30岁以后发病的Leber遗传性视神经病变临床观察

来源 :2014年中国眼底病论坛暨全国眼底病专题学术研讨会 | 被引量 : 0次 | 上传用户:bingdongfenxing
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  Objective Sum up the clinical features of Leber hereditary optic neuropathy(LHON)patients over 30 years old.Methods Retrospectively studied Clinical data of 9 LHON patients over 30 years old who were all male,aged from 34 to 56(average age 45.22 ± 6.91)years old.Results of mtDNA test,medical history,family history,visual acuity,visual field,fundus image,other clinical exams,and the follow-up information are collected.Results 77.78%(7/9)of the patients have family history..7(77.78%)of the LHON patients had mutation G11778A,1(11.11%)had mutation G11696A,and 1(11.11%)had mutation T14484C.4 patients had both eyes involed successively,the interval was berween 15 days to 9 months.5 of the patients receiced corticosteroid shock treatment.11 eyes had visual acuity lower than 0.1(61.11%),3 between 0.12 and 0.3(16.67%),4 better than 0.4(22.22%).The duration of disease was between 7 days to 21 months,the median duration of 6 months.12 eyes had a course less than 6 months,7 of which had normal optic disc,3 eyes had blurred optic disc border and telangiectasia,and 2 eyes had pale optic.6 eyes had a course more than 6 months,5 eyes had pale optic disc and 1 had normal optic disc.After a follow-up of 12 months,visual acuity of 9 eyes improved and 2 decreased.Others were stable.Conclusion Elderly onset LHON patients were more common in men,most patients had mutation G11778A,We should pay attention to the diagnosis of optic nerve in patients with genetic testing in middle age.
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