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BACKGROUND: “Spaceflight associated neuro-ocular syndrome” (SANS) represents a challenging health condition in modern space medicine. Forty-eight percent of astronauts are diagnosed with SANS after long-term space missions. The pathophysiological mechanism seems to be multifactorial, and yet remains unknown. In this proof-of-concept study we plan to investigate retinal microcirculatory changes in weightlessness and aim to identify their role in the development of SANS. METHODS AND DESIGN: Healthy individuals will take part in a parabolic flight campaign, which recreates fractioned total weightlessness periods. The airplane is specifically equipped, and designed for the execution of parabolic flight maneuvers and scientific research in microgravity. Retinal microcirculation will be assessed with a modified fundus camera, which allows dynamic vessel analysis. We will additionally measure intra-ocular pressure and hemodynamic changes during each phase of the flight. Blood samples will be analyzed at baseline, one hour and 24 hours after exposure to weightlessness. CONCLUSIONS: This pilot study aims to investigate the feasibility of retinal microcirculation assessment during varying gravity. Results of this study may generate insights whether venous stasis in the eye, surrogated by the dilatation of retinal vessels and increase in intraocular pressure as signs of venous insufficiency, may potentially contribute to the development of SANS.

Zusammenfassung Hintergrund Im Verlauf der letzten Jahre werden zunehmend mehr OP-Mikroskope mit OCT-Modul (intraoperatives OCT, iOCT) verfügbar, die in Echtzeit hochauflösende Aufnahmen des OP-Situs liefern. Nachdem eine Erhebung 2018 an unserer Klinik nur in 2,4% aller Operationen einen intraoperativen Zusatznutzen der iOCT ergab, der Hersteller aber die Hardware und Software überarbeitet hat, führten wir erneut eine Nutzenanalyse der Technologie durch. Material und Methoden Prospektive monozentrische Analyse zu Einsatz und Anwenderfreundlichkeit eines EnFocus Ultra-Deep OCT (Leica Microsystems) über einen Zeitraum von 25 (2018) bzw. 20 Werktagen (2021). Anhand eines standardisierten Fragebogens wurde die Verwendung der iOCT auf den OP-Verlauf durch die Operateure bewertet. Ergebnisse 2018 wurden über einen Zeitraum von 25 Tagen 118 Operationen und 2021 über 20 Tage 92 Operationen durchgeführt. 2018 wurde die iOCT-Funktion in 24,6% und 2021 in 48,9% aller Eingriffe eingesetzt, wobei sich in beiden Jahren die iOCT nach Einschätzung durch die Operateure in 2,4% bzw. 3,3% als „entscheidend“ für den OP-Verlauf erwies. Hierbei handelte es sich um Operationen, bei denen der intraokulare Einblick limitiert war, wie z. B. bei dekompensierter Hornhaut, Glaskörperblutung oder nach Voroperationen wie z. B. nach perforierender Keratoplastik. Schlussfolgerung Durch die Weiterentwicklung der Nutzeroberfläche wurde eine Verbesserung der Bedienbarkeit erzielt und das iOCT deutlich häufiger verwendet. Die iOCT erwies sich in vergleichbar wenigen Operationen, insbesondere bei komplexen Ausgangslagen, als verlaufsentscheidend.

Zusammenfassung Hintergrund Eine regelmäßige ophthalmologische Untersuchung ist für immer mehr Menschen mit Diabetes gefordert, sei es zur Früherkennung und Kontrolle einer diabetischen Retinopathie. In manchen Ländern kommen bereits automatische Verfahren der Bildauswertung zur Detektion einer behandlungsbedürftigen diabetischen Retinopathie zur Anwendung. Ziel dieser Studie ist es, die Früherkennung diabetischer Augenerkrankungen ohne direkten Arzt-Patienten-Kontakt in Deutschland zu evaluieren und mögliche Barrieren für Patienten zu erkennen. Material und Methoden In dieser prospektiven monozentrischen Studie wurden bisher 93 Patienten eingeschlossen, die sich für ein Screening auf eine diabetische Retinopathie vorstellten. Die Studienteilnehmer nahmen ein Angebot zu einer Untersuchung (Visuserhebung, non-mydriatische kamerabasierte Fundusuntersuchung) ohne Arzt-Patienten-Kontakt wahr. Mittels Fragebogen wurde die Zufriedenheit der Patienten erhoben. Ergebnisse Das durchschnittliche Alter der eingeschlossenen Patienten (n = 93; 49,5 % weiblich) betrug 53,5 Jahre (29 bis 76 Jahre, SD: 13,6 Jahre). Bei 17 Augen (18,3 %) wurde mittels kamerabasierter Fundusuntersuchung eine diabetische Retinopathie festgestellt. In der Stichprobe musste sich kein Patient aufgrund schlechter Bildqualität erneut vorstellen. Insgesamt berichteten die Patienten eine hohe bis sehr hohe Zufriedenheit. Die Akzeptanz des Untersuchungsangebots war hoch. Diskussion In dieser Studie konnte mit einer Screeninguntersuchung ohne direkten Arztkontakt eine hohe Akzeptanz erreicht werden. Qualifiziertes Fachpersonal ermöglichte eine hohe Bildqualität der erhobenen Fundusbilder, sodass der Einsatz von automatischen Algorithmen (künstliche Intelligenz) das Potenzial hat, die bisherigen Angebote zu ergänzen.

Faricimab is a bispecific antibody that acts through dual inhibition of both angiopoietin-2 and vascular endothelial growth factor A. We report primary results of two phase 3 trials evaluating intravitreal faricimab with extension up to every 16 weeks for neovascular age-related macular degeneration (nAMD).TENAYA and LUCERNE were randomised, double-masked, non-inferiority trials across 271 sites worldwide. Treatment-naive patients with nAMD aged 50 years or older were randomly assigned (1:1) to intravitreal faricimab 6·0 mg up to every 16 weeks, based on protocol-defined disease activity assessments at weeks 20 and 24, or aflibercept 2·0 mg every 8 weeks. Randomisation was performed through an interactive voice or web-based response system using a stratified permuted block randomisation method. Patients, investigators, those assessing outcomes, and the funder were masked to group assignments. The primary endpoint was mean change in best-corrected visual acuity (BCVA) from baseline averaged over weeks 40, 44, and 48 (prespecified non-inferiority margin of four letters), in the intention-to-treat population. Safety analyses included patients who received at least one dose of study treatment. These trials are registered with ClinicalTrials.gov (TENAYA NCT03823287 and LUCERNE NCT03823300).Across the two trials, 1329 patients were randomly assigned between Feb 19 and Nov 19, 2019 (TENAYA n=334 faricimab and n=337 aflibercept), and between March 11 and Nov 1, 2019 (LUCERNE n=331 faricimab and n=327 aflibercept). BCVA change from baseline with faricimab was non-inferior to aflibercept in both TENAYA (adjusted mean change 5·8 letters [95% CI 4·6 to 7·1] and 5·1 letters [3·9 to 6·4]; treatment difference 0·7 letters [-1·1 to 2·5]) and LUCERNE (6·6 letters [5·3 to 7·8] and 6·6 letters [5·3 to 7·8]; treatment difference 0·0 letters [-1·7 to 1·8]). Rates of ocular adverse events were comparable between faricimab and aflibercept (TENAYA n=121 [36·3%] vs n=128 [38·1%], and LUCERNE n=133 [40·2%] vs n=118 [36·2%]).Visual benefits with faricimab given at up to 16-week intervals demonstrates its potential to meaningfully extend the time between treatments with sustained efficacy, thereby reducing treatment burden in patients with nAMD.F Hoffmann-La Roche.

