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Neuromyelitis optica also known as Devic’s disease is an uncommon, immune mediated demyelinating condition of the central nervous system affecting predominantly the spinal cord and optic nerves. Since 1894 when Eugene Devic summarised 17 known cases of optic neuritis and myelitis the relation between neuromyelitis optica and multiple sclerosis (MS) has been controversial. The overlapping clinical features and the propensity for patients with neuromyelitis optica to express a range of auto-antibodies can result in misdiagnosis, of both MS and other autoimmune disorders. However, clinical, radiological, and immunopathological studies suggest neuromyelitis optica is distinct from MS. The recent identification of an apparent disease specific antibody—termed NMO-IgG (against the aquaporin-4 water channel) implicates humoral immunity.2,3 This further differentiates the disorder from MS and suggests that treatment to prevent relapses should be aimed primarily at humoral B cell mediated immunity if a pathogenic role for NMO-IgG is confirmed.
Neuromyelitis optica is an uncommon disorder in Western populations; based on observed cases among a population of 3 million over 10 years in North West England we estimated a minimum incidence of 0.4/million/year and a prevalence of 4/million, representing only one in 200 patients with demyelinating disease in this population.4 This contrasts with a much higher incidence in populations of Asian, Afro-Caribbean, and South American descent implying underlying genetic mechanisms in the expression of demyelinating disease. In all populations there is a strong female predominance, of >3:1 compared with males.4,5 The mean age of onset is around 40, although cases have been reported in childhood.
The cardinal clinical features of the disorder are transverse myelitis, which is often longitudinally extensive, and optic neuritis. These two index events can occur simultaneously, in rapid succession, or they can be separated by many years. The optic neuritis can be unilateral or …