Inflammatory, antibody mediated, immunologic disease of the central nervous system that causes demyelination of the optic nerve and spinal cord
Aetiology
Greater incidence in females (5:1)
Average age of onset - mid 30s
Pathophysiology
Involves demyelination and inflammation of multiple spinal cord segments and the optic nerves
Clinical features
Patients can initially present with an acute flu-like illness (fever, myalgia, and headache)
Later, more suggestive and specific signs and symptoms of NMO may start to develop including optic neuritis or myelitis
As opposed to MS related optic neuritis, patients with NMOSD may have a worse visual prognosis after optic neuritis and many patients are left with residual visual disability
Investigations
Diagnostic criteria
Core clinical criteria:
Optic neuritis
Longitudinal transverse myelitis
Area prostrema syndrome
Bloods: anti-aquaporin 4 antibodies (99% specific but only 75% sensitive)
Diagnosis - 2 core clinical with aquaporin 4
Management
Steroids - IV methyprednisolone, prolonged oral course