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Related Experiment Videos

Multiple exercise-related mononeuropathy with abdominal colic.

U Trockel, J M Schröder, K H Reiners

    Journal of the Neurological Sciences
    |August 1, 1983
    PubMed
    Summary

    Hereditary neuropathy with liability to pressure palsies (NLPP) presents with recurrent nerve damage from minor pressure. This variant causes painful palsies and abdominal colic, possibly due to a metabolic factor affecting myelin and autonomic nerves.

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    Area of Science:

    • Neurology
    • Genetics
    • Pathology

    Background:

    • Hereditary neuropathy with liability to pressure palsies (NLPP) is a rare genetic disorder.
    • It causes recurrent sensory-motor deficits triggered by minor pressure.
    • This report details a unique variant observed in siblings.

    Observation:

    • Siblings experienced painful palsies after strenuous activity and abdominal colic.
    • Symptoms mimicked acute intermittent porphyria.
    • Electrophysiological studies revealed typical NLPP nerve conduction abnormalities.

    Findings:

    • Microscopic examination showed characteristic NLPP lesions, including myelin sheath swelling.
    • Frequent observation of non-compacted, "loose" myelin lamellae and distended Schmidt-Lantermann incisures.

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  • Non-compacted myelin was a significant feature of this demyelinating neuropathy.
  • Implications:

    • Suggests an unknown metabolic factor contributes to this NLPP variant.
    • This factor may cause peripheral nerve demyelination and autonomic nerve dysfunction.
    • The metabolic factor could explain both the nerve palsies and abdominal pain episodes.