Neuromyelitis optica spectrum disorder (NMOSD), also known as Devic disease, is a rare immune-mediated inflammatory disorder of the CNS, characterized by concurrent inflammation and demyelination of the optic nerve and the spinal cord.
The term neuromyelitis optica (derived from neuro-myélite optique aiguë) was first described by Eugène Devic and his doctoral student Fernand Gault in 1894. The disease was therefore previously referred to as Devic’s disease. Until recently it was unclear whether neuromyelitis optica was a separate disease or merely a more severe form of ‘optico-spinal’ multiple sclerosis (MS). It was not until 2004 when the putative antigenic target, the aquaporin-4 water channel was identified, and the two diseases could be reliably distinguished through the detection of AQP4-Abs. The latest iteration of diagnostic guidelines unify antibody-negative and positive forms under the umbrella of NMOSDs.
In more than 90% of patients, NMO is a relapsing disease with attacks of ON, myelitis or both, occurring unpredictably
Myelitis (spinal cord inflammation)
Severe optic neuritis
Area postrema syndrome (bouts of intractable vomiting and hiccoughs)
International diagnostic criteria for neuromyelitis optica spectrum disorder (2015):
Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack.
When a diagnosis of NMO is confirmed or suspected, any acute exacerbation should ideally be treated promptly with a high dose of IVMP for 3–5 days