scholarly journals Electrophysiological and Structural Changes in Chinese Patients with LHON

2020 ◽  
Vol 2020 ◽  
pp. 1-9 ◽  
Author(s):  
Min Wang ◽  
Hong Guo ◽  
Shiying Li ◽  
Gang Wang ◽  
Yanling Long ◽  
...  
Keyword(s):  
Ganglion Cell ◽  
Optic Disc ◽  
Retinal Ganglion Cell ◽  
Structural Changes ◽  
Chinese Patients ◽  
Fundus Photography ◽  
Inner Plexiform Layer ◽  
Retinal Ganglion ◽  
Group 2 ◽  
Group 1

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.

scholarly journals Repeatability of Perimacular Ganglion Cell Complex Analysis with Spectral-Domain Optical Coherence Tomography

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Dorothy S. K. Ng ◽  
Preeti Gupta ◽  
Yih Chung Tham ◽  
Chye Fong Peck ◽  
Tien Yin Wong ◽  
...  
Keyword(s):  
Ganglion Cell ◽  
Retinal Ganglion Cell ◽  
Intraclass Correlation ◽  
Plexiform Layer ◽  
Cell Complex ◽  
Ganglion Cell Complex ◽  
Inner Plexiform Layer ◽  
Retinal Ganglion ◽  
Cell Parameters ◽  
Ganglion Cell Analysis

Purpose. To assess the repeatability of spectral-domain optical coherence tomography to measure macular and perimacular ganglion cell complex thicknesses and compare retinal ganglion cell parameters between algorithms.Methods. Ninety-two nonglaucomatous eyes from 92 participants underwent macular and perimacular ganglion cell complex thickness measurement using OCT-HS100 Glaucoma 3D algorithm and these measurements were repeated for 34 subjects. All subjects also had macular ganglion cell-inner plexiform layer thickness measured by Cirrus HD-OCT Ganglion Cell Analysis algorithm. Intraclass correlation coefficient and Pearson’s correlation analyses were performed.Results. Subfields of both macular and perimacular ganglion cell complex thicknesses had high intraclass correlation coefficient values between 0.979 (95% confidence interval [CI]: 0.958–0.989) and 0.981 (95% CI: 0.963, 0.991) and between 0.70 (95% CI: 0.481–0.838) and 0.987 (95% CI: 0.956–0.989), respectively. The overall average ganglion cell complex and macular average ganglion cell-inner plexiform layer thicknesses were strongly correlated(r=0.83, P<0.001).  Conclusions. The assessment of macular and perimacular retinal ganglion cell parameters by OCT-HS100 Glaucoma 3D algorithm is highly repeatable, and strongly correlates to retinal ganglion cell parameters assessed by Ganglion Cell Analysis algorithm. A comprehensive evaluation of retinal ganglion cells may be possible with OCT-HS100.

Retinal ganglion cell layer and visual function in a patient with optic disc drusen

1998 ◽  
Vol 236 (12) ◽  
pp. 904-915 ◽  
Author(s):  
M.-M. Gellrich ◽  
Stephan Neumaier ◽  
Claudia Auw-Hädrich ◽  
Nils-Claudius Gellrich ◽  
Lutz Lothar Hansen
Keyword(s):  
Ganglion Cell ◽  
Optic Disc ◽  
Retinal Ganglion Cell ◽  
Visual Function ◽  
Ganglion Cell Layer ◽  
Cell Layer ◽  
Retinal Ganglion ◽  
Optic Disc Drusen ◽  
Retinal Ganglion Cell Layer

scholarly journals Active MS is associated with accelerated retinal ganglion cell/inner plexiform layer thinning

Neurology ◽  
2012 ◽  
Vol 80 (1) ◽  
pp. 47-54 ◽  
Author(s):  
J. N. Ratchford ◽  
S. Saidha ◽  
E. S. Sotirchos ◽  
J. A. Oh ◽  
M. A. Seigo ◽  
...  
Keyword(s):  
Ganglion Cell ◽  
Retinal Ganglion Cell ◽  
Plexiform Layer ◽  
Inner Plexiform Layer ◽  
Retinal Ganglion

scholarly journals Interferon-β Is Less Effective Than Other Drugs in Controlling the Rate of Retinal Ganglion Cell Loss in MS

2021 ◽  
Vol 8 (3) ◽  
pp. e971
Author(s):  
Yuyi You ◽  
Michael H. Barnett ◽  
Con Yiannikas ◽  
John D.E. Parratt ◽  
Jim G. Matthews ◽  
...  
Keyword(s):  
Ganglion Cell ◽  
Nerve Fiber ◽  
Glatiramer Acetate ◽  
Retinal Ganglion Cell ◽  
Brain Mri ◽  
Cell Loss ◽  
Lesion Volume ◽  
Inner Plexiform Layer ◽  
Retinal Ganglion ◽  
Interferon Β

ObjectiveTo investigate the association between disease-modifying therapies (DMTs) and the rate of progressive retinal ganglion cell (RGC) and nerve fiber loss in MS.MethodsOne hundred five relapsing-remitting patients with MS were followed annually for a median of 4.0 years using optical coherence tomography. Twenty-five healthy subjects were also included as normal controls. The rates of global peripapillary retinal nerve fiber layer (pRNFL), temporal RNFL (tRNFL), and ganglion cell inner plexiform layer (GCIPL) thinning were analyzed according to DMT type using a linear mixed-effects model. Optic radiation lesion volume was measured on brain MRI and included as a covariate to minimize the effects of retrograde transsynaptic degeneration.ResultsThe annual rates of RNFL and GCIPL thinning were higher in patients treated with “platform” therapies (interferon-β and glatiramer acetate) compared with DMTs of higher clinical efficacy (including fingolimod, dimethyl fumarate, natalizumab, alemtuzumab, rituximab, and ocrelizumab) (difference = −0.22 μm/y, p = 0.02 for pRNFL; difference = −0.34 μm/y, p = 0.009 for tRNFL; and difference = −0.16 μm/y, p = 0.005 for GCIPL). Based on an analysis of individual treatments (interferon-β, glatiramer acetate, fingolimod, and natalizumab), interferon-β was associated with inferior RGC preservation, relative to the other drugs. No effect difference was found between glatiramer acetate, fingolimod, and natalizumab.ConclusionsProgressive loss of RGCs in patients with MS is more pronounced in patients treated with interferon-β than other DMTs. This finding may have implications for DMT selection in MS.Classification of EvidenceThis study provides Class IV evidence that for patients with MS, treatment with interferon-β compared with other DMTs leads to a more pronounced rate of retinal ganglion cell loss.

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