Visual rehabilitation training in a patient with large central scotoma due to Leber’s hereditary optic neuropathy

Abstract

Background. Leber’s hereditary optic neuropathy (LHON) is a disease associated with pathogenetic mutations of mitochondrial DNA which affects predominantly young adult males and leads to loss of central vision, low visual acuity and unstable fixation. The purpose of this study was to allow the patient to establish the best possible fixation in the best area of retinal sensitivity in LHON patient with central scotoma. Methods. A 17 years old patient with confirmed mitochondrial LHON 11778G > A mutation was included in the study. The patient underwent 16 visual rehabilitation sessions — two for each of 8 weeks using the training module available in the equipment — MAIA microperimeter. Visual acuity and standrad microperimetry examination were performed before and 8 weeks period of training. To measure the fixation stability with MAIA microperimeter, P1, P2 and Bivariate Contour Ellipse Area (BCEA) parameters were used. Results. Visual acuity of the trained better eye was 0.08 at the baseline versus 0.063 after 8 weeks training. Fixation stability parameter was P1-21%, P2-64% at the first session versus P1-60%, P2-90% at the last session. BCEA values 29.2°˛ before and 14.2°˛ after training, respectively. Conclusions. Visual training via microperimetry could potentially be a method that improves the fixation stability in patients with LHON.