From the Research
Definition and Characteristics of Neuromyelitis Optica (NMO)
- Neuromyelitis optica (NMO), also known as Devic's disease, is an autoimmune, inflammatory, and demyelinating central nervous system disorder that primarily affects the optic nerve and spinal cord 1, 2, 3, 4, 5.
- It is a distinct disorder from multiple sclerosis (MS), with different immunopathological, clinical, prognostic, and therapeutic characteristics 1, 2, 3, 4.
- The disease is characterized by the presence of antibodies directed against aquaporin-4 (anti-AQP4 or NMO-IgG) in the majority of cases, which distinguishes it from MS 1, 3, 4.
Clinical Features and Diagnosis
- NMO is a debilitating disorder that damages a person's health, with a relapsing course in most cases 1, 2, 3, 4.
- The disease predominantly targets the optic nerves and spinal cord, but can also affect certain regions of the brain 2, 3.
- Diagnostic criteria have been enriched with the recent description of the humoral disorder underlying NMO, including the presence of anti-AQP4 antibodies and characteristic magnetic resonance imaging (MRI) findings 1, 3, 4.
Treatment and Prognosis
- The treatment of NMO is still challenging, with current options being far from ideal 1.
- Treatment in the acute phase includes intravenous steroids and plasma exchange therapy, while immunosuppressive agents are recommended for prophylaxis of relapses 3, 4.
- Relapsing NMO has a poor prognosis, and therapy is necessary as early as possible in the disease course to prevent attack-related disability 4.