Search

A 64-year-old man presented with painless sequential bilateral vision loss, consistent with optic neuropathy, over the span of months. The significant decline in his visual function was out of proportion to the appearance of the optic nerves (which were not pale) or changes in his retinal nerve fiber layer thickness on optical coherence tomography. Neuroimaging revealed only mild T2 signal abnormality and faint enhancement in the left optic nerve. Extensive workup for potential infectious, metabolic, inflammatory, and ischemic etiologies was unremarkable. Empiric treatment with intravenous steroids did not slow or ameliorate the vision loss. Ultimately, genetic analysis revealed a missense m.11778Ggt;A mutation in mitochondrial MT-ND4 gene, consistent with Leber hereditary optic neuropathy. Initiation of multivitamin supplements and idebenone unfortunately did not result in recovery of vision.

Incidence of cataract, diabetic retinopathy, macular degeneration, and glaucoma will significantly increase by 2050. Visual impairment can increase morbidity and mortality in nonocular disease. There are different patterns of vision loss in cataract, diabetic retinopathy, age-related macular degeneration, and glaucoma. Internists and medical subspecialists play an important role in prevention, detection, and early treatment of eye disease. Awareness of screening guidelines for eye disease as well as a basic ocular history and simple penlight examination can decrease incidence of vision loss and its impact. Visual impairment places a significant financial burden on society.

Background. Primary open-angle glaucoma (POAG) is associated with systemic microvascular dysfunction including hemorrhages and other abnormalities of the nailfold capillary bed. This study aimed to verify the specificity of nailfold capillary hemorrhages and other abnormalities as risk factors for POAG. Methods. Nailfold video capillaroscopy was performed using a JH-1004 capillaroscope on the fourth and fifth digits of the nondominant hand in control (n = 277), POAG (n = 206), OHT (n = 57), and SG (n = 29) subjects. The number of hemorrhages, dilated capillaries >50 µm, and avascular zones ≥200 µm were counted and adjusted to counts per 100 capillaries. Descriptive analyses as well as univariate- and multivariable-adjusted logistic regression were performed comparing all groups with controls and POAG with OHT and SG. Subanalyses were conducted in POAG patients examining the association between nailfold capillary outcomes and previous glaucoma surgery, successful IOP control, or disease severity. Results. All nailfold capillary outcomes were significantly increased in POAG, no outcomes were increased in SG, and only hemorrhages were mildly increased in OHT. Hemorrhages were significantly more frequent in POAG compared with both OHT (P<0.0001) and SG (P=0.001). There were significant trends between higher numbers of hemorrhages and POAG compared with controls, OHT, and SG, with odds ratios of 18.3 (8.5–39.4), 9.1 (1.9–13.4), and 11.8 (1.7–7.3), respectively, for the presence of two or more hemorrhages per 100 capillaries. Hemorrhages were not significantly associated with previous glaucoma surgery, successful postoperative IOP control, or disease severity in POAG. Conclusions. These findings suggest that systemic microvascular dysfunction is frequent in POAG and occurs early in the disease process. The high specificity of nailfold hemorrhages makes them viable clinical risk factors for POAG.

Background Despite automated pupillometry's (AP) improved detection of relative afferent pupillary defects (RAPDs) compared with the Swinging Flashlight Test (SFT), AP remains uncommon in clinical practice. This study examined barriers to routine use of AP in evaluation of acute vision loss. Methods (1) Ophthalmologists and optometrists' perceptions of AP were captured via electronic survey. (2) Ophthalmologists were presented with clinical vignettes to assess their use of AP in clinical decision-making. (3) Patients presenting with decreased vision to an ophthalmology urgent care clinic underwent manual SFT and AP screening to evaluate ophthalmologists' perceptions of the device. Results Surveys indicated that clinicians were "neutral" to "somewhat likely" to use AP. In clinical vignettes, more physicians proceeded with workup for optic nerve pathology when presented with an RAPD by AP than SFT (77% vs 26%, P = 0.003). When SFT and AP results were discordant, more physicians proceeded with workup for optic nerve disease when AP was positive and SFT was negative than vice versa (61% vs 18%, P = 0.008). In the clinical study of 21 patients, 50% of RAPDs detected by AP were not detected by SFT, although ophthalmologists rated AP's usefulness as only "neutral" to "somewhat useful." Conclusion Clinicians value pupillary examination and trust AP over SFT; however, widespread adoption and perceived value of AP may depend on its impact on clinical outcomes. Within a comprehensive diagnostic device, AP may be an important tool, but is not necessary to screen for optic nerve disease or evaluate acute vision loss.

