Neuromyelitis Optica (Devic's Syndrome): an Appraisal

Area of Science:

• Neuroimmunology
• Central Nervous System Inflammatory Disorders

Background:

• Neuromyelitis optica spectrum disorders (NMOSD), previously Devic's syndrome, involve immune-mediated demyelination and axonal damage in CNS.
• These disorders predominantly affect optic nerves and spinal cord, often associated with NMO-IgG antibodies targeting aquaporin-4 (AQP4).
• Key clinical features include optic neuritis and transverse myelitis, typically with a relapsing course.

Purpose of the Study:

• To summarize recent advances in the diagnosis and treatment of NMOSD.
• To highlight the impact of new diagnostic criteria and treatment recommendations on patient prognosis.

Main Methods:

• Review of established consensus diagnostic criteria (revised 2015).
• Analysis of expert recommendations and uncontrolled trials for treatment guidelines.
• Assessment of treatment protocols for acute attacks and long-term management.

Main Results:

• Revised diagnostic criteria enhance diagnostic accuracy and evaluation of NMOSD.
• Acute attacks are treated with high-dose intravenous methylprednisolone or plasma exchange.
• Long-term management involves immunosuppressive agents such as azathioprine, methotrexate, mycophenolate, or rituximab for approximately 5 years.

Conclusions:

• Recent advances in diagnostic criteria and treatment have significantly improved the prognosis and outcomes for patients with NMOSD.
• Prompt treatment of acute attacks and sustained immunosuppression are crucial for managing NMOSD.