Neuromyelitis optica spectrum disorder
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Synopsis
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, with months to years between attacks, 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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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Multiple sclerosis
- Acute disseminated encephalomyelitis (ADEM)
- Systemic lupus erythematosus (SLE)
- Sjögren syndrome
- Neurosarcoidosis
- Behçet disease
- Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis
- Transverse myelitis
- Subacute combined degeneration of the spinal cord
- Spinal dural arteriovenous fistula
- Infection with human T-cell lymphotropic virus type 1
- Infection with HIV
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Therapy
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.Subscription Required
References
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Last Reviewed:10/13/2019
Last Updated:09/14/2022
Last Updated:09/14/2022