Altern ist ein multidimensionaler Prozess, der zu korperlichen, psychologischen und sozialen Veranderungen fuhrt. Einschrankungen der peripheren Sensorik und ihrer zentralen Verarbeitung haben hieran einen erheblichen Anteil. Selektive Literaturrecherche. Neben spezifischen Erkrankungen der Sinnesorgane sind im fortgeschrittenen Lebensalter zusatzlich unspezifische altersbedingte degenerative Prozesse fur die hohe Pravalenz von Sinneseinschrankungen in dieser Altersgruppe verantwortlich; diese konnen zu einer erheblichen Reduktion der Lebensqualitat bei den Betroffenen fuhren. Einschrankungen der Gleichgewichtsfunktion, des Horvermogens, Sehens, Riechens und der Somatosensorik sind mit einer Erhohung der Sturzhaufigkeit und einer erhohten Mortalitat alterer Menschen assoziiert. Daruber hinaus gibt es Hinweise fur einen Zusammenhang zwischen dem Vorliegen einer Horstorung und der Wahrscheinlichkeit der Entwicklung einer Demenz. Neben der Funktionsfahigkeit jedes einzelnen Sinnesorgans fur sich allein spielt die Fahigkeit der Integration teilweise widerspruchlicher Sinneswahrnehmungen eine zunehmende Rolle unter dem Einfluss altersbedingter Sinneseinschrankungen. Im Umgang mit alteren und alten Menschen ist gezielt auf die Entwicklung von Einschrankungen sensorischer Funktionen zu achten, um durch eine moglichst fruhzeitige und ggf. interdisziplinare Therapie die daraus resultierenden Folgeerkrankungen zu minimieren. Daneben konnten multimodale Therapieansatze dazu beitragen, die Plastizitat des Gehirns im Alter anzuregen.

An increasing number of patients suffering from diabetes require regular ophthalmological check-ups to diagnose and/or treat potential diabetic retinal disease. Some countries have already implemented systematic fundus assessments including artificial intelligence-based programs in order to detect sight-threatening retinopathy. The aim of this study was to improve the detection of diabetic fundus changes in Germany without examination by a doctor and to create an easy access to ophthalmological examinations.In this prospective monocentric study 93 patients in need for a routine check-up for diabetic retinopathy were included. The study participants took up an offer of an examination (visual examination, non-mydriatic camera-based fundus examination) without doctor-patient contact. Patient satisfaction with the organization and examinations was assessed using a questionnaire.The mean age was 53.5 years (SD 13.6 years, 49.5% female) and 17 eyes (18.3%) showed a diabetic retinopathy which was detected using a camera-based examination. Within the small sample, no patient had to repeat the examination due to poor image quality. All categories of the questionnaire showed a good to very good satisfaction, indicating a high acceptance of the other examination form that took place at the ophthalmologist's premises.In our study in an ophthalmological practice a high level of acceptance among the patients interested in the screening for diabetic retinopathy without any direct patient-doctor contact was achieved. Our study shows a very good acceptance and feasibility. Future use of artificial intelligence in clinical practice may help to be able to screen many more patients as in this study imaging quality was very good.HINTERGRUND: Eine regelmäßige ophthalmologische Untersuchung ist für immer mehr Menschen mit Diabetes gefordert, sei es zur Früherkennung und Kontrolle einer diabetischen Retinopathie. In manchen Ländern kommen bereits automatische Verfahren der Bildauswertung zur Detektion einer behandlungsbedürftigen diabetischen Retinopathie zur Anwendung. Ziel dieser Studie ist es, die Früherkennung diabetischer Augenerkrankungen ohne direkten Arzt-Patienten-Kontakt in Deutschland zu evaluieren und mögliche Barrieren für Patienten zu erkennen.In dieser prospektiven monozentrischen Studie wurden bisher 93 Patienten eingeschlossen, die sich für ein Screening auf eine diabetische Retinopathie vorstellten. Die Studienteilnehmer nahmen ein Angebot zu einer Untersuchung (Visuserhebung, non-mydriatische kamerabasierte Fundusuntersuchung) ohne Arzt-Patienten-Kontakt wahr. Mittels Fragebogen wurde die Zufriedenheit der Patienten erhoben.Das durchschnittliche Alter der eingeschlossenen Patienten (n = 93; 49,5 % weiblich) betrug 53,5 Jahre (29 bis 76 Jahre, SD: 13,6 Jahre). Bei 17 Augen (18,3 %) wurde mittels kamerabasierter Fundusuntersuchung eine diabetische Retinopathie festgestellt. In der Stichprobe musste sich kein Patient aufgrund schlechter Bildqualität erneut vorstellen. Insgesamt berichteten die Patienten eine hohe bis sehr hohe Zufriedenheit. Die Akzeptanz des Untersuchungsangebots war hoch.In dieser Studie konnte mit einer Screeninguntersuchung ohne direkten Arztkontakt eine hohe Akzeptanz erreicht werden. Qualifiziertes Fachpersonal ermöglichte eine hohe Bildqualität der erhobenen Fundusbilder, sodass der Einsatz von automatischen Algorithmen (künstliche Intelligenz) das Potenzial hat, die bisherigen Angebote zu ergänzen.

