Peribulbar Corticosteroids for Ocular Myasthenia Gravis.

BACKGROUND: Ocular myasthenia gravis is treated predominantly by oral medications, with the potential for systemic adverse effects. Successful treatment has been achieved using peribulbar dexamethasone. We assessed the effect of peribulbar dexamethasone or triamcinolone (40-mg Triesence), a longer-acting corticosteroid, targeting the peribulbar area as opposed to directly injecting the affected extraocular muscle. This more convenient and secure approach holds the potential for straightforward integration within clinical environments. METHODS: Retrospective chart review. RESULTS: Five patients were identified that were treated with peribulbar corticosteroids. In 4 of the 5 cases, ophthalmoparesis was unilateral. One case had isolated ptosis, and 4 had both ptosis and ophthalmoparesis. Three of these 4 cases reported complete resolution of symptoms within weeks of a single injection. Improvement lasted between 5 to 6 months, and all patients responded to repeated injections. CONCLUSIONS: Peribulbar corticosteroids can be effective in ocular myasthenia gravis. We suggest that longer-acting agents such as triamcinolone are preferable, to reduce injection frequency.

A Consensus Statement on the Terminology for Automated Visual Field Abnormalities.

BACKGROUND: A multitude of terms have been used to describe automated visual field abnormalities. To date, there is no universally accepted system of definitions or guidelines. Variability among clinicians creates the risk of miscommunication and the compromise of patient care. The purposes of this study were to 1) assess the degree of consistency among a group of neuro-ophthalmologists in the description of visual field abnormalities and 2) to create a consensus statement with standardized terminology and definitions. METHODS: In phase one of the study, all neuro-ophthalmologists in Israel were asked to complete a survey in which they described the abnormalities in 10 selected automated visual field tests. In phase 2 of the study, the authors created a national consensus statement on the terminology and definitions for visual field abnormalities using a modified Delphi method. In phase 3, the neuro-ophthalmologists were asked to repeat the initial survey of the 10 visual fields using the consensus statement to formulate their answers. RESULTS: Twenty-six neuro-ophthalmologists participated in the initial survey. On average, there were 7.5 unique descriptions for each of the visual fields (SD 3.17), a description of only the location in 24.6% (SD 0.19), and an undecided response in 6.15% (SD 4.13). Twenty-two neuro-ophthalmologists participated in the creation of a consensus statement which included 24 types of abnormalities with specific definitions. Twenty-three neuro-ophthalmologists repeated the survey using the consensus statement. On average, in the repeated survey, there were 5.9 unique descriptions for each of the visual fields (SD 1.79), a description of only the location in 0.004% (SD 0.01), and an undecided response in 3.07% (SD 2.11%). Relative to the first survey, there was a significant improvement in the use of specific and decisive terminology. CONCLUSIONS: The study confirmed a great degree of variability in the use of terminology to describe automated visual field abnormalities. The creation of a consensus statement was associated with improved use of specific terminology. Future efforts may be warranted to further standardize terminology and definitions.

Low-Density Lipoprotein Receptor-Related Protein 4-Positive Myasthenia Gravis in a Double-Seronegative, Electromyography-Negative Patient.

We describe a patient with ocular myasthenia gravis, where single-fiber electromyography and testing for acetylcholine receptor and muscle-specific kinase antibodies were negative. However, antibodies to low-density lipoprotein receptor-related protein 4 (LRP4) were positive, and this prompted appropriate management. We recommend that testing for LRP4 antibodies be considered when the clinical suspicion for myasthenia gravis is high despite negative conventional diagnostic tests.

Intermittent Horner syndrome in a pediatric patient.

Intermittent Horner syndrome is uncommon in both the adult and pediatric population. We describe a case of a pediatric patient with an intermittent Horner syndrome. Infrared photography and videography were used to help establish the diagnosis.

False Positive Hemianopia.

A 70-year-old woman was urgently referred for neuro-ophthalmic evaluation when a routine visual field test demonstrated a pattern of bilateral nasal hemianopia. Detailed inspection of the visual field study revealed the hemianopias to be artifactual for the following reasons: (1) it was performed with an excessive number of false positive responses; (2) the grey scale plot had white patches, consistent with abnormally high sensitivity; (3) the total deviation probability maps were normal, indicating that no tested points had poor scores. Confrontational visual field testing was normal in all zones for both eyes. Repeated visual field testing showed no evidence of a true hemianopia. Even automated visual fields with highly specific abnormalities can merely be testing artifact. Scrutinize all components of the report before determining the clinical implications.

