Sharp Edge Eye Syndrome: A Case Report and Survey of Self-Identified Individuals

Sharp edge eye syndrome (SEES), sometimes known as visual looming syndrome, is a condition in which the patient experiences ocular pain or discomfort when viewing or mentally picturing sharp objects and edges. Patients may present for medical care because they perceive the condition to represent an ophthalmic problem or a sign of a more serious underlying condition. An individual case report of SEES is included to aid in illustrating syndrome characteristics. Our aim is to describe the syndrome, vision-related quality of life (VRQOL), and psychosocial characteristics in patients with self-identified SEES.

Original Contribution Section Editors: Clare Fraser, MD Susan Mollan, MD Sharp Edge Eye Syndrome: A Case Report and Survey of Self-Identified Individuals Merrick S. Reynolds, MS, Bradley J. Katz, MD, PhD, Kathleen B. Digre, MD, Ben J. Brintz, PhD, Lenora M. Olson, PhD, Judith E. A. Warner, MD Background: Sharp edge eye syndrome (SEES), sometimes known as visual looming syndrome, is a condition in which the patient experiences ocular pain or discomfort when viewing or mentally picturing sharp objects and edges. Patients may present for medical care because they perceive the condition to represent an ophthalmic problem or a sign of a more serious underlying condition. An individual case report of SEES is included to aid in illustrating syndrome characteristics. Our aim is to describe the syndrome, vision-related quality of life (VRQOL), and psychosocial characteristics in patients with self-identified SEES. Methods: A cross-sectional web-based survey was made available on social media webpages dedicated to SEES. The study included 22 questions developed by the research team, demographic questions, and 4 standardized questionnaires [ID Migraine, the National Eye Institute’s Visual Function Questionnaire (NEI-VFQ-25), General Anxiety Department of Ophthalmology and Visual Sciences (MSR, BJK, KBD, and JEAW), John A Moran Eye Center, University of Utah Health Sciences Center, Salt Lake City, Utah; Department of Neurology (BJK, KBD, and JEAW), University of Utah Health Sciences Center, Salt Lake City, Utah; Division of Critical Care (BJB), Department of Pediatrics, University of Utah Health Sciences Center, Salt Lake City, Utah; and, Division of Epidemiology (LMO), Department of Internal Medicine, University of Utah Health Sciences Center, Salt Lake City, Utah. M. S. Reynolds was supported by the Rural & Underserved Utah Training Experience (RUUTE) program at the University of Utah School of Medicine. This investigation was supported by a generous gift from the Mostaghel family. The survey construction and data analyses were supported in part by the University of Utah Population Health Research (PHR) Foundation, with funding in part from the National Center for Research Resources, the National Center for Advancing Translational Sciences, and National Institutes of Health, through Grant UL1TR002538 (formerly 5UL1TR001067-05, 8UL1TR000105, and UL1RR025764). Supported in part by an Unrestricted Grant from Research to Prevent Blindness, New York, NY, to the Department of Ophthalmology & Visual Sciences, University of Utah. This research was presented in part at the Annual Meeting of the North American Neuro-Ophthalmology Society, February 20-23, 2021. The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Web site (www. jneuro-ophthalmology.com). Address correspondence to Judith E. A. Warner, John A. Moran Eye Center, 65 Mario Capecchi Drive, Salt Lake City, UT 84132; E-mail: [e-mail]. 524 Disorder-2 (GAD-2), and Patient Health Questionnaire (depression) Scale-2]. Results: Seventy-seven respondents had an average age of 29 and were 57% male. 92% reported symptoms before age 18. The main site of pain or discomfort was the eyes, with onset resulting from viewing or thinking of sharp objects and edges. Symptoms lasted from seconds to hours and could be prolonged even after closing eyes or avoiding viewing the trigger. The composite and subscale scores on the NEI-VFQ25 were low, with a mean composite score of 78 and selected subscores of general health (61), general vision (73), ocular pain (68), driving (79), mental health (61), and role difficulties (72). Anxiety was reported in 58% of participants, and depression in 57%. Migraine or headache was reported in 46% of participants. Participants reported Alice in Wonderland syndrome, visual snow, obsessive– compulsive disorder, attention deficit hyperactivity disorder, stripe-induced visual