Stummer et al. established fluorescence-guided surgery (FGS) for glioblastoma (GBM) using 5-aminolevulinic acid (5-ALA). Its metabolite, protoporphyrin IX (PPIX), is also a photosensitizer and can be used for photodynamic therapy (PDT) using a laser beam of 635 nm. The porphyrin derivate verteporfin (VP) was discovered to have properties to penetrate the brain, pharmacologically target glioma cells, and is approved for PDT of choroidal neovascularization in wet age-related macular degeneration at 689 nm.To elucidate whether GBM cell lines are susceptible to PDT with second-generation photosensitizer VP.Human glioma cell lines LN229, HSR-GBM1, and a low-passage patient-derived GBM cell line P1 were treated with variable concentrations of VP for 24 h, followed by PDT at 689 nm using a diode laser light. Cell viability was measured using the MTT assay and VP uptake was measured using a desktop cytometer.Significantly higher cell death following PDT with VP compared to VP treatment alone or no treatment was detected in all cell models (LN229, HSR-GBM1, P1). Flowcytometric measurements revealed a concentration-dependent cellular uptake of VP after 24 h incubation up to 99% at 10 µM (HSR-GBM1).This study demonstrates that PDT with VP causes cell death in GBM cells at marginal concentrations. Additionally, red spectrum fluorescence was detected at therapeutic concentrations in all cell lines, validating the cellular uptake of VP in GBM cells. VP, therefore, is not only a potential drug for targeting GBM pharmacologically but can be used as an optical imaging dye in surgery and photosensitizer to make GBM susceptible to PDT.

The aim of this study was to evaluate results after Descemet membrane endothelial keratoplasty (DMEK) and DMEK with cataract surgery (triple DMEK) in eyes with endothelial dysfunction and concomitant macular pathology.A monocentric, prospective clinical observational study of patients who underwent DMEK or triple DMEK surgery at the Department of Ophthalmology, University Hospital Düsseldorf, Germany, from June 2013 to February 2016 was conducted. Sex, age, best-corrected visual acuity (BCVA), central corneal thickness, endothelial cell density, and central retinal thickness in the 1 millimeter zone were analyzed.A total of 209 eyes were included in the study. Forty-two eyes (20.1%) had vision-limiting concomitant maculopathies. These were age-related macular degeneration (n = 17, 8.1%), epiretinal gliosis (n = 13, 6.2%), chronic macular edema (n = 7, 3.3%), macular holes (n = 3, 1.4%), and macular scarring (n = 2, 1.0%). BCVA significantly increased in patients without maculopathy from 0.6 ± 0.33 logMAR to 0.1 ± 0.15 logMAR (P0.001) and also in patients with maculopathy from 0.9 ± 0.38 logMAR to 0.27 ± 0.23 logMAR 12 months postoperatively (P0.001). There was a significant central retinal thickness increase of 34.1 μm 6 weeks after triple DMEK in the central 1-mm zone (P = 0.011). This increase was insignificant after DMEK. Postoperative macular edema occurred in 5.9% of cases after DMEK and 8.1% after triple DMEK, which was not significantly different.DMEK and triple DMEK significantly increase BCVA in eyes with concomitant maculopathy. Postoperative macular edema is a common disorder after lamellar keratoplasty; therefore, prophylactic treatment with nonsteroidal antiinflammatory drugs should be considered. Maculopathies did not predispose the development of postoperative macular edema.

Aims/hypothesis Hyperbaric oxygen (HBO) therapy may improve hyperglycaemia in humans with type 2 diabetes, but underlying mechanisms are unclear. Our objective was to examine the glucometabolic effects of HBO on whole-body glucose disposal in humans with type 2 diabetes. Methods In a randomised placebo-controlled crossover trial located at the German Diabetes Center, 12 male individuals with type 2 diabetes (age 18–75 years, BMI 2, HbA1c 42–75 mmol/mol [6–9%]), randomly allocated by one person, underwent 2-h HBO, once with 100% (240 kPa; HBO) and once with 21% oxygen (240 kPa; control, CON). Insulin sensitivity was assessed by hyperinsulinaemic–euglycaemic clamps with d-[6,6-2H2]glucose, hepatic and skeletal muscle energy metabolism were assessed by 1H/31P-magnetic resonance spectroscopy, while high-resolution respirometry measured skeletal muscle and white adipose tissue (WAT) mitochondrial capacity. All participants and people assessing the outcomes were blinded. Results HBO decreased fasting blood glucose by 19% and increased whole-body, hepatic and WAT insulin sensitivity about one-third (ppp Conclusions/interpretation HBO-mediated improvement of insulin sensitivity likely results from decreased endoplasmic reticulum stress and increased mitochondrial capacity, possibly leading to low-dose reactive oxygen species-mediated mitohormesis in humans with type 2 diabetes. Trial registration ClinicalTrials.gov NCT04219215 Funding German Federal Ministry of Health, German Federal Ministry of Education and Research, North-Rhine Westfalia Ministry of Culture and Science, European-Regional-Development-Fund, German-Research-Foundation (DFG), Schmutzler Stiftung Graphical abstract

A 65-year-old woman was referred for sudden bilateral loss of vision. She was vaccinated a few days earlier. The ophthalmological examination showed a massive optic disc swelling on both eyes. Magnetic resonance imaging (MRI) revealed suspected bilateral optic neuritis without cerebral involvement or transverse myelitis. After serological detection of anti-MOG (myelin oligodendrocyte glycoprotein) antibodies, the patient was treated with high-dose corticosteroid pulse therapy until vision recovered. Discussion: an atypical optic neuritis may indicate a neuromyelitis optica spectrum disorder (NMOSD), which should be further characterized by determination of Aquaporin 4(AQP4)-IgG and MOG-IgG.Eine 65-jährige Frau wurde wegen plötzlicher beidseitiger Sehminderung überwiesen, nachdem sie kurz zuvor geimpft wurde. Augenärztlich zeigte sich beidseits eine ausgeprägte Papillenschwellung. In der Magnetresonanztomografie (MRT) fand sich keine zerebrale Beteiligung oder transverse Myelitis. Serologisch konnten wir Myelin-Oligodendrozyten-Glykoprotein(MOG)-IgG nachweisen, sodass wir mit Hochdosiskortikosteroidpulstherapie behandelten. Diskussion: Bei atypischer Optikusneuritis muss an eine Neuromyelitis-optica-Spektrum-Erkrankung (NMOSD) gedacht werden, die durch die Bestimmung von Aquaporin 4(AQP4)- und MOG-IgG weiter charakterisiert werden sollte.

The frequency of diabetic retinopathy increases and is a leading cause of blindness in the working population. Recommended exams are testing of the visual acuity, ophthalmoscopy with dilated pupils, fundus fluorescein angiography and optical coherence tomography (OCT). By OCT the diagnosis and monitoring of diabetic macular edema has vastly improved. By intravitreal medications such as intravitreal anti-VEGF or intravitreal steroids macular edema can be treated successfully. Panretinal laser photocoagulation is the gold-standard in the therapy of proliferative diabetic retinopathy. Patients benefit from good co-working of general practitioner and ophthalmologist concerning screening and treatment.

