeaxanthin and omega-3 fatty acids for age-related macular degeneration: the Age-Related Eye Disease Study 2 (AREDS2) randomized clinical trial. JAMA 2013;309(19):2005–2015. 3. NIRI US : Mass+Food+Drug. 52 dernières semaines terminées le 29 novembre 2015. 4. Rapport MarketTrack de Nielsen pour les É.-U. et le Canada, 52 dernières semaines terminées le 29 novembre 2015, par volume de vente de toutes les vitamines oculaires.

Réservé Réservé à un usage usage professionnel. professionnel. Bausch & Lomb Canada, Vaughan (Ontario) L4K 4B4 © Valeant Canada LP MD/MC sont des marques de commerce de Bausch & Lomb Incorporated ou de ses affiliés.

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Clinical & Refractive Optometry Quebec is pleased to present this continuing education (CE) article by Dr. Mark Landig and Dr. Pauline F. Ilsen, West Los Angeles Veterans Affairs Healthcare Center, Optometry Clinic, Los Angeles, CA. This article has been approved for 1 Category A, UFC credit in Ocular Health by the Ordre des Optométristes du Québec. In order to obtain your credit, please refer to page 104 for complete instructions.

What an Optometrist Needs to Know About Lupus Erythematosus Mark L. Landig, OD; Pauline F. Ilsen, OD, FAAO

ABSTRACT Background: Lupus erythematosus (LE) is a chronic autoimmune disease that elicits a type III hypersensitivity reaction in which antibody-immune complexes precipitate and causes further immune response. There are three types of LE: discoid, systemic, and drug-induced. Systemic lupus erythematosus (SLE) is among the most common and concerning of the three. SLE harms mostly the heart, joints, skin, blood vessels, liver, kidneys, and nervous system. In addition to systemic issues, LE uniquely manifests itself in and around the eye. Secondary Sjögren’s syndrome (SS) and severe dry eyes are common among LE patients. Currently, there is no cure for LE; patients are often treated with an immunosuppressant or antimalarials, such as hydroxychloroquine. Case Reports: A patient who appeared to have discoid lupus erythematosus (DLE) with secondary SS was referred to eye clinic by rheumatology for hydroxychloroquine screening. He presented with severe dry eyes. Treatment for dry eyes was initiated and screening for hydroxychloroquine toxicity was conducted. Another patient presented to the eye clinic for reduced vision in his right eye. He was diagnosed with systemic lupus with no treatment. Ophthalmic examination revealed a unilateral macular edema secondary to a central retinal vein occlusion likely to active systemic lupus. Immediate referral to rheumatology and ophthalmology

M. L. Landig — Resident, West Los Angeles Veterans Affairs Healthcare Center, Los Angeles, CA; P. F. Ilsen — Professor, Marshall B. Ketchum University, West Los Angeles Veterans Affairs Healthcare Center, Los Angeles, CA Correspondence to: Dr. Pauline F. Ilsen, Marshall B. Ketchum University, West Los Angeles Veterans Affairs Healthcare Center, Optometry Clinic (123) Bldg. 304, Room 2-123, 11301 Wilshire Blvd., Los Angeles, CA USA 90073; E-mail: Pauline.Ilsen@va.gov This article has been peer-reviewed.

Clinical & Refractive Optometry Quebec 1:3, 2016

was made. Conclusion: Since patients with LE may have many ocular manifestations, optometrists should be able to recognize them and ensure that the patient receives appropriate treatment. Communication with ophthalmology and rheumatology is important for the management of these cases.

INTRODUCTION Lupus erythematosus is a chronic inflammatory disease that affects the body in multiple levels, targeting different organs. There are three different types of lupus erythematosus: skin, known as discoid lupus; drug-induced; and systemic lupus.1 Discoid lupus is often diagnosed after biopsy of a rash that manifests on the face, neck, or scalp.1 Cases of discoid tend to be limited to the skin, but may also have symptoms which manifest systemically. Certain prescribed medications can induce similar symptoms to those of systemic lupus, known as drug-induced lupus. Medications that are known to cause drug-induced lupus are: hydralazine — a direct acting smooth muscle relaxant, procainamide — an antiarrhythmic agent, and isoniazid — an anti-tuberculosis medication.1 The mechanism is unclear, but some theories include abnormal oxidative drug metabolism, the drugs may act as haptens, and/or drugs may nonspecifically activate lymphocytes.2 Systemic lupus erythematosus is the most common form and tends to be the most severe type of lupus; it can affect any organ in the body.1 The skin, joints, blood, kidneys, lungs, and the heart are some examples of organs that are vulnerable.1-3 This review will mainly focus on systemic lupus, but it is important for optometrists to understand that there are other types of the disease. The prevalence of SLE is 40 cases per 100,000 persons among Northern Europeans to more than 200 per 100,000 persons among African-Americans.1 In the United States, the number of people diagnosed with lupus has reached to at least 250,000.2 The cause of lupus is still unknown, but studies have shown that a genetic predisposition and the environment play important roles in the disease process.1-3 Environmental factors that are known to stimulate the disease process include, but not limited to, ultraviolet radiation, infection, antibiotics in particular sulfa and penicillin family, stress, hormones, and drugs.1-3

Table I Laboratory results of Case 1

Table II Anti-body results of Case 1

Labs

Value

Normal Range

Antibody

Results

Units

Normal Values

Complement C3 Complement C4 Complement total (CH50) Creatinine idms (spt Ur) Protein, Urine Western ESR Lymphocytes PLT WBC

115 mg/dL 22 mg/dL >60 mg/dL (H) 110.2 8.2 14 mm/hr 69% (H) 104 k/ul (L) 6. 6 k/ul

79-152 16-38 31-60 — — 0-20 20-40 150-440 4.5-11.0

Anti-Sm

0.7

EU/mL

0-16

Anti-Sm/RNP

1.9

EU/mL

0-16

Anti-SSA

19.1 (H)

EU/mL

0-16

Anti-SSB

1.7

EU/mL

0-16

Anti-Scl-70

2.1

UE/mL

0-25

Anti-dsDNA (EIA)

2.1

IU/mL

0-25

ANA (DIFA)

1+Pos

Neg

Fig. 1 Discoid lesion over the patient’s right hand (left) and discoid lesion just above the patient’s lips (right).

Case 1 A 66-year-old African-American male was referred to the eye clinic by rheumatology for his annual hydroxychloroquine eye screening. He reported no changes in his vision in the past year. He was diagnosed with discoid lupus and Sjögren syndrome 14 years ago. His body mass index (BMI) was about 29 and his weight was 191 pounds. His dry mouth and dry eyes were controlled with oral pilocarpine and carboxymethylcellulose eye drops, respectively. In his last rheumatologic examination, he denied any oral ulcers, sun sensitivity, Raynaud’s arthralgia, alopecia, abdominal pain, or new skin rash involvement elsewhere. He maintained a regular exercise regimen and was eating well. His medical history was remarkable for discoid lupus erythematosus, tinea unguium, nocturia, Sjögren syndrome, and hearing loss. His most recent blood pressure was 112/81 mmHg. Medications included carboxymethylcellulose 0.5% solution instilled 4 times daily for dry eyes, cholecalciferol 1000 unit tablet for bone strengthening, gabapentin 300 mg for nerve pain, hydroxychloroquine sulfate 200 mg for lupus, and pilocarpine HCl 5 mg for dry mouth. He applied sunscreen-35 PABA-free combination cream on the affected areas every 2 to 3 hours when out in the sun. He had a positive biopsy of the skin, negative antinuclear antibody (ANA), and positive Sjögren’s syndrome A (SSA). He was followed by his rheumatologist every

Fig. 2 Spectralis OCT of right and left eye shows average central macular thickness. Intra-retinal layers, RPE/Bruch’s membrane complex are intact with appropriate foveal contour.

6 months. Tables I and II show his pertinent laboratory test results. Ophthalmic examination revealed best-corrected visual acuities of 6/6 (20/20) OD and 6/6 (20/20) OS. Pupils were round, equal and reactive to light. Extraocular muscles were smooth, accurate, full and extensive. Cover tests was orthophoria at distance and 4 exophoria at near. His confrontation fields were full to finger counting. On exterior exam, he had a small, approximately 5 mm in size rash, on his right hand and an 8 mm elliptical shape rash above his left lip (Fig. 1). Findings in the anterior segment examination were all within normal limits for his age with the exception of grade 1 superficial punctate keratopathy and reduced tear breakup time to 4 seconds for both eyes. He has non-visually significant cataracts OU. Schirmer’s test with anesthesia was performed at this visit and was mildly reduced to 13 mm right eye and 14 mm left eye. His intraocular pressure was 15 mmHg both right and left eye at 11:30 a.m. with Goldmann applanation method. Posterior segment examination was within normal limits. View in was clear. Optic nerve head was average in size, pink and healthy with distinct margins. The cup-to-disc

What an Optometrist Needs to Know About Lupus Erythematosus — Landig, Ilsen

Fig. 3 Humphrey Visual Field 10-2 Swedish Interactive Threshold Algorithm pattern deviation plot revealed to be reliable with no signs of paracentral scotoma in both (A) left and (B) right eyes. Table III Laboratory results of Case 2 Laboratory

Results

Normal Values

ANA (EIA) Anti-dsDNA Anti-Smith/RNP Anti-SSA Anti-SSB CRP

4+ Positive 41.3 H IU/mL 144.5 H 3 EU/mL 0.6 EU/mL 0.09 mg/dL

Negative 0-25 0-16 0-16 0-16 <0.744-0.744

ratio was measured to be 0.50 round on both right and left eye. His retinal blood vessels were of good caliber. His Humphreys central 10-2 visual field revealed no paracentral scotoma. Spectralis optical coherence tomography (OCT) showed appropriate foveal contour, intraretinal layers were intact, and the RPE/Bruchâ&#x20AC;&#x2122;s complex was intact (Figs. 2, 3). All 14 color plates (Ishihara) were correctly identified when tested monocularly. Since his dry eyes and dry mouth were controlled well with pilocarpine pills and carboxymethylcellulose eye drops, he was instructed to continue using them and to return for routine follow-up in 1 year. Case 2 A 24-year-old Asian-American male patient reported for his yearly eye examination requesting new eyeglasses. He reported moderate visual changes in his right eye that started 7 days ago. His ocular history was significant for moderate dry eyes and myopia. His medical history was remarkable for systemic lupus erythematosus, hypertension, neck pain, hyperlipidemia, and acute renal impairment. He had arthritis, malar rash, Raynaudâ&#x20AC;&#x2122;s phenomenon, and proteinuria. He had several systemic signs of lupus flare: low-grade fever, polyarthritis, and diarrhea. Systemic medications included aspirin; atovaquone to prevent infection; cholecalciferol for vitamin D supplementation; docusate sodium for constipation; furosemide for water retention; gabapentin and hydrocodone for pain, Lisinopril for blood pressure; simvastatin for cholesterol;

Clinical & Refractive Optometry Quebec 1:3, 2016

and sunscreen-35 PABA-free combination for sun protection. He had no significant family ocular history. He denied any headache, eye trauma, or ocular surgery in the past. Table III shows a summary of significant laboratory test results. Ophthalmic examination revealed reduced bestcorrected visual acuities of 6/30 (20/100) OD and 6/6 (20/20) OS. A pinhole did not improve the visual acuity of the right eye. Pupils were round, equal, and reactive to light with no relative afferent pupillary defect. His extraocular muscles were smooth, accurate, full and extensive. Ocular alignment was orthophoria at distance and near. Confrontation fields were normal to finger counting. Gross assessment of his orbits revealed no evidence of trauma, ptosis, and Hirschberg were centered in both eyes. Slit lamp biomicroscopy showed grade 1 meibomian gland dysfunction and mild blepharitis, with unremarkable findings for the conjunctiva, sclera, iris, anterior chamber, and lens OU. The right cornea revealed diffuse punctate epithelial erosions and reduced tear breakup time to 8 seconds both eyes. Color vision test and visual fields were not tested at the initial visit. Dilated fundus examination revealed normal, well perfused discs, with a cup-to-disc ratio of 0.30 round on both eyes. The macula of the right eye appeared to be elevated with no foveal reflex, while the left eye was flat and intact. Spectralis macular OCT scan revealed an increased central macula thickness of 319 microns of the right eye and normal 283 microns of the left eye. There were many scattered cotton wool spots and dot-blot hemorrhages throughout the posterior pole of the right eye, while the left eye was homogenous. The peripheral retina was attached 360 degrees with no holes, tears, or breaks (Figs. 4, 5). A diagnosis of macular edema secondary to a central retinal vein occlusion likely due to an inflammatory process such as lupus was made. The patient was referred to rheumatology immediately for an evaluation of his lupus and he was scheduled for a next-day follow-up

Fig. 4 Macular Spectralis OCT of the right eye of Case 2 revealed intraretinal cystic space indicative of macular edema secondary to central retinal vein occlusion due to untreated Systemic Lupus.