Decompensated Superior Oblique Palsy Secondary to Bilateral Nutritional Optic Neuropathy Following Bariatric Surgery.

Optic neuropathy can occur secondary to nutritional deficiencies in patients who have undergone bariatric surgery. We present a unique case of a 39-year-old man, claiming to be generally healthy, who presented with intermittent vertical diplopia and bilateral decreased vision in each eye. Visual acuity was 6/18 in the right eye and 6/12 in the left eye. Ishihara testing was defective for both eyes. Automated visual fields showed a severe generalized reduction in sensitivity in both eyes. The patient had a left head tilt and a right intermittent hypertropia of 30 prism diopters in primary position. CT of the orbits revealed a right superior oblique of small caliber. On further questioning, the patient admitted to a history of bariatric surgery 7 years prior to presentation with failure to take any nutritional supplements. Blood work demonstrated deficiencies in folate, thiamine, and copper. Within 6 months of initiating nutritional supplements, the vision in each eye was markedly improved and the diplopia resolved. There was an associated normalization of thiamine and copper, but folate levels remained low. We believe that the nutritional deficiency caused a bilateral optic neuropathy and the resulting vision loss precipitated a manifestation of a congenital superior oblique palsy that had previously just been a phoria. The case emphasizes the importance of considering occult sensory etiologies of acquired strabismus.

Artifactual Visual Field Defects Identified on Technically "Reliable" Visual Field Studies in a Neuro-Ophthalmology Practice.

PURPOSE: To assess the reliability of automated visual field studies with neurological abnormalities and normal reliability indices that were inconsistent with the remainder of the neuro-ophthalmic assessment. METHODS: Retrospective observational study from the clinical practice of a neuro-ophthalmologist at a tertiary referral center. RESULTS: From 2230 patient charts, ten cases were identified that met the inclusion criteria. In eight of the cases repeat visual field testing had no reproducible abnormality. Four of these cases were concerning for a bitemporal or homonymous hemianopia. None of the patients, including the two cases with a reproducible defect, developed any convincing manifestations of an organic disease related to the visual field defect. CONCLUSION: Our findings suggest that even marked neurological abnormalities on reliable automated visual field tests can be false. When the remainder of the neuro-ophthalmic evaluation is inconsistent with the test result, we recommend that clinicians attempt to immediately repeat the visual field study.

A Novel Method of Video Recording Ophthalmic Surgical Procedures.

BACKGROUND: Modern surgical microscopes are equipped with video recording and broadcasting capability. We present a simple method for utilizing these systems even in circumstances where the surgeon is operating with surgical loupes. METHODS: A divergent lens is suspended immediately below the objective of the microscope, thereby increasing the microscope's working distance. The microscope can be suspended high above the patient, out of the surgeon's field of view, yet still provide excellent video recording of the surgical procedure. RESULTS: The technique has been used successfully in over 30 surgical cases. CONCLUSION: This method offers a simple solution for recording surgical procedures that do not use the operating microscope. The implications are relevant to surgeons who operate with surgical loupes.

The "Crab" Memory Tool for the Actions of the Extraocular Muscles.

INTRODUCTION: Understanding the primary, secondary and tertiary actions of the vertical recti and oblique muscles is important in the diagnosis of various types of strabismus (superior oblique palsy, A- and V-patterns). Unfortunately, learning these actions can be very challenging. We designed a visual memory tool, and assessed its usefulness for medical students. METHODS: Medical students undergoing their rotation in ophthalmology were taught the actions of the extraocular muscles either without the memory aid (control group) or with it (test group). The students were surveyed one week and one month later to determine their ability to recall the muscle actions and asked to subjectively rate the usefulness of the memory aid. RESULTS: Approximately 40% of the test group used the memory tool. Eighty-seven percent of the respondents recommended that the memory aid be taught in the future. Overall, there was no significant difference in the ability to recall the actions of the muscles between the control and test groups. However, those students who found the memory aid helpful had significantly better recall than those who did not. CONCLUSION: The memory aid may be useful to a significant proportion of students.

A Novel Method of Visual Field Assessment for Patients with Unilateral Severely Limited Central Vision: A Pilot Study.