Background The senses serve as the crucial interface between the individual and the environment. They are subject to aging and disease processes. Methods This review is based on pertinent publications retrieved by a selective search in the Medline and Cochrane Library databases. Results Approximately 40% of persons aged 70 to 79 manifest dysfunction in at least one, and more than 25% in multiple senses. Sensory changes are accompanied by diverse comorbidities which depend on the particular sense(s) affected. The presence of sensory deficits is associated with an increased risk of developing dementia (OR: 1.49 [95% confidence interval: 1.12; 1.98] for dysfunction in a single sensory modality, 2.85 [1.88; 4.30] for dysfunction in three or more sensory modalities). The risk of developing depressive symptoms is elevated as well (OR 3.36 [2.28; 4.96]). The individual's ability to cope with the demands of everyday life is largely determined by the ability to carry out multisensory integration, in which the perceptions of the different senses are bound together. This function itself is subject to age-related changes that can be either adaptive or maladaptive; it can, therefore, serve as an indicator for pathological aging processes. Conclusion Sensory dysfunction in old age should be detected as early as possible. This implies the need for close collaboration of all of the involved disciplines. It would be desirable to develop sensory screening tests as well as a procedure for testing multisensory integration in routine clinical practice.

Journal of Clinical Medicine (17), 5101 (2022). doi:10.3390/jcm11175101, Published by MDPI, Basel

Summary Background The human cytokine erythropoietin conveys neuroprotection in animal models but has shown ambiguous results in phase 2 clinical trials in patients with optic neuritis. We assessed the safety and efficacy of erythropoietin in patients with optic neuritis as a clinically isolated syndrome in a multicentre, prospective, randomised clinical trial. Methods This randomised, placebo-controlled, double-blind phase 3 trial, conducted at 12 tertiary referral centres in Germany, included participants aged 18–50 years, within 10 days of onset of unilateral optic neuritis, with visual acuity of 0·5 or less, and without a previous diagnosis of multiple sclerosis. Participants were randomly assigned (1:1) to receive either 33 000 IU erythropoietin or placebo intravenously for 3 days as an adjunct to high-dose intravenous methylprednisolone (1000 mg per day). Block randomisation was performed by the trial statistician using an SAS code that generated randomly varying block sizes, stratified by study site and distributed using sealed envelopes. All trial participants and all study staff were masked to treatment assignment, except the trial pharmacist. The first primary outcome was atrophy of the peripapillary retinal nerve fibre layer (pRNFL), measured by optic coherence tomography (OCT) as the difference in pRNFL thickness between the affected eye at week 26 and the unaffected eye at baseline. The second primary outcome was low contrast letter acuity at week 26, measured as the 2·5% Sloan chart score of the affected eye. Analysis was performed in the full analysis set of all randomised participants for whom treatment was started and at least one follow-up OCT measurement was available. Safety was analysed in all patients who received at least one dose of the trial medication. This trial is registered at ClinicalTrials.gov, NCT01962571. Findings 108 participants were enrolled between Nov 25, 2014, and Oct 9, 2017, of whom 55 were assigned to erythropoietin and 53 to placebo. Five patients were excluded from the primary analysis due to not receiving the allocated medication, withdrawn consent, revised diagnosis, or loss to follow-up, yielding a full analysis set of 52 patients in the erythropoietin group and 51 in the placebo group. Mean pRNFL atrophy was 15·93 μm (SD 14·91) in the erythropoietin group and 14·65 μm (15·60) in the placebo group (adjusted mean treatment difference 1·02 μm; 95% CI −5·51 to 7·55; p=0·76). Mean low contrast letter acuity scores were 49·60 (21·31) in the erythropoietin group and 49·06 (21·93) in the placebo group (adjusted mean treatment difference −4·03; −13·06 to 5·01). Adverse events occurred in 43 (81%) participants in the erythropoietin group and in 42 (81%) in the placebo group. The most common adverse event was headache, occuring in 15 (28%) patients in the erythropoietin group and 13 (25%) patients in the placebo group. Serious adverse events occurred in eight (15%) participants in the erythropoietin and in four (8%) in the placebo group. One patient (2%) in the erythropoietin group developed a venous sinus thrombosis, which was treated with anticoagulants and resolved without sequelae. Interpretation Erythropoietin as an adjunct to corticosteroids conveyed neither functional nor structural neuroprotection in the visual pathways after optic neuritis. Future research could focus on modified erythropoietin administration, assess its efficacy independent of corticosteroids, and investigate whether it affects the conversion of optic neuritis to multiple sclerosis. Funding German Federal Ministry of Education and Research (BMBF).

Purpose The primary endpoint results from the comparing alternative ranibizumab dosages for safety and efficacy in retinopathy of prematurity (CARE-ROP) core study identified ranibizumab as an effective treatment to control acute retinopathy of prematurity (ROP). This study reports the 1- and 2-year follow-up data focusing on long-term functional outcomes and safety. Methods The CARE-ROP trial compared 0.12 mg versus 0.20 mg ranibizumab in 20 infants with ROP in a multicentric, prospective, randomized, double-blind, controlled study design. Sixteen patients entered the follow-up period. An ophthalmologic assessment at one year postbaseline was acquired from all 16 patients and a neurodevelopmental assessment at two years postbaseline was acquired from 15 patients. Results Fifteen of 16 infants were able to fixate and follow moving objects at one year postbaseline treatment. One child progressed to stage 5 ROP bilaterally between the end of the core study and the 1-year follow-up (first seen at PMA 75 weeks). Mean spherical equivalents were -1.9 diopters (D) and -0.75 D in the 0.12 mg and the 0.20 mg treatment arms. Strabismus was present in seven and nystagmus in five out of 16 infants. Mental development scores were within normal limits in six out of ten patients with available data. No statistically significant difference was observed between the two treatment arms. Conclusion Neurodevelopmental and functional ocular outcomes 1 and 2 years after treatment with ranibizumab are reassuring regarding long-term safety. Late reactivation of ROP, however, represents a challenge during the follow-up phase and it is of utmost importance that regular follow-ups are maintained.