Many ophthalmology residents struggle with the acquisition of surgical skills, but a large majority are successfully remediated before graduation using supportive measures and modern ophthalmic surgical training.

To determine if variables of the pupillary light response mature with age and sex in a healthy pediatric cohort and the utility of pupillometry in assessment among pediatric participants. After 1 min in a dark room to establish baseline, pupillometry was performed on 323 healthy, pediatric participants (646 eyes; 2–21 years; 175 females). Variables included initial pupil diameter, pupil diameter after light stimulus, percent pupillary constriction, latency to onset of constriction, average constriction velocity, maximum constriction velocity, average dilation velocity, and time from light stimulus to 75% of the initial pupil diameter. Data analyses employed ANOVAs and non-linear regressions. Analyses of age group differences revealed that participants 12–21 years old had a larger initial pupil diameter and pupil diameter after light stimulus, with males aged 12–18 years demonstrating a larger pupil diameter than all younger participants (ps

A 3-month-old girl with no past medical history presented with new-onset intermittent upbeat nystagmus. Episodes were triggered by movement into the supine position as well as by bright light flashes. They lasted a few seconds and were not associated with any distress. Neurological examination and work-up, including magnetic resonance imaging of the brain and abdominal ultrasonography, were normal. The eye movements completely resolved after 3 months.

Incidence of cataract, diabetic retinopathy, macular degeneration, and glaucoma will significantly increase by 2050. Visual impairment can increase morbidity and mortality in nonocular disease. There are different patterns of vision loss in cataract, diabetic retinopathy, age-related macular degeneration, and glaucoma. Internists and medical subspecialists play an important role in prevention, detection, and early treatment of eye disease. Awareness of screening guidelines for eye disease as well as a basic ocular history and simple penlight examination can decrease incidence of vision loss and its impact. Visual impairment places a significant financial burden on society.

Neuro-ophthalmic manifestations of cancer are vast and early recognition of a serious ocular condition due to either cancer or its therapy is important for both vision preservation as well as providing valuable treatment and prognostic information regarding the underlying malignancy. This review focuses on direct and indirect effects of cancer on the eye and its adnexa, hematologic malignancy, complications of traditional and novel oncologic therapies, and paraneoplastic syndromes as they relate to the eye as these disorders can lead to potentially devastating or irreversible vision loss.PubMed was searched primarily for the following topics: optic nerve infiltration, primary vitreoretinal lymphoma (PVRL), ocular paraneoplastic disorders, and ophthalmic complications of cancer therapeutics. Literature was selected based on historical significance and landmark studies (e.g., Cross et al. series of paraneoplastic optic neuritis patients; Chan's textbook on primary intraocular lymphoma) as well as publications published after 2000. References from select studies were additionally included. Given the sparsity of literature on many subjects, most publications were included during this time frame in our review.There are several ophthalmic entities that the oncologist should be aware of including leukemic optic nerve infiltration, PVRL, paraneoplastic syndromes as they related to the eye, and adverse effects of therapeutics. Unfortunately, given the rarity of some of these entities [e.g., paraneoplastic optic neuropathy (PON), cancer-associated retinopathy (CAR)], diagnosis can be difficult and treatment options are often limited.Oncologists can develop a set of basic ophthalmology examination skills that will help to triage and manage patient eye complaints. In certain instances, oncologists have the potential to avert devastating vision loss with early recognition of neuro-ophthalmic complications.

Purpose In this study, we set out to better understand the factors that influenced current ophthalmology residents' internship selection. We then tested the hypothesis that certain clinical or research experiences in medical school and internship may influence residents' confidence upon entering ophthalmology residency. Furthermore, we investigated whether completing internship at the same program as one's residency is correlated with confidence at the start of residency. Design Observational, cross-sectional, multicenter survey. Participants U.S. ophthalmology residents (Post Graduate Year 2/3) belonging to the class of 2018. Eighty surveys were submitted of which 63 were analyzed based on established inclusion criteria. Methods Residents responded to a 22-question online survey addressing how residents chose their internship, internship curriculum, exposure to ophthalmology in medical school and during internship, confidence level entering ophthalmology residency, confidence in managing various ocular pathologies, and factors that built confidence prior to ophthalmology residency. A Likert scale format was used for the majority of survey questions. Kruskal–Wallis testing and Fisher's exact testing were used to compare outcome variables among three groups defined by sense of confidence entering ophthalmology training. Main Outcome Measures Level of confidence at the start of ophthalmology residency. Results Quality of life and geographic location were found to be the most important factors in choosing internship programs, while obtaining ophthalmology skills was least. Although 32.3% of residents either agreed or strongly agreed that they felt confident at the start of ophthalmology residency, 42.9% disagreed or strongly disagreed. Residents who felt most confident for ophthalmology training spent more time on ophthalmology rotations in medical school (p = 0.05) or internship (p = 0.02) and worked up patients independently during their internship ophthalmology rotation (s) (0.01). Completing one's internship at the same institution as one's ophthalmology residency did not correlate with confidence entering residency. Conclusions Residents chose internships based on quality-of-life factors rather than enhancing ophthalmology training. Residents who felt confident at the start of ophthalmology residency had more hands on clinical ophthalmology experience than residents who did not feel confident. No statistically significant correlation was found between completion of internship at the same institution as one's ophthalmology residency and confidence entering residency.

