Explanipedia

Measuring Rod- and Cone-Photoreceptor–Specific Vision in Inherited Retinal Diseases Using a Commercial Perimeter Open

Vivian Wu, Alejandro J. Román, Emma L. Galsterer, Georg Ansari, Inbar Erdinest , et al. · 2025

Photoreceptor-specific function can be measured over a large dynamic range using a turnkey commercial perimeter and a relatively short, practical protocol that may be introduced into the clinic, translational work, and clinical trials.

Flavoprotein Fluorescence Imaging in Stargardt Disease: Linking Metabolic Stress to Structural Damage Open

David A. Merle, Vicente Villanueva, Giulia Righetti, Ronja Jung, Melanie Kempf , et al. · 2025

This study demonstrates that FPF imaging is a promising tool for detecting early metabolic changes in Stargardt disease, potentially serving as a non-invasive biomarker for monitoring disease progression and treatment response. However, cu…

Clinical manifestations of dual-gene variants involving ABCA4 in retinal dystrophies Open

Lasse Wolfram, David A. Merle, Laura Kuehlewein, Milda Reith, Melanie Kempf , et al. · 2025

Background This study investigates the clinical manifestations of inherited retinal diseases (IRD) associated with dual-gene variant constellations involving biallelic ABCA4 variants. Methods We assess four cases for their unique phenotypi…

S-cone contribution to oscillatory potentials in patients with blue cone monochromacy Open

Giulia Righetti, Melanie Kempf, Susanne Kohl, Bernd Wissinger, Laura Kühlewein , et al. · 2024

Purpose The aim of this exploratory study is to investigate the role of S-cones in oscillatory potentials (OPs) generation by individuals with blue-cone monochromacy (BCM), retaining S-cones, and achromatopsia (ACHM), lacking cone function…

Influencing Factors on Pupillary Light Responses as a Biomarker for Local Retinal Function in a Large Normative Cohort Open

Ricarda Jendritza, Krunoslav Stingl, Torsten Straßer, Ronja Jung, Felix Tonagel , et al. · 2024

Stimulus eccentricity had the most relevant impact on relMCA and latency of the PLR during L-cone- and rod-favoring stimulation. Latency is prolonged ≥60 years under scotopic conditions. Considering the large study collective, a representa…

Adaptive optics retinal imaging in patients with usher syndrome Open

Melanie Kempf, Susanne Kohl, Krunoslav Stingl, Fadi Nasser, Katarína Štingl , et al. · 2024

Purpose To determine the structure of the cone photoreceptor mosaic in the macula in eyes with retinitis pigmentosa related to Usher syndrome using adaptive optics fundus (AO) imaging and to correlate these findings with those of the stand…

Comparison of Full-Field Stimulus Threshold Measurements in Patients With Retinitis Pigmentosa and Healthy Subjects With Dilated and Nondilated Pupil Open

Milda Reith, Katarína Štingl, Laura Kühlewein, Melanie Kempf, Krunoslav Stingl , et al. · 2024

Our data provide useful information for the clinical use of FST.

Defining reference values of arterioles in healthy individuals for studies with adaptive optics imaging Open

Constanze Kortuem, David A. Merle, Milda Reith, Laura Kuehlewein, Ronja Jung , et al. · 2024

Purpose To investigate age-dependent wall to lumen ratio (WLR) reference values for healthy individuals in adaptive optics imaging (AO). WLR serves as an objective, dimensionless parameter for the evaluation of structural changes in vessel…

Chorioretinal Atrophy Growth After Voretigene Neparvovec Retinotopically Is Connected to Retinal Functional Rescue Open

Krunoslav Stingl, Melanie Kempf, Ronja Jung, Katarína Štingl · 2024

PURPOSE: Chorioretinal atrophy growth after voretigene neparvovec has been reported recently with its positive correlation with successful treatment. This finding raised the question on long-term effects and the etiology of the chorioretin…

Multi-luminance mobility testing after gene therapy in the context of retinal functional diagnostics Open

Ronja Jung, Melanie Kempf, Saskia Holocher, Friederike Kortüm, Krunoslav Stingl , et al. · 2023

A morphometric analysis of the retinal arterioles with adaptive optics imaging in RPE65‐associated retinal dystrophy after treatment with voretigene neparvovec Open

Constanze Kortuem, Melanie Kempf, David A. Merle, Laura Kuehlewein, Lisa Pohl , et al. · 2023

Purpose To investigate the changes in retinal arterial architecture after treatment with voretigene neparvovec in patients with retinal dystrophy caused by bi‐allelic mutations in the RPE65 gene. Methods Sixteen eyes treated with voretigen…

Rod and Cone Function Measured Objectively by Chromatic Pupil Campimetry Show a Different Preservation Between Distinct Genotypes in Retinitis Pigmentosa Open

Carina Kelbsch, Melanie Kempf, Ronja Jung, Friederike Kortüm, Milda Reith , et al. · 2023

Rod and cone function measured objectively by CPC showed a different preservation between genotypes in RP. However, heterogeneity inside the same genotype was present. CPC data correlated with FST, but structural OCT parameters seem to be …

Photoreceptor-Specific Temporal Contrast Sensitivities in RP1L1-Associated Occult Macular Dystrophy Open

Cord Huchzermeyer, Julien Fars, Jan Kremers, Laura Kühlewein, Melanie Kempf , et al. · 2023

OMD was characterized predominantly by deterioration of L- and M-cone-cone driven function in the perifovea. Rod-driven functions were normal. Differences in the photoreceptor signals were further modified by postreceptoral filters.