Aging is a multidimensional process that may lead to physical, psychological and social changes. This is predominantly due to a decline of sensory functions and their central processing.Selective literature search in Medline and the Cochrane Library.In addition to specific disorders of the sensory organs, unspecific age-related degenerative processes are responsible for the high prevalence of sensory limitations in older people. This can lead to a significant reduction in the quality of life. Balance impairment, decreasing function of hearing, vision, smell and the somatosensory system are associated with an increased risk of falling and an increased mortality in older people. Furthermore, there is evidence for a link between hearing loss and cognitive decline. In addition to the functional ability of every sense on its own, the integration of multiple sensory perceptions plays an increasing role in age-related sensory limitations.Sensory impairments have to be considered when working with older people. Early detection and an interdisciplinary therapeutic approach can reduce the negative consequences. Multimodal stimulation appears to stimulate brain plasticity which helps to compensate age-related changes.HINTERGRUND: Altern ist ein multidimensionaler Prozess, der zu körperlichen, psychologischen und sozialen Veränderungen führt. Einschränkungen der peripheren Sensorik und ihrer zentralen Verarbeitung haben hieran einen erheblichen Anteil.Selektive Literaturrecherche.Neben spezifischen Erkrankungen der Sinnesorgane sind im fortgeschrittenen Lebensalter zusätzlich unspezifische altersbedingte degenerative Prozesse für die hohe Prävalenz von Sinneseinschränkungen in dieser Altersgruppe verantwortlich; diese können zu einer erheblichen Reduktion der Lebensqualität bei den Betroffenen führen. Einschränkungen der Gleichgewichtsfunktion, des Hörvermögens, Sehens, Riechens und der Somatosensorik sind mit einer Erhöhung der Sturzhäufigkeit und einer erhöhten Mortalität älterer Menschen assoziiert. Darüber hinaus gibt es Hinweise für einen Zusammenhang zwischen dem Vorliegen einer Hörstörung und der Wahrscheinlichkeit der Entwicklung einer Demenz. Neben der Funktionsfähigkeit jedes einzelnen Sinnesorgans für sich allein spielt die Fähigkeit der Integration teilweise widersprüchlicher Sinneswahrnehmungen eine zunehmende Rolle unter dem Einfluss altersbedingter Sinneseinschränkungen.Im Umgang mit älteren und alten Menschen ist gezielt auf die Entwicklung von Einschränkungen sensorischer Funktionen zu achten, um durch eine möglichst frühzeitige und ggf. interdisziplinäre Therapie die daraus resultierenden Folgeerkrankungen zu minimieren. Daneben könnten multimodale Therapieansätze dazu beitragen, die Plastizität des Gehirns im Alter anzuregen.

Objective Studies using conventional full‐field visual evoked potentials (ffVEP) have reported subtle abnormalities in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We hypothesize that these abnormalities can be detected in the majority of CIDP patients using enhanced methods. Methods We performed a cross‐sectional noninterventional study comparing 18 CIDP patients and 18 matched healthy controls using multifocal VEP (mfVEP) as a technique with enhanced sensitivity to detect conduction abnormalities across the spectrum of optic nerve fibers. Patients with confounding diseases (ophthalmologic, diabetes mellitus) were excluded. Results The mean amplitude and latency, as well as the low‐contrast visual acuity, did not differ between CIDP patients and controls. Subanalyses revealed latency differences concerning the superior sector of the visual field. Severity markers of CIDP (ODSS, motor nerve conduction velocity) were associated with mfVEP latency delay. Interpretation We could not adduce evidence for clinically or diagnostically relevant visual pathway involvement in CIDP. The latency differences identified were very subtle and restricted to the superior visual field which cannot be readily explained biologically, anatomically, or pathologically. In summary, we conclude that our study revealed no relevant differences in mfVEP parameters between CIDP patients and controls.

Die Biomechanik der Akkommodation ist besonders mit Hinblick auf die Ursachen der Presbyopie und auf die Funktionsweise von Intraokularlinsen von Interesse. Ziel der vorliegenden Arbeit ist die detaillierte modellhafte Abbildung der Mechanismen der Akkommodation. Es wird der gegenwartige Stand bei der Anwendung biomechanischer Modelle der Akkommodation vorgestellt, welche ein Verstandnis des Akkommodationsvorgangs ermoglichen. Die etablierten Modelle konnen bei Anwendung der Voraussetzungen entsprechend der Helmholtz-Theorie den Akkommodationsvorgang plausibel erklaren. Sie gestatten damit weiterfuhrende Untersuchungen zur Presbyopiegenese und zur Entwicklung akkommodierbarer Intraokularlinsen und -implantate. Allerdings sind immer Messungen notwendig, um die Simulationsergebnisse mit der Realitat abzugleichen und um Eingabedaten wie Materialdaten und geometrische Abmessungen der Bestandteile des Auges bereitzustellen.

The objectives of the study were to investigate the value of optical coherence tomography in detecting papilledema in patients with idiopathic intracranial hypertension (IIH), a disease which is difficult to monitor and which can lead to permanent visual deficits; to analyze retinal changes over time. In this non-interventional case-control study, spectral-domain optical coherence tomography (SD-OCT) was used to analyze the retinal and optic nerve head (ONH) morphology of 21 patients with IIH and 27 age- and sex-matched healthy controls over time. We analyzed the ONH volume using a custom-made algorithm and employed semi-automated segmentation of macular volume scans to assess the macular retinal nerve fiber layer (RNFL) and ganglion cell layer and inner plexiform layer complex as well as the total macular volume. In IIH patients, the ONH volume was increased and correlated with cerebrospinal fluid (CSF) pressure. The ONH volume decreased after the initiation of treatment with acetazolamide. The macular RNFL volume decreased by 5% in 3.5 months, and a stepwise multivariate regression analysis identified CSF pressure as the main influence on macular RNFL volume at diagnosis. The only factor predicting macular RNFL volume loss over time was ONH volume. SD-OCT can non-invasively monitor changes in retinal and ONH morphology in patients with IIH. Increased ONH volume leads to retinal atrophy in the form of macular RNFL volume loss, presumably due to mechanic jamming of the optic nerve at the disc and subsequent axonal loss.

Der subbasale Nervenplexus (SNP) der Kornea bietet die Moglichkeit, periphere Nervenstrukturen nichtinvasiv in vivo mithilfe der Konfokalmikroskopie (CCM) zu untersuchen. Morphologische Veranderungen des SNP konnen so unmittelbar detektiert und quantitativ charakterisiert werden. Aufgrund der inhomogenen Verteilung der Nervenfasern reicht eine einzelne CCM-Aufnahme fur eine valide Diagnose nicht aus. Gesucht werden daher Verfahren zur grosflachigen Erfassung des SNP. Dieser Beitrag gibt einen Uberblick uber publizierte Ansatze zur Losung dieses Problems. Aktuelle Entwicklungsarbeiten am Karlsruher Institut fur Technologie und der Universitatsaugenklinik in Rostock lassen zukunftig eine vereinfachte Handhabung der Technologie und eine weitere Verbesserung der Bildqualitat erwarten.

