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Neuromyelitis optica spectrum disorder
Other Resources UpToDate PubMed

Neuromyelitis optica spectrum disorder

Contributors: Andrea Wasilewski MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic disease or neuromyelitis optica (NMO), are inflammatory conditions resulting in severe demyelination of the central nervous system (CNS). Classic symptoms include optic neuritis (often bilateral) and transverse myelitis with extremity weakness, sensory loss, and sphincter dysfunction. Brain stem syndromes, such as narcolepsy or area postrema syndrome with intractable nausea, vomiting, and hiccups, can also occur.

NMOSD is associated with disease-specific antibodies including aquaporin (AQP)-4-IgG and, less commonly, anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. Thought to be mediated by the humoral immune system, NMOSD has a relapsing course, and disability is based on accumulation of deficits from attacks. Incidence is up to 10 times higher in women than men, with onset typically in the third to fourth decade, although the disease can be seen in children and older adults. NMOSD is more prevalent in those of African, East Asian, and Latin American descent than in those of Northern European descent.

Prognosis is variable and depends on the number and severity of attacks, although rates of disability and mortality are high.

Codes

ICD10CM:
G36.0 – Neuromyelitis optica [Devic]

SNOMEDCT:
25044007 – Neuromyelitis optica

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Last Reviewed:10/13/2019
Last Updated:11/17/2019
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Neuromyelitis optica spectrum disorder
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Neuromyelitis optica spectrum disorder : Nausea/vomiting, Recurring episodes or relapses, Hiccups, Sudden onset of visual loss, Extremity weakness
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