Clinical Characteristics of m.11778G>A Leber Hereditary Optic Neuropathy with Favorable Outcomes.

The prognosis of 11,778 mitochondrial mutations in Leber hereditary optic neuropathy (LHON) is poor. Patients with favorable outcomes (visual acuity better than 20/100) who could be observed for more than 6 months were analyzed. Among 74 patients (57 male, 17 female), 6 (8.1%) showed improvement in visual acuity of 20/100 or higher. The patients with favorable outcomes have better visual acuity at nadir (logMAR 0.98 ± 0.69 in the favorable patients and logMAR 2.32 ± 0.93 in the unfavorable patients, p = .003). Among the favorable group, four patients (36, 32, 19, and 7 years of age at onset) took idebenone within 6 months of onset. However, fifty-one percent of the patients with unfavorable outcomes took idebenone (p = .008). Although the age at onset in the favorable patients is relatively younger than that of the unfavorable patients (20.3 ± 10.8 versus 28.8 ± 12.8 years), a significant difference was not found (p = .138). In conclusion, better visual acuity in nadir and administration of idebenone may affect vision recovery.

Evaluation of angle-to-angle and spur-to-spur using swept source optical coherence tomography in different refractive error.

PURPOSE: To measure angle-to-angle (ATA) and spur-to-spur (STS) distances along six meridians using swept-source optical coherence tomography (SS-OCT) and compare with horizontal white-to-white (WTW) distance in different refractive error. METHODS: Overall, 126 eyes were assessed with the Anterion SS-OCT (Heidelberg Engineering, Heidelberg, Germany). ATA and STS distances were obtained using SS-OCT at 0, 30, 60, 90, 120, and 150 degrees. WTW was measured at 0 degree with built-in infrared camera. One way ANOVA test, pearson correlation coefficient, and stepwise multivariate regression analysis were used to compare ATA and STS distances with age, anterior chamber depth (ACD), axial length (AL), and simulated keratometric values (Sim K) in different refractive error groups. RESULTS: The mean MRSE refraction was +0.05 ± 0.23 D in the emmetropic group (41 eyes), -3.42 ± 3.04 D in the myopic group (44 eyes), and +1.33 ± 0.64 D in the hyperopic group (31 eyes). There was no statistical difference in the WTW of the emmetropic (11.62 ± 0.44 mm), myopic (11.79 ± 0.46 mm), and hyperopic groups (11.80 ± 0.49 mm) using one-way ANOVA (p = 0.007). ATA and STS were vertically oval in all groups. The correlation between ATA, STS and age, ACD, AL, and K values showed different significance for each meridian according to the refractive error. ATA increased as the horizontal WTW, ACD, and AL increased and Sim K decreased. STS shows relatively smaller explanatory power than ATA in the stepwise multivariate regression analysis. CONCLUSIONS: This study is the first to analyze the relationship between ATA and STS compared to WTW by different refractive error. The difference between the horizontally oval WTW and vertically oval anterior chamber can be large, especially in myopia. ATA showed a greater positive correlation than STS with AL and ACD.