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Demyelinating peripheral neuropathy in Devic disease.

Y Aimoto1, K Ito, F Moriwaka

  • 1Department of Neurology, Hokkaido University School of Medicine, Sapporo, Japan.

The Japanese Journal of Psychiatry and Neurology
|December 1, 1991
PubMed
Summary

This case report details a 20-year-old man with Devic disease (neuromyelitis optica) and peripheral neuropathy. Nerve biopsy revealed significant demyelination, suggesting shared pathways in central and peripheral nervous system damage.

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Area of Science:

  • Neuroimmunology
  • Neuropathology

Background:

  • Devic disease, also known as neuromyelitis optica (NMO), is a rare autoimmune disorder primarily affecting the optic nerves and spinal cord.
  • Co-occurrence of NMO and peripheral neuropathy is uncommon, presenting diagnostic and therapeutic challenges.

Observation:

  • A 20-year-old male presented with clinical features consistent with Devic disease.
  • The patient also exhibited symptoms of demyelinating peripheral neuropathy.
  • Sural nerve biopsy demonstrated a significant reduction in large-diameter myelinated fibers.

Findings:

  • Teased fiber analysis revealed segmental demyelination and remyelination in 50% of examined peripheral nerve fibers.
  • Pathological findings indicated concurrent demyelination in the optic nerves, spinal cord, and peripheral nerves.

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Implications:

  • The simultaneous central and peripheral nervous system involvement suggests a potential common pathogenetic mechanism in this patient's condition.
  • This case highlights the importance of considering peripheral nerve involvement in patients diagnosed with Devic disease.
  • Further research into shared pathways could lead to novel therapeutic strategies for NMO spectrum disorders.