Background A characteristic disease pattern may be reflected by retinal layer thickness changes in non-arteritic anterior ischaemic optic neuropathy measured using spectraldomain optical coherence tomography. Retinal layer segmentation is enabled by advanced software. In this study, retinal layer thicknesses in acute and chronic non-arteritic anterior ischaemic optic neuropathy were compared. Design A single-centre cross-sectional analysis was used. Participants A total of 27 patients (20 age-matched healthy eyes) were included: 14 with acute (

Background: The diagnosis of trigeminal neuralgia (TN) is challenging due to the lack of objective diagnostics. Corneal confocal microscopy (CCM) is a non-invasive ophthalmic imaging technique, which allows quantification of corneal nerve fibers arising from the trigeminal ganglion and may allow the assessment of neurodegeneration in TN. Methods: CCM was undertaken in 11 patients with TN and 11 age-matched healthy controls. Corneal nerve fiber density (CNFD), corneal nerve branch density, corneal nerve fiber length (CNFL), corneal nerve fiber width, corneal nerve fiber area, and dendritic cell and non-dendritic cell density with or without nerve fiber contact were quantified. Results: Patients with TN had significantly lower CNFD and CNFL but no difference for any other corneal nerve or dendritic cell parameter in the ipsilateral and the contralateral cornea compared to the control group. There was no significant difference in corneal nerve and cell parameters between patients with TN with and without involvement of the ophthalmic nerve (V1) or with nerve vessel conflict. Conclusion: Corneal confocal microscopy is a rapid non-invasive imaging technique that identifies symmetrical corneal nerve loss in patients with TN.

Background: Visual field defects are a common and disabling consequence of stroke and a negative prognostic factor of patient's quality of life. They result from lesions in different parts of the visual system, most commonly the visual cortex and optic radiation. An important pathophysiological mechanism is transsynaptic retrograde degeneration (TRD). Methods: In a case series 21 patients with posterior cerebral artery (PCA) territory infarctions were analyzed by spectral-domain optical coherence tomography (SD-OCT) and multifocal visual evoked potentials (mfVEPs) cross-sectionally and longitudinally for up to 6 months. In OCT, symptomatic affected nasal and temporal sectors and corresponding visual fields in mfVEPs were compared to the contralateral side. Results: SD-OCT revealed a significant reduction (−2.92 ±2.53 μm, mean ± SD) of the symptomatic nasal macular retinal nerve fiber layer (RNFL) thickness and of the symptomatic temporal peripapillary RNFL after 6 months compared to baseline whereas the symptomatic temporal macular quadrant already showed a significantly thinner RNFL at baseline. The mfVEP first peak latency at baseline was significantly different (nasal visual field +11.69 ±11.17 ms, mean ± SD; temporal visual field +16.63 ±7.97 ms, mean ± SD) on the symptomatic compared to the asymptomatic field. The nasal visual fields partly recovered in amplitude and first peak latency of mfVEPs over the following 6 months compared to baseline. Conclusion: The dynamics of OCT and mfVEP outcomes for degeneration and recovery after PCA infarction differ between the nasal and temporal retinal sector. We postulate that retinal sectors may differ in their temporal pattern of TRD over time after retrogeniculate cerebral infarction.

Diabetes mellitus type 2 (T2D) is characterized by insulin resistance of liver and skeletal muscle, which is at least partly due to impaired muscle mitochondrial function. Long-term HBO therapy, as applied for treating the diabetic foot syndrome, has been shown to improve blood glucose concentrations. To study the underlying mechanisms, we examined the short-term effect of HBO treatment on insulin sensitivity and mitochondrial oxidative capacity and production of reactive oxygen species (ROS) in a randomized, double blinded, placebo-controlled trial. Volunteers with type 2 diabetes (n=4; age 61±4 years; body mass index 30.2±2.2 kg/m2; HbA1c 7.0±0.7%) underwent two 2-hours sessions in a hyperbaric chamber of once with application of 100% oxygen (1.4 bar over atmospheric pressure [barO] for 20 min; HBO) and once with ambient air as placebo (21% oxygen; 1.4 barO; PLC). Tissue-specific insulin sensitivity was assessed by hyperinsulinemic-euglycemic clamps with stable isotope dilution, while muscle oxygen fluxes were measured with high resolution respirometry. After HBO treatment, hepatic insulin sensitivity was higher (insulin-mediated suppression of endogenous glucose production: 69.7±0.1% vs. PLC 55.3±3.2%, p In conclusion, a single session of HBO treatment already improves hepatic insulin resistance and stimulates muscle ROS release, which may activate antioxidative defense, both of which could contribute to improved glycaemia upon chronic treatment. Disclosure T. Sarabhai: None. E. Anadol-Schmitz: None. H. Katsuyama: None. D.F. Markgraf: Research Support; Self; Sanofi. L. Busch: None. R. Guthoff: None. J. Schneppendahl: None. M. Roden: Advisory Panel; Self; Boehringer Ingelheim Pharmaceuticals, Inc., Poxel SA, Servier. Board Member; Self; Eli Lilly and Company. Research Support; Self; Boehringer Ingelheim Pharmaceuticals, Inc., Sanofi. Speaker's Bureau; Self; Novo Nordisk Inc.

Background: Symptomatic carotid artery disease (CAD) may cause modified blood supply to the retina possibly leading to retinal structure changes. Results of previous studies in asymptomatic CAD were heterogeneous in retinal layer changes measured by OCT. The objectives of this prospective, non-interventional study were to investigate if structural retinal changes occur in symptomatic CAD patients with macroangiopathic ischemic stroke or transient ischemic attack (TIA). Methods: We used spectral-domain optical coherence tomography (SD-OCT) to cross-sectionally and longitudinally analyze the retinal morphology of CAD patients with macroangiopathic ischemic stroke or TIA not permanently affecting the visual pathway. We employed semi-automated segmentation of macular volume scans to assess the macular retinal layers' thickness and peripapillary ring scans to determine the peripapillary retinal nerve fiber layer thickness using the contralateral eye and eyes of microangiopathic ischemic stroke patients with matched age, gender, and vascular risk factors as control. Visual function and visual field deficits were assessed by multifocal visual evoked potentials (mfVEP). Results: Neither the thickness of retinal layers measured by SD-OCT in 17 patients nor the mfVEP latency or amplitude in 10 patients differed between the symptomatic stenotic, the contralateral internal carotid artery (ICA) side and the control group of 17 microangiopathic stroke patients at baseline. Furthermore, longitudinal investigations of 10 patients revealed no significant changes of any retinal layer 4 months after ischemic stroke or TIA. Conclusion: In conclusion, our study revealed no evidence for an impact of symptomatic carotid artery disease on retinal structure or functional impairment of the visual pathway.