discomfort, and synesthesia. Conclusion: From this survey, we have the beginnings of an understanding of the characteristics of SEES, as well as VRQOL impacts. These survey responses lead us to postulate that SEES may be a distinct visual phenomenon and to propose SEES criteria. Systematic studies of this condition’s clinical features and treatment responses will be additional steps toward improving patient care. Journal of Neuro-Ophthalmology 2022;42:524–529 doi: 10.1097/WNO.0000000000001650 © 2022 by North American Neuro-Ophthalmology Society S harp edge eye syndrome (SEES) is a condition in which the sufferer experiences ocular pain or discomfort when viewing or mentally imaging a sharp object or edge. Although we were unable to identify any published reports of this condition, an Internet search for “Sharp Edge Eye Syndrome” or “Visual Looming Syndrome” (used interchangeably) reveals several blog posts and Internet forums discussing the condition (1–4). Posts report that SEES may make it difficult to fall asleep, drive, or use a computer. Patients with SEES have often consulted health care professionals without satisfaction (1). Case Report MSR (primary author) is a healthy 28-year-old man who has experienced discomfort associated with viewing sharp objects for as long as he can remember. The earliest instance occurred Reynolds et al: J Neuro-Ophthalmol 2022; 42: 524-529 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution as a child, while watching the movie, Matilda. His eyes became extremely uncomfortable when Matilda was placed in a spiked Iron Maiden. He describes his pain as a “sinus ache” and deep “discomfort,” as if “the object is actually poking my eye, slowly.” There is both a physical component of the head or eye discomfort and a psychological discomfort. He feels the strong urge to look away, hold his hands to his eyes, and engage in mental distraction. The severity ranges from mild to moderate, with varying degree of severity. The severity has not changed over time, although symptoms once resolved for 3 months. Triggers include table corners, cutlery, writing implements, display hooks, branches, and spears (See Supplemental Digital Content, Figure S1, http://links.lww.com/WNO/ A613), among many others. Objects pointed toward his eyes centrally or peripherally induce symptoms, but thinking about these objects can also cause discomfort. He denies a fear or phobia of these objects. Once triggered, an episode may range from seconds to hours. Exacerbating factors include fatigue and stress. Alleviating factors include placing blunting objects over triggers, sleeping with a soft cloth or eye mask with moderate pressure, squinting, pressing his fingers firmly on his eyelids, and distracting thoughts. He denies any disability stemming from this condition. MSR’s younger brother suffers from similar symptoms. MSR denies a family history of eye disease except refractive error. His father has a history of migraines. MSR has attention deficit hyperactivity disorder (ADHD) but no other medical conditions. His ophthalmic examination is normal, including 20/15 uncorrected visual acuity in each eye, external, anterior segment, fundus examinations, and intraocular pressure. Noncontrasted MRI was normal. This study aims to explore the features of SEES, propose a definition of SEES, better understand its associated features and comorbidities, and begin to determine its effect on vision-related quality of life (VRQOL). METHODS Selection of Study Subjects A cross-sectional web-based survey was made available on social media pages dedicated to SEES. The survey was made available to persons 18 years or older who experience eye pain or discomfort when viewing or imagining sharp objects (2–4). The study protocol was approved by the University of Utah Institutional Review Board (UofU IRB). The study followed the tenets of the Declaration of Helsinki, and informed consent was obtained from participants before their completion of the study instruments. The study was available from July 9–21, 2020. whether viewing or thinking of sharp objects causes discomfort. Participants had to answer in the affirmative to one of these 2 questions to continue. Demographics collected included age, gender, race, and geographic location. The survey included 22 de novo questions developed by the research team. These were created from descriptions from online forums dedicated to SEES and from the first author’s experiences (1,2). Additional questions explored whether this condition may be related to other conditions such as Alice in