PURPOSE: Reliable visual field testing requires the tested eye to be fixated on a central target. This poses a major obstacle for eyes with severe central vision loss. This pilot study assesses whether it may be feasible to examine such patients with a modified method. METHODS: A green filter was placed over the fellow eye. A FASTPAC algorithm was used with a red stimulus. The green filter prevented transmission of the red stimuli but allowed visualization of the yellow fixation light. Subjects were tested by both the conventional and the novel method, performed in a randomized order. We compared the reliability indices and also the precision of the two methods. RESULTS: We present results from six patients. The novel method was associated with an 85% reduction in fixation losses (P=0.028) and a 58% reduction in eye motion on gaze tracking (P=0.007). Further, specialized testing in one of the volunteers demonstrated that the novel technique could more precisely define a small zone of preserved peripheral vision (P=0.008). CONCLUSION: The results of this pilot study suggest that the novel method described may be a feasible strategy for visual field testing in patients with unilateral severe central vision loss.

Ophthalmology practice during the COVID-19 pandemic.

OBJECTIVE: To present an established practice protocol for safe and effective hospital-setting ophthalmic practice during the coronavirus disease 2019 (COVID-19) pandemic. METHODS AND ANALYSIS: Literature was reviewed to identify articles relevant to COVID-19 pandemic and ophthalmology. The following keywords were used: COVID-19, SARS-CoV-2 and telemedicine, combined with eye, ophthalmology, conjunctivitis and tears. Data were extracted from the identified manuscripts and discussed among subspecialists to obtain consensus evidence-based practice. RESULTS: A protocol for ophthalmic practice in the era of COVID-19 pandemic was established. The protocol covered patient screening, clinic flow, required personal protective equipment and modifications of ophthalmic equipment for improved safety. CONCLUSION: Important literature emerged with respect to the practice of ophthalmology in the era of COVID-19. An evidence-based ophthalmic practice protocol was established and should be modified in the future to accommodate new insights on the COVID-19 pandemic.

Systematic approaches for reviewing neuro-imaging scans in ophthalmology.

Neuroimaging is an important tool in ophthalmology, but many ophthalmologists are uncomfortable evaluating actual scans. Unfortunately, exclusive reliance on a radiologist's report can lead to diagnostic and management errors. We outline a methodology for equipping ophthalmologists with the skills necessary to read neuroimaging studies with respect to specific clinical questions.

Vestibular Schwannoma Presenting with Bilateral Papilledema Without Hydrocephalus: Case Study.

Bilateral papilledema secondary to obstructive or communicating hydrocephalus in patients with vestibular schwannomas is a known presentation; however, papilledema in the absence of hydrocephalus is rarely reported and its mechanism is poorly understood. We report a case of a 20-year-old woman presenting with visual deterioration and bilateral papilledema on fundoscopy. Magnetic resonance imaging (MRI) revealed a giant vestibular schwannoma with no sign of hydrocephalus. The only imaging evidence of increased pressure on preoperative imaging studies was seen on a T2-weighted MRI, where there was subtle dilatation of the arachnoid space of the optic sleeve. We presume that this patient developed papilledema by some mechanism not connected to hydrocephalus. In a young patient, papilledema may be a sign preceding hydrocephalus, or she may have had pseudotumor cerebri concomitant with her vestibular schwannoma. In either case, removal of the vestibular schwannoma solved the problem. She had complete visual recovery, irrespective of the mechanism.

Ocular flutter as the presenting sign of lung adenocarcinoma.

Ocular flutter is a rare ophthalmic finding that could represent paraneoplastic phenomena. In adults it is most commonly associated with small cell lung cancer (SCLC). Most patients also present with other neurological defects. We report the case of a 75-year-old woman who presented with isolated ocular flutter. The ensuing workup was significant for an early lung adenocarcinoma that would not have been biopsied otherwise due to its small size. To our knowledge, this is the first reported case of isolated ocular flutter as the presenting symptom of non-SCLC.

An update on the genetics of comitant strabismus.

Genetics play a significant role in the development of comitant strabismus and elucidating the relevant mechanisms that cause it may lead to the development of new therapeutic options. The genetics of strabismus are complex and involve the interactions of multiple genes. This article reviews the progress that has been made in the understanding of the genetic causes of comitant strabismus including linkage studies that have identified a variety of candidate sites, RNA and protein studies that have identified genes with altered regulation, and a study that establishes a role for genetic imprinting in comitant strabismus.

Neuro-imaging: a review for the general ophthalmologist.

The diagnosis of many neuro-ophthalmic conditions is facilitated with neuro-imaging. The two main modalities are Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). Clinicians who refer patients for either of these techniques must not only know which of them to choose, but also where the imaging should be performed (e.g. brain, orbit), whether or not contrast is indicated, and if angiography should be supplemented. These complexities often result in imaging studies that are either unneeded or unhelpful. The goal of this manuscript is to provide a practical set of guidelines for the general ophthalmologist of how to choose the correct parameters for neuro-imaging studies.