PURPOSE: To determine the associative value of selected questions from the U. S. National Health Interview Survey (NHIS) for screening adults older than 18 years at risk of injurious falls. METHODS: Data from adults 18 years of age and older were extracted from the NHIS for 2011 relevant to an injurious fall within the preceding year. A multivariate logistic regression model was used to determine associations of self-reported injurious falls with key social-demographic, health, and physical function variables. Outcomes were reported as odds ratio (OR) with 95% confidence intervals (CIs). RESULTS: Self-reported injurious fall within the preceding year was associated with difficulty climbing 10 steps without special equipment (OR, 3.22; 95% CI, 2.32-4.46), loss of dependence for an activity of daily living (OR, 1.85; 95% CI, 1.17-2.91), pain in legs and below the knees (OR, 1.68; 95% CI, 1.23-2.30), and moderate visual impairment (OR, 1.59; 95% CI, 1.18-2.15). Women were at greater risk than men and those aged 75 years and older (OR, 1.46; 95% CI, 1.02-2.09). CONCLUSIONS: A subset of the NHIS questions are positively associated with injurious falls in the previous 12 months and may be of use in identifying adults at greater risk of future falls. The NHIS questions may serve to identify persons in need of targeted preventive services. Language: en

Functional (nonorganic) visual loss is frequently encountered in ophthalmic and neurologic practice, occurring in 0.5–5% of patients presenting with vision loss. Patients with functional visual loss may be misdiagnosed because of the paucity of objective findings on examination. Identifying such patients is extremely important to avoid unnecessary laboratory testing and to facilitate secondary gain. Likewise, it is also imperative to be able to identify those patients with organic vision loss with superimposed functional vision loss. Although the characterization of the psychological profile of patients with functional vision loss is helpful, for the ophthalmologist or neurologist, a familiarity with diagnostic strategies used to identify functional vision loss is more important. In this chapter, terminology is clarified, then patient types and their potential interactions with the examiner are described. The methods used to diagnose functional vision loss are reviewed in detail, followed by a brief description of other neuro-ophthalmologic manifestations of nonorganic disease.

As in any field of medicine, the neuro-ophthalmic history guides the physician's examination and differential diagnosis. From the beginning of the history taking, the physician should attempt to categorize the patient's problem. This chapter reviews the various elements of the neuro-ophthalmic history in the context of neuro-ophthalmic disorders.

Visual hallucinations and illusions comprise some of the most vivid and sometimes bizarre symptoms in neuro-ophthalmology. Hallucinations are defined as perceptions that occur in the absence of a corresponding external sensory stimulus. Visual hallucinations can be classified as unformed/simple (e.g., dots, flashes, zig-zags) or formed/complex (actual objects or people). In contrast, illusions are misinterpretations of a true sensory stimulus. Visual hallucinations and illusions are generally positive phenomena, in contrast to visual loss, which is a negative phenomenon. The causes of visual hallucinations and illusions can be grouped into several major categories: migraine, release phenomena (in the setting of impaired vision), entoptic (ocular) phenomena, alcohol and drug-related, seizures, neurogenerative disease, central nervous system lesions, psychiatric disease, and narcolepsy. This chapter details the various categories, but first the history, examination, and diagnostic and therapeutic considerations in patients with hallucinations and illusions are reviewed.

An acute neurologically isolated sixth nerve palsy in an adult over age 50 with vasculopathic risk factors is most commonly due to microvascular ischemia. Traditionally, clinical recommendation has been to defer immediate neuroimaging in favor of “watchful waiting” anticipating spontaneous recovery of the ocular motor palsy, as imaging is usually unrevealing. However, this approach may result in a missed or delayed diagnosis of an alternative, potentially (or perceived) life threatening or treatable cause of sixth nerve palsy, such as a space occupying mass or infarction. This chapter reviews the causes of isolated sixth nerve palsy, current guidelines for diagnostic evaluation, and the studies on the yield of neuroimaging for this condition.

