Homonymous hemianopia due to Erdheim-Chester disease.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis typically affecting multiple organ systems. We report 2 patients who presented with homonymous hemianopia and were ultimately diagnosed with biopsy-confirmed ECD. We review the spectrum of ECD and its treatment as well as histopathological and immunohistochemical differentiation from other histiocytic disorders.

Posterior Cortical Atrophy: Characteristics From a Clinical Data Registry.

Background: Posterior cortical atrophy (PCA) is a neurodegenerative syndrome that presents with higher-order visual dysfunction with relative sparing of memory and other cognitive domains, and it is most commonly associated with Alzheimer's disease pathology. There is a lack of data regarding the presentation of PCA to non-cognitive specialists. Therefore, we collected clinical data from neuro-ophthalmologists regarding the presentation of PCA to their practices and compared data to published cohorts and a published survey of cognitive specialists. Methods: Members of the North American Neuro-Ophthalmology Society Listserv (NANOSnet) were invited to complete an online, retrospective, chart-review data-entry survey regarding their patients with PCA, and REDCap was used for data collection. Results: Data for 38 patients were entered by 12 neuro-ophthalmologists. Patient mean age at presentation was 67.8 years, and 74% of patients were women. Difficulty reading was reported at presentation by 91% of patients, and poor performance on color vision, stereopsis, and visual field testing (performed reliably by 36/38 patients) were common findings. Most patients who were treated were treated with donepezil and/or memantine. Conclusions: Compared to published data from cognitive specialists, patients presenting to neuro-ophthalmology with PCA were more likely to be older and female and have a reading complaint. Reliable visual field testing was the norm with homonymous defects in the majority of patients. The neuro-ophthalmologist plays an important role in diagnosing PCA in older adults with unexplained visual signs and symptoms, and future studies of PCA should involve multiple specialists in order to advance our understanding of PCA and develop effective treatments.

Web-based gerontology courses: how do they measure up?

This study compared Web-based and lecture-based Gerontology and Psychology of Aging courses in terms of student performance, demographic and academic characteristics of students enrolled in the courses, and extent to which these characteristics differentially predicted outcomes of learning in the two course types. Participants for this study were 289 undergraduate students who completed a Psychology of Aging or Gerontology course, taught in either a Web-based or lecture-based format. Evaluation of student performance indicated percentage of points earned in the two course types did not differ significantly. Although students in Web-based courses tended to be older and were more likely to be upperclassmen than students in lecture-based courses, student profiles did not differ in terms of gender, race, grade point average, and Scholastic Assessment Tests (SAT) scores. Additionally, demographic and academic variables did not differentially predict course performance. The results of this study suggest a similar market in terms of demographic and academic characteristics of students for Web-based and lecture-based gerontology courses. Moreover, the findings suggest student learning outcomes in the two course formats are comparable.

Endogenous hypercortisolism inducing reversible ocular hypertension.

PURPOSE: To describe the clinical findings of two patients with reversible ocular hypertension secondary to endogenous hypercortisolism. DESIGN: Retrospective, observational case series. SUBJECTS: A 65-year-old man (patient 1) and a 21-year-old woman (patient 2) were both found to have Cushing's syndrome after presentation to our clinic with elevated intraocular pressures (IOP). METHODS: Clinical histories, ophthalmic examinations including IOP measurements, optical coherence tomography of the retinal nerve fiber layer, visual field testing, magnetic resonance imaging and computerized tomography of two patients were reviewed between 2007 and 2019. OBSERVATIONS: Patient 1 demonstrated elevated IOP (maximum 26 mmHg OD and 22 mmHg OS) and bilateral disc edema. Following diagnosis of Cushing's syndrome, the patient underwent two pituitary resections and bilateral adrenalectomy, with subsequent resolution of his hypercortisolism and ocular hypertension (OHT). Patient 2 presented with blurred vision and found to have OHT (maximum 32 mmHg OU). Following diagnosis of Cushing's disease and two resections of her adrenocorticotropic hormone (ACTH) producing pituitary adenoma, her IOPs normalized. Both patients maintained normal IOPs after resolution of their endogenous hypercortisolism and discontinuation of topical IOP-lowering medication. CONCLUSIONS AND IMPORTANCE: Ocular hypertension induced by endogenous hypercortisolism is, in some cases, fully reversible following normalization of cortisol levels. These findings suggest that the physiologic changes to the trabecular meshwork induced by endogenous hypercortisolism may be fully reversible.

Oral lipoic acid as a treatment for acute optic neuritis: a blinded, placebo controlled randomized trial.

