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Purpose To explore the pupil redilation during persistent light exposure (pupillary escape phenomenon) at the macula and periphery with monochromatic light stimuli. Methods Forty healthy subjects aged 18–64 years (24 females) were examined by chromatic pupil campimetry (CPC) using red and blue 4-s stimuli of 10° radius at the center and 20°-peripheral locations one per quadrant. One glaucoma patient and one achromatopsia patient served as disease models. For statistical analyses, linear mixed-effects models were performed followed by post hoc t-tests. Results A distinct pupillary escape could be demonstrated peripherally (blue 0.099%*s, red 0.153%*s); at the central healthy retina, there was no relevant escape, neither for blue nor red stimulation. Comparing central versus peripheral stimulation revealed highly significant differences in the escape (difference blue 0.100 ± 0.013, red 0.144 ± 0.013, < 0.0001, respectively). In the periphery, the escape was significantly more pronounced for red compared with blue stimulation (difference 0.054 ± 0.013; p = 0.0001). Enhanced pupillary escape outside of the 95% confidence interval of the linear mixed-effects model of the healthy population could be exemplarily shown in a patient with glaucomatous ganglion cell damage. In the achromatopsia example, no relevant escape was found for blue stimulation, but for red stimulation in the periphery in a comparable range to healthy controls. Conclusion The results emphasize that an intact inner retinal network of nerve fibers arising from the central macular region is necessary for maintaining pupillary constriction during a bright 4-s light stimulus and preventing increase of pupillary escape. Increasing receptive field sizes towards the periphery on the level of retinal ganglion cells and less input from central 1:1 connections could be one of the driving mechanisms for pupillary escape.

Purpose To assess the effect of central and peripheral stimulation on the pupillary light reflex. The aim was to detect possible differences between cone- and rod-driven reactions. Methods Relative maximal pupil constriction amplitude (relMCA) and latency to constriction onset (latency) to cone- and rod-specific stimuli of 30 healthy participants (24 ± 5 years (standard deviation)) were measured using chromatic pupil campimetry. Cone- and rod-specific stimuli had different intensities and wavelengths according to the Standards in Pupillography. Five filled circles with radii of 3°, 5°, 10°, 20° and 40° and four rings with a constant outer radius of 40° and inner radii of 3°, 5°, 10° and 20° were used as stimuli. Results For cone-and rod-specific stimuli, relMCA increased with the stimulus area for both, circles and rings. However, increasing the area of a cone-specific ring by minimizing its inner radius with constant outer radius increased relMCA significantly stronger than the same did for a rod-specific ring. For cones and rods, a circle stimulus with a radius of 40° created a lower relMCA than the summation of the relMCAs to the corresponding ring and circle stimuli which combined create a 40° circle-stimulus. Latency was longer for rods than for cones. It decreased with increasing stimulus area for circle stimuli while it stayed nearly constant with increasing ring stimulus area for cone- and rod-specific stimuli. Conclusion The effect of central stimulation on relMCA is more dominant for cone-specific stimuli than for rod-specific stimuli while latency dynamics are similar for both conditions.

Although non-functioning pituitary tumors are frequent, diagnostic and therapeutic concepts are not well standardized. We here present the first German multidisciplinary guideline on this topic. The single most important message is to manage the patients by a multidisciplinary team (consisting at least of an endocrinologist, a neurosurgeon, and a (neuro-) radiologist). The initial diagnostic work-up comprises a detailed characterization of both biochemical (focusing on hormonal excess or deficiency states) and morphological aspects (with magnetic resonance imaging of the sellar region). An ophthalmological examination is only needed in presence of symptoms or large tumors affecting the visual system. Asymptomatic, hormonally inactive tumors allow for a 'wait and scan' strategy. In contrast, surgical treatment by an experienced pituitary surgeon is standard of care in case of (impending) visual impairment. Therapeutic options for incompletely resected or recurrent tumors include re-operation, radiotherapy, and observation; the individual treatment plan should be developed multidisciplinary. Irrespective of the therapeutic approach applied, patients require long-term follow-up. Patient with larger pituitary tumors or former surgery/radiotherapy should be regularly counseled regarding potential symptoms of hormonal deficiency states.

