Retinal Ganglion Cell and Inner Plexiform Layer Loss Correlate with Visual Acuity Loss in LHON: A Longitudinal, Segmentation OCT Analysis

Moster, Stephen J; Moster, Mark L.; Bryan, Molly Scannell; Sergott, Robert C.

doi:10.1167/iovs.15-17328

Cited by 38 publications

(18 citation statements)

References 19 publications

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“…Central field defects and blind spot enlargement were common findings, in keeping with previous investigations [18]. e current study showed that that the thickness of the RNFL around the optic disc and surrounding the macula did not reduce in the acute stage but reduced significantly in the chronic stage of the disease, corroborating previous reports [19,20]. e pattern ERG N95 and approximately 70% of P50 originate in the retinal ganglion cells.…”

Section: Discussionsupporting

confidence: 92%

Electrophysiological and Structural Changes in Chinese Patients with LHON

Wang

Guo

et al. 2020

Journal of Ophthalmology

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Objective. To review retrospectively the electrophysiological and structural changes in 13 Chinese patients with Leber hereditary optic neuropathy (LHON). Methods. 26 eyes of 13 patients with a genetically confirmed diagnosis of LHON were categorized into two groups according to the duration of the disease: group 1 (duration less than 3 months) and group 2 (duration between 3 months and 18 years). Clinical history, comprehensive visual electrophysiology, optical coherence tomography (OCT), and color fundus photography were performed. Results. Fundoscopy showed optic disc hyperemia in group 1 and optic atrophy in group 2. OCT measures of retinal nerve fiber layer (RNFL) thickness around the optic disc and surrounding macula were normal in group 1 but reduced in group 2 (10 of 10 eyes). The thickness of the retinal ganglion cell layer (GCL) plus inner plexiform layer (IPL) surrounding the macula reduced significantly in group 1 and group 2 compared with a healthy control group. Pattern ERG (PERG) P50 amplitude was normal, but the N95/P50 ratio reduced in most of group 1 (4 of 5 eyes) and in all of group 2 (11 eyes). PERG P50 peak time was abnormally short in group 2. Multifocal electroretinography (mfERG) showed subnormal responses associated with ring 1 (the central area) and ring 2 in group 1 and reductions in rings 1, 2, and 3 in group 2. Conclusion. The study highlights differences in retinal structure and function between the acute and chronic stages of LHON in a group of Chinese patients. There is PERG evidence of retinal ganglion cell dysfunction and OCT evidence of GCL + IPL thinning in both groups, but there is additional peripapillary RNFL loss in the chronic stage, associated with more severe RGC dysfunction. There is multifocal ERG evidence of localized macular dysfunction in both acute and chronic groups. The study highlights the importance of comprehensive electrophysiological and structural assessments of the retina in LHON and is pertinent to studies that aim to monitor disease progression or the effects of future therapeutic interventions.

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Section: Discussionsupporting

confidence: 92%

Electrophysiological and Structural Changes in Chinese Patients with LHON

Wang

Guo

et al. 2020

Journal of Ophthalmology

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“…Some studies previously reported a relationship between visual acuity and GCIPL thickness that was related to the pathophysiology of each optic neuropathy. Kim et al 16 demonstrated that thinning of the superior and inferior GCIPL was significantly correlated with visual acuity in severe glaucoma, and Moster et al 17 reported that a decrease in GCIPL thickness was associated with worsening vision in the acute phase of LHON. 17 In addition to those optic neuropathies, we found a significant effect of GCIPL thickness on the potential for visual recovery in patients with EON.…”

Section: Discussionmentioning

confidence: 99%

“…Kim et al 16 demonstrated that thinning of the superior and inferior GCIPL was significantly correlated with visual acuity in severe glaucoma, and Moster et al 17 reported that a decrease in GCIPL thickness was associated with worsening vision in the acute phase of LHON. 17 In addition to those optic neuropathies, we found a significant effect of GCIPL thickness on the potential for visual recovery in patients with EON. In our results, as the thickness of the remaining GCIPL increased and the damage to the GCIPL decreased during 6 months of follow-up, the possibility of visual acuity recovery before permanent retinal ganglion cell death increased.…”

Section: Discussionmentioning

confidence: 99%

Ganglion Cell Layer and Inner Plexiform Layer as Predictors of Vision Recovery in Ethambutol-Induced Optic Neuropathy: A Longitudinal OCT Analysis

Lee

Choi

Park

et al. 2018

Invest. Ophthalmol. Vis. Sci.

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“…Other OoC devices with higher degrees of complexity and multi-compartmental organization, such as brain tissues (e.g., blood-brain-barrier (BBB) chips) and the human eye (eye-on-a-chip) [ 88 , 89 ], exhibit design challenges beyond what is shown here for the HoC. For example, to recapitulate the membrane structure of blood capillaries in the brain or to recapitulate the functions of the multi-layer cellular assemblies of the cornea [ 29 ] and retina [ 90 ] or the blinking function of the eyelid [ 91 ] requires elaborate design efforts. Especially, the integration with the surrounding biopolymers (ECMs and membranes) forming scaffolds for realizing the 3D architecture of the organ then becomes an essential function in the design.…”

Section: Discussionmentioning

confidence: 99%

Conceptual Design of Micro-Bioreactors and Organ-on-Chips for Studies of Cell Cultures

Mandenius

2018

Bioengineering

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Engineering design of microbioreactors (MBRs) and organ-on-chip (OoC) devices can take advantage of established design science theory, in which systematic evaluation of functional concepts and user requirements are analyzed. This is commonly referred to as a conceptual design. This review article compares how common conceptual design principles are applicable to MBR and OoC devices. The complexity of this design, which is exemplified by MBRs for scaled-down cell cultures in bioprocess development and drug testing in OoCs for heart and eye, is discussed and compared with previous design solutions of MBRs and OoCs, from the perspective of how similarities in understanding design from functionality and user purpose perspectives can more efficiently be exploited. The review can serve as a guideline and help the future design of MBR and OoC devices for cell culture studies.

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Retinal Ganglion Cell and Inner Plexiform Layer Loss Correlate with Visual Acuity Loss in LHON: A Longitudinal, Segmentation OCT Analysis

Cited by 38 publications

References 19 publications

Electrophysiological and Structural Changes in Chinese Patients with LHON

Electrophysiological and Structural Changes in Chinese Patients with LHON

Ganglion Cell Layer and Inner Plexiform Layer as Predictors of Vision Recovery in Ethambutol-Induced Optic Neuropathy: A Longitudinal OCT Analysis

Conceptual Design of Micro-Bioreactors and Organ-on-Chips for Studies of Cell Cultures

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