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Leber's hereditary optic neuropathy can cause rapid deterioration of vision
Invest Ophthalmol Vis Sci. 2009;50(7):3112-3115.
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Leber's hereditary optic neuropathy can cause significant vision loss, perhaps worse compared with other ophthalmic disorders, and may be typified by rapid onset of vision loss and a poor long-term prognosis.
According to a survey of patients with Leber's congenital optic neuropathy, 74.5% of affected patients were men. Mean age at onset was 27.9 years, with an average disease duration of 15.5 years at the time of the survey.
The survey, which evaluated 196 affected individuals and 206 genetic carriers unaffected by the rare condition, found significantly lower scores on the VF-14, a visual function test that asks patients to self-report their ability to perform 14 vision-dependent activities in daily life. Subjects are graded from 0 (unable to do) to 4 (no difficulty).
Affected patients scored 25.1, which was significantly lower than the 97.3 reported by unaffected subjects. Patients with the m.14484T>C mutation had significantly higher scores on the VF-14 than patients with either subtype m.3460G>A or m.1178G>A.
However, according to the study, there was no correlation between disease duration and VF-14 score. In fact, visual impact was notable within 1 year of disease onset. Among 21 affected individuals with onset within the prior year, the mean VF-14 score was 23.4 compared with 25.3 among individuals with a duration longer than 1 year.
"Reading small print and reading a newspaper or book were the two activities of daily living that cause the greatest subjective difficulty," according to the survey.