periaqueductal grey matter) which are particularly rich in aquaporin 4 8.Įarly in the disease, demyelinating regions will demonstrate similar findings to MS, such as macrophage/microglia activation and axonal damage. This accounts for some of the predilection for the circumventricular organs (e.g. In approximately 70% (sensitivity of 70-90% specificity of 90%) of patients with established NMO, a specific immunoglobulin can be isolated (AQP4-IgG which targets a transmembrane water channel ( aquaporin 4) present on astrocyte foot processes abutting the limiting membrane 5,8. The current diagnostic criteria are defined by the International Panel for NMO Diagnosis. Additionally, it is now recognised that some patients present with unilateral optic nerve involvement.Īlthough NMO was initially thought of as a monophasic illness, it is now evident that, as with MS, it is usually a relapsing-remitting disease with symptomatic events separated by many years 5.įurthermore, NMOSD also encompasses non-neurological manifestations in anti-AQP4 antibody seropositive patients including systemic lupus erythematosus (SLE) and Sjögren syndrome 13. Although the two usually present concurrently, it is not uncommon for one to precede the other by up to several weeks 3. NMO is characterised by bilateral optic neuritis and myelitis resulting in blindness and paraplegia.
It is found more frequently in patients of Asian, Indian, and African descent 8. Neuromyelitis optica is typically found in patients somewhat older than those with multiple sclerosis (MS), with an average age of presentation of 41 years, and there is an even stronger female predilection (F:M 6.5:1) 6,8. Neuromyelitis optica (NMO) was previously referred to as Devic disease, and traditionally NMO was thought to have limited if any intracranial manifestations. Over the past decade, however, a far wider range of manifestations have been recognised as belonging together and thus the term NMOSD has been proposed to encompass them all.
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