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Electrophysiological and Structural Changes in Chinese Patients with LHON

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...

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Autores principales: Wang, Min, Guo, Hong, Li, Shiying, Wang, Gang, Long, Yanling, Meng, Xiaohong, Liu, Bo, Liu, Yong, Robson, Anthony G., Yin, Zheng Qin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7152967/
https://www.ncbi.nlm.nih.gov/pubmed/32318281
http://dx.doi.org/10.1155/2020/4734276
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author Wang, Min
Guo, Hong
Li, Shiying
Wang, Gang
Long, Yanling
Meng, Xiaohong
Liu, Bo
Liu, Yong
Robson, Anthony G.
Yin, Zheng Qin
author_facet Wang, Min
Guo, Hong
Li, Shiying
Wang, Gang
Long, Yanling
Meng, Xiaohong
Liu, Bo
Liu, Yong
Robson, Anthony G.
Yin, Zheng Qin
author_sort Wang, Min
collection PubMed
description 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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spelling pubmed-71529672020-04-21 Electrophysiological and Structural Changes in Chinese Patients with LHON Wang, Min Guo, Hong Li, Shiying Wang, Gang Long, Yanling Meng, Xiaohong Liu, Bo Liu, Yong Robson, Anthony G. Yin, Zheng Qin J Ophthalmol Research Article 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. Hindawi 2020-03-30 /pmc/articles/PMC7152967/ /pubmed/32318281 http://dx.doi.org/10.1155/2020/4734276 Text en Copyright © 2020 Min Wang et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Wang, Min
Guo, Hong
Li, Shiying
Wang, Gang
Long, Yanling
Meng, Xiaohong
Liu, Bo
Liu, Yong
Robson, Anthony G.
Yin, Zheng Qin
Electrophysiological and Structural Changes in Chinese Patients with LHON
title Electrophysiological and Structural Changes in Chinese Patients with LHON
title_full Electrophysiological and Structural Changes in Chinese Patients with LHON
title_fullStr Electrophysiological and Structural Changes in Chinese Patients with LHON
title_full_unstemmed Electrophysiological and Structural Changes in Chinese Patients with LHON
title_short Electrophysiological and Structural Changes in Chinese Patients with LHON
title_sort electrophysiological and structural changes in chinese patients with lhon
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7152967/
https://www.ncbi.nlm.nih.gov/pubmed/32318281
http://dx.doi.org/10.1155/2020/4734276
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