Fig. 5 Spectralis Macular OCT of the right and left eye at initial presentation. Central macular thickness of the right eye is elevated due to macular edema secondary to central retinal vein occlusion from lupus retinopathy. Visual acuity of the right eye was 6/30 (20/100) and of the left eye was 6/6 (20/20).

DISCUSSION

positive for SLE; however, an approach that is often utilized by many rheumatologists to differentiate SLE from other systemic disease is a questionnaire (Table IV).4,5 If three or more questions are answered with a “yes,” SLE is a possibility and an ANA is warranted.4 Sometimes a patient may have had SLE for years but has remained undiagnosed for a variety of reasons. Primary care physicians and rheumatologists usually look for classic clinical signs: such as discoid rash, oral ulcers, and malar rash.5,6

Diagnosis of Lupus Erythematosus The diagnosis of LE is contingent upon iconic clinical features and supportive laboratory results. Early in the disease process, patients who are suspected to have SLE often experience classic symptoms such as unexplained nonspecific fever, fatigue, or weight loss.1 A photosensitivity rash may develop on the skin, primarily in areas that are exposed to sunlight. Raynaud phenomenon, arthritis, lung and heart inflammation, and alopecia are examples of early manifestations.1,3 Primary care providers who have patients with these manifestations will typically order a panel of laboratory testing, including screening for specific auto-antibodies. Complete blood count (CBC), metabolic profile, creatine kinase, erythrocyte sedimentation rate, and urinalysis are the common laboratory tests that are often ordered.4 Autoantibody testing include, but not limited to, antinuclear antibodies, antiphospholipid antibodies, antibodies to double-stranded deoxyribonucleic acid (dsDNA), and anti-Smith (Sm) antibody. For further evaluation, biopsies of the affected organs may also be performed.4 For example, if discoid lupus is suspected, a skin biopsy may be done.4 Clinical features, laboratory results, and antibody testing are all key in determining whether or not a patient is

General Pathophysiology Lupus is characterized as a type-III hypersensitivity reaction, which occurs when antigen-antibody complexes are not adequately cleared out by the innate immune system.3 There are many proposed mechanisms for lupus pathophysiology, making it a very complex disease. Lupus disease activity is thought to involve three main processes: activation of adaptive immunity, activation of innate immunity, and ineffective clearance of immune complexes.3-5 When a cell undergoes apoptosis, it induces surface blebs, which leak intracellular content into the extracellular space.3-5 Due to the ineffective clearance in lupus patients, more DNA is released. T-cells recognize and bind with the major histocompatibility complex (MHC) molecule on the surface nuclear antigen-presenting cell.3-5 However, this binding is not strong enough to stimulate the T-cell. In this mechanism, there have been theories of co-stimulatory molecular pairs, such as CD40-CD40 ligand and CD28-B7, which generates the second signal in activating the T-cell.3 This, in turn, will activate the B cell, releasing antibodies, which is targeted for double-stranded DNA. Normally C4 of the complement system inhibits the production of self-antigen.7

with retina clinic. He was started on hydroxychloroquine 200 mg b.i.d. by mouth by rheumatology. After 8 months of close follow-up with rheumatology, his macular edema resolved. He was corrected to 6/6 (20/20) in both right and left eyes with a low myopic prescription (Fig. 6).

What an Optometrist Needs to Know About Lupus Erythematosus — Landig, Ilsen

Table IV Questions for diagnosing systemic lupus erythematosus Questionnaire Do you have rheumatism or arthritis for at least 3 months? Are your fingers uncomfortable in the cold or feel numb? Have you had sores in the month for at least 2 weeks? Are you diagnosed with anemia, leukocytopenia, or thrombocytopenia? 5. Have you had a rash on the face for at least 1 month and has not improved? 6. Do you have skin reaction after being in the sun? 7. Do you have difficulty with taking deep breaths? 8. Do you have proteinuria? 9. Have you loss hair due to an autoimmune disease? 10. Have you had any seizure attack? 1. 2. 3. 4.

Fig. 6 Spectralis Macular OCT of the right and left after 8 months of close follow-up with rheumatology and hydroxychloroquine treatment. Best corrected visual acuity was 6/6 (20/20) in both right and left eye.

In lupus, complement system C1q, C2, C3 and C4 are deficient. Current researchers are looking into ways to inhibit the co-stimulatory molecular pairs as way to treat lupus.7,8 The normal action of the complement system is to promote immune response. The complement cascade ultimately forms C3b complex, an opsonin that promotes phagocytosis, and C3a, acting as a chemo-attractant for immune cells. If this complex fails, they eventually lead to the formation of membrane attack complex that lyses cells.7-10 In innate immunity, dendritic cells have toll-like receptors (TLR) that generally recognize foreign material in the body.7,8 For patients who have lupus, dsDNA antibody will be recognized by TLR. In addition, nucleosomes, which are combination of histones and DNA, are also recognized by TLR. Upon the binding, cytokines will be released (TNF-alpha, IL-10, IL-17, BAFF), which causes inflammation and recruitment of other cells, such as macrophages, T-cells, other dendritic cells.7-10 Inflammation is the culprit that causes permanent tissue damage in patients. The dsDNA antibody can accumulate and travel to different parts of the body, such as the lungs, brain, kidneys, skin, and joints.7-10 C1q is responsible for clearing out immune complex by phagocytosis.11 Consequently there will be an overproduction of growth factors and oxidative agents, which can lead to fibrosis of tissues.11 In addition to autoantibodies to dsDNA, SLE is also associated with autoantibodies to red blood cells (RBC), white blood cells (WBC), and platelets, which can lead to hemolytic anemia, leukocytopenia, and thrombocytopenia respectively.3,12,13 If someone is diagnosed with lupus, physicians often recommend patients to stop smoking, as there is a strong correlation between smoking and the disease process. The

Clinical & Refractive Optometry Quebec 1:3, 2016

most common treatment of lupus is hydroxycholoroquine, an anti-malarial drug.5 Other treatment modalities are nonsteroidal anti-inflammatory drug, systemic corticosteroids, methotrexate, cyclophosphamide, and azathioprine.4-6 Ocular Manifestation of Lupus Systemic lupus erythematosus can affect the eye quite extensively, including the adnexa, corneal surface, retina, and optic nerve. More common findings among SLE patients are keratoconjunctivitis sicca (KCS) and, if the disease process is not controlled, retinopathy.14,15 According to Sobrin et al, there have been cases where an ocular manifestation has manifested well in advance of the definitive diagnosis of systemic lupus.16 However, ocular manifestation is not part of the diagnostic scoring scale. External Involvement The classic sign of discoid lupus is a raised, scaly lesion on the skin. The lesions may appear like chronic blepharitis if they appear around the eyelid.16 A definitive diagnosis of discoid lupus can be established after a biopsy has been performed and several conservative topical therapies have been applied with no resolution.15,16 Keratoconjunctivitis sicca (KCS) is one of the most common ocular manifestation lupus patients may experience. According to Jensen et al, 12 out of their 20 patients with lupus reported at least one symptom of dry eyes.17 In a retrospective study of a cohort of 55 patients, KCS occurred in approximately 25% of patients.18 Other external manifestations that optometrists need to be mindful of are interstitial keratitis and conjunctivitis, though both are rare.17,18 Episcleritis and scleritis are manifestation of SLE, and according to Sobrin et al, they can precede diagnosis of SLE by as much as 3 years.16 Necrotizing and nonnecrotizing scleritis may manifest, but necrotizing type is less common with SLE as compared to rheumatoid arthritis. According to Situla, the incidence of episcleritis in adults

with SLE is 2.4%.19 In general, episcleritis is benign, with associated symptoms of red eye, dull sensation around the orbit, and tearing. Scleritis is more significant and is more of a concern for vision loss.16,19 Retinal Involvement Retinopathy is a common ocular manifestation among SLE patients. According to a prospective study by StaffordBrady, 88% of patients with lupus retinopathy had active disease.20 Common clinical retinal findings are cotton wool spots (CWS), which are microinfarctions of the nerve fiber layer with the cessation of axoplasmic flow, resulting in accumulation of mitochondria.21 CWS may be isolated or associated hemorrhages may appear. Contrasting with hypertensive and diabetic retinopathies, where arteries tend to be tortuous, SLE retinopathy displays relative dilation of the arteries.22 Other findings consist of hemorrhages and vasculitis.22 In Ushiyama et al’s clinical cross-sectional study of 69 patients, 7 of the 69 patients (10%) presented with retinopathy. In this study, the average age of onset of SLE with and without retinopathy was 34.2 and 31.9 years old, respectively.23 Findings from this study shown that patients with retinopathy also presented with proteinuria and central nervous system (CNS) disease. From the seven patients with retinopathy, four of them presented with proteinuria (57%). According to the study, patients with retinopathy had higher chance of developing CNS disease: 71%, with retinopathy compared to 13%, without retinopathy. The study suggests that retinopathy tends to develop with SLE patients who have renal or CNS disease, or both.22,23 The earliest manifestation of lupus retinopathy is hemorrhages and cotton wool spots. The pathogenesis is the deposit of immune complexes, such as immunoglobulin and complement deposits, in the vessel walls.24,25 Retinal vasculitis, inflammation of retinal arterioles and venules, tends to manifest in an acute process of the disease and in most cases has a poorer prognosis in terms of visual outcome.26 Hall et al’s study in 1984 reported the first positive antiphospholipid antibodies and retinal vasculopathy.27 Since then, more studies have supported a relationship between antiphospholipid antibody titer and the disease process.27 There has been a case report that identified central retinal artery/vein occlusion, vitreous hemorrhage, and neovascularization as a result of retinal vasculopathy in a lupus patient.26 Lupus retinopathy that leads to a central vein occlusion tends to manifest unilaterally. In Montehermoso et al’s study,28 among the SLE patients who had retinal involvement, 77% of them had positive antiphospholipid antibody titers, compared to patients who had no retinal involvement, of whom only 29% had positive titers. Rarely does lupus retinopathy become proliferative, but when it does with severe vasculitis, visual prognosis is quite poor. According to Jabs et al, more than 50% of the

affected eyes have visual acuity of 6/60 (20/200) or worse.29 With diffuse arteriolar occlusion and extensive capillary nonperfusion, retinal neovascularization will manifest.28,29 Often times the severity of retinopathy is linked directly to the activity of the disease process; however, there have been a few cases of proliferative retinopathy in patients with quiescent disease.29 Immunosuppression has been shown to improve retinopathy.27 Unfortunately, visual acuity loss from severe retinopathy is permanent, due to retinal ischemia.26 In addition to immunosuppressant, other treatment that was utilized to help visual outcome presented in literature were plasmapheresis and plasma exchange.30,31 Vitrectomy, panretinal photocoagulation, intravitreal antivascular endothelial growth factor agents were used to treat retinopathy involving retinal ischemia.26 Retinal vein occlusion is rare in patients with SLE, but has occurred in several reported cases with active lupus retinopathy. In patients who have proliferative retinopathy, pan-retinal photocoagulation is warranted using the same criteria from diabetes and branch retinal vein occlusion.32 A new case study conducted by Doruk et al showed positive outcome both visually and anatomically with intravitreal ranibizumab injection prior to PRP.33 Choroidal Involvement According to Palejwala, lupus choroidopathy is a rare finding, with only about 40 patients reported in the literature.26 Lupus choroidopathy is often linked to significant nephropathy, uncontrolled blood pressure, and CNS vasculitis.26 Sophisticated ocular imaging, such as Indocyanine green angiography (ICG) has been utilized to identify a change in choroidal circulation in patients who have active lupus.26,34 Fluorescein angiography demonstrates areas of focal early transient hypofluorescence, intermediate and late diffuse choroidal hyperfluorescence along with distortion of the large choroidal vessels.26,34,35 One study discussed six patients with active lupus who had multifocal serous elevation of the retina pigment epithelium (RPE) and sensory retina. Improvement of the serous detachment in three patients occurred with control of the systemic disease.34 Another study showed two cases of active lupus and correlation of multifocal RPE and serous retinal detachments.27 One of these patients showed deposits of immune complexes in Bruch’s membrane leading researchers to believe that the insult was due to anti-RPE antibodies.28 Using ICG, Baglio et al discovered a relationship between choroidopathy and active nephropathy.36 This finding was significant; however, several studies indicated that the pathophysiology of choroidopathy is multifactorial: uncontrolled hypertension,37 antiretinal pigment epithelium antibodies,38 and immune complex deposition into the choriocapillaris.39 Choroidopathy has been shown