Purpose To study the characteristics of Medicare beneficiaries hospitalized for ophthalmic conditions. Design Cross-sectional study. Methods The 2015 National Medicare 100% Inpatient Limited Dataset was analyzed to identify all patients with either an admitting or primary diagnosis for an ophthalmic condition using ICD-9-CM codes. All other hospitalized Medicare patients served for comparison. Comorbidities were calculated using the Elixhauser Comorbidity Index. Multivariable logistic regression was used to determine odds of primary ophthalmic hospitalization after controlling for patient characteristics and medical comorbidities. Results For 2015, there were a total of 13 152 Medicare patients with ocular hospitalizations compared to 6 621 005 patients with nonophthalmic events. Most ophthalmic patients were emergent admissions (73.19%) with routine discharges (75.50%) and low rates of inpatient mortality (0.62%). The top admitting diagnoses for nontraumatic and traumatic eye conditions were diplopia (11.69%) and closed fracture of the orbital floor (3.76%), respectively. Patients admitted for eye conditions were more likely to be younger, to be African American, and to have hypertension, valvular heart disease, diabetes, hypothyroidism, AIDS, lymphoma, solid tumor without metastasis, rheumatologic diseases, alcohol and drug abuse, psychoses, and depression compared to the general Medicare inpatient population. Conclusions Most inpatient admissions for US Medicare beneficiaries with primary ophthalmic diagnoses were for nontraumatic disorders of the eye and adnexa. Ophthalmic admissions were on average shorter in duration and had lower rates of inpatient mortality compared to nonophthalmic admissions. Patients admitted for eye conditions were more likely to have comorbidities such as hypertension, diabetes, and depression compared to the general Medicare inpatient population.

Introduction Retinal structural changes in subjects with mild cognitive impairment (MCI) remain a subject of controversy. Methods We investigated the correlation between optical coherence tomography (OCT) of the retinal sublayers, including the retinal nerve fiber layer (RNFL), and cognitive function in subjects with amnestic MCI and compared the OCT findings with matched controls. We also performed a meta‐analysis of the world literature using a random‐effects model. Results We found no statistically significant differences in OCT between amnestic MCI (aMCI) and controls. In aMCI subjects, we found an inverse relationship between RNFL thickness and two cognitive tests (delayed story recall and a word‐list learning test and the word‐list test). The meta‐analysis revealed a statistically significant decrease in RNFL thickness in MCI subjects. Discussion The inverse relationship between cognitive testing and RNFL thickness suggests that retinal involvement may include paradoxically increased thickness of the RNFL, which could suggest gliotic reactive changes.

A test of validation study was conducted to investigate the use of a novel computerized portable pupillometer to detect and quantify relative afferent pupillary defects (RAPDs).Binocular pupillary response curves were recorded in patients with RAPDs clinically graded by an examiner (n = 32) and in normal subjects (n = 31) with RAPDs simulated using quantifiable dimmed light intensities. In 14 normal subjects, testing was repeated within two months. Pupillary constriction amplitude (CA), velocity (CV) and onset latency (COL) were used to calculate RAPDs.RAPDs in normal subjects were 0.16 ± 0.12 log units (LU) (range = 0-0.38). In retested normals, inter-visit variability was 0.21 ± 0.12 LU (range = 0-0.42). Significant correlation was found between RAPD values and dimmed light intensity in normal subjects (Pearson's r = 0.87, p 0.0001) and between clinician and pupillometer grading of RAPDs in patients (r = 0.81, p 0.0001). Using the upper limit of the one-sided 95% confidence interval (CI) of the ratio of percentage change in CA (LU) as determined from normals, 21/23 (91%) patients with RAPDs ≥ 0.5 LU were distinguished from normals. The area under the receiver operating characteristic curve for distinguishing RAPDs ≥ 0.5 LU was 0.98 (95% CI = 0.95-1.00). RAPDs calculated using CA and CV correlated more strongly with the clinician's grading compared to COL (Steiger's test p 0.0001).This novel pupillometer correlated strongly with an expert examiner's clinical grading of RAPDs and detected clinically significant RAPDs with high sensitivity and specificity, suggesting it may have a prominent role as an objective clinical tool in the screening of patients with vision loss.