BACKGROUND: Lipoic acid, an antioxidant, has beneficial effects in experimental acute optic neuritis and autoimmune encephalomyelitis. Optical coherence tomography can detect retinal nerve fiber layer thinning, representing axonal degeneration, approximately 3-6 months after acute optic neuritis. OBJECTIVE: To determine whether lipoic acid is neuroprotective in acute optic neuritis. METHODS: A single-center, double-blind, randomized, placebo controlled, 24-week trial. Intervention included 6 weeks of once daily lipoic acid (1200 mg) or placebo within 14 days of acute optic neuritis diagnosis. The primary outcome was the mean difference in affected eye retinal nerve fiber layer (RNFL) thickness from baseline to 24 weeks. RESULTS: We enrolled 31 subjects (placebo n=16; lipoic acid n=15; average age 38.6 years (standard deviation (SD) 10.3)). Affected eye mean global RNFL thickness (µm) in the lipoic acid group decreased from 108.47 (SD 26.11) at baseline to 79.31 (SD 19.26) at 24 weeks. The affected eye RNFL in the placebo group decreased from 103.67 (SD 18.04) at baseline to 84.43 (SD 20.94) at 24 weeks. Unaffected eye RNFL thickness did not significantly change in either group over 24 weeks. CONCLUSION: Six weeks of oral lipoic acid supplementation after acute optic neuritis is safe and well tolerated; however, because of insufficient recruitment, we could not conclude that lipoic acid treatment was neuroprotective in acute optic neuritis.

Vision in Alzheimer's disease: a focus on the anterior afferent pathway.

Visual dysfunction has long been recognized as a manifestation of Alzheimer's disease (AD), particularly in the form of visuospatial impairment during all stages of disease. However, investigations have revealed findings within the anterior (i.e., pregeniculate) afferent visual pathways that rely on retinal imaging and electrophysiologic methodologies for detection. Here we focus on the anterior afferent visual pathways in AD and the measures used for assessment, including optical coherence tomography, electrophysiology, color vision testing and threshold visual field perimetry. A brief summary of higher order visual dysfunction is also included to allow the reader to keep in context the broader findings of afferent visual dysfunction in AD.

Retinal Blood Flow Response to Hyperoxia Measured With En Face Doppler Optical Coherence Tomography.

PURPOSE: To use multiplane en face Doppler optical coherence tomography (OCT) to measure the change in total retinal blood flow (TRBF) in response to hyperoxia. METHODS: One eye of each healthy human participant (n = 8) was scanned with a commercial high-speed (70-kHz) spectral OCT system. Three repeated scans were captured at baseline and after 10 minutes of oxygen (hyperoxia) by open nasal mask. The procedure was performed twice on day 1 and once more on day 2. Blood flow of each vein was estimated using Doppler OCT at an optimized en face plane. The TRBF was summed from all veins at the optic disc. The TRBF hyperoxic response was calculated as the TRBF percent change from baseline. RESULTS: Participants experienced a 23.6% ± 10.7% (mean ± standard deviation [SD]) decrease (P < 0.001, paired t-test) in TRBF during hyperoxia. The within-day repeatability of baseline TRBF was 4.1% and the between-day reproducibility was 10.9% coefficient of variation (CV). Between-grader reproducibility was 3.9% CV. The repeatability and reproducibility (pooled SD) of hyperoxic response were 6.1% and 6.4%, respectively. CONCLUSIONS: The multiplane en face Doppler OCT algorithm was able to detect, in all participants, a decreased TRBF in response to hyperoxia. The response magnitude for each participant varied among repeated trials, and the averaging of multiple trials was helpful in establishing the individual response. This technique shows good potential for the clinical investigation of vascular autoregulation.

Autoantibodies and Sjogren's Syndrome in multiple sclerosis, a reappraisal.

BACKGROUND: Rheumatologic diseases may cause neurologic disorders that mimic multiple sclerosis (MS). A panel of serum autoantibodies is often obtained as part of the evaluation of patients suspected of having MS. OBJECTIVES: To determine, in light of recently revised diagnostic criteria for MS, neuromyelitis optica, and Sjogren's Syndrome, if testing for autoantibodies in patients with a confirmed diagnosis of MS would reveal a frequency or demonstrate a clinical utility divergent from previous reports or lead to identification of undiagnosed cases of Sjogren's Syndrome. METHODS: Convenience sample cross-sectional study of MS patients recruited from the OHSU Multiple Sclerosis Center. RESULTS: Autoantibodies were detected in 38% (35/91) of patients with MS and were not significantly associated with disease characteristics or severity. While four patients had SSA antibodies, none met diagnostic criteria for Sjogren's Syndrome. CONCLUSIONS: Rheumatologic autoantibodies are frequently found in MS patients and are not associated with disease severity or systemic rheumatologic disease. Our demonstration of the low specificity of these autoantibodies suggests that the diagnostic utility and cost-effectiveness of testing is not supported when there is strong clinical suspicion of MS and low clinical suspicion of rheumatologic disease.

Initiation of anti-TNF therapy and the risk of optic neuritis: from the safety assessment of biologic ThERapy (SABER) Study.