Purpose In September 2015, the first and so far only medication for treatment of Leber’s hereditary optic neuropathy (LHON) was approved in the EU. The drug in question is idebenone (©Raxone) and has been given to all newly diagnosed patients of the University Eye Hospital Tuebingen since the approval of the drug. The aim of the study was to find out whether regular administration of the drug led to an improvement in vision. We retrospectively examined 2 cohorts of consecutive patients with newly occurred visual impairment and LHON diagnosis: One with the initial diagnosis made from January 2010 until April 2014 and a second from October 2015 until January 2020. Methods Retrospective, observational cohort study. All electronic medical files of newly diagnosed and genetically confirmed LHON patients of the University Eye Hospital Tuebingen from January 2010 until April 2014 (cohort 1) and October 2015 until January 2020 (cohort 2) with at least 12 months of follow-up examinations have been analyzed. Results Five patients were included in the first and 7 patients in the second cohort. Patients of cohort 1 received no medication; patients of cohort 2, a daily dose of 900 mg idebenone. The primary visual acuity (VA) ranged between 0.03 and 0.5 in cohort 1 and did not improve during the observation period (median 60 months, range 23–87 months). The patients of cohort 2 have been observed for a median of 23 months (range 12–35 m). The primary VA ranged from 0.01 to 0.16. A recovery in one or both eyes with a final VA from 0.8 to 1.0 was experienced in 3 out of 7 patients. All patients showing a recovery of VA carried the m.11778G>A mutation. Conclusion The observed improvement in the treated cohort may be considered as a hint on the efficacy of idebenone in LHON. The time course of improvement suggests that idebenone should be given 1.5 years in newly diagnosed LHON cases.

The swinging flashlight test is a standard diagnostic procedure to detect relative afferent pupillary defects. The advantages of the test lie within its objectivity and minimal effort. However, its value depends on its correct execution and interpretation. This questionnaire-based survey investigates whether this is given among German speaking ophthalmologists.A multiple-choice questionnaire with 14 questions on the use of the swinging flashlight test was designed. It was presented to German speaking ophthalmology specialists (primary data) and orthoptists (secondary data) on specialist conferences or by telephone interviews.249 ophthalmologists and 76 orthoptists participated in the survey. Only 2% of ophthalmologists answered all 14 questions correctly. On average 66% (range 29 - 100%) of the questions were answered correctly by the ophthalmologists. The question with the lowest result had a rate of 19%, the question with the highest result was correctly answered by 95%. The orthoptists achieved similar results.The rate of correct answers appears disturbingly low. The swinging flashlight test being a basic tool and an obligatory test in a number of guidelines should rather be performed nearly error-free. In light of the high error rates, misdiagnoses and treatment errors must be feared. Better training seems necessary.Der Swinging-Flashlight-Test ist eine ophthalmologische Standarduntersuchung zur Ermittlung eines relativen afferenten Pupillendefekts (RAPD). Die Vorzüge des Tests liegen in seinem minimalen technischen Aufwand und seiner Objektivität. Sein Nutzen hängt jedoch von der korrekten Durchführung und richtigen Interpretation ab. Inwieweit dies gegeben ist, untersuchte die vorliegende Befragungsstudie unter deutschsprachigen Ophthalmologen.Es wurde ein Multiple-Choice-Fragebogen mit 14 Fragen zur Anwendung des Swinging-Flashlight-Tests entwickelt. Dieser wurde deutschsprachigen Augenfachärzten (Hauptauswertung) und Orthoptistinnen (Nebenauswertung) auf Kongressen vorgelegt oder mittels Telefoninterview abgefragt.249 Augenfachärzte und 76 Orthoptistinnen nahmen an der Befragung teil. Nur 2% der Fachärzte beantworteten alle 14 Fragen richtig. Durchschnittlich wurden 66% (Spanne 29 – 100%) der Fragen richtig beantwortet. Die Frage mit dem schlechtesten Ergebnis wurde von 19%, die mit dem besten Ergebnis von 95% der Teilnehmer richtig beantwortet. Die Ergebnisse der Teilgruppe der Orthoptistinnen stimmten annähernd mit denen der Fachärzte überein.Die Rate an Richtigantworten erscheint beunruhigend niedrig. Für eine klinische Untersuchungsmethode, die als augenärztliches Basishandwerkszeug gilt und in ophthalmologischen Leitlinien als obligate Untersuchung geführt wird, wäre eine nahezu fehlerfreie Anwendung zu fordern. Angesichts der hohen Fehlerrate sind Fehldiagnosen und -behandlungen zu befürchten. Verbesserungen der Weiter- und Fortbildung sind hier unabdingbar.