What an Optometrist Needs to Know About Lupus Erythematosus — Landig, Ilsen

to respond to corticosteroids and immunosuppression. Choroidopathy is a good indication of aggressive disease process and systemic work-up is warranted with a rheumatologist.36-39 Neuro-Ophthalmic Involvement Optic nerve disease is possible in severe disease and manifests as optic neuritis or ischemic optic neuropathy, and occurs in about 1% of patients.40,41 Patients who have optic neuritis due to lupus will have very poor visual acuity; most of them will see worse than 6/60 (20/200).41 Optic neuritis secondary to lupus is different compared to that of optic neuritis secondary to multiple sclerosis (MS). Lupus induced optic neuritis is thought to be an ischemic process that leads to demyelination and axonal necrosis, which directly correlate to vision.41 In MS patients, the primary cause of optic neuritis is the inflammation causing a demyelinating process.41 The Optic Neuritis Treatment Trial (ONTT), 87% of patients recovered better than 6/7.5 (20/25) at 5 years follow-up. Comparing it to Lin et al study, only 50% of patients recovered to visual acuity better than 6/7.5 (20/25); 37.5% remained worse than 6/60 (20/200).42 Similar to optic neuritis due to MS, optic neuritis secondary to lupus responds well with high dose corticosteroids.41,42 Optic neuropathy is possible if the small blood vessels supplying the optic nerve head and retrobulbar nerve are affected by the lupus disease process.41,42 Patients may complain of a sudden vision loss and visual field testing will show altitudinal field loss.42 Optic neuropathy may or may not be associated with disc edema. Optic neuropathy often occurs bilaterally and responds well with high dose intravitreal corticosteroids following taper.42 Ocular motility can also be affected in the active disease process. According to Keane et al, eye movement abnormality has been reported in about 29% of patients.43 the cause is microvascular disease in the brainstem. Intern clear ophthalmoplegia is the most common cause of conjugate gaze abnormalities, while sixth nerve palsy is most common among dysconjugate gaze abnormalities.44-48 Ophthalmic Side Effects from Treatment Treatment for lupus can also cause ophthalmic morbidity, and optometrists need to be aware of what to watch for when patients are undergoing certain therapies. Corticosteroids are often used to treat lupus and may cause cataracts and steroid induced glaucoma. Another treatment modality that is very popular in the rheumatology community is hydroxychloroquine. If a patient is susceptible to hydroxychloroquine toxicity, and no intervention is done, irreversible blindness can result.28,29 Hydroxychloroquine Toxicity The American Academy of Ophthalmology recommendation for screening of chloroquine (CQ) and hydroxychloroquine (HCQ) retinopathy were updated in 2011

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with improved screening tools and new knowledge on the prevalence of the retinopathy. The prevalence of toxicity within the first 5 years of use was quite low and increased after 5 to 7 years of HCQ use or a cumulative dose of 1000 g to 1%.49 The risk is even higher after 15 to 20 years of use. One study found that risk increases 4 times after 7 years of use.50 In a retrospective medical record review performed at Doheny Eye Institute at the University of Southern California and at Northwestern University Sorrel Rosin Eye Center in Chicago, the average mean age toxicity development was 10.4 years, with a range of 3 to 10 years.51 There are also other factors that optometrists should be aware of that may increase the risk of retinopathy. Some risk factors include the duration of more than 5 years of use; cumulative dose of greater than 1000 g of HCQ and greater than 460 g of CQ; daily dose exceeding 400 mg/day of HCQ and greater than 250 mg/day of CQ; complications with the kidney or liver; and current presentation of maculopathy.49,51,52 The classic sign of toxicity is bilateral bullâ&#x20AC;&#x2122;s eye maculopathy. During funduscopic examination, there will be a ring of RPE depigmentation that spares a foveal island. The mechanism of CQ and HCQ toxicity is not fully understood but scientists have speculated that both drugs have a sudden effect on metabolism of retinal cells as they both bind to melanin in the retinal pigment epithelium (RPE) and this binding is known to prolong the toxic effect to the retinal structures.51-53 However, Mititelu et alâ&#x20AC;&#x2122;s study concluded that preserving the external limiting membrane (ELM) has some positive outcome because they believe that toxic effects may be associated with regeneration of the photoreceptor layer with improvement in visual function.51 The American Academy of Ophthalmology (AAO) recommends performing a baseline examination within 1 year of initiating treatment and annual screenings after 5 years of treatment in all patients. Humphrey central 10-2 visual field is recommended because it is readily available and can elucidate the degree of functional loss.52 Marmor et al emphasized the importance of a 10-2 visual field; their study concluded that about 10% of patients who had early HCQ toxicity showed a ring scotoma on the visual field in spite of a normal macular spectral domain OCT.53 Among the 2,000 patients who were enrolled in the study from Stanford and Kaiser Permanente who had used HCQ with cumulative doses larger than 1000 g, 150 had toxicity.53 All 2,000 patients had no history of macular disease.53 In addition to a 10-2 visual field, objective testing is also recommended. Spectral domain OCT (SD-OCT) is recommended since it is widely available, sensitive, and easy to use. Multifocal electroretinogram (mfERG) and fundus autofluorescence (FAF) are also sensitive and can detect early changes; however, their availability is sparse.52,54 Furthermore, the relative sensitivity and specificity have not been fully established with these tests.53

If a patient presents with new visual symptoms or any abnormalities are revealed with screening tests, a more careful evaluation is warranted. If a patient presents with possible early toxicity, the patient may elect to discontinue the medication with consultation with a rheumatologist, or to be monitored in 3- to 6-month intervals until there is enough evidence to rule out toxicity. Currently there is no definition of “early toxicity,” so any changes in the macula, especially the parafoveal regions, and changes in visual field should be taken seriously.52 If a patient presents with probable toxicity or “clear evident toxicity,” a consultation with the patient’s rheumatologist needs to be done immediately with recommendations to discontinue the medication.52,53 Manifestation with probable toxicity or clearly evident toxicity is defined as having either bilateral bull’s eye scotoma, bilateral paracentral mfERG, bilateral depigmentation, or parafoveal abnormalities on fundus autofluorescence.52 There should be a close follow-up regimen of 3 months to assess the progression and ensure that the patient understands the risks of the medication.52,56 Patients with probable toxicity would benefit with fullfield ERG and referring patients to medical centers who can perform such tests is recommended. When medication is discontinued, the patient should be re-examined in 3 months after discontinuation and then annually until findings are stable.52

CONCLUSION Ocular manifestations of lupus may be sight-threatening and can be an indicator of active disease. Very close follow-ups are warranted for patient who has active disease or history thereof. Ocular pain or a decrease in visual acuity warrants an immediate consultation with an eye specialist. The serious manifestations such as scleritis, lupus retinopathy, and choroidopathy need systemic work up. As primary eye care providers, one needs to identify when an appropriate, timely referral to a rheumatologist for SLE workup is warranted.56 When HCQ therapy is utilized, patients should be educated extensively regarding the importance of regular check-ups. Patients need to be advised to return to clinic with new visual symptoms, including, but not limited to the following: a decrease in visual sensitivity, reading difficulty, blind spots, or changes in systemic health, such as major weight changes, kidney or liver disease. Early detection of toxicity is crucial to prevent irreversible blindness. ❏

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Lahita RG, Phillips RH. Lupus Q&A revised and updated, 3rd edition: Everything You Need to Know. Avery Trade; 2014. Rubin RL. Drug-induced lupus. Toxicology 2005; 209: 135. Rahman A, Isenberg D. Systemic lupus erythematosus. New Engl J Med 2008; 358: 9. Liang MH, Meenan RF, Cathcart ES, Schur PH. A screening strategy for population studies in systemic lupus erythematosus. Series design. Arthritis Rheum 1980; 23: 153.

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Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1998; 40: 1725. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40: 1725. Von Muhlen CA, Tan EM. Autoantibodies in the diagnosis of systemic rheumatic disease. Semin Arthritis Rheum 1995; 24: 323. Tan EM, Feltkamp TE, Smolen JS, et al. Range of antinuclear antibodies in “healthy” individuals. Arthritis Rheum 1997; 40: 1601. Shiel WC Jr, Jason M. The diagnostic associations of patients with antinuclear antibodies referred to a community rheumatologist. J Rheumatol 1989; 16: 782. Smeenk R, Brinkman K, van den Brink H, et al. Antibodies to DNA in patients with systemic lupus erythematosus. Their role in the diagnosis, the follow-up and the pathogenesis of the disease. Clin Rheumatology 1990; 9: 100. Botto, M, Dell’Agnola C, Bygrave AE et al. Homozygous C1q deficiency causes glomerulonephritis associated with multiple apoptotic bodies. Nat Genet 1998; 19: 56-59. Eaton RB, Schnneider G, Schur PH. Enzyme immunoassay for antibodies to native DNA. Specificity and quality of antibodies. Arthritis Rheum 1983; 26: 52. Munves EF, Schur PH. Antibodies to Sm and RNP. Prognosticators of disease involvement. Arthritis Rheum 1983; 26: 848. Von Feldt JM. Systemic Lupus Erythematosus. Recognizing its various presentations. Postgrad Med 1995; 97(4): 79, 83, 86 passim. Cervera R, Khamashta MA, Font J, et al. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. Medicine (Baltimore) 1971; 50: 85. Sobrin L, Foster S. Systemic lupus erythematosus. Contemporary Ophthalmology 2006; 5(4): 1-8. Jensen JL, Bergem HO, Gilboe IM, Husby G, Axel T. Oral and ocular sicca symptoms and findings are prevalent in systemic lupus erythematosus. Oral Pathol Med 1999; 28: 317-322. Zufferey P, Meyer OC, Bourgeois P, et al. Primary systemic Sjögren’s syndrome preceding systemic lupus erythematosus: a retrospective study of four cases in cohort of 55 patients. Lupus 1995 ; 4: 23. Sitaula R, Shah DN, Singh D. The spectrum of ocular involvement in systemic lupus erythematosus in a tertiary eye care center in Nepal. Ocul Immuno Inflamm 2011; 19(6): 422-425. F. J. Stafford-Brady, M. B. Urowitz, D. D. Gladman, and M. Easterbrook, Lupus retinopathy. Patterns, associations, and prognosis. Arthritis Rheum 1988; 31(9): 1105-1110. Schwab IR, Dubielzig RR, Schobert C. Evolution’s witness: how eyes evolved. New York: Oxford University Press, 2012. Lanham, JG, Barrie T, Kohner M, Hughes RV. SLE Retinopathy: evaluation by fluorescein angiography. Ann Rheum Dis 1982; 41: 473-478. Ushiyama O, Ushiyama K, Koarada S, et al. Retinal disease in patients with systemic lupus erythematosus. Ann Rheum Dis 2000; 59(9): 705-708. Aiello JS. Ocular findings in lupus erythematosus. Am J Ophthalmol 1952; 35(6): 837-843.