PURPOSE: To evaluate the incidence of optic neuritis (ON) in patients using anti-tumor necrosis factor (TNF) alpha therapy. DESIGN: Retrospective, population-based cohort study. METHODS: We identified new users of anti-TNF therapy (etanercept, infliximab, or adalimumab) or nonbiologic disease-modifying antirheumatic drugs (DMARDs) during 2000-2007 from the following data sources: Kaiser Permanente Northern California, Pharmaceutical Assistance Contract for the Elderly, Tennessee Medicaid, and National Medicaid/Medicare. Within this cohort, we used validated algorithms to identify ON cases occurring after onset of new drug exposure. We then calculated and compared ON incidence rates between exposure groups. RESULTS: We identified 61 227 eligible inflammatory disease patients with either new anti-TNF or new nonbiologic DMARD use. Among this cohort, we found 3 ON cases among anti-TNF new users, occurring a median of 123 days (range, 37-221 days) after anti-TNF start. The crude incidence rate of ON across all disease indications among anti-TNF new users was 10.4 (95% CI 3.3-32.2) cases per 100 000 person-years. In a sensitivity analysis considering current or past anti-TNF or DMARD use, we identified a total of 6 ON cases: 3 among anti-TNF users and 3 among DMARD users. Crude ON rates were similar among anti-TNF and DMARD groups: 4.5 (95% CI 1.4-13.8) and 5.4 (95% CI 1.7-16.6) per 100 000 person-years, respectively. CONCLUSION: Optic neuritis is rare among those who initiate anti-TNF therapy and occurs with similar frequency among those with nonbiologic DMARD exposure.

Clinical spectrum of tuberculous optic neuropathy.

PURPOSE: Tuberculous optic neuropathy may follow infection with Mycobacterium tuberculosis or administration of the bacille Calmette-Guerin. However, this condition is not well described in the ophthalmic literature. METHODS: Ophthalmologists, identified through professional electronic networks or previous publications, collected standardized clinical data relating to 62 eyes of 49 patients who they had managed with tuberculous optic neuropathy. RESULTS: Tuberculous optic neuropathy was most commonly manifested as papillitis (51.6 %), neuroretinitis (14.5 %), and optic nerve tubercle (11.3 %). Uveitis was an additional ocular morbidity in 88.7 % of eyes. In 36.7 % of patients, extraocular tuberculosis was present. The majority of patients (69.4 %) had resided in and/or traveled to an endemic area. Although initial visual acuity was 20/50 or worse in 62.9 % of 62 eyes, 76.7 % of 60 eyes followed for a median of 12 months achieved visual acuities of 20/40 or better. Visual field defects were reported for 46.8 % of eyes, but these defects recovered in 63.2 % of 19 eyes with follow-up. CONCLUSION: Visual recovery from tuberculous optic neuropathy is common, if the diagnosis is recognized and appropriate treatment is instituted. A tuberculous etiology should be considered when evaluating optic neuropathy in persons from endemic areas.

Gass plaques and fluorescein leakage in Susac Syndrome.

INTRODUCTION: Susac Syndrome (SS) consists of the triad of encephalopathy, branch retinal artery occlusion, and hearing loss. It is an autoimmune endotheliopathy that primarily affects young women. Two funduscopic findings, Gass plaques (GP) and arteriolar wall hyperfluorescence (AWH), have recently been described and are not only useful in making the SS diagnosis but also point to the endothelium as the site of autoimmune injury. In this report we wish to raise awareness of GP and AWH with this disorder. METHODS: Four selected SS cases are presented with fundus photographs revealing GP. Fluorescein angiographic photographs are shown describing AWH. RESULTS: GP are shown in several cases. These GP are unique in that they are yellow, sometimes refractile, and located distant from retinal arteriolar bifurcations unlike Hollenhorst plaques which are orange and located at retinal arteriolar bifurcations. Fluorescein angiography displays AWH of the retinal arterioles of patients with SS distant from affected vessels which has not been demonstrated in other retinal vasculitides. CONCLUSION: Small punctuate yellow GP are almost unique to this disorder and their characteristic location and color should assist in confirming the diagnosis. Fluorescein angiography should be performed in all patients with an unexplained encephalopathy to look for the characteristic AWH pattern that occurs in this illness.

Ischemic optic neuropathy following spine surgery in a 16-year-old patient and a ten-year-old patient.

Peri-operative ischemic optic neuropathy typically occurs in middle-aged or older patients. We report this condition in two patients aged 16 and 10 years. Only six other cases of peri-operative ischemic optic neuropathy have been reported in patients aged less than 30 years, all but one occurring after spinal surgery. Although the visual prognosis appears to be more favorable in younger patients, the pathogenesis of this rare complication of surgery is likely to be the same as that affecting older individuals.