There are many disease patterns that are treated jointly by neurologists and ophthalmologists, for which optical coherence tomography (OCT) is of important differential diagnostic significance. In this context neurologists are mainly confronted by two patient collectives: patients with an acute ischemic event, who present with an acute but painless monocular visual deterioration (for central retinal artery occlusion) or with a monocular visual field defect (for arterial branch occlusion or anterior ischemic optic neuropathy). The second collective is patients without ophthalmological symptoms but with conspicuous optic nerve findings (papilledema or optic disc drusen). In this overview article both patient collectives are considered separately. In addition, the most important OCT findings for optic neuritis are presented. Before the disease patterns are described in detail, the normal OCT findings and the diagnostic possibilities of OCT are explained.Es gibt viele Krankheitsbilder, die von Neurologen und Ophthalmologen gemeinsam behandelt werden, bei denen die optische Kohärenztomographie (OCT) von wichtiger differenzialdiagnostischer Bedeutung ist. Hierbei werden Neurologen hauptsächlich mit zwei Patientenkollektiven konfrontiert: zum einen Patienten mit einem akut ischämischen Geschehen, welche sich mit einer akuten, schmerzlosen monokularen Sehverschlechterung (bei Zentralarterienverschluss) bzw. mit einem monokularen Gesichtsfelddefekt (bei Arterienastverschluss oder anteriorer ischämischer Optikusneuropathie) präsentieren und zum anderen Patienten ohne ophthalmologische Symptomatik, aber mit auffälligen Sehnervenbefunden (Stauungspapille oder Drusenpapille). Diese beiden Patientenkollektive möchten wir in dieser Übersichtsarbeit getrennt voneinander betrachten. Zudem werden wir die wichtigsten OCT-Befunde bei der Optikusneuritis darstellen. Bevor die Krankheitsbilder im Detail beschrieben werden, sollen der OCT-Normalbefund sowie die diagnostischen Möglichkeiten der OCT erläutert werden.

Recently, we proposed a method to assess cell-specific retinal functions based on the frequency-dependent responses to sinusoidal transcorneal electrostimulation. In this study, we evaluated the alterations in responsiveness in achromatopsia patients to explore the frequency-selectivity of photoreceptors. The electrical stimulation was applied to one eye of genetically confirmed achromatopsia patients via corneal electrodes. The stimulus was composed of amplitude-modulated sine waves with variable carrier frequencies (4-30 Hz) and a steady low-frequency envelope. The retinal responsiveness across the spectrum was calculated based on the velocity and the synchronicity of the electrically evoked pupillary oscillations. Achromats displayed a characteristic peak in responsiveness in the 6-10 Hz range. In contrast, stimulus frequencies above 16 Hz elicited only weak pupil responses and weak phosphenes. Compared to the tuning curve of the healthy retina, responses to low-frequency stimulation appear to reflect mainly rod activation while higher frequencies seem to activate cones. The possibility to examine cell-specific retinal functions independently from their responses to light may improve our understanding of the structural changes in the retina induced by gene therapy.

