Purchase this article with an account.
Norihiro Yamada, Shoji Kishi; Attenuation Of Nerve Fiber And Ganglion Cell Layer In The Macular Area In Leber’S Hereditary Optic Neuropathy. Invest. Ophthalmol. Vis. Sci. 2012;53(14):806.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
Leber’s hereditary optic neuropathy (LHON) is caused by a point mutation of the mitochondrial gene, whose primary lesion is considered to be retinal ganglion cells. We report four cases of LHON whose nerve fiber layer and ganglion cell in the macular area is attenuated.
Case 1: A 33-year-old man presented decreased vision and central scotoma. At first visit, his best corrected visual acuity (BCVA) was 0.8 in right and 1.2 in left eye with bilateral central scotoma. His BCVA progressively declined and became 0.15 in right and 0.08 in left eye 2 months after the initial visit. LHON was diagnosed by the G11778A point mutation of mitochondrial gene. On Spectral domain optical coherence tomography (SD-OCT), nerve fiver layer and ganglion cell layer was attenuated, which resulted in swallowing of foveal pit. Case 2: A 17-year-old boy presented decreased vision with central scotoma in his left eye. Goldmann perimeter showed central scotoma and enlargement of blind spot in his left eye. His BCVA was 1.2 in right and 0.05 in left eye at first visit. He has a family history of LHON. His BCVA declined to 0.2 and 0.08 one month after. Mitochondrial gene showed the G11778A point mutation. SD-OCT showed attenuation of nerve fiber layer and ganglion cell layer. Case 3: A 14-year-old boy was treated as bilateral optic neuritis with steroid pulse. At his first visit, BCVA was 0.08 in right and 0.04 in left eye. He had a point mutation of G11778A. SD-OCT revealed thinning of nerve fiber layer and ganglion cell layer in the macular area. Case 4: A 62-year-old man was treated as right optic neuritis with steroid pulse. His BCVA declined to 0.06 and 0.07 11 months after. Mitochondrial gene showed the G11778A point mutation. SD-OCT showed attenuation of nerve fiber layer and ganglion cell layer.
All 4 cases of LHON showed thinning of nerve fiber layer and ganglion cell layer in a 6 mm scan of macular area in SD-OCT.
The OCT finding correlated with a previously reported pathological report of LHON.
This PDF is available to Subscribers Only