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25. Aronson AJ, Ordonez NG, Diddie KR, Ernest JT. Immunecomplex deposition in the eye in systemic lupus erythematosus. Arch Intern Med 1979; 139(11): 1312-1313. 26. Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: a review of literature. Autoimmune Dis 2012; Vol. 2012, Article ID 290898, doi:10.1155/2012/290898. 27. Hall S, Buettner H, Luthra HS. Occlusive retinal vascular disease in systemic lupus erythematosus. J Rheumatol 1984; 11(6): 846-850. 28. Montehermoso A, Cervera R, Font J, et al. Association of antiphospholipid antibodies with retinal vascular disease in systemic lupus erythematosus. Semin Arthritis Rheum 1999; 28(5): 326-332. 29. Jabs DA, Fine SL, Hochberg MS, et al. Severe retinal vaso-occlusive disease in systemic lupus erythematosus. Arch Ophthalmol 1986; 104: 558. 30. Papadaki TG, Zacharopoulos IP, Papaliodis G, et al. Plasmapheresis for lupus retinal vasculitis. Arch Ophthalmol 2006; 124(11): 1654-1656. 31. Damato E, Chilov M, Lee R, et al. Plasma exchange and rituximab in the management of acute retinal vaso-occlusive disease in systemic lupus erythematosus. Ocul Immunol Inflamm 2011; 19(5): 379-381. 32. Vine AK, Barr CC. Proliferative lupus retinopathy. Arch Ophthalmol 1984; 102(6): 852-854. 33. Doruk HC, Cetin P, et al. Bilateral intravitreal ranibizumab injection and panretinal photocoagulation in a 16-year-old girl with vaso-occlusive lupus retinopathy. Ophthalmol Med 2013; Vol. 2013, Article ID 817186, doi: 10.1155/ 2013/817186. 34. Jabs DA, Hanneken AM, Schachat AP, Fine SL. Choroidopathy in systemic lupus erythematosus. Arch Ophthalmol 1988; 106(2): 230-234. 35. Matsuo t, Nakayama T, Koyama T, Matsuo N. Multifocal pigment epithelial damage with serous retinal detachment in systemic lupus erythematosus. Ophthalmologica 1987; 195: 97. 36. Baglio V, Gharbiya M, Balacco-Gabrieli C, et al. Choroidopathy in patients with systemic lupus erythematosus with or without nephropathy. J Nephrol 2011; 24(4): 522-529. 37. Hannouche D, Korobelnik JF, Cochereau I, et al. Systemic lupus erythematosus with choroidopathy and serous retinal detachment. Int Ophthalmol 1995; 19(2): 125-127. 38. Matsuo T, Nakayama T, Koyama T, Matsuo N. Multifocal pigment epithelial damages with serous retinal detachment in systemic lupus erythematosus. Ophthalmologica 1987; 195(2): 97-102. 39. Schwartz MM, Roberts JL. Membranous and vascular choroidopathy: two patterns of immune deposits in systemic lupus erythematosus. Clin Immunol Immunopathol 1983; 29(3): 369-380. 40. Hochberg MC, Boyd RE, Ahearn JM, et al Systemic lupus erythematosus: a review of clinico-laboratory features and immunogenetic markers in 150 patients with emphasis on demographic subsets. Medicine 1985; 64: 285-295.

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41. Feinglass EJ. Arnett FC, Dorsch CA, Zizic TM, Stevens MB. Neuropsychiatric relationship to other features of systemic lupus erythematosus: diagnosis, clinical spectrum and relationship to other features of the disease. Medicine 1976; 55: 323-339. 42. Smith CH. Optic neuritis. In: Miller NR, Newman NJ, eds. Walsh and Hoytâ&#x20AC;&#x2122;s Clinical Neuro-Ophthalmology, 6th Edition. Philadelphia: Lippincott Williams & Wilkins, 2005; Vol. 1: 293-347. 43. Keane JR. Eye movement abnormalities in systemic lupus erythematosus. Arch Neurol 1995; 52(12): 1145-1149. 44. Lin YC, Wang AG, Yen MY. Systemic lupus erythematosus-associated optic neuritis: clinical experience and literature review. Acta Ophthalmologica 2009; 87(2): 204-210. 45. Galindo M, Pablos JL, Gomez-Reino JJ. Internuclear ophthalmoplegia in systemic lupus erythematosus. Semin Arthritis Rheum 1998; 28(3): 179-186. 46. Cogen MS, Kline LB, Duvall ER. Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus. J Clin Neuroophthalmol 1987; 7(2): 69-73. 47. Meyer MW, Wild JH. Letter: unilateral internuclear ophthalmoplegia in systemic lupus erythematosus. Arch Neurol 1975; 32(7): 486. 48. Jarius S, Jacobi C, de Seze J, et al. Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders. Multiple Sclerosis Journal 2011; 17(9): 1067-1073. 49. Wolfe F, Markmor MF. Rates and predictors of hydroxychloriquine retinal toxicity in patients with rheumatoid arthritis and systemic lupus erythematosus. Arthritis Care Res (Hoboken) 2010; 62: 775-784. 50. Lyons JS, Secerns ML. Detection of early hydroxychloroquine retinal toxicity enhanced by ring ratio analysis of multifocal electroretinography. Am J Ophthalmol 2007; 143: 801-809. 51. Mititelu M, Wong BJ, Brenner M, Bryar PJ, Jampol LM, Fawzi AA. Progression of hydroxychloroquine toxic effects after drug therapy cessation: new evidence from multimodal imaging. JAMA Ophthalmol 2013; 131(9): 1187- 1197. 52. Marmor MF, Kellner U, Lai TY, Lyons JS, Mieler WF. Revised recommendations on screening for chloroquine and hydroxychloroquine retinopathy. Ophthalmology 2011; 118(2): 415-422. 53. Marmor MF, Melles RB. Disparity between visual fields and optical coherence tomography in hydroxychloroquine retinopathy. Ophthalmology 2014; 121(6): 1257-1262. 54. Lyons JS, Severns ML. Using multifocal ERG ring ratios to detect and follow Plaquenil retinal toxicity: a review of mfERG ring ratios in Plaquenil toxicity. Doc Ophthalmol 2009; 118: 29-36. 55. Kellner S, Weinitz S, Kellner U. Spectral domain optical coherence tomography detects early stages of chloroquine retinopathy similar to multifocal electroretinography, fundus autofluorescence and near infrared autofluorescence. Br J Ophthalmol 2009; 93: 1444-1447. 56. Vanderzee G, Tassi D. New trends in early diagnosis of hydroxychloroquine toxic retinopathy. Optometry 2012; 83(5): 200-207.

CATEGORY A UFC C REDIT A PPLICATION F ORM

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_______________________________________________________________________________ City

Office Phone: (

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QUESTIONNAIRE What an Optometrist Needs to Know About Lupus Erythematosus Mark L. Landig, OD; Pauline F. Ilsen, OD, FAAO 1. ❑ ❑ ❑ ❑

What is the prevalence of system lupus erythematosus among Northern Europeans? 10 cases per 100,000 persons 20 cases per 100,000 persons 30 cases per 100,000 persons 40 cases per 100,000 persons

2. ❑ ❑ ❑ ❑

In Case 1 of the paper presented, all of the following describe the patient’s medical history, EXCEPT: Hearing loss Pain above the left eye Tinea unguium Nocturia

All of the following describe the patient’s exterior ophthalmic exam in Case 1 of the paper presented, EXCEPT: Grade 2 superficial punctate keratopathy 5 mm rash on right hand Reduced tear breakup time Non-visually significant cataracts OU

❑ ❑ ❑ ❑

Clinical & Refractive Optometry Quebec 1:3, 2016

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4. ❑ ❑ ❑ ❑

In Case 2 of the paper presented, all of the following describe the findings of the patient’s ophthalmic examination, EXCEPT: Evidence of trauma Orthophoria at distance and near Confrontational fields were normal to finger counting Mild blepharitis

5. ❑ ❑ ❑ ❑

In Case 2 of the paper presented, all of the following describe the patient’s medical history, EXCEPT: Moderate dry eye Myopia Arthritis Discoid lupus erythematosus

6. ❑ ❑ ❑ ❑

Which of the following factors has been shown to be contributory to lupus erythematosus? Heredity Increasing age Smoking Raynaud’s disease

In a retrospective study of 55 patients, keratoconjunctivitis sicca (KCS) occurred in approximately what percentage of patients? 15% 20% 25% 30%

❑ ❑ ❑ ❑ 8. ❑ ❑ ❑ ❑

In Ushiyama et al’s study, what was the average age of onset of system lupus erythematosus with retinopathy? 25.2 30.2 33.2 34.2

9. ❑ ❑ ❑ ❑

Which of the following is one of the earliest manifestations of lupus retinopathy? Hemorrhages Ocular pain Loss of visual acuity Severe eye redness

10. According to Jabs et al, what percentage of eyes affected with severe vasculitis have visual acuity of 6/60 (20/200) or worse? ❑ >35% ❑ >45% ❑ >50% ❑ >55%

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CLICK HERE TO PRINT THIS UFC CREDIT ARTICLE AND TEST

Clinical & Refractive Optometry Quebec is pleased to present this continuing education (CE) article by Dr. Stacey Chong et al, University of Waterloo, School of Optometry and Vision Science, Waterloo, ON. This article has been approved for 1 Category A, UFC credit in General Optometry by the Ordre des Optométristes du Québec. In order to obtain your credit, please refer to page 112 for complete instructions.

Blind Spot Mapping: A Case Report Stacey Chong, OD, MSc, BSc; Patricia K. Hrynchak, OD, MScCH(HPTE); Michelle J. Steenbakkers, BScH, OD, FAAO; Derek Y. Ho, MD; Natalie Hutchings, MCOptom, PhD; Nadine M. Furtado, OD, MSc, FAAO

ABSTRACT The blind spot is an essential element in static perimetry as the absolute scotoma produced is used to ensure that the patient is fixating steadily throughout the test. If the optic nerve is anomalous, the scotoma might not be in the predicted location. This case outlines a few potential pitfalls in perimetry in a patient with an anomalous optic nerve head.

INTRODUCTION Visual field reliability indexes are essential to ensuring the validity of the test.1 The reliability indices are monitored throughout an automated Humphrey visual field test and include fixation losses, false positives and false negatives. Fixation is monitored to determine if the patient is shifting their point of gaze. False positives occur when a patient has responded when a stimulus was not presented. False negatives occur when the patient does not respond to a stimulus brighter than the predetermined threshold. It has been found that when reliability criteria are compared between normal patients and those with glaucoma, 30% and 45%, respectively had unreliable fields as determined by a failure to meet the criterion outlined by the manufacturer of the Humphrey Visual Field Analyzer (HFA).2 The manufacturer has indicated that fixation losses should not be greater than 20%, and false positives and negatives should not be greater than 33%. Nearly half of the instances where fixation losses were greater than 20% were caused by technical artifacts including improper initial determination of the blind spot and high false positives.3 Re-plotting the blind spot during testing has the potential to decrease the frequency S. Chong, P.K. Hrynchak, M.J. Steenbakkers, D.Y. Ho, N. Hutchings, N.M. Furtado — University of Waterloo, School of Optometry and Vision Science, Waterloo, ON Correspondence to: Dr. Stacey Chong, 85 Finch Avenue East, Toronto, ON M2N 4R4; E-mail: stacey.chong.od@gmail.com The authors have no financial interests. This article has been peer reviewed.

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of unreliable test results secondary to excessive fixation losses from 33% to 14%.3 The blind spot of the patient is plotted at the start of visual field testing. Fixation losses are monitored throughout testing by presenting a suprathreshold stimulus in the area of the predetermined blind spot. If the stimulus is detected, it is assumed the patient has lost fixation. When fixation losses are high, re-plotting of the blind spot may address the issue. Determination and tracking of the blind spot as a reliability index are used in both in kinetic perimetry and static perimetry.

