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Dejerine-Sottas disease revisited

R Satran

    Archives of Neurology
    |February 1, 1980
    PubMed
    Summary

    Dejerine-Sottas disease, a rare inherited hypertrophic neuropathy, was first described over a century ago. This review revisits early pathologic findings in light of contemporary research.

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    Area of Science:

    • Neurology
    • Pathology
    • Genetics

    Background:

    • Dejerine-Sottas disease (DDS) is a rare, severe, inherited hypertrophic neuropathy.
    • Early clinical and pathologic descriptions of DDS date to the late 19th and early 20th centuries.

    Purpose of the Study:

    • To recall and review the foundational pathologic observations of DDS.
    • To contextualize historical findings within current scientific understanding of hypertrophic neuropathies.

    Main Methods:

    • Review of seminal historical publications on Dejerine-Sottas disease.
    • Comparative analysis of early pathologic descriptions with recent research findings.

    Main Results:

    • Early studies identified characteristic nerve hypertrophy in infants with DDS.
    • Historical observations on nerve structure and function remain relevant to understanding DDS.

    Conclusions:

    • The fundamental pathology of Dejerine-Sottas disease was accurately described in early reports.
    • Revisiting historical data provides valuable insights for ongoing research into hypertrophic neuropathies.

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