Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China

Purpose. To evaluate the clinical classification and characteristics of hereditary optic neuropathy patients in a single center in China. Method. Retrospective case study. Patients diagnosed with hereditary optic neuropathy between January 2014 and December 2015 in the neuro-ophthalmology division i...

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Main Authors: Dekang Gan, Mengwei Li, Jihong Wu, Xinghuai Sun, Guohong Tian
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Journal of Ophthalmology
Online Access:http://dx.doi.org/10.1155/2017/6186052
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author Dekang Gan
Mengwei Li
Jihong Wu
Xinghuai Sun
Guohong Tian
author_facet Dekang Gan
Mengwei Li
Jihong Wu
Xinghuai Sun
Guohong Tian
author_sort Dekang Gan
collection DOAJ
description Purpose. To evaluate the clinical classification and characteristics of hereditary optic neuropathy patients in a single center in China. Method. Retrospective case study. Patients diagnosed with hereditary optic neuropathy between January 2014 and December 2015 in the neuro-ophthalmology division in Shanghai Eye and ENT Hospital of Fudan University were recruited. Clinical features as well as visual field, brain/orbital MRI, and spectrum domain optical coherence tomography (SD-OCT) were analyzed. Results. Eighty-two patients diagnosed by gene test were evaluated, including 66 males and 16 females. The mean age of the patients was 19.4 years (range, 5–46 years). A total of 158 eyes were analyzed, including 6 unilateral, 61 bilateral, and 15 sequential. The median duration of the disease was 0.5 year (range, 0.1–20 years). Genetic test identified 68 patients with Leber hereditary optic neuropathy, 9 with dominant optic neuropathy, and 2 with a Wolfram gene mutation. There was also one case of hereditary spastic paraplegia, spinocerebellar ataxia, and polymicrogyria with optic nerve atrophy, respectively. Conclusion. Leber hereditary optic neuropathy is the most common detected type of hereditary optic neuropathy in Shanghai, China. The detection of other autosomal mutations in hereditary optic neuropathy is limited by the currently available technique.
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series Journal of Ophthalmology
spelling doaj-art-3d0d0a236a3c4f12b4df03343884ddb22025-08-20T03:55:33ZengWileyJournal of Ophthalmology2090-004X2090-00582017-01-01201710.1155/2017/61860526186052Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, ChinaDekang Gan0Mengwei Li1Jihong Wu2Xinghuai Sun3Guohong Tian4Department of Ophthalmology, Eye, Ear, Nose and Throat Hospital of Fudan University, Shanghai, ChinaDepartment of Ophthalmology, Eye, Ear, Nose and Throat Hospital of Fudan University, Shanghai, ChinaDepartment of Ophthalmology, Eye, Ear, Nose and Throat Hospital of Fudan University, Shanghai, ChinaDepartment of Ophthalmology, Eye, Ear, Nose and Throat Hospital of Fudan University, Shanghai, ChinaDepartment of Ophthalmology, Eye, Ear, Nose and Throat Hospital of Fudan University, Shanghai, ChinaPurpose. To evaluate the clinical classification and characteristics of hereditary optic neuropathy patients in a single center in China. Method. Retrospective case study. Patients diagnosed with hereditary optic neuropathy between January 2014 and December 2015 in the neuro-ophthalmology division in Shanghai Eye and ENT Hospital of Fudan University were recruited. Clinical features as well as visual field, brain/orbital MRI, and spectrum domain optical coherence tomography (SD-OCT) were analyzed. Results. Eighty-two patients diagnosed by gene test were evaluated, including 66 males and 16 females. The mean age of the patients was 19.4 years (range, 5–46 years). A total of 158 eyes were analyzed, including 6 unilateral, 61 bilateral, and 15 sequential. The median duration of the disease was 0.5 year (range, 0.1–20 years). Genetic test identified 68 patients with Leber hereditary optic neuropathy, 9 with dominant optic neuropathy, and 2 with a Wolfram gene mutation. There was also one case of hereditary spastic paraplegia, spinocerebellar ataxia, and polymicrogyria with optic nerve atrophy, respectively. Conclusion. Leber hereditary optic neuropathy is the most common detected type of hereditary optic neuropathy in Shanghai, China. The detection of other autosomal mutations in hereditary optic neuropathy is limited by the currently available technique.http://dx.doi.org/10.1155/2017/6186052
spellingShingle Dekang Gan
Mengwei Li
Jihong Wu
Xinghuai Sun
Guohong Tian
Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China
Journal of Ophthalmology
title Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China
title_full Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China
title_fullStr Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China
title_full_unstemmed Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China
title_short Analysis of Genetic Mutations in a Cohort of Hereditary Optic Neuropathy in Shanghai, China
title_sort analysis of genetic mutations in a cohort of hereditary optic neuropathy in shanghai china
url http://dx.doi.org/10.1155/2017/6186052
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