CASE REPORT A 46-year-old Egyptian man presented for a full eye exam with the complaint of intermediate blurred vision. Medical history and family ocular history were unremarkable. He was not taking any systemic medications and he had no known drug allergies. Ocular history included bilateral LASIK surgery performed in Egypt in 2002. Pre-surgical refractive error was approximately -11.00 DS with -2.00 D of astigmatism in each eye, as reported by the patient. Best-corrected visual acuities were 6/6 (20/20) in the right (-0.25 -1.75 x 160) and left eye (+0.75 -1.50 x 015) for distance and near. Cover test was normal, eye movements were unrestricted and confrontation fields were full to finger counting in each eye. Pupils were round and reactive to light with no relative afferent pupillary defect detected. Slit lamp biomicroscopy was unremarkable. Intraocular pressures (IOPs, at 3:20 pm), measured with Goldmann applanation tonometry, were 20 mmHg in the right eye and 23 mmHg in the left eye. Gonioscopy revealed that the angles in both eyes were open to posterior trabecular meshwork in all quadrants. Pupils were dilated using one drop of 1% tropicamide and one drop of 2.5% phenylephrine. Dilated fundus exam revealed mild nuclear sclerosis. Asymmetric optic nerve cupping was noted with vertical cup-to-disc ratios (C/D) of 0.45 in the right eye and 0.60 in the left eye; C/D assessment was difficult due to a large tilt of the nerves. The optic nerve in the right eye followed the ISNT rule and there was a suspicion of thinning of the inferior rim of the left nerve. In addition, the right nerve was rotated 40 degrees clockwise and the left nerve 30 degrees counterclockwise. There was no evidence of pallor or edema of the neuroretinal rim in either eye. The vasculature of the fundus

Fig. 1 First Humphrey visual field of the right eye with a temporal defect.

Fig. 2 First Humphrey visual field of the left eye showing a temporal defect along with a superior nasal defect.

was normal and there were no holes, tears or breaks of the retina in either eye. Baseline Humphrey (740i, Carl Zeiss Meditec, Dublin, CA) automated 30-2 threshold SITA-standard visual fields (Figs. 1, 2) were carried out with good reliability (right: fixation losses 3/19, false positives 2%, false negatives 0%; left: fixation losses 2/22, false positives 1%, false negatives 0%). The right eye was within normal limits and the left eye was outside normal limits as determined by the Glaucoma Hemifield Test, which compares sectors of static threshold visual field results from above and below the horizontal meridian for symmetry based on the anatomy of the retinal nerve fiber layer.4 A temporal defect was found in the right eye and a temporal as well as superior nasal defect was found in the left eye. The mean deviations (MD) were -4.97 dB (right) and -8.54 dB (left), indicating the overall depression of the field from the normative database.5 The pattern standard deviations (PSD) were 3.13 dB (right) and 3.59 dB (left), representing the degree of difference between the measured visual field pattern and the normal hill of vision.5 The visual field index (VFI) was 92% (right) and 83% (left), referring to the estimation of the rate of change of glaucoma as a percentage of field loss relative to a group of normal observers.6 The diagnosis of tilted optic discs was made. A tilted optic nerve does not insert at a 90-degree angle into the eye, but rather obliquely, and typically is accompanied by a rotation along the anterior-posterior axis.7 Sequelae of tilted discs typically include myopia and astigmatism, as well as a temporal visual field loss that does not respect the midline, both of which were present in this patient.7 The differential diagnoses included glaucoma, a tumor of the anterior visual pathway and papilledema. The optic nerves did not show any signs of edema or papilledema, color vision was normal and the visual field defects did not respect the midline as would be expected with tumor involvement. The patient was also diagnosed as a glaucoma suspect due to the following risk factors: high myopia,

asymmetric C/D ratios, small optic nerve head with large cupping, rim tissue that did not follow the ISNT rule (left eye), and high IOPs (likely underestimated due to thin corneal tissue or decreased corneal hysteresis secondary to LASIK correction). A repeat Humphrey visual field 30-2 SITA standard test, Spectralis Optical Coherence Tomography (OCT; Heidelberg Engineering, Carlsbad, CA) of the optic nerve and macula, and pachymetry were ordered to further evaluate the risk of glaucoma. Follow-Up #1 (2 Weeks Later) A follow-up appointment revealed IOPs to be 19 mmHg in the right eye and 18 mmHg in the left eye at 2:45 pm. The slight asymmetry of the vertical optic nerve cupping was confirmed by a different clinician with stereoscopic fundus exam and photography (Figs. 3, 4). Humphrey visual field testing (Figs. 5, 6) showed good reliability of the right eye and 19/19 fixation losses in the left eye (gaze tracker was turned off). Similar although smaller defects on the visual field tests were found and the defects were much less dense than previous testing. A repeat visual field was scheduled, along with pachymetry and OCT. Follow Up #2 (10 Weeks Following the First Assessment) The IOPs, by Goldmann tonometry, at 5:13 pm were 24 mmHg in the right eye and 26 mmHg in the left eye; pachymetry was 496 µm in the right eye and 495 µm in the left eye. The vertical C/D ratios were judged to be 0.7 in the right eye and 0.8 in the left eye by a third clinician and verified with stereoscopic fundus photos. Humphrey visual field testing (Figs. 7, 8) of the right eye continued to be reliable, while the left eye once again showed high fixation losses of 5/19 (26%) and low reliability, despite re-plotting of the blind spot midway through the test. Humphrey visual field analysis over the three visits showed a consistent temporal defect in the right eye that became smaller and less dense over time and with practice. The left eye also showed a consistent temporal

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Fig. 3 Stereo photos of the patient’s right optic nerve. Note the large tilt (40 degrees clockwise) of the optic nerve and no evidence of pallor or edema of the neuroretinal rim.

Fig. 4 Stereo photos of the patient’s left optic nerve. Again, note the tilt (30 degrees counterclockwise) of the optic nerve and no evidence of pallor or edema of the neuroretinal rim.

Fig. 5 Second Humphrey visual field of the right eye showing a mild temporal defect.

Fig. 6 Second Humphrey visual field of the left eye showing a temporal defect. The previously noted nasal defect was not found. Note gaze tracking turned off and high fixation losses (19/19).

defect, along with what was thought to be a superior nasal defect that became less apparent over time. Imaging with the Heidelberg Retinal Tomography (HRT; Heidelberg Engineering, Carlsbad, CA) and OCT were attempted. Due to the axial length of the patient’s eye and the tilt of the optic nerve, HRT imaging was not possible. The OCT using the Heidelberg Spectralis (Fig. 9) was accomplished with fairly good quality (right: Q = 24, left: Q = 22). The OCT revealed bilateral small optic nerve heads (right: 1.30 mm2, left: 1.44 mm2). The nerve fiber layer thickness plots (TSNIT plots) demonstrated the general shape of two large peaks, although positioned closer together than average for each eye. The positioning of the peaks caused the nerves to be classified as outside normal limits (when compared to the Caucasian database) with thin superior and inferior rim tissue relative to the normative database. When the TSNIT plots were directly compared between the right and left eye, they were quite symmetrical and the height of each peak would have fallen in the normal range had the peaks been recorded in the appropriate location. When comparing the physical appearance of the optic nerve rim (structure) to the trend of visual field

defects detected by the three tests performed to this point (function), the temporal defects detected corresponded to tilted nerves. The left eye initially (first field) showed the start of an arcuate defect superiorly which would align with the initial speculation that there was inferior thinning of the left optic nerve. The suspected defect was not repeatable, although reliability of the tests was low due to high fixation losses. The OCT showed thinning of the superior nerve fiber layer (NFL) in the left eye and potentially both superior and inferior temporal thinning for both eyes. The latter would potentially correspond to an inferior field defect as well as nasal defects, which were not demonstrated by the visual field tests of the patient.

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Follow Up #3 (5 Months Later) At a fourth appointment, plotting of the blind spot (Octopus 900; Haag Streit, Mason, OH) using static perimetry (Fig. 10) showed only two absolute defects corresponding to the patient’s blind spot (right eye) that were not within the shaded area for the typical optic nerve, but were shifted closer to the fovea. The minimum number of points that may encompass the average optic nerve (blind spot) with this method was 11, which is

Fig. 7 Third Humphrey visual field of the right eye with a temporal defect.

Fig. 8 Third Humphrey visual field of the left eye with a temporal defect. High fixation losses (5/19) causing test to have low reliability.

of the blind spot indicated that it was located at (right eye) and/or just below (left eye) the horizontal meridian. Visual field testing (Figs. 12, 13) was repeated with the Octopus 900 and the right eye was performed reliably with a temporal defect. The test of the left eye was also reliable with a temporal defect and scattered nasal defects.

DISCUSSION

Fig. 9 OCT using the Heidelberg Spectralis showing symmetrical TSNIT plots with shifted peaks, although the nerves were classified as outside normal limits.

far greater than the two points that were absolute defects for this patient. In the left eye, the absolute defect was also closer to the fovea than the outlined normal optic nerve and there was only one spot that defined the patient’s blind spot. However, the majority of the points had a relative defect. When using the kinetic module (Octopus 900, Fig. 11) for plotting the blind spot, it was found to be less than a few degrees round in both the right and left eye, as well as shifted towards the foveae in each eye. The speed of the target was slightly higher than that typically used and this could account for the small size of the nerve, although this finding was consistent on both tests, static and kinetic. Both tests for determining the size

The patient was being monitored for glaucoma due to his risk factors that included high myopia, high IOPs, large cupping in a small disc and loss of the ISNT rule of the neuroretinal rim. Visual fields are integral for monitoring glaucoma progression and can be quite difficult to interpret when the results are unreliable. Fixation losses are a common cause of poor reliability and can be attributed to the patient’s ability to perform the test or due to technical artifacts.2 In this case, they were likely due to the patient’s very small blind spot from which fixation was monitored. When the test was set up accurately (Figs. 1, 2) and the patient was able to maintain fixation, there was good reliability. If not positioned correctly, fixation losses were high (Fig. 6), although the depth of the defects was shown to decrease slightly. By re-plotting the blind spot mid-way through testing, the fixation losses decreased but results were still unreliable (Fig. 8). Fixation losses, measured using the Heijl-Krakau method, can occur for other reasons than unsteady gaze, such as the initial blind spot plotted at the edge of a scotoma, a head tilt or an alteration in head position.8 It is suggested that when fixation losses are high, the other reliability indices should be taken into consideration to determine whether the visual field should be included for analysis.8 The assertion that the small blind spots were causing high fixation losses was hypothesized due to the severity of the patient’s tilted disc on fundus exam. The patient’s optic nerves were found to be structurally smaller than average by OCT, which was confirmed functionally when the blind spots were found to be smaller and closer to the fovea using the static and kinetic perimetry available on the Octopus 900. The OCT measurements of the optic

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Fig. 10 Mapping of the blind spot using static perimetry, left and right eye.

Fig. 11 Mapping of the blind spot using static perimetry on the Octopus 900, left and right eye showing very small areas of absolute defect corresponding to the blind spot.

Fig. 12 First visual field using the Octopus 900 of the right eye showing a temporal defect.

