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Peripheral nerve involvement in Batten-Spielmeyer-Vogt's disease.

B B Lyon

    Journal of Neurology, Neurosurgery, and Psychiatry
    |February 1, 1975
    PubMed
    Summary

    Peripheral nerve conduction is impaired in Batten-Spielmeyer-Vogt's disease. Electromyography revealed slowed sensory nerve conduction velocity in patients with this rare genetic neurological disorder.

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    Area of Science:

    • Neurology
    • Neurophysiology
    • Genetics

    Background:

    • Batten-Spielmeyer-Vogt's disease, also known as juvenile amaurotic idiocy or cerebral ceroid lipofuscinosis, is a rare inherited neurodegenerative disorder.
    • The disease primarily affects the central nervous system, leading to progressive vision loss and cognitive decline.

    Purpose of the Study:

    • To investigate peripheral nerve function in patients with Batten-Spielmeyer-Vogt's disease.
    • To assess electrophysiological parameters, specifically nerve conduction velocities, in affected individuals.

    Main Methods:

    • Electromyography (EMG) and sensory and motor nerve conduction studies were performed.
    • Nerve conduction velocities were measured in both median and sural nerves.

    Main Results:

    • A slight to moderate slowing of sensory conduction velocity was observed in the median and sural nerves.
    • This slowing was more pronounced in the distal nerve segments compared to proximal segments.

    Conclusions:

    • The electrophysiological findings suggest impaired peripheral nerve transmission in Batten-Spielmeyer-Vogt's disease.
    • These results contribute to understanding the broader neurological impact of this condition beyond the central nervous system.

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