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Related Experiment Videos

The early phase in Leber hereditary optic atrophy

E Nikoskelainen, R L Sogg, A R Rosenthal

    Archives of Ophthalmology (Chicago, Ill. : 1960)
    |June 1, 1977
    PubMed
    Summary

    Early signs of Leber hereditary optic atrophy (LHON) appear in the retinal nerve fiber layer before vision loss. This optic nerve dysfunction progresses despite treatments, indicating limited therapeutic options for LHON.

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    Area of Science:

    • Ophthalmology
    • Neuroscience
    • Genetics

    Background:

    • Leber hereditary optic atrophy (LHON) is a maternally inherited mitochondrial disease.
    • It primarily affects the optic nerve, leading to vision loss.

    Observation:

    • Clinical, ophthalmoscopic, perimetric, and color vision tests were performed.
    • Visual evoked responses were recorded in symptomatic and asymptomatic family members.
    • Photographic documentation captured retinal nerve fiber degeneration.

    Findings:

    • Presymptomatic eyes showed retinal nerve fiber layer abnormalities.
    • Optic nerve dysfunction preceded central vision failure in LHON patients.
    • Degeneration of retinal nerve fibers, especially in the papillomacular bundle, was observed post-symptom onset.

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    Implications:

    • Early detection of LHON is possible through retinal nerve fiber layer analysis.
    • The findings highlight the progressive nature of optic nerve damage in LHON.
    • Current treatments like prednisone and hydroxocobalamin were ineffective in halting vision impairment.