[Devic's neuromyelitis optica and related neurological disorders]

Area of Science:

• Neuroimmunology
• Demyelinating diseases of the central nervous system (CNS)

Context:

• Devic's neuromyelitis optica (DNMO) is a rare inflammatory CNS disorder affecting the spinal cord and optic nerves.
• The distinction between DNMO and multiple sclerosis (MS) has been debated since 1894.
• Recent data strongly support DNMO and MS as separate entities with different prognoses and treatments.

Purpose:

• To highlight the critical differences in prognosis and treatment between DNMO and MS.
• To emphasize the autoimmune and antibody-mediated nature of DNMO.
• To discuss the role of NMO-IgG and its target, Aquaporin-4, in DNMO pathogenesis and diagnosis.

Summary:

• DNMO requires early immunosuppressive treatment; standard MS immunomodulators are ineffective and potentially harmful.
• DNMO is now recognized as an autoimmune disease, particularly antibody-mediated, with the identification of NMO-IgG targeting Aquaporin-4.
• NMO-IgG aids in differentiating DNMO from MS, expands the clinical spectrum of DNMO, and may predict relapses or conversion.

Impact:

• Accurate diagnosis and distinction between DNMO and MS are crucial for appropriate patient management.
• NMO-IgG is a key biomarker for DNMO diagnosis and potentially for predicting disease course.
• Understanding NMO-IgG's pathogenic role offers insights into DNMO immunopathology and potential therapeutic targets.