Fig. 13 First visual field using the Octopus 900 of the left eye showing a temporal defect with scattered nasal defects.

disc surface area were 1.30 mm2 in the right eye and 1.44 mm2 in the left eye, which is much smaller than the average optic disc surface of 2.69 mm2.9 Re-plotting of he blind spot multiple times throughout testing may be necessary to ensure a reliable test result. The patient performed the initial Humphrey visual field well but fixation losses on every additional field were very high and caused the field to be labeled unreliable. The variability could be attributed partially to the difference in skill between technicians administering the tests. The visual field carried out on the Octopus did not indicate any fixation losses but this was due to the type of fixation monitoring system used. The Octopus 900 has a monitoring system that includes fixation control and automated eye tracking to detect and compensate for patient’s blinking, and an automatic chin rest adjustment to account for a patient’s movement during testing. The HFA used was equipped with a gaze tracking system but the newer models (HFAII-i 750i) also have eye tracking that includes the ability to automatically align the head and eye as well as a vertex monitor to indicate if the patient has moved too far away. Older HFA models use the HeijlKrakau method that initializes a point for the blind spot and continues to check the same spot periodically throughout testing.8

Overall, the visual field tests performed by this patient demonstrated temporal defects as well as inconsistent nasal defects. The temporal defects did not respect the midline, which made a neurological etiology less likely. The defects were becoming more distinct and smaller over a period of time, which could be attributed to the patient’s learning curve with the test. When reviewing MD and the PSD for the right and left eyes separately, they were fairly consistent between visits and between the two different visual field units used. The average MD, from the Humphrey visual fields, for the right eye (Figs. 1, 5, 7) was -4.27 ± 1.21 dB (± standard deviation) and the average PSD was 2.87 ± 0.23 dB (± standard deviation). These values were very similar to those found on the Octopus visual field, MD of -4.20 dB and PSD of 3.40 dB (Fig. 12). The average MD from the Humphrey visual fields for the left eye (Figs. 2, 6, 8) was -7.70 ± 1.02 dB (± standard deviation) and the average PSD was 3.19 ± 0.39 dB (± standard deviation). These values were also very similar to those found on the Octopus visual field, MD of -7.00 dB and PSD of 4.00 dB (Fig. 13). The PSD values on both units were quite similar between the eyes and the asymmetry of the MD was consistent on both units. This depression may be due to a reduction in retinal sensitivity due to a potentially diffusely hypoplastic nerve that occurs with a tilted disc.7

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Of note, the blind spots were missing on all the grey scales of the Humphrey visual fields. The OCT images confirmed that the patient’s optic nerves were small which correlated well with the small blind spots on perimetry. The normative databases of the OCT are not useful when analyzing an atypical optic nerve head and nerve fiber layer, which was the case for this patient. It is referred to as “red disease” when an area is marked as abnormal and said to be outside the range of the database due to a misinterpretation.10 When the OCT was further analyzed including the average thicknesses of the superior and inferior nerve fiber layer, a slightly different pattern of the TSNIT plot than normal was found, although symmetry was noted between the right and left eye which appeared to indicate that the patient likely did not have glaucoma. Upon investigation, the peaks on our scan, the OCT misinterpreted the NFL thickness and included a blood vessel in its calculation. Retinal blood vessels create a barrier on the OCT scan so that imaging cannot be done on tissue located beyond the blood vessel; it appears as if there is only white space below the blood vessel. The peaks of tissue for our patient corresponded to the areas where blood vessels are found. This accounts for the initial symmetry that was noted and does not correctly identify the NFL. Of note, the OCT NFL scan has a standard 3.4 mm diameter circle location. The position of the scan circle can have an effect on the measurements of the thickness of the NFL and the peaks, which typically indicate the thickest area of NFL can be shifted and will not align with that of the printout.11 The nature of the scan would create peaks that fell further apart than the normative database with a small optic nerve head and closer together for a large optic nerve head. Ultimately the OCT did not definitively indicate the thickness of the patient’s NFL. However, since the OCT will most likely continue to scan this patient in the same manner on each visit, it can still be used as a tool for monitoring change in apparent thickness of the NFL. This patient had several risk factors for glaucoma, including high myopia, high IOPs (potentially higher than recorded because of the altered cornea post-LASIK) and large C/D ratios in small optic nerves. The patient’s recorded IOP values were in the upper range of normal (18 to 26 mmHg). This actually may have been underestimated due to the decrease in corneal hysteresis, a biomechanical property of the cornea or the difference in the bending properties as the pressure is assessed using applanation also known as the elasticity.12 Studies have shown that a decreased corneal hysteresis is commonly associated with a decreased central corneal thickness. Both are risk factors for the development of glaucoma, independent of IOP, because they may represent weakness in the structure of the eye which could encompass the lamina cribrosa.12 This patient did not have a family history of glaucoma and had no systemic risk factors. Both the visual field defects and the OCT findings were attributed

to the patient’s tilted optic nerve anatomy. The absence of a blind spot on the HFA was likely due to the small size of the optic nerve combined with the tilt of the optic disc, confirmed by blind spot mapping using the Octopus visual field and OCT imaging. This patient has not yet been diagnosed with glaucoma, but due to the risk factors, warrants close monitoring. Comparisons with the patient’s own data (rather than with normative data) will be the best way to establish the presence of glaucomatous changes, which will include both serial visual field and OCT measurements. Both visual field and OCT measurements are expected to remain stable in a patient with a tilted disc, but are expected to progress in the presence of glaucoma. If visual field tests continue to be unreliable, serial OCTs may be the best tool for monitoring. A diurnal measurement of IOPs should also be considered to determine the extent of pressure fluctuation.

CONCLUSION It is important to remember that there can be anomalies identified by technology that defy typical interpretation, especially when the patient does not fall within the parameters defining normative databases. The information that we obtain from our various instruments should be critically analyzed and integrated to extract the necessary valuable information. ❏

REFERENCES 1.

Newkirk MR, Gardiner SK, Demirel S, Johnson CA. Assessment of false positives with the Humphrey Field Analyzer II perimeter with the SITA Algorithm. Invest Ophthalmol Vis Sci 2006: 47(10): 4632-4637. 2. Katz J, Sommer A. Reliability indexes of automated perimetric tests. Arch Ophthalmol 1988; 106(9): 1252-1254. 3. Sanabria O, Feuer WJ, Anderson DR. Pseudo-loss of fixation in automated perimetry. Ophthalmology 1991: 98(1); 76-78. 4. Asman P, Heijl A. Glaucoma Hemifield Test. Automated visual field evaluation. Arch Ophthalmol 1992; 110(6): 812-819. 5. Yaqub M. Visual fields interpretation in glaucoma: a focus on static automated perimetry. Community Eye Health 2012; 25(79-80): 1-8. 6. Artes PH, O’Leary N, Hutchison DM, Heckler L, et al. Properties of the Statpac Visual Field Index. Glaucoma 2011; 52(7): 4030-4038. 7. Witmer MT, Margo CE, Drucker M. Tilted optic disks. Surv Ophthalmol 2010; 55(5): 403-428. 8. Nema HV, Nema N. Diagnostic Procedures in Ophthalmology. New Delhi: Jaypee Brothers Medical Publishers, 2003. 9. Jonas JB, Gusek GC, Naumann GO. Optic disc, cup and neuroretinal rim size, configuration and correlations in normal eyes. Invest Ophthalmol Vis Sci 1988; 29(7): 1151-1158. 10. Chong GT, Lee RK. Glaucoma versus red disease: imaging and glaucoma diagnosis. Curr Opin Ophthalmol 2012; 23(2): 79-88. 11. Gabriele ML, Ishikawa H, Wollstein G, Bilonick RA, et al. Optical coherence tomography scan circle location and mean retinal nerve fiber layer measurement variability. Invest Ophthalmol Vis Sci 2008; 49(6): 2315-2321. 12. Abitbol O, Bouden J, Doan S, Hoang-Xuan T, et al. Corneal hysteresis measured with the Ocular Response Analyzer® in normal and glaucomatous eyes. Acta Ophthalmologica 2010; 88(1): 116-119.

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QUESTIONNAIRE Blind Spot Mapping: A Case Report Stacey Chong, OD, MSc, BSc; Patricia K. Hrynchak, OD, MScCH(HPTE); Michelle J. Steenbakkers, BScH, OD, FAAO; Derek Y. Ho, MD; Natalie Hutchings, MCOptom, PhD; Nadine M. Furtado, OD, MSc, FAAO 1. ❑ ❑ ❑ ❑ 2. ❑ ❑ ❑ ❑

When comparing normal patients and those with glaucoma, what percentage had unreliable fields as determined by a failure to meet the criterion outlined by the manufacturer? 15% and 35%, respectively 20% and 40%, respectively 30% and 45%, respectively 45% and 50%, respectively With the Humphrey Visual Field Analyzer (HFA), where fixation losses were greater than 20%, what amount was caused by technical artifacts? Nearly one-quarter Nearly one-third Nearly half Nearly three-quarters

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3. ❑ ❑ ❑ ❑ 4.

Re-plotting the blind spot during testing has the potential to decrease the frequency of unreliable test results secondary to excessive fixation losses by what amount? From 25% to 10% From 33% to 14% From 35% to 13% From 40% to 10%

❑ ❑ ❑ ❑

The manufacturer of the HFA has stated that false positives and negatives should not be greater than what amount? 15% 20% 25% 33%

5. ❑ ❑ ❑ ❑

All of the following clinical signs describe the patient in the Case Report presented, EXCEPT: Abnormal cover test Unrestricted eye movements No relative afferent pupillary defect Unremarkable slit lamp biomicroscopy

6. ❑ ❑ ❑ ❑

In the Case Report presented, dilated fundus exam revealed all of the following, EXCEPT: Mild nuclear sclerosis Left nerve was rotated 40 degrees clockwise Asymmetric optic nerve cupping Right nerve was rotated 40 degrees clockwise

7. ❑ ❑ ❑ ❑

In the Case Report presented, what was the patient’s visual field index (VFI)? 75% (right) and 63% (left) 82% (right) and 75% (left) 90% (right) and 73% (left) 92% (right) and 83% (left)

8. ❑ ❑ ❑ ❑

The Case Report presented revealed all of the following optic nerve findings, EXCEPT: Abnormal color vision No signs of edema No signs of papilledema Normal color vision

9. ❑ ❑ ❑ ❑

In follow-up #2 in the Case Report presented, Humphrey visual field testing revealed what percentage of fixation losses? 22% 25% 26% 28%

10. ❑ ❑ ❑ ❑

The manufacturer of the HFA has stated that fixation losses should not be greater than what percentage? 10% 15% 18% 20%

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Clinical & Refractive Optometry Quebec is pleased to present this continuing education (CE) article by Kathryn Dailey and Dr. Leonid Skorin, Jr., Mayo Clinic Health System, Albert Lea, MN. This article has been approved for 1 Category A, UFC credit in Ocular Health by the Ordre des Optométristes du Québec. In order to obtain your credit, please refer to page 122 for complete instructions.

Homonymous Hemianopia in a Young Adult: A Case Report Kathryn Dailey, BS; Leonid Skorin Jr., OD, DO, MS, FAAO, FAOCO

ABSTRACT Background: When a patient presents with acute visual field loss correlating the pattern of loss with the area of visual pathway damaged is critical to proper patient management. Homonymous hemianopia field loss can occur with damage to any area of the retrochiasmal visual pathway and is often devastating for patients. In older adults the most common cause of homonymous hemianopia is ischemic infarction of the occipital lobe. In younger adults causes of homonymous hemianopia are more heterogeneous and multi-factorial.1,2 This often presents the clinician with a diagnostic challenge and indicates the need for a full systemic health evaluation. Although homonymous hemianopia is rarely reversible, determination of the underlying cause is especially important in young adults to help prevent a repeat vascular event, stroke, or myocardial infarction. Case Report: A 34-year-old male with sudden left-sided visual field loss and an occipital headache was referred for evaluation. Magnetic resonance imaging (MRI) revealed ischemic infarction of the right occipital lobe. A subsequent full stroke evaluation failed to uncover an etiology for the stroke. The patient was started on daily aspirin and clopidogrel and various methods to help the patient adapt to his visual field loss are being trialed. Conclusion: Eye care providers should be aware of management options for helping patients cope with field loss and the importance of determining the underlying etiology of homonymous hemianopia to maintain patient health. L. Skorin, Jr. — Consultant, Department of Surgery, Community Division of Ophthalmology, Mayo Clinic Health System, Albert Lea, MN K. Dailey — Pacific University College of Optometry, OD candidate, degree to be conferred May 2016 Correspondence to: Dr. Leonid Skorin, Jr., Mayo Clinic Health System, 404 West Fountain Street, Albert Lea, MN 56007; E-mail: skorin.leonid@mayo.edu This article has been peer reviewed.