Significance Horizontal visual field extension was assessed for red and white stimuli in subjects with protanopia using semi-automated kinetic perimetry. In contrast to a conventional anomaloscope, the “red/white dissociation ratio” (RWR) allows to describe protanopia numerically. For the majority of subjects with protanopia a restriction for faint red stimuli was found. Purpose Comparing the horizontal visual field extensions for red and white stimuli in subjects with protanopia and those with normal trichromacy and assessing the related intra-subject intra-session repeatability. Methods The subjects were divided into groups with protanopia and with normal trichromacy, based on color vision testing (HMC anomaloscope, Oculus, Wetzlar/FRG). Two stimulus characteristics, III4e and III1e, according to the Goldmann-classification, were presented with semi-automated kinetic perimetry (Octopus 900 perimeter, Haag-Streit, Köniz/CH). They moved along the horizontal meridian, with an angular velocity of 3°/s towards the visual field center, starting from either the temporal or nasal periphery. If necessary, a 20° nasal fixation point offset was chosen to capture the temporal periphery of the visual field. For each condition the red/white dissociation ratio (RWR); Pat Appl. DPMA DRN 43200082D) between the extent of the isopter for red (RG610, Schott, Mainz/ FRG) and white stimuli along the horizontal meridian was determined. Results All data are listed as median/interquartile range: Five males with protanopia (age 22.1/4.5 years) and six males with normal trichromacy (control group, age 30.5/15.2 years) were enrolled. The RWR is listed for the right eye, as no clinically relevant difference between right and left eye occurred. Protanopes’ RWR for mark III4e (in brackets: control group) was 0.941/0.013 (0.977/0.019) and for mark III1e 0.496/0.062 (0.805/0.051), respectively. Conclusions In this exploratory “proof-of-concept study” red/white dissociation ratio perimetry is introduced as a novel technique aiming at assessing and quantifying the severity of protanopia. Further effort is needed to understand the magnitude of the observed red-/white dissociation and to extend this methodology to a wider age range of the sample and to anomalous trichromacies (protanomalia) with varying magnitude.

BACKGROUND Visual phenomena are of many different kinds; their differential diagnosis is usually possible with directed history-taking. In this review, we describe common visual phenomena that must be distinguished from a migraine aura. METHODS This review is based on publications retrieved by a selective search in PubMed and the Web of Knowledge/Science, with special attention to the current classification of the International Headache Society and the recommendations of the German Migraine and Headache Society. The following search terms were used: "visual phenomena/phenomenon," "migraine aura," and "persistent/complication/long-lasting/ongoing." RESULTS The most helpful questions for differential diagnosis are whether the symptoms are present in one eye only or in both, whether their onset was sudden or over minutes or days, and whether the phenomenon has occurred only once or repeatedly, or is persistently present. A visual aura associated with migraine must be distinguished, in rare cases, from an isolated epileptic aura, from cerebral/retinal ischemia, or from visual snow. Further differential diagnoses include a persisting perceptual disturbance after hallucinogen use (HPPD, "hallucinogen persisting perception disorder") and the Charles Bonnet syndrome (CBS); the latter arises as a consequence of severely impaired vision. Posterior reversible encephalopathy syndrome (PRES) is rare and generally reveals itself over its further course through the appearance of additional clinical manifestations. Primary ophthalmological causes can usually be readily identified and classified by ophthalmological examination. CONCLUSION Patients with visual phenomena typically consult physicians from various medical specialties. A correct differential diagnosis can be made based on the history if the physician views the symptoms in their overall context to determine the particular disease entity that is responsible.

Studies on the electrical excitability of retinal neurons show that photoreceptors and other cell types can be selectively activated by distinct stimulation frequencies in vitro. Yet, this principle still needs to be validated in humans in vivo. As a first step, this study explored the frequency preferences of human rods by means of transcorneal electrostimulation (TES), using the electrically-elicited pupillary responses (EEPRs) as an objective readout. The stimulation paradigm contained a 1.2 Hz sinusoidal envelope, which was superimposed on variable carrier frequencies (4-30 Hz). These currents were delivered to one of the participant's eyes via a corneal electrode and consensual pupillary reactions were recorded from the contralateral eye. The responsiveness of the retina at each frequency was assessed based on the EEPR dynamics. Differences between healthy participants and patients with retinitis pigmentosa were evaluated to identify the preferred frequency range of rods. The responsiveness of healthy individuals revealed a clear peak around 6-8 Hz. In contrast, the pupillary responses of patients were significantly reduced in the lower frequency range. These findings suggest that the responses in this frequency bin were selectively mediated by rods. This work provides evidence that different retinal cell types can be selectively activated via TES in vivo, and that this effect can be captured noninvasively using EEPRs. This knowledge may be exploited for the diagnostics and therapy of retinal diseases, e.g., to design cell-specific functional tests for the degenerating retina, or to optimize stimulation paradigms which are currently used by retinal prostheses.