Wonderland syndrome (5), visual snow syndrome (6), stripe-induced visual discomfort (7), aichmophobia (needle phobia) (8), or synesthesia (9). A focus group of 4 individuals with self-identified SEES helped refine the questions. These individuals were excluded from the study. The three-item ID-Migraine questionnaire (10) was included twice in the survey: First, the survey asked if SEES symptoms were accompanied by migraines or headaches. If a respondent self-reported that their symptoms were accompanied by migraine, or by headache scoring 2 or 3 on the IDMigraine questionnaire, we concluded their symptoms were likely accompanied by migraine and reported the sum of these individuals. Later in the survey, we asked if individuals experienced migraines independent of SEES symptoms. Likewise, we reported the number of subjects who experience migraine independent of symptoms as the sum of the individuals who self-reported migraine and the individuals who self-reported headache and scored 2 or 3 on the ID-Migraine questionnaire. We used the 25-item National Eye Institute Visual Function Questionnaire (11) (NEI-VFQ-25). Items that used the phrase “because of your eyesight” were reworded to “because of your eye discomfort with sharp objects.” This change was based on similar changes made by researchers adapting the NEI-VFQ-25 to blepharospasm (12). We used the General Anxiety Disorder-2 (13) (GAD-2) and the Personal Health Questionnaire Scale-2 (14) (PHQ-2). We categorized participants as having moderate-to-severe anxiety if they had a GAD-2 score of $3 or if they self-identified as having anxiety. We categorized participants as having moderate-to-severe depression if they had a PHQ-2 score of $3 or if they self-identified as having depression. Statistical Analyses Data were tabulated (Excel 2020 for Windows), and R version 3.6.2 was used for data analyses (15). Data are presented as a fraction of the respondents who chose to answer the question. RESULTS Survey Instrument The survey instrument may be viewed in Supplemental Digital Content (see Figure S2, http://links.lww.com/ WNO/A628). It begins with 2 screening questions, asking Reynolds et al: J Neuro-Ophthalmol 2022; 42: 524-529 Between July 9 and 21, 2020, 77 participants completed the survey. Although the consent form and survey invitation stated that the survey was for individuals 18 years of age or older, 4 participants were younger than 18. We included 525 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution these responses after further review by and approval from the UofU IRB. Although not every participant answered every question, every question in the survey was answered by at least 73 of the 77 respondents. Demographics The mean age was 29 years. Survey responses came from 17 different countries. Most participants identified as Caucasian. Results are summarized in Table 1. Site of Pain and Discomfort Eighty-four percent reported discomfort or pain in both eyes, 49% reported discomfort located in between the eyes, and 42% reported discomfort in the front of the head. Age of Onset Ninety-two percent reported symptom onset before the age of 18 years (mean 10 years). Triggering Objects Although discomfort is triggered by viewing sharp objects or edges, 78% of respondents indicated that visualizing a triggering object could induce pain or discomfort. 58% reported that viewing a triggering object on a screen could induce symptoms. Table 2 includes a list of triggering objects. When triggered, 82% of participants described the discomfort as feeling like a needle was being held up to their eyes and slowly moved forward, creating an urgent desire to look away. 57% indicated that it may feel like the object was physically poking their eyes. When a sharp object was pointed toward them, 78% reported needing to look TABLE 1. Demographics and disease characteristics of survey respondents Age, mean (years) Age at symptom onset, mean (years) Female:male:nonbinary within group Region of origin, n (%) Europe North America Central and South America Central Asia and Middle East Southeast Asia Oceania Africa Race*, n (%) American Indian/Alaska Native Asian Black/African American Hispanic/Latino Native Hawaiian or Pacific Islander Middle Eastern/North African Caucasian Prefer not to say 29 10 32:43:1 20 (26) 44 (58) 4 (5) 3 (4) 1 (1.3) 3 (4) 1 (1.3) 1 (1) 5 (7) 3 (4) 9 (12) 1 (1) 3 (3.9) 62 (82) 1 (1) Percentages are expressed as a fraction of the number of participants who responded to the question. *Participants had the option of selecting more than one race. 526 away