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Devic disease: clinical course, pathophysiology, and management.

Alireza Minagar1, J Steven Alexander, Marjorie R. Fowler

  • 1Department of Neurology, Louisiana State University Health Sciences Center, 1501 Kings Highway, 71130, Shreveport, LA, USA

Pathophysiology : the Official Journal of the International Society for Pathophysiology
|October 19, 2002
PubMed
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Devic disease, also known as neuromyelitis optica spectrum disorder, involves optic neuritis and myelitis but lacks brain lesions or specific spinal fluid markers. Its cause and pathogenesis remain unknown, posing diagnostic and therapeutic challenges.

Area of Science:

  • Neurology
  • Neuroimmunology
  • Ophthalmology

Background:

  • Devic disease, or neuromyelitis optica spectrum disorder (NMOSD), is a rare autoimmune disorder targeting the central nervous system.
  • It is characterized by severe attacks of optic neuritis and necrotizing myelitis.
  • Unlike multiple sclerosis, Devic disease typically lacks brain lesions and specific cerebrospinal fluid (CSF) findings like oligoclonal bands.

Purpose of the Study:

  • To provide a comprehensive review of Devic disease.
  • To discuss the current understanding of its nature, clinical course, and pathogenesis.
  • To outline the available and emerging therapeutic options for managing Devic disease.

Main Methods:

  • Literature review of scientific articles and clinical studies on Devic disease.

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  • Synthesis of information regarding diagnostic criteria, clinical presentation, and epidemiological data.
  • Analysis of research on the underlying mechanisms and treatment strategies.

    • Main Results:

    • Devic disease remains a diagnostic and therapeutic challenge due to unknown etiology and pathogenesis.
    • The review consolidates current knowledge on the clinical manifestations, including optic neuritis and myelitis.
    • It highlights the importance of differentiating Devic disease from other demyelinating disorders.

    Conclusions:

    • Devic disease requires a multidisciplinary approach for accurate diagnosis and effective management.
    • Further research into its pathogenesis is crucial for developing targeted therapies.
    • Advances in understanding the disease are leading to new treatment strategies, including immunomodulatory therapies.