Neoplastic infiltration of the extraocular muscle (EOM) is a rare condition which can pose a diagnostic dilemma due to its rarity and overlapping ultrasonographic features with orbital myositis. The ultrasonographic features of neoplastic enlargement of EOM have not been systematically studied and previously have been described in only a few case reports. Orbital ultrasonography, in conjunction with the pattern of ocular misalignment, was assessed for its potential role in identifying patients with neoplastic EOM enlargement. Retrospective chart review of patients with neoplasm and myositis. The clinical features of 8 patients with neoplastic infiltration of the EOM were compared to 15 patients with myositis. In the neoplastic group the width of the EOM was (10.5 mm) almost twice the normal width of the muscle with myositis (p < 0.001). All the muscles in the neoplastic category were low to medium reflective. Paretic deviation was seen in 4/8(50%), purely restrictive in 2/8 (25%) and combined pattern in 2/8 (25%) were noted. In the myositis group the average EOM enlargement was 5.8 mm and all muscles showed low reflectivity. Although ultrasonographic features overlapped between the 2 groups paretic deviations were more common in the neoplastic group versus the myositis group (50% versus 7%). Neoplastic muscle enlargement tends to be larger with paretic deviations of ocular motility seen clinically. These findings in a patient with EOM enlargement should raise the suspicion of neoplasm as the etiology and further work up should be considered.

A 66-year-old man with chronic kidney disease presented with painless unilateral vision loss and bilateral optic disc swelling. Vision loss progressed in the affected eye and developed in the fellow eye. Evaluation for infectious, neoplastic, ischemic, and inflammatory disorders was unrevealing. High-dose corticosteroids appeared to stabilize his vision temporarily. Eventually he was diagnosed with papilledema in the setting of transverse venous sinus stenosis and dural arteriovenous fistula. His papilledema resolved, and his vision improved following stenting of the venous sinus stenosis and embolization of the fistula.

Our objective was to present clinicopathologic evidence of anterior visual pathway involvement in patients with amyotrophic lateral sclerosis (ALS) secondary to a C9orf72 mutation. Two related patients from an extended pedigree with ALS and GGGGCC hexanucleotide repeat expansion in the C9orf72 gene (C9-ALS) underwent neuro-ophthalmologic examination. Following death and tissue donation of the younger ALS patient, histopathologic examination of the retina, optic nerve and central nervous system (CNS) was performed. Ophthalmologic examination revealed contrast sensitivity impairment in the younger C9-ALS patient. Immunohistochemistry performed on this patient’s donor tissue demonstrated p62-positive, pTDP43-negative perinuclear inclusions in the inner nuclear layer of the retina and CNS. Further colocalization with GLT-1 and recoverin suggested that the majority of retinal p62-positive inclusions are found within cone bipolar cells as well as some amacrine and horizontal cells. In conclusion, this is the first report that identifies disease-specific pathologic inclusions in the anterior visual pathway of a patient with a C9orf72 mutation. Cone bipolar cell involvement within the inner nuclear layer of the retina may explain the observed subtle visual function deficiencies in this patient. Further clinical and histopathologic studies are needed to fully characterize a larger population of C9-ALS patients and explore these findings in other forms of ALS.

PURPOSE: To evaluate the effectiveness of a new binocular infrared computerized pupillometer in the quantitative measurement of the relative afferent pupillary response in patients with glaucoma by assessing the correlation of the intereye difference in visual function as measured by standard automated perimetry (SAP) with the intereye difference in the afferent pupillary response. METHODS: Twenty-three patients with glaucoma underwent examination with a prototype, automated, binocular pupillometer. Correlation between the intereye difference in the afferent pupillary response and the intereye difference in mean deviation (MD) was explored. RESULTS: Within 7 months of pupillography, all patients underwent SAP using the Humphrey Field Analyzer IIi, 24-2, Swedish Interactive Threshold Algorithm. The intereye differential pupillary response was 0.69±0.59 (log units, mean±SD). The intereye difference in MD was 5.67±5.29 dB (mean±SD). There was a strong correlation between the intereye difference in the afferent pupillary response and the intereye difference in MD (Spearman correlation coefficient, r = -0.77; P

Purpose To estimate the proportion of patients presenting with isolated third, fourth, or sixth cranial nerve palsy of presumed microvascular origin versus other causes. Design Prospective, multicenter, observational case series. Participants A total of 109 patients aged 50 years or older with acute isolated ocular motor nerve palsy. Testing Magnetic resonance imaging (MRI) of the brain. Main Outcome Measures Causes of acute isolated ocular motor nerve palsy (presumed microvascular or other) as determined with early MRI and clinical assessment. Results Among 109 patients enrolled in the study, 22 had cranial nerve III palsy, 25 had cranial nerve IV palsy, and 62 had cranial nerve VI palsy. A cause other than presumed microvascular ischemia was identified in 18 patients (16.5%; 95% confidence interval, 10.7–24.6). The presence of 1 or more vasculopathic risk factors (diabetes, hypertension, hypercholesterolemia, coronary artery disease, myocardial infarction, stroke, and smoking) was significantly associated with a presumed microvascular cause ( P = 0.003, Fisher exact test). Vasculopathic risk factors were also present in 61% of patients (11/18) with other causes. In the group of patients who had vasculopathic risk factors only, with no other significant medical condition, 10% of patients (8/80) were found to have other causes, including midbrain infarction, neoplasms, inflammation, pituitary apoplexy, and giant cell arteritis (GCA). By excluding patients with third cranial nerve palsies and those with GCA, the incidence of other causes for isolated fourth and sixth cranial nerve palsies was 4.7% (3/64). Conclusions In our series of patients with acute isolated ocular motor nerve palsies, a substantial proportion of patients had other causes, including neoplasm, GCA, and brain stem infarction. Brain MRI and laboratory workup have a role in the initial evaluation of older patients with isolated acute ocular motor nerve palsies regardless of whether vascular risk factors are present. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