and 79% reported that the discomfort might continue after closing their eyes. 38% of participants were unable to sleep without covering their eyes. Despite these symptoms, when directly asked, “Would you say you have an unreasonable or excessive fear of sharp objects?”, 72% of respondents indicated they did not. Accompanying Symptoms Seven participants indicated that they experienced migraine when triggered. An additional 23 participants indicated they experienced headaches when triggered. Five of these 23 participants scored $2 on the ID-Migraine Questionnaire. These 12 participants (16%) were categorized as having accompanying migraine (Table 3). Thirty-six percent of participants reported light sensitivity. Duration of Discomfort When asked how long the discomfort lasted, 34% indicated “seconds,” 53% “minutes,” and 12% “hours.” When asked the longest time of freedom from symptoms, 16% indicated “hours,” 40% “days,” 36% “months,” and 9% “years.” In the last year, 29% experienced worsening, 14% improvement, and 57% no change of symptoms. Exacerbating and Alleviating Factors Participants found their discomfort to be worse when tired (70%), when stressed (57%), when the object was viewed in bright light (35%), or when the object was shiny (21%). Participants reported that their pain could be improved if they looked away from the trigger (79%), put pressure on both eyes (69%), covered the trigger with something soft (53%), or thought of something else in an effort to ignore the sharp object (43%). They reported they could improve their pain by covering one eye (29%), being well-rested (26%), applying pressure to the temples (20%), wearing glasses or sunglasses (17%), or wearing a hat (5%). Some respondents reported TABLE 2. Objects that may trigger discomfort Furniture (table and nightstand) corners Pencils/pens Knives Needles Open cabinet door corners Forks Department store display hooks Scissors Straws Laptop corners Tree branches Umbrellas Windshield wipers Computer cursor Others 78 68 62 61 60 53 52 52 38 36 34 27 27 12 26 Common objects that may trigger symptoms reported as a percent of respondents. Reynolds et al: J Neuro-Ophthalmol 2022; 42: 524-529 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution half of the participants were aware of a family history of migraine. TABLE 3. Accompanying symptoms, comorbid conditions, and family history Accompanying symptoms, n(%) Migraine (ID-Migraine + self-reported) Headache Light sensitivity Co-morbid conditions, n(%) Anxiety (GAD-2 + self-reported) Depression (PHQ-2 + self-reported) Migraines (ID-Migraine + self-reported) Headaches Alice in Wonderland syndrome Visual snow Obsessive–compulsive disorder Attention deficit hyperactivity disorder Restless leg syndrome Stripe-induced visual discomfort Synesthesia Family history, n(%) Family history of migraines Family history of SEES 12 (16) 23 (30) 26 (36) 43 42 19 14 21 21 17 14 9 8 6 (58) (57) (26) (19) (28) (27) (23) (19) (12) (10) (8) 32 (42) 15 (20) Survey respondents were asked if their SEES symptoms were associated with migraines, headaches, and light sensitivity. Respondents were also asked if they had migraines or headaches independent of SEES symptoms. Respondents were asked about mental health conditions, other disturbances of visual perception, and a family history of migraine or SEES. Percentages are expressed as a fraction of the number of participants who responded to the question. GAD-2, General Anxiety Disorder-2; PHQ-2, Personal Health Questionnaire Scale-2; SEES, sharp edge eye syndrome. sleeping with an eye mask (12%) or with a soft cloth over the eyes (17%) to improve their discomfort. Comorbid Conditions Anxiety, depression, and other mental health conditions Table 3 shows a significant number of respondents reporting moderate-to-severe anxiety, moderate-to-severe depression, obsessive–compulsive disorder (OCD), and ADHD. Migraine Thirteen participants indicated that they experienced migraines independent of SEES symptoms. 