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INTRODUCTION Damage to any portion of the visual pathway from the globe to the visual cortex will result in characteristic visual field changes. Homonymous hemianopia, or vision loss to the right or left hemifield of both eyes, occurs with damage to any portion of the visual pathway posterior to the optic chiasm (optic tract, lateral geniculate nucleus, optic radiations and visual cortex). Homonymous hemianopia is often highly disabling for patients causing problems with reading and visual scanning. In addition, these patients cannot drive in most states resulting in significant consequences for their vocational and personal lives. Based upon one retrospective case review study of 904 cases of homonymous hemianopia, men and women are equally affected. The mean age at onset in the study was 51 years old, although cases were reported in patients aged two to 92 years old. Right and left homonymous hemianopias were equally as common but incomplete homonymous hemianopia (60%) was more common than complete (40%). Approximately 45% were isolated with no other neurologic signs or symptoms. Lesions were most commonly located in the occipital lobe (45%) and the optic radiations (32%).1 In older adults the most common etiology of a permanent homonymous hemianopia is vascular (cerebral infarction and intracranial hemorrhage), accounting for 42% to 89% of cases.1,2 Other common causes include brain tumors, trauma, surgery and central nervous system disease. Uncommon causes include multiple sclerosis, infections, dementia, seizures, severe hyperglycemia and migraine.1-3 Clinically, patients with homonymous hemianopia may or may not be aware of their visual field loss. Visual acuity is preserved in most cases; but, patients often complain of difficulty with reading due to inability to scan into the blind field. Because the occipital lobe is the most common reported site of damage resulting in homonymous hemianopia, most patients will not have any other neurologic symptoms aside from visual field loss. Lesions of the optic tract can result in a relative afferent pupillary defect contralateral to the side of lesion. Another pupillary phenomenon reported secondary to lesions of the optic tract is pupillary hemiakinesia.2 This occurs when light shone onto the blind hemifield

results in a diminished or absent pupillary response; but, light shone onto the seeing hemifield results in a normal pupillary response. Lesions of the optic radiations will sometimes result in additional neurologic defects aside from field loss. Damage to the temporal lobe may also cause aphasia, memory deficits and auditory or visual hallucinations. Damage to the parietal lobe can result in additional sensory deficits or hemifield neglect if the nondominant hemisphere is damaged.2 Diagnosis of homonymous hemianopia occurs based upon confrontation visual field testing and automated perimetry. Automated perimetry should always be completed to allow for a full assessment of the pattern of field loss and to follow visual field changes. Neuroimaging is indicated in all patients diagnosed with homonymous hemianopia.4 MRI is most commonly used due to its superior ability to image soft tissue.

CASE REPORT A 34-year-old white male was referred to our clinic by the emergency room (ER) for evaluation of sudden vision loss. The patient initially presented to the ER reporting that half of his vision had disappeared suddenly while watching television 11 hours earlier and had not returned. He also reported a pounding headache located at the back of his head. It had started around the same time as the vision loss and gotten progressively worse. Other positive symptoms included nausea and photophobia. Negative symptoms included dizziness, numbness, tingling and weakness. The patient had recently completed a boot camp and reported that he was in good physical condition. He also denied any recent head injuries or illness. The patientâ&#x20AC;&#x2122;s past ocular history was unremarkable for any eye diseases or surgeries. His medical history was significant for depression, cervical spine fusion for neck pain, borderline hypertension, asthma and migraine headaches. The patient also reported having a stroke four years earlier but no magnetic resonance imaging (MRI) or other work up had been done to confirm the event. His symptoms after the stroke included left-sided weakness, short-term memory problems and a stutter. He stated that his symptoms had resolved after rehabilitation therapy. Current medications included an albuterol inhaler used as needed, clonazepam and nortriptyline. The patient reported allergies to droperidol, gabapentin, methylprednisolone and metoclopramide. The patientâ&#x20AC;&#x2122;s social history was positive for smoking one pack of cigarettes per day for 20 years. He denied any recent alcohol or illicit drug use. Upon presentation at the ER, peripheral pulse rate was measured to be 80 beats per minute, respiratory rate was 16 cycles per minute and blood pressure was 149 mmHg/85 mmHg. The patient was reported to be

alert and oriented to person, place and time with no focal neuro-logical deficits. Pupils were equal, round and reactive to light and accommodation. Extraocular motilities were full with no restrictions. A computed tomography (CT) scan of the head without contrast was completed and no abnormalities were reported. Laboratory work included a complete blood count (CBC) with differential and a basic metabolic panel. These laboratory findings were unremarkable. Upon presentation at our office later that day, uncorrected distance visual acuities were 6/30 (20/100) [with pinhole 6/12 (20/40)] in the right eye and 6/60 (20/200) [with pinhole 6/9 (20/30)] in the left eye. Uncorrected near visual acuities were 6/6 (20/20) in both right and left eyes. The patient reported normally wearing glasses but did not bring them with him. Pupils were equal, round and reactive to light without a relative afferent pupillary defect. Extraocular muscles were full but with excessive refixations. Cover testing at distance and near revealed orthophoric ocular alignment. Confrontation visual fields revealed left homonymous hemianopic field loss. Slit lamp examination of the anterior segment was unremarkable. No eyelid ptosis was present. A dilated fundus examination was also unremarkable. A Humphrey central 24-2 threshold visual field test confirmed a complete left homonymous hemianopia (Figs. 1 and 2). The left field loss was dense with most points being absolute defects. There were also scattered defects in the right visual field of both eyes but with no apparent pattern. Due to the density and completeness of the left homonymous hemianopia it was suspected to be secondary to a cerebrovascular accident affecting the right occipital lobe. The patient was referred back to the ER for a stroke evaluation. All vital signs, neurologic testing and laboratory work was normal during this second ER visit. Additional testing included MRI with and without contrast, magnetic resonance angiogram (MRA) and magnetic resonance venography (MRV) of the brain. The MRI revealed recent infarcts predominantly involving the right occipital lobe with smaller infarcts in the right thalamus and left occipital lobe (Fig. 3). The CT scan from earlier in the day was re-evaluated by the radiologist and a small area of hypodensity in the right occipital lobe corresponded to the larger area of infarction shown on the MRI (Fig. 4). The MRA and MRV revealed no abnomalities. There was no evidence of intracranial aneurysm, large vessel occlusion or stenosis or dural venous sinus thrombosis. Due to MRI evidence of several strokes in the bilateral posterior cerebral artery distributions, the patient was admitted to the neurology stroke service. The patient was given 324 mg aspirin and directed to begin 81 mg of aspirin daily. No intravenous (IV) tissue plasminogen activator (TPA) was administered since the symptom onset was

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Fig. 1 Humphrey central 24-2 threshold visual field test of the right eye showing dense left hemianopia with scattered inferior nasal defects.

Fig. 2 Humphrey central 24-2 threshold visual field test of the left eye showing dense left hemianopia with scattered inferior and superior nasal defects.

Fig. 3 Axial diffusion weighted imaging (DWI) MRI showing large area of infarction in the right occipital lobe and a focal area of infarction in the left occipital lobe.

Fig. 4 Axial CT scan showing subtle area of hypodensity that corresponds to the large area of infarction in the right occipital lobe shown on MRI.

greater than four and a half hours prior. The patient was also not started on statin therapy because there was no evidence of coronary heart disease. Nicotine cessation counseling was completed and nicotine replacement therapy was started. Stroke labs, including a fasting lipid panel, hemoglobin A1c, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and a urine toxicology screen were completed and no abnormalities were found. Blood cultures to rule out infection predisposing to endocarditis were normal. Chest x-rays to rule out aspiration were normal. An electrocardiogram (ECG) showed normal sinus rhythm with a ventricular rate of 67 beats per minute. A transesophageal echocardiogram (TEE) also showed normal sinus rhythm with a ventricular rate of 66 beats per minute. There was no evidence of intracardiac mass or thrombus. The ventricular chamber size and heart valves were normal. No heart murmurs were appreciated on auscultation. A computed tomography angiography (CTA) of the neck was ordered to rule out a vertebral or carotid artery dissection. The CTA was negative for any significant vascular abnormalities in the neck.

No definite etiology of the stroke was established; but, the mechanism was thought to be cardioembolic. The patient was discharged and asked to start the 81 mg aspirin therapy and wear a Holter monitor for 24 hours to assess for intermittent atrial fibrillation. If no defects were found with the Holter monitor, prolonged cardiac monitoring was recommended. Two days after discharge from the stroke center the patient presented back to the ER with concerns of further vision changes. He reported that his vision became fuzzy around the edges and got dark just after doing pushups. The vision change lasted approximately 45 minutes. Associated symptoms included a throbbing biparietal and bitemporal headache that he reported had been continuous since his first presentation at the ER three days earlier. All vital signs and neurological testing were again normal. Pupils were equal, round and reactive to light. Extraocular muscles were full with no restrictions. Another CT scan of the head without contrast was ordered. No additional infarcts or defects other than those noted previously were seen. Another ECG was completed

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and no electrical defects were found. The data from the Holter monitor showed basic sinus rhythm with one episode of second-degree heart block. The patient was put on 75 mg of clopidogrel in addition to the 81 mg of aspirin he had been taking and was discharged with instructions to see his primary care physician (PCP) within one week. Later that week, the patient’s PCP ordered another ESR and an antinuclear antibody test (ANA) to rule out vasculitis as a contributing factor to the stroke. These tests came back normal. The PCP recommended placing the patient on an event monitor for prolonged cardiac monitoring. Additionally, she recommended a follow-up with our clinic in two months’ time for a visual field recheck. The patient was advised to continue taking the 81 mg aspirin and 75 mg clopidogrel in addition to his regular medications. Just over one week after his appointment with his PCP, the patient presented back at our clinic requesting to be fit with contact lenses so he could wear sunglasses more often. Since the stroke he had become highly photosensitive and reported that being outside gave him headaches. He also reported difficulty adapting to the left-sided visual field loss. He was not driving but felt uncomfortable moving around outdoors where there was more motion in his surroundings. Habitual spectacle corrected distance visual acuities were 6/6-1 (20/20-1) in both the right and left eyes. The manifest refraction was -1.25-0.50x147 in the right eye and -2.00 DS in the left eye. Best corrected visual acuities were 6/4 (20/15) in the distance for both the right and left eyes and 6/6 (20/20) at near for each eye. The patient had a history of successful Acuvue Oasys 8.4 mm base curve contact lens wear so he was refit into these lenses. A -1.25 DS lens on the right eye and a -1.75 DS lens on the left eye resulted in 6/6 (20/20) acuities for each eye at both distance and near. Slit lamp examination of the contact lenses showed an excellent fit with good centration, coverage and movement in primary and up-gaze in both eyes. The patient was also counseled regarding modalities available for coping with his field loss. He was interested in the idea of yoked prism. Ten prism diopters base left before each eye was trialed in office. With the prism the patient reported being able to see the doors on his left as he walked down the hallway in the office. He did not notice that his right visual field was any smaller but did note that the prism made him feel slightly nauseated. After a long discussion with the patient, prism lenses were not prescribed at this appointment. Both insurance issues and the patient's desire to have more time to adapt to the field loss contributed to our decision to wait. The patient returned for his one week contact lens follow-up with no complaints. A contact lens prescription was

released. The patient was scheduled for a follow-up visual field, as requested by his PCP, in eight weeks’ time. As suggested by his PCP, the patient wore an event monitor for 30 days to allow for prolonged cardiac monitoring. The results of the event monitor were unremarkable. The etiology of the patient’s stroke is still unknown.