Zusammenfassung Hintergrund Es wurde vermutet, dass die mit Drusenpapillen assoziierten Gesichtsfeldausfälle im Laufe des Lebens stetig langsam zunehmen oder aber in der Adoleszenz entstehen und sich im Laufe des Lebens nur unwesentlich verschlechtern. Wir überprüften diese Annahmen. Material und Methoden Aus dem Patientenverwaltungssystem der Universitäts-Augenklinik Tübingen wurden 255 konsekutive Fälle mit Drusenpapillen identifiziert, die Richtigkeit der Diagnose verifiziert und, soweit vorhanden und qualitativ verwertbar, die Gesichtsfelder quantifiziert. Zusätzlich wurde der Visus ausgewertet. Ergebnisse In 104 Fällen lagen beidseitig qualitativ gute, quantifizierbare Gesichtsfelder vor. Der Anteil an Patienten mit ausgeprägten Gesichtsfeldausfällen fiel insgesamt gering aus. Nur 3 Patienten zeigten beidseitig einen Gesichtsfeldverlust von ≥ 50%. Beide Augen waren meistens vergleichbar betroffen. Ein höheres Alter war mit größeren Gesichtsfeldausfällen korreliert. Lediglich 1 Patient (0,4%) erreichte beidseitig nicht den Visus 0,3. Diskussion Anhand unseres Patientenkollektivs ist eine kontinuierliche leichte Verschlechterung des Gesichtsfelds mit dem Alter anzunehmen. Sehr ausgeprägte Gesichtsfeldausfälle waren selten. Dasselbe galt auch für den Visus, der erst im Alter ab 60 Jahren leichte Beeinträchtigungen aufwies.

ZusammenfassungMeningeome sind langsam wachsende Tumoren, die entweder durch Sehverschlechterung oder Doppelbilder symptomatisch werden oder aber zufällig entdeckt werden. Nicht jedes Meningeom bedarf der Behandlung. Diese besteht entweder in der neurochirurgischen Entfernung oder, wenn inoperabel, in der Strahlentherapie. Ob dies notwendig ist, richtet sich bei Meningeomen der vorderen Sehbahn hauptsächlich nach dem augenärztlichen Befund. Dieser kann und muss mittels OCT und klinischer Würdigung des Gesamtbilds auch Aussagen zur Prognose machen. Diese Arbeit gibt einen Überblick über die Überlegungen, die bei Meningeomen der vorderen Sehbahn anzustellen sind. Sie beruht im Wesentlichen auf eigenen Erfahrungen.

ZusammenfassungDie Beurteilung der Pupille ist ein wichtiger Teil der neuroophthalmologischen Diagnostik. Der Nachweis einer afferenten oder efferenten Pupillenstörung lässt Rückschlüsse auf ein weites Spektrum relevanter Erkrankungen des zentralen und peripheren Nervensystems zu. Dieses Update erfasst einen wesentlichen Teil der Pupillenstörungen der alltäglichen Praxis des Augenarztes. Die Bedeutung der Pupillenuntersuchung hingegen reicht weit über das Fach der Augenheilkunde hinaus.

To assess the pupil light response (PLR) to chromatic stimulation in patients with different types of X-linked congenital stationary night blindness (CSNB). Eight patients with CSNB due to CACNA1F and NYX mutations were exposed to blue and red light stimuli, and PLR was evaluated using infrared video pupillography. Pupil responses were compared between CSNB patients and healthy subjects (n = 34) at baseline, at maximum of constriction, for post-illumination pupil responses (PIPR) and the slope of redilation using Cohen’s d. A subgroup comparison was performed descriptively between CACNA1F and NYX associated CSNB patients using the same parameters. In CSNB, smaller baseline pupil diameters compared to healthy subjects were measured both before blue and red light stimulation (d = 1.44–1.625). The maximum constriction to blue light stimuli was smaller for the CSNB group compared to healthy subjects (d = 1.251) but not for red light stimuli (d = 0.449). Pupil response latencies were prolonged in CSNB for both light stimuli (d = −1.53 for blue and d = −1.011 for red stimulation). No relevant differences were found between the CSNB group and healthy subjects for PIPR (d = 0.01), but the slope of redilation was smaller for CSNB patients (d = 2.12). Paradoxical pupil constriction at light offset was not seen in our patients. A reduced redilation and smaller baseline pupil diameters for patients with CSNB indicate a disinhibition of intrinsically photosensitive retinal ganglion cells due to affected post-photoreceptor transduction via bipolar cells and can explain the pupillary behavior in our patient group.