The role of optic nerve sheath fenestration (ONSF) in the management of idiopathic intracranial hypertension remains controversial, with indications, risks, and benefits compared to cerebro-spinal fluid diversion procedures not fully elucidated. We report a retrospective record review of 37 patients (50 eyes) which had undergone ONSF by a single surgeon. Visual acuity (VA) improved in 22% of operated eyes and 17% of fellow eyes; stabilized in 54% of operated and 74% of fellow eyes; and deteriorated in 24% of operated and 9% of fellow eyes. Better pre-operative VA (p = 0.01), colour vision (p = 0.002), and earlier intervention (p = 0.04) were associated with stabilization. We conclude that ONSF often stabilizes vision and visual fields. Our results were best in patients with better pre-operative vision and in those with earlier intervention.

Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disorder that may have anterior visual pathway involvement. In this study, we compare the macular structure of patients with ALS to healthy controls and examine correlations between macular sub-layer thickness measurements and pulmonary function tests and disease duration. ALS patients underwent optical coherence tomography (OCT) imaging to obtain macular cube scans of the right eye. Macular cube OCT data from age-matched healthy subjects were provided by the OCT reading center. Semi-automated retinal segmentation software was used to quantify macular sub-layers. Pulmonary function tests and time since symptom onset were collected retrospectively from the electronic medical records of ALS patients. Macular retinal nerve fiber layer was significantly thinner in ALS patients compared to healthy controls (P P

To determine if clinical and histopathologic findings were present in the eyes of patients with amyotrophic lateral sclerosis (ALS) and explore correlations to an animal model of ALS.Two patients with ALS were studied histopathologically as well as the retinas of ALS/dementia transgenic mice with dysfunctional ubiquilin2, UBQLN2(P497H). Clinical study 1, an observational, cross-sectional study, was performed using optical coherence tomography (OCT) to obtain and compare mean total macular thickness and average and quadrant specific peripapillary retinal nerve fiber layer (pRNFL) scans from 16 patients with ALS to controls. Correlation analysis was performed to evaluate the association with disease duration. Clinical study 2 consisted of measuring visual acuity, color vision, contrast sensitivity, and quality of life in 12 patients.Histopathologic studies demonstrated intraretinal inclusions in one patient and loss of ganglion cell axons in another. Mouse eyes had intraretinal inclusions in the inner plexiform layers. Total macular volume was thinner in patients compared to controls (P.05), and 37.5% of patients with ALS had an average pRNFL below the 1st percentile. Total macular and pRNFL thickness correlated inversely with disease duration.Histopathologic analysis of ALS eyes and mice with the UBQLN2(P497H) mutation, as well as OCT measurements, supports involvement of the anterior visual pathway. We identified pathologies, including intraretinal deposits and axonal loss. pRNFL and total macular thinning found on OCT correlated with disease duration. A pattern of vision loss specific for ALS was not identified. This study confirms ocular involvement in patients and transgenic animals with ALS/dementia.

Purpose To report a surgical approach to managing hypotropia associated with thyroid eye disease (TED) that uses adjustable sutures. Methods The medical records of consecutive patients with TED and hypotropia who underwent adjustable suture inferior rectus muscle recession with or without superior rectus muscle recession of the hypertropic eye were reviewed retrospectively. The initial goal was undercorrection in primary gaze and no hyperdeviation in downgaze. Outcomes were rated according to the presence of diplopia in primary and reading positions as excellent (none), good (no diplopia with Δ correction), and poor (diplopia). Results Fifty-four patients were included. The initial median primary position deviation was 16 Δ . Of the 54 patients, 39 (72%) underwent unilateral inferior rectus muscle recession; 15 (28%), bilateral; and 24 (44%), superior rectus muscle recession. On mean follow-up of 38 weeks, 35 patients (65%) had excellent results (30 aligned in primary and reading position, 5 of whom could fuse small deviations [average, 3 Δ ]); 17 (31%), good results (13 Δ ); and 2 (3.7%), poor results, but aligned after reoperation (6 Δ overcorrection, 16 Δ undercorrection). Of the 24 misaligned patients (ie, those with small deviations, good results, and poor results), 13 were undercorrected an average of 4.9 Δ and 11 (20%) were overcorrected an average of 6.2 Δ . Adjustment timing, number of muscles recessed, preoperative deviation, and previous orbital decompression did not affect outcomes. Conclusions In this series, patients with hypotropia and TED were effectively managed by the use of adjustable sutures and by surgeons operating on multiple muscles, anticipating drift toward overcorrection of the recessed inferior rectus muscle, and using prism for residual deviation.