BACKGROUND: Susac syndrome is characterised by the triad of encephalopathy with or without focal neurological signs, branch retinal artery occlusions and hearing loss. Establishment of the diagnosis is often delayed because the triad is complete only in a minority of patients at disease onset. This leads to a critical delay in the initiation of appropriate treatment. Our objective was to establish criteria for diagnosis of either definite or probable Susac syndrome. METHOD: The establishment of diagnostic criteria was based on the following three steps: (1) Definition of a reference group of 32 patients with an unambiguous diagnosis of Susac syndrome as assessed by all interdisciplinary experts of the European Susac Consortium (EuSaC) team (EuSaC cohort); (2) selection of diagnostic criteria, based on common clinical and paraclinical findings in the EuSaC cohort and on a review of the literature; and (3) validation of the proposed criteria in the previously published cohort of all Susac cases reported until 2012. RESULTS: Integrating the clinical presentation and paraclinical findings, we propose formal criteria and recommend a diagnostic workup to facilitate the diagnosis of Susac syndrome. More than 90% of the cases in the literature fulfilled the proposed criteria for probable or definite Susac syndrome. We surmise that more patients could have been diagnosed with the recommended diagnostic workup. CONCLUSIONS: We propose diagnostic criteria for Susac syndrome that may help both experts and physicians not familiar with Susac syndrome to make a correct diagnosis and to prevent delayed treatment initiation.

Zytologische und histologische Methoden sind vergleichsweise selten eingesetzte, aber wichtige Hilfsmittel bei der Diagnostik von Erkrankungen der Bindehaut und Hornhaut. Die Impressionszytologie bzw. mit Bürsten gewonnene zytologische Abstriche der Konjunktiva sind einfach durchzuführen, kostengünstig und effizient. Als nicht invasive Verfahren stellen sie nur geringe Anforderungen an die Patientencompliance und das manuelle Geschick des Untersuchers. Sie können rasch in Tropfanästhesie durchgeführt und bei Bedarf problemlos wiederholt werden. Ein weiteres Mittel stellt die histologische Untersuchung dar, die im Regelfall zwar eine lokale Betäubung und Exzision erfordert, aber auch eine höhere diagnostische Aussagekraft besitzt.

Background Even though macular edema (ME) in patients with retinal vein occlusion (RVO) is resolved after intravitreal treatment with anti-vascular endothelial growth factor (VEGF), impairment of visual acuity (VA) often persists. Objective A qualitative and quantitative evaluation of spectral domain optical coherence tomography (SD-OCT) images was carried out in patients with RVO and resolved ME to investigate a correlation between retinal morphology and functional results. Material and methods Foveal SD-OCT scans of 13 patients with RVO and resolved ME after treatment were retrospectively evaluated. The thickness of inner retinal layers up to the external limiting membrane (ELM) and up to the photoreceptors in the retinal pigment epithelium (RPE) was measured by automatic segmentation software. Foveal continuity of the four outer hyperreflective bands, the ellipsoid zone of the inner segments (ISe), the ELM, the interdigitation zone (IZ), the RPE and the location of the initial ME were evaluated. Patients with good (≤ 0.3 logMAR, n = 10) and poor VA (≥ 1.0 logMAR, n = 3) were compared. Results Inner retinal layers up to ELM were thinner in the the poor VA group. In the good VA group the initial ME was significantly more often above the ISe and after resolution of ME the ISe tended to be intact more frequently. Conclusion In patients with poor VA despite resolved ME the inner retinal layers up to the ELM were significantly thinner, which could be a sign of atrophy. Qualitative differences were seen at the photoreceptor level, which could be explained by ischemia or an involvement of the outer retina during initial ME that leads to permanent destruction of the ISe.

AIM: To investigate indications, surgical challenges, and outcome of Descemet-membrane endothelial keratoplasty (DMEK) in patients with retinal comorbidities (RC). METHODS: In a prospective cohort study, 8 eyes of 8 DMEK-patients with known RC were compared to 38 eyes of 38 DMEK-patients without RC. The duration of surgery, the degree of difficulty graded by the surgeon, and the complications through DMEK-surgery were analyzed for each patient. The best-corrected visual acuity (BCVA), the endothelial cell count, the intraocular pressure, and the subjective satisfaction was evaluated after a 6-month follow-up. Data were compared applying the non-parametric Wilcoxon-, Chi-square- and Fisher´s-exact-test with P≤0. 05 as level of significance. RESULTS: RC-patients had dry age-related macular degeneration (n=4) or history of pars-plana vitrectomy (n=4). The main indication for DMEK was pain due to bullous keratopathy for the RC-patients (n=7, 88%) and visual impairment due to Fuchs endothelial keratoplasty for the non-RC-patients (n=33, 87%). The BCVA increased for both groups (P=0.01, P

Introduction: Macular edema after cataract surgery (Irvine-Gass syndrome) or pars plana vitrectomy is a postoperative complication which can lead to permanent visual loss. Increased inflammatory substances, such as prostaglandins and cytokines, are discussed to be causative. Currently, there are no evidence-based guidelines for the treatment of postoperative macular edema. Intravitreal dexamethasone (DEX) could be effective by its anti-inflammatory effect. We examined the functional and morphological results of treatment with 0.7 mg intravitreal DEX implant (Ozurdex®). Methods: In an observational study, we analyzed visual acuity (logMAR), intraocular pressure (IOP), clinical findings, and the central macular thickness (CMT, optical coherence tomography [OCT] Spectralis®, Heidelberg Engineering, 30° macular scan, 19 scans) of 12 eyes before and 1 month after the last DEX implantation (off-label use, Ozurdex®, Allergan, Inc., Irvine, CA, USA) for macular edema after cataract surgery or vitrectomy. Re-implantation was performed when OCT showed new intraretinal fluid along with a decrease in the patient's visual acuity. The mean follow-up was 14.4 ± 10.6 months. Results: Twelve eyes of 12 patients (4 female, 8 male) with a mean age of 62.6 ± 11.9 years were treated with a mean of 2.5 ± 1.6 intravitreal DEX implant injections. Prior to injection, the visual acuity was 0.74 ± 0.34 logMAR and the CMT was 608 ± 129 µm. One month after the last injection (after a mean of 437 ± 322 days), the CMT normalized (300 ± 90 µm, p < 0.01) in all cases with a visual acuity of 0.49 ± 0.43 logMAR (p < 0.01). After 8.1 ± 5.3 months, recurring macular edema could be completely reduced by re-injection in 66% (8 patients). Four patients had no recurrence. Postinjection, the mean IOP was 17.4 ± 6.8 mm Hg. Postinjection, 7 patients required topical antiglaucomatous therapy. Conclusions: Treatment with an intravitreal DEX implant is an effective therapy for postoperative macular edema. Each injection leads to a complete resorption of the edema with a significant increase in visual acuity.