Pupillographic campimetry allows measuring the visual field objectively by analyzing the pupil response to perimetric stimuli. One of the drawbacks of this technique, similar to static perimetry, is the need of reliable fixation of the subject. By using stimulus sizes comparable to static perimetry and applying gaze tracking, we enable a retinotopic visual field examination regardless of fixation problems and with an increased stability and improved spatial resolution. Here, we present the results of applying the method in eight normal sighted subjects as well as in three patients suffering from diseases usually diagnosed by perimetry. The results in normal sighted subjects show a reduction in the amplitude of the pupil response with increasing eccentricity as expected. We also demonstrate that gaze-controlled campimetry is able to detect organic visual field defects objectively in a patient group and classify the visual field defects without an organic background. Moreover, we show that our method is able to evaluate the visual field sensitivity loss beyond classical perimetry in patients with late-stage retinitis pigmentosa. Thus, gaze-controlled pupil campimetry can be used in addition to classical perimetry, allowing for an objective monitoring of disease progression, rendering it as a biomarker for novel treatments.

Background/objectives The correct classification of a slowly progressing optic atrophy can be challenging. The aim of this work was to find out if the characteristics of peripapillary retinal nerve fiber layer (RNFL) thickness loss differ between open angle glaucoma (POAG), optic nerve sheath meningioma (ONSM), and sphenoid wing meningioma (SWM). Methods A total of 45 patients with POAG, ONSM, and SWM were included in the retrospective study. The peripapillary RNFL thickness measured by spectral-domain optical coherence tomography was analyzed using the Heidelberg Engineering glaucoma module©. Results Each group consisted of 15 patients. The temporal sector of the RNFL thickness showed a median decrease of − 17 µm in glaucoma patients (range + 6/–34 µm), − 43 µm in ONSM (range − 19/ − 52 µm), and − 44 µm in SWM patients (range − 25/ − 52 µm). The RNFL thickness of the temporal sector of glaucoma patients differed significantly from the other groups (p Conclusion The peripapillary RNFL thickness of the temporal sector of patients with beginning to moderate POAG is usually inside normal limits or borderline. In contrast, patients with ONSM and SWM are much more likely to show a considerable reduction in RNFL thickness of the temporal sector. RNFL thickness of the temporal sector marked outside normal limits occurred exclusively in meningioma patients. Considering the presence of this condition as a predictor for meningioma, sensitivity and specificity were 0.8 and 1.0, respectively. In patients with significant reduction in RNFL thickness of the temporal sector, magnetic resonance imaging of the head should be considered to rule out compression of the optic nerves.

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).

As optic nerve sheath meningiomas (ONSM) are rare, there are no prospective studies. Our retrospective analysis focusses on a cohort of patients with uniform disease characteristics all treated with the same radiotherapy regimen. We describe treatment decision making, radiotherapy planning and detailed neuro-ophthalmological outcome of the patients. 26 patients with unilateral ONSM extending only to the orbit and the optic canal were evaluated for neuro-ophthalmological outcome. Radiation treatment was planned in a simultaneous integrated boost approach to gross tumor volume (GTV) + 2 mm / 5 mm to 54 Gy / 51 Gy in 1.8 Gy / 1.7 Gy fractions. Follow-up was done by specialized neuro-ophthalmologists. Visual acuity and visual field defects were evaluated after therapy as well as during follow-up. Interdisciplinary treatment decision for patients with ONSM follows a rather complex decision tree. Radiation treatment planning (equivalent uniform dose (EUD), maximum dose to the optic nerve) improved with experience over time. With this patient selection visual acuity as well as visual field improved significantly at first follow-up after treatment. For visual acuity this also applied to patients with severe defects before treatment. Long term evaluation showed 16 patients with improved visual function, 6 were stable, in 4 patients visual function declined. Interdisciplinary case discussion rated the visual decline as radiation-associated in two patients. With stringent patient selection radiotherapy for unilateral primary ONSM to 51 Gy / 54 Gy is safe and leads to significantly improved visual function. Interdisciplinary treatment decision and experience of the radiation oncology team play a major role.

