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...
Saved in:
| Main Authors: | , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2020-01-01
|
| Series: | Journal of Ophthalmology |
| Online Access: | http://dx.doi.org/10.1155/2020/4734276 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832560884907507712 |
|---|---|
| author | Min Wang Hong Guo Shiying Li Gang Wang Yanling Long Xiaohong Meng Bo Liu Yong Liu Anthony G. Robson Zheng Qin Yin |
| author_facet | Min Wang Hong Guo Shiying Li Gang Wang Yanling Long Xiaohong Meng Bo Liu Yong Liu Anthony G. Robson Zheng Qin Yin |
| author_sort | Min Wang |
| collection | DOAJ |
| 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. |
| format | Article |
| id | doaj-art-9bf9febc4e504b089766460154053a88 |
| institution | Kabale University |
| issn | 2090-004X 2090-0058 |
| language | English |
| publishDate | 2020-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of Ophthalmology |
| spelling | doaj-art-9bf9febc4e504b089766460154053a882025-02-03T01:26:25ZengWileyJournal of Ophthalmology2090-004X2090-00582020-01-01202010.1155/2020/47342764734276Electrophysiological and Structural Changes in Chinese Patients with LHONMin Wang0Hong Guo1Shiying Li2Gang Wang3Yanling Long4Xiaohong Meng5Bo Liu6Yong Liu7Anthony G. Robson8Zheng Qin Yin9Southwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Military Medical University), Chongqing, ChinaDepartment of Medical Genetics, Third Military Medical University (Army Military Medical University), Chongqing, ChinaSouthwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Military Medical University), Chongqing, ChinaSouthwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Military Medical University), Chongqing, ChinaSouthwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Military Medical University), Chongqing, ChinaSouthwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Military Medical University), Chongqing, ChinaSouthwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Military Medical University), Chongqing, ChinaSouthwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Military Medical University), Chongqing, ChinaDepartment of Electrophysiology, Moorfields Eye Hospital, London, UKSouthwest Hospital/Southwest Eye Hospital, Third Military Medical University (Army Military Medical University), Chongqing, ChinaObjective. 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.http://dx.doi.org/10.1155/2020/4734276 |
| spellingShingle | Min Wang Hong Guo Shiying Li Gang Wang Yanling Long Xiaohong Meng Bo Liu Yong Liu Anthony G. Robson Zheng Qin Yin Electrophysiological and Structural Changes in Chinese Patients with LHON Journal of Ophthalmology |
| 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 |
| url | http://dx.doi.org/10.1155/2020/4734276 |
| work_keys_str_mv | AT minwang electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT hongguo electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT shiyingli electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT gangwang electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT yanlinglong electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT xiaohongmeng electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT boliu electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT yongliu electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT anthonygrobson electrophysiologicalandstructuralchangesinchinesepatientswithlhon AT zhengqinyin electrophysiologicalandstructuralchangesinchinesepatientswithlhon |