Objective There is an unmet need for better diagnostic tools to further delineate clinical subsets of heterogeneous chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) to facilitate treatment decisions. Corneal confocal microscopy (CCM) is a noninvasive and reproducible nerve imaging technique. This study evaluates the potential of CCM as a diagnostic surrogate in CIDP and MMN. Methods In a cross-sectional prospective approach, 182 patients and healthy controls were studied using CCM to quantify corneal nerve damage and immune cell infiltration. Results Patients with CIDP and MMN had a reduction in corneal nerve fiber (CNF) measures and an increase in corneal immune cell infiltrates. In CIDP, CNF parameters decreased with increasing duration of disease. The number of dendritic cells in proximity to CNFs was increased in patients with early disease and correlated with the degree of motor affection. A further reduction in CNF parameters and an increase in nondendritic cells were observed in patients with painful neuropathy. In CIDP patients with antineuronal antibodies the number of nondendritic cells was increased. Interpretation Our findings suggest that CNF loss may reflect severity of neuropathy and quantification of distinct cells around the CNF plexus may help in stratifying CIDP subtypes, clinical course, and disease activity. However, further longitudinal studies are required before CCM can be considered as a valid surrogate endpoint for patients with CIDP and MMN.

Purpose Recent studies highlighted that early diabetic neurodegeneration is present before microvascular changes are visible. Retinal neurodegeneration can decrease retinal layer thickness. We aimed to determine whether decreased retinal layer thickness is present already in the early time course of disease. Methods A cross-sectional analysis of patients and healthy adults from the German Diabetes Study (GDS, ClinicalTrials.gov Identifier number: CT01055093, https://clinicaltrials.gov/ct2/show/NCT01055093). Inclusion criteria were a diagnosis of diabetes mellitus (DM) within the last 12 months. Retinal layers thickness in the nasal pericentral segment was measured by spectral domain ocular coherence tomography (SD-OCT). For statistical analysis proc mixed (sas-version 9.4) was used. Results One hundred and seventy-eight eyes of 89 patients with type 1 DM (58 males, age 36 ± 11 years, BMI 25.5 ± 4.2 kg/m²) and 242 eyes of 121 patients with type 2 DM (84 males, age 53 ± 10 years, BMI 31.9 ± 6.3 kg/m²) with a disease duration of less than 1 year were compared to 76 eyes of 38 controls (27 males, age 41 ± 16 years, BMI 27.3 ± 6.4 kg/m²). Analysis of retinal layer thickness and visual function did not reveal a significant difference between patients and controls. Conclusion In the early course of DM potential, neurodegeneration does not relate to measureable changes of retinal layer thickness.

Optical coherence tomography (OCT) of the retina and corneal confocal laser scanning microscopy (CLSM) of the subbasal nerve plexus (SBP) are noninvasive techniques for quantification of the ocular neurodegenerative changes in individuals with type 1 diabetes mellitus (T1DM). In adult T1DM patients these changes are hardly related to T1DM only. Instead, ageing and/or lifestyle associated comorbidities have to be considered as putative confounding variables. Therefore, we investigated pediatric T1DM patients (n = 28; 14.2 ± 2.51 y; duration of disease: 5.39 ± 4.16 y) without clinical signs of diabetic retina disease, neuropathy, vasculopathy or nephropathy and compared our findings with those obtained in healthy controls (n = 46; 14.8 ± 1.89 y). The SBP was characterized by the averaged length, thickness, and tortuosity of nerve fibers as well as the number of branching and connecting points. OCT was used to determine the total thickness of the retina (ALL) and the thickness of each retinal layer. Both methods revealed signs of early neurodegenerative changes, e.g. thinning of distinct retinal layers at the pericentral ring and shortening of corneal nerve fibers that are already present in pediatric T1DM patients. Standardization of instruments and algorithms are urgently required to enable uniform comparison between different groups and define normative values to introduce in the clinical setting.

The aim of this non-interventional study was to characterize retinal layer pathology in Susac syndrome (SuS), a disease with presumably autoimmune-mediated microvessel occlusions in the retina, brain, and inner ear, in comparison to the most important differential diagnosis multiple sclerosis (MS).Seventeen patients with SuS and 17 age- and sex-matched patients with relapsing-remitting MS (RRMS) and healthy controls (HC) were prospectively investigated by spectral-domain optical coherence tomography (OCT) including intraretinal layer segmentation in a multicenter study. Patients with SuS additionally received retinal fluorescein angiography (FA) and automated perimetry.Patchy thinning of the retinal nerve fiber layer, ganglion cell layer, inner plexiform layer, inner nuclear layer, and outer plexiform layer compared to corresponding sectors in RRMS and HC eyes (p0.003 for SuS vs RRMS and HC) was observed in 23/34 (68%) SuS eyes, particularly in temporal quadrants. The outer nuclear layer (ONL) and photoreceptor layers (PRL) were not affected. FA performed in 15/17 patients with SuS was negative for disease-specific branch retinal artery occlusions in all but 1 eye at the time of OCT examination and revealed no additional vascular abnormalities, even in severely damaged OCT areas. In a subset of patients with SuS, associations of visual field data with distinct retinal layers were observed.Distinct OCT patterns of scattered, scar-like intraretinal pathology in SuS eyes, sparing the ONL and PRL, suggest a retinal, but not choroidal, vascular pathomechanism and clearly differentiate SuS from RRMS. Depending on the disease stage, OCT and FA provide specific complementary diagnostic information in SuS.

Amyotrophic lateral sclerosis (ALS) is characterized by neuro-ophthalmological abnormalities beyond disturbed oculomotor control such as decreased visual acuity and disturbed visual evoked potentials. Here we report retinal alterations in a cohort of 24 patients with clinically definite (n = 20) or probable (n = 4) ALS as compared to matched controls. High-resolution spectral domain optical coherence tomography with retinal segmentation revealed a subtle reduction in the macular thickness and the retinal nerve fiber layer (RNFL) as well as a marked thinning of the inner nuclear layer (INL). Our data indicate an unprecedented retinal damage pattern and suggest neurodegeneration beyond the motor system in this disease.