Purpose To assess the developments in contrast sensitivity, color vision, and subjective perception after Descemet membrane endothelial keratoplasty (DMEK) in patients with Fuchs endothelial corneal dystrophy (FECD). Methods Included in this study were pseudophakic, unilateral DMEK patients with bilateral FECD having a follow-up period of 6 months (n = 23). The mean age at surgery was 70 years (range: 52-81 years). Pseudophakic eyes without history of other ocular pathology or surgery served as a control (n = 10). Pelli-Robson contrast sensitivity and Panel-D15 color vision tests were used. Best-corrected visual acuity, modified visual functioning questionnaire-25, central corneal thickness, and endothelial cell density were assessed. We visualized the subjective impression of patients with bilateral FECD after unilateral DMEK in a subgroup using Photoshop CS6. Results Contrast sensitivity improved significantly from 1.35 ± 0.26 to 1.64 ± 0.17 (P = 0.002; control eyes: 1.92 ± 0.09). No difference in the color vision error score was observed for preoperative and postoperative eyes (P = 0.063). The best-corrected visual acuity improved significantly after surgery (P = 0.001). The average values in the Logarithm of the Minimum Angle of Resolution were 0.59 ± 0.42 preoperatively and 0.1 ± 0.10 postoperatively (control eyes: 0.01 ± 0.03). Examinations revealed a decrease of the central corneal thickness and endothelial cell density after surgery (P = 0.001; P = 0.001, respectively). Scores in the general and the driving questionnaire were significantly higher after surgery (P = 0.001; P = 0.005, respectively). Conclusions This study showed significant improvement in subjective patient satisfaction and contrast sensitivity. Spontaneous subjective color vision improvement might be explained by significantly improved contrast sensitivity. Contrast sensitivity might be considered as a parameter in preoperative decision-making and evaluation of surgical outcome.

Purpose Analysis of a cohort of pediatric optic neuritis patients concerning the epidemiology, disease progression, and association with multiple sclerosis (MS). Methods Retrospective, observational cohort study. From 2004 to 2018, all electronic medical files of patients younger than 18 years referred to a tertiary care clinic in Germany with the diagnosis optic neuritis have been analyzed. Results Sixty-nine patients were referred in the study period, 16 did not suffer under optic neuritis and were excluded. The median visual acuity of the remaining 53 patients was 0.07 at the baseline examination and 1.0 at the latest follow-up examination (decimal notation, median 2.1 years after baseline). Forty-two percent of the patients developed MS during the study period. Female sex (p = 0.028) as well as higher age (p = 0.0082) proved to be statistically significant risk factors for MS development. Conclusion The prognosis for restoring vision in pediatric optic neuritis was favorable. During the observation period, the risk of developing MS was overall 42% and 8% for patients younger than 11 years. The percentage of MS as underlying cause of optic neuritis does not differ remarkably between children older 10 years and adults.

ZusammenfassungNeuroophthalmologische Anamnesen können sehr umfangreich und schwierig sein. Dieser Beitrag gibt 14 Ratschläge, wie man die Anamnese möglichst effizient gestalten und das Ansammeln irrelevanter Informationen vermeiden kann.

ZusammenfassungAkute Doppelbilder können von harmlosen Störungen des optischen Systems bis hin zu gefährlichen intrakraniellen oder intraorbitalen Prozessen mit entsprechenden Bewegungseinschränkungen der Augen verschiedenste Ursachen haben. Entsprechend schwierig kann die Entscheidung bezüglich der Erfordernisse weiterer diagnostischer Abklärungen erscheinen. Dieser Beitrag soll eine kurze, strukturierte Übersicht über die häufigsten Differenzialdiagnosen akuter Doppelbilder liefern und deren Abklärung sowie Behandlungsoptionen aufzeigen.