14 participants indicated they experienced headaches independent of SEES, of whom 6 scored $2 on the ID-Migraine Questionnaire. These 19 (26%) participants were categorized as having migraine in Table 3. Other Disturbances of Visual Perception Participants reporting Alice in Wonderland syndrome, visual snow, stripe-induced discomfort, and synesthesia are documented in Table 3. Family History of Sharp Edge Eye Syndrome and Migraine Twenty percent of our respondents reported at least one biologically related family member with SEES, and almost Reynolds et al: J Neuro-Ophthalmol 2022; 42: 524-529 Seeking Medical Attention for Sharp Edge Eye Syndrome Twenty-six percent of participants reported that they had consulted a medical professional about their pain syndrome. Most expressed feeling frustrated by these medical encounters when no diagnosis or treatment was rendered. Many participants reported feeling that the medical consultant dismissed their symptoms. Others reported that they were referred to other specialists. Some people with SEES, having researched their condition on the internet, found many comments describing the futility of a medical consultation, and for this reason did not seek medical attention. Vision-Related Quality of Life Seventy-six of the 77 participants completed the NEI-VFQ25, and their responses are tabulated in Table 4. The mean composite score as well as the subscores for general health, general vision, ocular pain, driving, and mental health were all below 80. DISCUSSION Proposed Diagnostic Criteria Based on the syndrome characteristics that at least twothirds of the respondents reported, we propose the following diagnostic criteria for SEES: A. Ocular pain or discomfort when viewing sharp objects or edges. TABLE 4. Vision-related quality of life assessed in survey participants with SEES using the 25-Item National Eye Institute Visual Function Questionnaire (NEI-VFQ-25) Participants completing the NEI-VFQ-25, n NEI-VFQ-25 composite score, mean NEI-VFQ-25 subscale score, means General health General vision Ocular pain Near activities Distance activities Driving Color vision Peripheral vision Vision-specific Social functioning Mental health Role difficulties Dependency 76 78 61 73 68 82 88 79 97 85 90 61 72 89 SEES, sharp edge eye syndrome. 527 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution B. Presence of at least 2 of the following: 1. Thinking of sharp objects causes pain or discomfort. 2. Triggered pain or discomfort involves both eyes. 3. Viewing sharp objects causes a sensation as if a needle is pointed at the eye(s) and is moving toward them. 4. Discomfort continues even after closing one’s eyes. 5. Discomfort improves when looking away from the object. C. Triggers: 1. Sharp edges or corners. 2. Sharp or pointed objects. D. Exacerbating and alleviating factors: 1. Symptoms are worse when tired or stressed. 2. Symptoms are improved by placing pressure over the eyes. E. Age of onset before the age of 18 years. F. Symptoms are not better explained by another disease, disorder, or syndrome. Mental Health and Other Visual Perceptual Conditions A significant proportion of our respondents seem to have anxiety, depression, OCD, and/or ADHD. By comparison, the worldwide prevalence of anxiety is estimated to be 7.8% (16) and the prevalence of depression is estimated to be 3.4% (17). The prevalence of OCD and ADHD are estimated to be 2.1% (18) and 5.3% (19), respectively. Our respondents reported features of other syndromes affecting visual perception, suggesting that this group of individuals may be prone to perturbation of visual perception and that these disturbances of visual perception may share some common etiology. The role of psychoactive or other drugs was not considered for this study, given the community-reported young age of onset. ocular conditions (26), and the most common neurologic cause of eye pain in a tertiary neurology clinic was migraine. Although 16% of our participants had migraine in conjunction with SEES symptoms, and 26% had migraine separate from SEES events (with only 3 people experiencing both SEES and non-SEES–related migraine), migraine was by no means a universal symptom and, therefore, not the sole explanation for SEES symptoms. Inflammatory eye conditions and migraine both involve activation of the trigeminal pathway (27). SEES may be unique because the sensation of pain appears to originate in the visual cortex, where the triggering object is recognized. Previous research has demonstrated that photographs of the sun can cause pupillary constriction (28), suggesting that neural pathways beginning in the visual cortex may project to subcortical brain areas. While the phenomena of visually induced sensations such as stripe-induced discomfort and synesthesia have been investigated, this study is the first to report a condition in which a visual construct (present or mentally produced) may produce pain. MRI and EEG are important diagnostic tools, and multiple patients have anecdotally reported normal imaging following workup with their individual providers. MSR had a normal MRI. Because participants were not