ascorbic-acid and Optic-Atrophy--Hereditary--Leber

Patients with Leber hereditary optic neuropathy (LHON) have a progressive decrease of their visual acuity which can deteriorate to <0.1. Some patients can have a partial recovery of their vision in one or both eyes. One prognostic factor associated with a recovery of vision is an early-age onset. The purpose of this study was to determine other clinical factors that are predictive of a good visual recovery.. Sixty-one Japanese LHON patients, with the 11,778 mutation and a mean age of 23.1 ± 12.1 years at the onset, were studied. All patients were initially examined at an acute stage of LHON and were followed for 3 to 10 years. At 1 year after the onset, the lowest visual acuity was <0.1 in all eyes. We studied the following parameters of patients with/without a final visual acuity of ≥ 0.2: sex; heavy consumption of cigarettes and alcohol; taking idebenone; mean age at onset; mean lowest visual acuity; and distribution of the lowest and the final visual acuity.. Fifteen (24.6%) of the 61 patients or 25 (20.5%) of the 122 eyes had a recovery of their visual acuity to ≥ 0.2. The mean age at onset of these 15 patients with visual recovery to ≥ 0.2 was 17.5 ± 7.7 years, and that of the 46 patients without visual recovery to ≥ 0.2 was 25.0 ± 12.8 years (P = 0.02, Mann-Whitney U test). The mean lowest visual acuity of the 25 eyes with visual recovery ≥ 0.2 was 0.04, and that of the 97 eyes without visual recovery to ≥ 0.2 was 0.015 (P < 0.001, Mann-Whitney U test). Fifty percent (15/30) of the eyes whose lowest visual acuity was ≥ 0.04 during 1 year after the onset had a visual recovery to ≥ 0.2, while 11% (10/92) of the eyes whose the lowest visual acuity was ≤ 0.03 had a visual recovery to ≥ 0.2 (P < 0.001, χ. A final visual acuity of ≥ 0.2 was associated with a less severe reduction of the visual acuity at 1 year after the onset. Our findings can be used to predict the visual prognosis in LHON patients.

Topics: Adolescent; Adult; Age of Onset; Aged; Antioxidants; Ascorbic Acid; Child; DNA Mutational Analysis; DNA, Mitochondrial; Female; Follow-Up Studies; Humans; Male; Middle Aged; Optic Atrophy, Hereditary, Leber; Point Mutation; Polymerase Chain Reaction; Prognosis; Recovery of Function; Retrospective Studies; Riboflavin; Ubiquinone; Vision Disorders; Visual Acuity; Visual Field Tests; Vitamin B Complex; Young Adult

To ascertain the efficacy of idebenone and multivitamin treatment in Leber's hereditary optic neuropathy (LHON).. Two patients diagnosed of unilateral LHON were treated with megadoses of idebenone, vitamin C and riboflavin for one year. They were examined clinically before, during and after treatment.. No improvement of visual function was observed. Despite the idebenone treatment, in both cases the second eye became involved.. Despite previous reports of visual recovery with idebenone in patients with LHON, our experience shows that an effective treatment for Leber's disease remains to be found.

Topics: Adult; Antioxidants; Ascorbic Acid; Benzoquinones; Female; Humans; Male; Optic Atrophy, Hereditary, Leber; Riboflavin; Treatment Failure; Ubiquinone; Vitamin B Complex; Vitamins