OBJECTIVE The purpose of this study was to evaluate the pupil response to chromatic stimuli in patients with lesions in the dorsal midbrain and possibly gain new insights into the afferent pupillary pathways. METHODS Color pupillography was performed in 5 patients with dorsal midbrain syndrome (DMS), and their results were compared with those of 20 healthy control subjects. We used full-field red stimuli (605 nm) that primarily address the rod/cone system and blue stimuli (420 nm) that preferentially activate intrinsically photosensitive retinal ganglion cells (ipRGCs) directly, with a duration of 4 seconds and a stimulus intensity of 28 lx corneal illumination under mesopic conditions. One eye was stimulated, and the consensual pupil response was recorded and analyzed. RESULTS The pupillary light reflex in patients with DMS was reduced, differed in shape, and showed a prolonged latency time compared to normal subjects. The blue response was less affected than the red response: the mean maximal relative amplitude (M) was 15.8% (SD = 7.8) in patients with DMS compared with 43.0% (SD = 5.5) in normal subjects for red stimulation, and M = 40.8%, SD = 8.4 (DMS) with M = 58.3%, SD = 4.8 (normals) for blue stimulation. The reduction was 63% for red stimulation but only 30% for blue stimulation in patients with DMS. Moreover, there was a preserved postillumination pupil response to blue stimulation in DMS patients. CONCLUSIONS In DMS, the melanopsin-mediated ipRGC pathway appeared relatively preserved.

It has been assumed that visual field defects in optic disc drusen slowly increase with age or occur during adolescence and do not change substantially in later years. In our study, we aimed to validate these assumptions.255 consecutive cases with optic disc drusen were identified from the patient records of the University Eye Hospital Tübingen; the diagnosis was verified and visual fields were quantified as long as available and of sufficient quality. Additionally, visual acuity was evaluated.In 104 cases, quantifiable visual fields of sufficient quality for both eyes were available. In general, few patients with marked visual field defects could be detected. Only three patients showed visual field defects of ≥ 50% in both eyes. Both eyes were usually involved to approximately the same extent. Older age was correlated with more visual field defects. Only one patient remained below visual acuity of 0.3 in both eyes.By means of our patient base, a continuous slight decline in the visual field with age can be assumed. Marked visual field defects were rare. The same was true for visual acuity, which showed some mild decline above the age of 60 years.Es wurde vermutet, dass die mit Drusenpapillen assoziierten Gesichtsfeldausfälle im Laufe des Lebens stetig langsam zunehmen oder aber in der Adoleszenz entstehen und sich im Laufe des Lebens nur unwesentlich verschlechtern. Wir überprüften diese Annahmen.Aus dem Patientenverwaltungssystem der Universitäts-Augenklinik Tübingen wurden 255 konsekutive Fälle mit Drusenpapillen identifiziert, die Richtigkeit der Diagnose verifiziert und, soweit vorhanden und qualitativ verwertbar, die Gesichtsfelder quantifiziert. Zusätzlich wurde der Visus ausgewertet.In 104 Fällen lagen beidseitig qualitativ gute, quantifizierbare Gesichtsfelder vor. Der Anteil an Patienten mit ausgeprägten Gesichtsfeldausfällen fiel insgesamt gering aus. Nur 3 Patienten zeigten beidseitig einen Gesichtsfeldverlust von ≥ 50%. Beide Augen waren meistens vergleichbar betroffen. Ein höheres Alter war mit größeren Gesichtsfeldausfällen korreliert. Lediglich 1 Patient (0,4%) erreichte beidseitig nicht den Visus 0,3.Anhand unseres Patientenkollektivs ist eine kontinuierliche leichte Verschlechterung des Gesichtsfelds mit dem Alter anzunehmen. Sehr ausgeprägte Gesichtsfeldausfälle waren selten. Dasselbe galt auch für den Visus, der erst im Alter ab 60 Jahren leichte Beeinträchtigungen aufwies.