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Visually evoked potentials in Friedreich's ataxia

W Wenzel, L Camacho, D Claus

    Advances in Neurology
    |January 1, 1982
    PubMed
    Summary

    Visual evoked potentials (VEP) reveal optic nerve damage in 55% of Friedreich

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    Area of Science:

    • Neuroscience
    • Ophthalmology
    • Genetics

    Background:

    • Friedreich's ataxia is a rare inherited disease that causes progressive nervous system damage.
    • Optic nerve involvement is a known complication, but its prevalence is debated.

    Purpose of the Study:

    • To investigate the incidence of optic nerve lesions in Friedreich's ataxia using visual evoked potentials (VEP).
    • To assess the utility of VEP in diagnosing and monitoring optic nerve involvement in this patient population.

    Main Methods:

    • Pattern-reversal visual evoked potentials (VEP) were recorded in 45 patients diagnosed with Friedreich's ataxia.
    • VEP results were analyzed for latency abnormalities and compared with clinical data (visual acuity, age, disease duration).

    Main Results:

    • Pathological VEP results, indicative of optic nerve dysfunction, were found in 25 patients (55%).
    • Six patients (13%) had no obtainable response due to severe optic atrophy.
    • No significant correlation was found between VEP abnormalities and visual acuity, age, or disease duration.

    Conclusions:

    • Optic nerve lesions are more common in Friedreich's ataxia than previously assumed.
    • VEP is a sensitive tool for detecting subclinical optic nerve damage in Friedreich's ataxia.
    • VEP cannot differentiate Friedreich's ataxia from multiple sclerosis and is not helpful in distinguishing between the two.

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