In a small, pilot open-label trial, seven patients with confirmed dominant optic atrophy
(DOA) were treated with idebenone (270-1000 mg/day) for at least one year and the authors reported improved visual function in all patients when compared to baseline .
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TABLE 1 Etiologies Number of Eyes with Percentage eyes cysts Hereditary optic atrophy (mitochondrial or autosomal 40 30 75% dominant optic atrophy) Ischemic optic neuritis 6 3 50% Drusen of Optic nerve 4 2 50% Compressive OA 9 4 44.4% Inflammatory optic neuropathy 27 12 32% Undetermined origin OA 27 5 18.5% Primary open angle glaucoma 85 15 17.6% Idiopathic intracranial hypertension 2 0 0% Juxtapapillary toxoplasmic 1 0 0% TABLE 2 Optic nerve RNFL RNFL with cyst RNFL/normal P value Superior 74.28 113 P < 0.001 Inferior 71.95 115.90 P < 0.001 Nasal 52.90 65.54 P < 0.05 Temporal 35.28 56.27 P < 0.001