Full-field Scotopic Threshold Improvement after Voretigene Neparvovec-rzyl Treatment Correlates with Chorioretinal Atrophy Open

Katarína Štingl, Krunoslav Stingl, Hillary Schwartz, Mark W. Reid, Melanie Kempf , et al. · 2023

Central retina plays a decisive role in the suppression of pupillary escape Open

Carina Kelbsch, Ricarda Jendritza, Torsten Straßer, Felix Tonagel, Paul Richter , et al. · 2023

Frequency-dependent retinal responsiveness to sinusoidal electrical stimulation in achromatopsia Open

Ronja Jung, Melanie Kempf, Lisa Pohl, Friederike Kortüm, Milda Reith , et al. · 2022

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 achromatops…

Therapy with voretigene neparvovec. How to measure success? Open

Krunoslav Stingl, Melanie Kempf, Ronja Jung, Friederike Kortüm, Giulia Righetti , et al. · 2022

Retinal gene supplementation therapy such as the first approved one, voretigene neparvovec, delivers a functioning copy of the missing gene enabling the protein transcription in retinal cells and restore visual functions. After gene supple…

Cell-specific electrical stimulation of human retinal neurons assessed by pupillary response dynamics in vivo Open

Ronja Jung, Carina Kelbsch, Helmut Wilhelm, Barbara Wilhelm, Torsten Straßer , et al. · 2022

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…

Evaluation of Local Rod and Cone Function in Stargardt Disease Open

Krunoslav Stingl, Carel C B Hoyng, Melanie Kempf, Susanne Kohl, Ronja Jung , et al. · 2022

The functional analysis of the macular region in STGD1 disease indicates reduced central cone function, corresponding to photoreceptor degeneration. In contrast, the rod function in the central area was not affected. Nevertheless, some alt…

How lesions at different locations along the visual pathway influence pupillary reactions to chromatic stimuli Open

Carina Kelbsch, Krunoslav Stingl, Ronja Jung, Melanie Kempf, Paul Richter , et al. · 2021

Effect of central and peripheral cone- and rod-specific stimulation on the pupillary light reflex Open

Anton Sonntag, Carina Kelbsch, Ronja Jung, Helmut Wilhelm, Torsten Straßer , et al. · 2021

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 (rel…

Oscillatory Potentials in Achromatopsia as a Tool for Understanding Cone Retinal Functions Open

Giulia Righetti, Melanie Kempf, Christoph Braun, Ronja Jung, Susanne Kohl , et al. · 2021

Achromatopsia (ACHM) is an inherited autosomal recessive disease lacking cone photoreceptors functions. In this study, we characterize the time-frequency representation of the full-field electroretinogram (ffERG) component oscillatory pote…

Short term morphological rescue of the fovea after gene therapy with voretigene neparvovec Open

Constanze Kortuem, Melanie Kempf, Ronja Jung, Susanne Kohl, Constanze Ilse Katharina Kortüm , et al. · 2021

Spatial and temporal resolution of the photoreceptors rescue dynamics after treatment with voretigene neparvovec Open

Krunoslav Stingl, Melanie Kempf, Karl Ulrich Bartz‐Schmidt, Spyridon Dimopoulos, Felix F. Reichel , et al. · 2021

Background Voretigene neparvovec is a gene therapeutic agent for treatment of retinal dystrophies caused by bi-allelic RPE65 mutations. In this study, we report on a novel and objective evaluation of a retinotopic photoreceptor rescue. Met…

Räumliche und zeitliche Auflösung der Wiederherstellungsdynamik der Photorezeptoren nach Behandlung mit Voretigen Neparvovec Open

Krunoslav Stingl, Melanie Kempf, Karl Ulrich Bartz‐Schmidt, Spyridon Dimopoulos, Felix Reichel , et al. · 2021

Hintergrund:Voretigen Neparvovec ist ein gentherapeutischer Wirkstoff zur Behandlung von Netzhautdystrophien, die durch biallelische RPE65-Mutationen verursacht werden. In der vorliegenden Studie berichten wir über eine neuar…

Chromatic Pupil Campimetry Reveals Functional Defects in Exudative Age-Related Macular Degeneration with Differences Related to Disease Activity Open

Carina Kelbsch, Jakob Lange, Helmut Wilhelm, Barbara Wilhelm, Tobias Peters , et al. · 2020

With the combination of short recording time, objectiveness of the measurement and gaze-correction for fixation problems, this method presents a suitable complement to the currently used clinical functional tests of the macula.

Chromatic Full-Field Stimulus Threshold and Pupillography as Functional Markers for Late-Stage, Early-Onset Retinitis Pigmentosa Caused by CRB1 Mutations Open

Krunoslav Stingl, Laura Kuehlewein, Nicole Weisschuh, Saskia Biskup, Frans P.M. Cremers , et al. · 2019

The functional measurements presented in this study present a valid clinical progression marker in late-stage early onset retinitis pigmentosa caused by biallelic CRB1 mutations. Additionally, they can be used as outcome measures fo…

Objective Measurement of Local Rod and Cone Function Using Gaze-Controlled Chromatic Pupil Campimetry in Healthy Subjects Open

Carina Kelbsch, Katarína Štingl, Melanie Kempf, Torsten Straßer, Ronja Jung , et al. · 2019

Chromatic pupil campimetry provides information about the local rod and cone function of the human retina with distinct pattern of distributions in an objective manner.

Objective Measurement of Functional Defects in Age-related Macular Degeneration by Pupil Campimetry Open

Carina Kelbsch, Jakob Lange, Helmut Wilhelm, Barbara Wilhelm, Tobias Peters , et al. · 2019

Evaluation of pupil responses to local stimuli in Patients with retinitis pigmentosa caused by USH2A mutations Open

Tobias Peters, Krunoslav Stingl, Karl Ulrich Bartz‐Schmidt, Laura Kuehlewein, Carina Kelbsch , et al. · 2019

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