MANAGEMENT Many studies have shown that the blind hemifields of patients with homonymous hemianopia often retain some visual functions and can improve spontaneously.2,5 The reported rate of spontaneous improvement varies between 18% and 67% but most studies agree that recovery is negligible after two to three months and unlikely after six months.5 Field defects of traumatic origin are most likely to show improvement while defects of vascular origin are least likely. If improvement is seen, it occurs in stages starting with perception of light, motion, form, color and finally depth.5 Although the prognosis for visual recovery is poor, treatments for patients with homonymous hemianopia can reduce disability associated with the field loss, improve patient confidence and facilitate independence. Treatment approaches include surgical revascularization, optical aids and cognitive rehabilitation techniques.4,5 Surgical revascularization is controversial and is based upon the idea that after a stroke there is an area of nonfunctioning but viable tissue that could recover function if blood flow is restored. There are case reports of neurologic defects reversing after surgical revascularization; but, there are no current guidelines to identify patients with neurologic deficits and viable tissue that could benefit from revascularization. In addition, the bypass procedures are associated with excess mortality.5 Optical aids are the most commonly used treatment modality for patients with homonymous hemianopia. One form of optical aid is the hemianopic spectacle. These glasses involve fitting a small mirror onto the spectacle frame that can be adjusted to allow the patient to see the reflection of objects in the blind field. The mirror is placed on the eye that coincides with the field loss. Prisms can be added unilaterally or bilaterally to a spectacle prescription in order to shift images from the blind field into the seeing field. Prescription of bilateral, yoked prism can result in reduced visual acuities due to decreased image quality. Prescription of unilateral prism results in central diplopia.4 In either case, the lenses will require adaptation time. Prism ranging from 10 to 20 prism diopters is usually used with the base oriented towards the side of the field loss. Prism used in this fashion does not result in a true expansion of the visual field but rather shifts the location of the field. An additional prism technique known as the Peli Lens utilizes sector press-on Fresnel prisms placed superior and inferior to the visual axis. This technique

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requires the patient to learn to scan into the prisms but results in true expansion of the visual field unlike the other two prism approaches. With all of the optical aid treatment options the patient must be highly motivated to practice with the device. Many patients are confused by the optics of these systems and can feel nauseated while wearing them. Reports on the effectiveness of optical aids vary; but, some patients report increased ability to complete their activities of daily living. It is also important to remember that while these aids may help with ambulation they cannot be used for driving. The final treatment modality used in patients with homonymous hemianopia is cognitive rehabilitation techniques. This involves strategies such as compensatory oculomotor training to enhance a patientâ&#x20AC;&#x2122;s ability to scan into their blind field. Computerized saccadic training is often beneficial for this. Helping patients develop strategies to cope with field loss while reading can also be very valuable. Right-sided hemianopia is generally more disturbing for English speakers due to the need to read left to right. However, left-sided hemianopia can also present difficulties due to the eye movements required to return to the beginning of a new line of text. Many patients benefit from simple adaptations such as using their finger or a ruler positioned under each line of text to help them keep their place. Others train themselves to read vertically and avoid the blind hemifield completely.4

DISCUSSION When a patient presents with acute visual field loss, the clinicianâ&#x20AC;&#x2122;s initial assessment must determine the presence of other neurological defects and the exact type of visual field damage. This will help localize the area of injury to the visual pathway and direct imaging studies. Damage to the visual pathway anterior to the chiasm will present as a monocular visual field defect. Lesions within the optic chiasm will most often show bitemporal defects and rarely binasal defects. Damage to the pathway posterior to the chiasm (optic tract, lateral geniculate nucleus, optic radiations and visual cortex) will present as homonymous defects. Homonymous field loss will be contralateral to the side of the intracranial lesion. Defects in the retrochiasmal pathway can cause hemianopias or quadrantanopias. Hemianopic field loss respects the vertical meridian but crosses the horizontal meridian of the visual field and can occur with large enough damage to any area of the retrochiasmal pathway. Quadrantanopic field loss respects the vertical and horizontal meridians of the visual field. Quadrantanopias indicate damage to either the superior or inferior bank of the occipital cortex or the optic radiations in either the parietal or temporal lobe. Both hemianopias and

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quadrantanopias can be classified as complete or partial and congruous or incongruous. A complete hemianopia or quadrantanopia indicates that the field loss respects the appropriate meridians and involves the entire hemifield or quadrant. All other defects are defined as partial or incomplete. Complete defects occur with lesions that damage all of the visual fibers in one area of the pathway. Only defects that are partial can be classified as congruous or incongruous.4 Traditionally, the more congruous or symmetric the field loss is between the two eyes, the further back in the retrochiasmal pathway the damage has occurred. Both the completeness and congruity of visual field loss are helpful to consider but are not always reliable indicators of the location of the damage.2 Stroke in Young Adults Although ischemic stroke is one of the most common reported causes of homonymous hemianopia, our patientâ&#x20AC;&#x2122;s presentation is unexpected due to his young age. Just fewer than 10% of all strokes occur in patients younger than 55 years of age.6 Despite survival and functional outcome from stroke being much better in younger adults, patients often have significant residual emotional, social or physical impairments that decrease quality of life and make employment difficult.7,8 One study reported that only 42% of young adults are able to return to work after having a stroke.9 Another study reported that 55% of young adults have some level of depression following stroke, ranging from borderline to extreme.8 Common causes of stroke in younger adults include congenital heart problems, hematologic conditions, vasculopathy (such as arterial dissection), metabolic disorders, pregnancy, migraine, and drug ingestion. This population may also have risk factors such as hypertension, hyperlipidemia and diabetes that are known to increase the risk of stroke in older adults.6,9 Up to 50% of strokes in young adults have also been linked to smoking.6,10 The potential causes of stroke in the younger population are multifactorial and more heterogeneous than in older patients. Despite a comprehensive work-up, stroke cause remains undetermined in up to 20% of cases.6 Determining stroke etiology is of the utmost importance because recurrence of vascular events occurs in up to 10% of patients within five years.6,9 The assessment of stroke in younger adults often requires a different approach than in older adults. A sequential diagnostic work-up is suggested in order to improve the yield of diagnostic tests and minimize risks and costs for the patient. The first line screening after ischemic stroke usually involves MRI of the brain and blood vessels and a cardiac assessment.6 These tests can rule out the most frequent causes of stroke in younger patients: cardioembolism (20%) and arterial dissection (15%).10 The next step of

testing should be guided by clinical suspicion based on patient medical history and the presence of other symptoms. Suggested tests include lumbar puncture, blood culture, Holter-ECG, transesophageal echocardiography, and HIV and syphilis serologies. More invasive tests such as angiography should be reserved for cases when all other diagnostics have been unsuccessful in establishing an underlying cause.6 A retrospective case study of ischemic stroke in 215 young adults between the ages of 18 and 45 reported that Holter monitoring, toxicology screening and a vasculitis panel had a relatively low diagnostic yield compared to echocardiography and angiography. It was also reported that stroke etiology was attributed to large-artery atherosclerosis and small-vessel disease in less than 10% of cases.6 This suggests that the vascular risk factors common in older adults may increase the susceptibility to stroke from other causes in young adults but are not typically the primary factor. Long-term risk of mortality is higher for young patients who suffered from a stroke secondary to large vessel disease than for those patients with stroke of unknown etiology.7 The overall risk of vascular death, repeat stroke or nonfatal myocardial infarction is approximately 15% over six years.9 Treatment for young adults after stroke most commonly includes antiplatelet or anticoagulant therapy. The ideal duration, safety, and efficacy of these medications has not been established for this population. Cholesterol lowering medications like the statins are used in a minority of patients and thrombolysis is used in a lower percentage of patients than in older adults.9 Studies show that a large percentage of patients make efforts to control modifiable risk factors such as smoking, oral contraceptive use and high blood pressure after stroke.7 In addition, up to 95% of young adults report satisfaction with post-stroke rehabilitation in physical therapy, speech therapy and occupational therapy.7,8

CONCLUSION MRI findings indicate that the cause of our patient’s left homonymous hemianopia was an ischemic stroke in the posterior cerebral artery distribution serving the right occipital lobe. While the visual field loss corresponds well to the area of damage in the occipital lobe, the etiology of our patient’s stroke is still undetermined. The extensive battery of tests completed on our patient has ruled out many of the common etiologies in young adults. The mechanism is postulated to be cardioembolic and our patient’s 20-year history of cigarette smoking is likely a contributing factor. Migraines have also been shown to be a risk factor for stroke and his positive history of migraines could also be contributory. The patient is continuing with 81 mg of aspirin and 75 mg of clopidogrel daily. He is also trying to quit smoking with the help of nicotine replacement therapy.

While determining stroke etiology is important to reduce the likelihood of future complications, it is also important for primary eye care providers to remember to help patients cope with their visual field loss. Interventions will depend upon each individual’s needs, activities and specific deficits and there are many variables that can be altered to allow significant improvements in functionality. Contact lenses, sunglasses, prism, reading strategies, rehabilitation and appropriate referrals can increase the quality of life of these patients and help them focus on making the most of their remaining vision. ❏

REFERENCES 1.

Zhang X, Kedar S, Lynn MJ, et al. Homonymous hemianopias. Clinical-anatomic correlations in 904 cases. Neurology 2006; 66(6): 906-910. http://neurology.org/ content/66/6/906.long. Accessed September 29, 2015. 2. Biousse V, Kedar S, and Newman N. Homonymous Hemianopia. http://www.uptodate.com/contents/homonymous-hemianopia. Updated February 2015. Accessed September 24, 2015. 3. Strowd RE, Wabnitz A, Balakrishnan N, et al. Clinical Reasoning: Acute-onset homonymous hemianopia with hyperglycemia: seeing is believing. Neurology 2014; 82(15): e129-133. www.neurology.org/content/82/15/ e129.fall.pdf. Accessed September 24, 2015. 4. Biousse V, Newman N. Visual Loss: An Overview. In: Biousse V and Newman N. Neuro-ophthalmology Illustrated. 1st ed. Thieme; 2009: 498-500. 5. Pambakian ALM, Kennard C. Can visual function be restored in patients with homonymous hemianopia? British Journal of Ophthalmology 1997; 81(4): 324-328. www.ncbi.nim.nih.gov/pmc/articles/pmc1722157/pdf/v08 1p00324.pdf. Accessed September 24, 2015. 6. Ruijun J, Schwamm L, Pervez M, et al. Ischemic Stroke and transient ischemic attack in young adults. Risk factors, diagnostic yield, neuroimaging, and thrombolysis. JAMA Neurology 2013; 70(1):51-57. doi: 10.1001/jamaneurol.2013.575. Accessed September 24, 2015. 7. Kappelle LJ, Adams HP, Heffner ML, et al. Prognosis of young adults with ischemic stroke. A long-term follow-up study assessing recurrent vascular events and functional outcome in the Iowa Registry of Stroke in Young Adults. Stroke 1994; 25: 1360-1365. doi: 10.1161/01.STR.25.7.1360. Accessed September 24, 2015. 8. Americai E, Poenaru D. The post-stroke depression and its impact on functioning in young and adult stroke patients of a rehabilitation unit. Journal of Mental Health 02/ 2015; 24(2): 1-5. http://www.tandfonline.com/doi/full/ 10.3109/09638237.2015.1022251. Accessed September 29, 2015. 9. Naess H, Waje-Andreassen U, Thomassen L, et al. Do all young ischemic stroke patients need long-term secondary preventive medicine? Neurology 2005; 65(4): 609-611. doi: http://dx.doi.org/10.1212/01.wnl. 0000173029. 89752.7b. Accessed September 24, 2015. 10. Smith S, Fox C. Ischemic stroke in children and young adults: Etiology and clinical features. http://www.uptodate. com/contents/ischemic-stroke-in-children-and-youngadults-etiology-and-clinical-features. Updated January 2015. Accessed September 24, 2015.

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QUESTIONNAIRE Homonymous Hemianopia in a Young Adult: A Case Report Kathryn Dailey, BS; Leonid Skorin Jr., OD, DO, MS, FAAO, FAOCO 1. ❑ ❑ ❑ ❑

Which of the following statements describing cases of homonymous hemianopia in a retrospective case review study are true? Men are more affected than women Women are more affected than men Men over age 55 are more affected Men and women are equally affected

2. ❑ ❑ ❑ ❑

What was the mean age of onset of homonymous hemianopia in the study cited in the paper? 45 years old 51 years old 55 years old 60 years old

In older adults, vascular etiology accounts for what percentage of patients with a permanent homonymous hemianopia? 21%-40% 40%-69% 41%-75% 42%-89%

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4. ❑ ❑ ❑ ❑

All of the following are possible causes of a permanent homonymous hemianopia, EXCEPT: Brain tumors Surgery Parkinson’s disease Infections

5. ❑ ❑ ❑ ❑

Damage to the temporal lobe may cause all of the following, EXCEPT: Speech deficits Aphasia Memory deficits Auditory hallucinations

6. ❑ ❑ ❑ ❑

In the Case Report presented, upon presentation to the ER the patient was found to have all of the following clinical signs, EXCEPT: Orientation to place and time No focal neurological deficits Lack of orientation to person Full extraocular motilities

7. ❑ ❑ ❑ ❑

What is the reported rate of spontaneous improvement in patients with homonymous hemianopia? Between 13% and 55% Between 18% and 67% Between 20% and 75% Between 25% and 80%

8. ❑ ❑ ❑ ❑

What is the incidence of stroke in patients younger than 55 years of age? Fewer than 5% Fewer than 8% Fewer than 10% Fewer than 15%

9. ❑ ❑ ❑ ❑

What percentage of young adults have some level of depression following stroke? 42% 50% 55% 60%

10. ❑ ❑ ❑ ❑

Stroke cause remains undetermined in what percentage of cases? Up to 15% Up to 20% Up to 30% Up to 35%

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