Background In cases of progressive optic neuropathy, diagnostic uncertainty often persists despite extensive work-up. Optic nerve biopsy (ONB) can be considered, especially when visual decline of the affected or fellow eye ensues despite empiric therapy. We aimed to evaluate both diagnostic and therapeutic utilities of ONB based on the long-term experience at a tertiary care institution. Methods This was a retrospective chart review of biopsies over 20 years at a single institution involving intrinsic or adherent optic nerve masses. Main outcome measures included the impact of tissue sampling on reaching a diagnosis and on guiding treatment. Secondary measures included vision in the eye of the ONB and the fellow eye. Results Fifteen patients with a mean age of 51.7 ± 17.4 years underwent biopsies. At the time of biopsy, visual acuity was no light perception in 8 (53%) eyes, light perception to counting fingers in 5 (33%), and 20/400 or better in 2 (13%). The fellow eye of 7 patients (47%) experienced some degree of sequential vision loss before biopsy. Seven specimens included en bloc biopsy of the nerve, 7 contained the dural sheath (usually with a portion of the optic nerve), and 1 only of the compressive mass. Six patients (40%) had tumors. Six of 8 inflammatory lesions biopsied required further clinical data to arrive at specific diagnoses. In one case, a clinical diagnosis could not be made. No patients experienced further vision loss in the fellow eye at last follow-up (median, 8 months). Conclusions In diverse circumstances of progressive optic neuropathy, ONB can be beneficial in establishing the diagnosis. ONB can help direct specific local or systemic treatment, particularly when infectious or inflammatory etiologies are identified. ONB, if considered early in the disease course, can potentially halt or prevent vision loss when the fellow eye is threatened.

Objective: To determine whether weight gain is associated with recurrence in idiopathic intracranial hypertension (IIH). Methods: Medical records of adult patients with IIH seen between 1993 and 2009 at 2 university hospitals were reviewed to identify those with and without recurrence. Patients with documented height and weight at presentation and at subsequent visits were studied. The Wilcoxon rank sum test was used to compare mean body mass index (BMI) and percent weight change between the groups of patients with recurrence and without recurrence. The signed-rank test was used for comparing BMI within groups at the various time points. Results: Fifty women with IIH were included in the analyses: 26 had IIH recurrence and 24 did not. Patients with recurrence had greater BMI at the time of recurrence compared to BMI at diagnosis ( p = 0.02, signed-rank test). They also demonstrated a greater degree of weight gain between initial resolution and recurrence (BMI change +2.0 kg/m 2 [−1.5 to 10.8]) compared to patients without recurrence (−0.75 kg/m 2 [−35 to 3.6], p = 0.0009, Wilcoxon rank sum test). Patients without recurrence demonstrated stable weights (0%[95% CI −9.6 to 10.1%]), while patients with recurrence demonstrated a 6% weight gain ([−3.5 to 40.2%], p = 0.005), with an average rate of BMI gain of 1.3 kg/m 2 /year vs −0.96 kg/m 2 /year in those without recurrence. Conclusion: Patients with IIH recurrence had significant increases in BMI compared to patients without recurrence in this cohort. Patients with resolved IIH should be advised that weight gain may be a risk factor for IIH recurrence.

Two patients with carotid cavernous aneurysms presented with uncommon patterns of third nerve involvement, one with an isolated elevation deficit without ptosis and the other with a depression deficit with possible pupillary involvement. These patterns are best explained by topographic organization of third nerve fibers within the cavernous sinus (prior to its anatomic bifurcation) that subserve individual extraocular muscles.

