Objective: Neuromyelitis optica is a severe inflammatory disease with selective involvement of the optic nerve and spinal cord and relative sparing of the brain. This paper will review the recent literature on neuromyelitis optica with special reference to the recently proposed changes in the diagnostic criteria and the broadening clinical spectrum of the disease.

Results: The revised proposed diagnostic criteria of neuromyelitis optica allow incorporation of the status of the recently discovered autoantibody, NMO-IgG, and the presence of brain abnormalities shown by magnetic resonance imaging. NMO-IgG has been found to have good sensitivity and specificity in distinguishing neuromyelitis optica from multiple sclerosis. About 60% of neuromyelitis optica patients can develop such brain abnormalities not typical of multiple sclerosis. The visual prognosis and ambulatory outcome of neuromyelitis optica is poor, and early, aggressive treatment may be warranted to prevent relapses resulting in severe disability.

Conclusions: The clinical spectrum of neuromyelitis optica has expanded since the original description of the disease. Clinicians need to be aware of the recent developments in the diagnostic approach to neuromyelitis optica.