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Hypertrophic neuropathy with spinal cord compression

L Carlin, J Biller, V Challa

    Surgical Neurology
    |October 1, 1982
    PubMed
    Summary
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    Dejerine-Sottas disease can cause spinal cord compression due to enlarged nerve roots. This case highlights characteristic myelographic changes associated with this rare neurological disorder.

    Area of Science:

    • Neurology
    • Neurosurgery
    • Medical Imaging

    Background:

    • Dejerine-Sottas disease is a rare, inherited demyelinating neuropathy.
    • Spinal cord compression is an uncommon but serious complication.
    • Hypertrophic nerve roots are a hallmark of the disease.

    Observation:

    • A 40-year-old patient presented with symptoms of spinal cord compression.
    • The compression was found to be caused by significantly enlarged (hypertrophic) nerve roots.
    • Review of six prior cases of Dejerine-Sottas disease with similar complications.

    Findings:

    • The presented case illustrates spinal cord compression secondary to hypertrophic nerve roots in Dejerine-Sottas disease.
    • Characteristic myelographic findings in Dejerine-Sottas disease leading to compression were identified.

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  • Analysis of previous literature supports the occurrence of this complication.

    • Implications:

    • Understanding these myelographic changes is crucial for diagnosing spinal cord compression in Dejerine-Sottas disease.
    • Early recognition and management of spinal cord compression may improve patient outcomes.
    • This case contributes to the limited literature on neurological complications of Dejerine-Sottas disease.