Objective Evaluate the effectiveness of an interactive cognitive computer simulation for teaching the hydrodissection portion of cataract surgery compared with standard teaching and to assess the attitudes of residents about the teaching tools and their perceived confidence in the knowledge gained after using the tools. Design Case-control study. Participants and Controls Residents at academic institutions. Methods Prospective, multicenter, single-masked, controlled trial was performed in 7 academic departments of ophthalmology (Harvard Medical School/Massachusetts Eye and Ear Infirmary, University of Iowa, Emory University, University of Cincinnati, University of Pennsylvania/Scheie Eye Institute, Jefferson Medical College of Thomas Jefferson University/Wills Eye Institute, and the Aravind Eye Institute). All residents from these centers were asked to participate and were randomized into 2 groups. Group A (n = 30) served as the control and received traditional teaching materials; group B (n = 38) received a digital video disc of the Virtual Mentor program. This program is an interactive cognitive simulation, specifically designed to separate cognitive aspects (such as decision making and error recognition) from the motor aspects. Both groups took online anonymous pretests (n = 68) and posttests (n = 58), and answered satisfaction questionnaires (n = 53). Wilcoxon tests were completed to compare pretest and posttest scores between groups. Analysis of variance was performed to assess differences in mean scores between groups. Main Outcome Measures Scores on pretests, posttests, and satisfaction questionnaires. Results There was no difference in the pretest scores between the 2 groups ( P = 0.62). However, group B (Virtual Mentor [VM]) scored significantly higher on the posttest ( P = 0.01). Mean difference between pretest and posttest scores were significantly better in the VM group than in the traditional learning group ( P = 0.04). Questionnaire revealed that the VM program was "more fun" to use (24.1% vs 4.2%) and residents were more likely to use this type of program again compared with the likelihood of using the traditional tools (58.6% vs 4.2%). Conclusions The VM, a cognitive computer simulation, augmented teaching of the hydrodissection step of phacoemulsification surgery compared with traditional teaching alone. The program was more enjoyable and more likely to be used repetitively by ophthalmology residents. Financial Disclosure(s) Proprietary or commercial disclosures may be found after the references.

To determine if there is any association in ophthalmology between the gender of the chairperson and residency program director and the gender of faculty and residents.An anonymous electronic survey was sent to 121 ophthalmology residency program directors. Demographic information pertaining to size, location, academic or community affiliation, and gender distribution of the faculty, residents, residency program directors and chairs was obtained.The response rate was 45.45% with 55 residency program directors responding to the survey. Academic programs comprised 53 (96%) of the programs studied. Male department chairs led all 53 (96%) programs. In terms of the residency program director gender, 37 (67%) were male, whereas 18 (34%) were female. Female faculty and residents comprised 313 (28%) and 270 (45%), respectively. Compared to departments with male chairs, departments with a female chair had a higher crude proportion of female faculty (35% vs. 28%; P = 0.300) and female residents (50% vs. 45%; P = 0.660), although there was no statistical difference. Departments with either a male or female residency program director had similar number of female faculty (28% vs. 28%; P = 0.991) and residents (44% vs. 46%; P = 0.689).We found no significant association between the gender of the residency program director and chairperson with the proportion of female faculty and residents. Given a higher ratio of female residents relative to female faculty, it is probable that graduating female residents are choosing not to pursue academic medicine, shrinking the potential pool of female candidates for positions of departmental leadership.

Purpose To compare the results of advanced visual field defects (VFD) measured with the conventional reference perimeter Octopus 101 (O-101) and the new portable Tübingen Mobile Campimeter (TMC). Methods Thirty-seven subjects (18 to 75 years), 13 with advanced arcuate scotomas, 12 with VFD respecting vertical meridians, 6 with concentric constriction and 6 healthy controls were included. First examination was with O-101: grid 30°-NO, 192 stimuli, 10 cd/m2 background luminance, stimulus size: Goldmann III (26’); second examination was with TMC: 84 stimuli (subset of grid 30°-NO), stimulus size 34’, stimulus luminance 320–370 cd/m2, background luminance 8–20 cd/m2. Point-wise accuracy (proportion of concordant locations), sensitivity, and specificity were estimated into 95% confidence intervals (CI) by averaging individual logits. Examination durations were compared. Results TMC results are highly concordant with O-101 results for all defect classes. For the entire sample, the percentage of discordant points (perceived with TMC but not with O-101) among all discordant points was 35% (CI: 30% to 40%). Analyzed by VFD pattern, accuracy was highest in healthy controls scotomas (97.9%; CI: 97% to 98.5%) and lowest in arcuate scotomas (80.6 %; CI: 77.3% to 83.5%). Sensitivity was highest in concentric constriction (94.5%; CI: 82.9% to 98.4%) and lowest in healthy controls (59.1%; CI: 26.3% to 85.3%). Specificity was highest in healthy controls (98.1%; CI: 96.6% to 98.9%) and lowest in concentric constriction (77.4%; CI: 62.1% to 87.7%). Mean examination time was 4.6 minutes (TMC) and 9.8 minutes (O-101). Conclusions The results indicate that the TMC is a feasible device for detection of VFD.