Abstract
The objective of this study is to investigate the characteristics and the evolution of visual field damage caused by Leber’s hereditary optic neuropathy (LHON) and to provide clinical data for the diagnosis of LHON. Parameters of visual field in 32 consecutive patients (49 eyes) with LHON who were confirmed by genetic diagnostic tests were retrospectively measured within 1 week, between three to six months, and at six months after onset. Visual field defects revealed central scotoma in 26 eyes (53.1 %), paracentral scotoma in 12 eyes (24.5 %), ceco-central defects in 6 eyes (12.2 %), blind spot enlargenment in 3 eyes (6.1 %), quadrantanopia in 2 eyes (4.1 %) within 1 week after onset. After 3 to 6 months, ceco-central defects were detected in 22 eyes (44.9 %), central isopter constriction in 10 eyes (20.4 %), hemianopia or quadrantanopia in 5 eyes (10.2 %), central scotoma in 4 eyes (8.2 %), and paracentral scotoma in 1 eye (2.0 %). After 6 months, central isopter constriction was observed in 18 eyes (36.7 %), diffuse defects in 21 eyes (42.9 %), ceco-central defects in 3 eyes (6.1 %), hemianopia or quadrantanopia in 5 eyes (10.2 %), and central scotoma in 2 eyes (4.1 %). LHON at different stages was characterized by different focal visual field defects: visual field defects in LHON patients within 1 week after onset were mostly central or paracentral scotoma, which was enlarged around the ceco-central defect, or connected to form a blind spot after 3–6 months. Diffuse and central isopter constriction defects were usually developed after 6 months. Damages firstly appeared in papillomacular bundle and gradually expanded outward. These characteristics of visual field defects reported in this study might provide a clinical basis for better diagnosis of LHON.
Highlights
Leber’s hereditary optic neuropathy (LHON) has a mitochondrial inheritance pattern
Sensitivity and stability of visual field Correlations between visual field index (VFI), mean defect (MD) and BCVA (LogMAR) were analyzed using Pearson correlation coefficient, It was found that MD was negatively correlated with BCVA (LogMAR) (r = −0.613, p = 0.011)
LHON at different stages was characterized by different focal visual field defects: visual field defects in LHON patients within 1 week after onset were mostly central or paracentral scotoma, which was enlarged around the ceco-central defect, or connected to form a blind spot after 3–6 months
Summary
Leber’s hereditary optic neuropathy (LHON) has a mitochondrial inheritance pattern. In 91–92 % of cases in mainland and Taiwan of China, the disease is associated with G11778A mitochondrial DNA (mtDNA) mutations (Lin et al 2012; Wang et al 2005). LHON typically affects young adult males who have the gene mutation presenting unilateral or bilateral subacute/acute painless loss of central vision. In the early stage of the disease, the unmyelinated retinal nerve fibre layer of optic nerve is firstly affected, especially the papillomacular bundle. With the development of the disease, the entire nerve fibre is affected and optic atrophy ensues. There is a cecocentral defect, and a larger central defect, often with a superior or inferior predilection (Wallace et al 1988; Yu-Wai-Man et al 2009)
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