administered MRI or EEGs as a part of this study, we cannot rule out seizure disorders or structural disease at this time. Future imaging studies of patients with SEES may help us to better understand what neural pathways are activated by looking at or thinking about sharp objects. Limitations We used a convenience sample, and the survey was open for 12 days. For these reasons, we cannot generalize our results to all persons who may have SEES. Furthermore, the associations we documented only represent a single time point. Conclusions Vision-Related Quality of Life Our data suggest that persons who identify as having SEES experience a significant reduction in VRQOL. Reference data from a sample of the German general population demonstrated scores .90 (20), but the mean composite score for our respondents on the NEI-VFQ-25 was 78. This mean may be compared with those reported for migraine (85) (21), multiple sclerosis with a history of optic neuritis (85) (22), idiopathic intracranial hypertension (82.4) (23), neuro-ophthalmic disorders other than multiple sclerosis (81) (24), hemifacial spasm (80) (12), and dry eye (80) (25). This observation indicates that SEES is not just a disturbance of visual perception but a health condition that results in a significant reduction in VRQOL. Ocular Pain Induced by a Visual Image We have previously reported that the most common ocular causes of eye pain in a tertiary eye clinic are inflammatory 528 The unique symptoms reported by our respondents lead us to conclude that SEES represents a distinct visual phenomenon. Our survey indicates that a significant number of SEES patients also suffer from mental health conditions and that a significant proportion suffer from migraines, light sensitivity, and other disturbances of visual perception. Although the pathophysiology of SEES is unknown, 20% of respondents reported a family history, suggesting a possible genetic predisposition. Patients with SEES appear to have substantially reduced VRQOL. Although there are no known treatments for SEES, physicians caring for these patients could suggest using some of the alleviating aids used by the survey respondents. Reassuring the patient that there are others with these symptoms and that they do not appear to be associated with any serious ophthalmic or neurologic condition may help relieve distress associated with SEES. Recognizing the association between SEES and mental health conditions Reynolds et al: J Neuro-Ophthalmol 2022; 42: 524-529 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Original Contribution can lead to increased screening for mental health conditions, which may also help decrease distress associated with SEES. Further study, perhaps with functional MRI or connectome, may help us better understand the pathophysiology of SEES and develop effective treatments for patients with SEES. STATEMENT OF AUTHORSHIP Conception and design: M. S. Reynolds, B. J. Katz, K. B. Digre, J. E. A. Warner; Acquisition of data: M. S. Reynolds, B. J. Katz; K. B. Digre, J. E. A. Warner; Analysis and interpretation of data: M. S. Reynolds, B. J. Katz; K. B. Digre, B. J. Brintz, L. M. Olson, J. E. A. Warner; Drafting the manuscript: M. S. Reynolds, B. J. Katz, K. B. Digre, B. J. Brintz, L. M. Olson, J. E. A. Warner; Revising the manuscript for intellectual content: M. S. Reynolds, B. J. Katz, K. B. Digre, B. J. Brintz, L. M. Olson, J. E. A. Warner; Final approval of the completed manuscript: M. S. Reynolds, B. J. Katz, K. B. Digre, B. J. Brintz, L. M. Olson, J. E. A. Warner. 11. 12. 13. 14. 15. 16. 17. ACKNOWLEDGMENTS The authors thank all the participants who took part in the survey and who have volunteered to share their symptoms and experiences. This study would not have been possible without their participation and support. Authors also thank Martin Witjes, Tina Kuustie, Phillip T. Dowdle, and Christopher Badger for providing valuable input regarding survey construction and implications of the survey results and Susan Schulman for assistance with editing the manuscript. REFERENCES 1. Mark1001. Why Do My Eyes Feel Weird Looking at Certain Objects? [MedHelp Web Site]. 2008. Available at: https://www. medhelp.org/posts/Eye-Care/Why-do-my-eyes-feel-weird-lookingat-certain-objects/show/681368. Accessed March 18, 2021. 2. Ophthalmologist. Visual Looming Syndrome [Facebook Web Site]. Available at: https://www.facebook.com/Visual-LoomingSyndrome-111883913685286/?eid=ARBx7ebsGNtjpn7Of_uqgGLUCCx33NVvsIFVr4-9YM7VX_fwJWd_ RE6eXSOg5UHxloVy8nfwyI6fWrI. 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