J Rheum Dis.  2014 Feb;21(1):4-8. 10.4078/jrd.2014.21.1.4.

The Systemic Rheumatologic Disease and Neuromyelitis Optica

Affiliations
  • 1Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea. hojinkim@ncc.re.kr

Abstract

Neuromyelitis optica (NMO), an autoimmune inflammatory disease of the central nervous system (CNS), is related with autoantibodies for aquaporin-4, which is the most abundant water channel in CNS. The clinical syndromes of NMO, such as longitudinally extensive transverse myelitis and optic neuritis, can occur in the context of systemic rheumatologic diseases, such as systemic lupus erythematosus and Sjogren syndrome. It is debatable as to whether NMO is a feature of genetic tendency toward humoral autoimmunity or a CNS complication of systemic rheumatologic diseases. Current evidence suggests that NMO coexists with systemic rheumatologic diseases rather than a complication from them. Early immunosuppressive therapies should be considered in these patients since just one or two attacks can cause severe neurological disability.

Keyword

Systemic rheumatologic disease; Neuromyelitis optica; Comorbidity; Complication

MeSH Terms

Autoantibodies
Autoimmunity
Central Nervous System
Comorbidity
Humans
Lupus Erythematosus, Systemic
Myelitis, Transverse
Neuromyelitis Optica*
Optic Neuritis
Sjogren's Syndrome
Water
Autoantibodies
Water

Figure

  • Figure 1. Spinal MRI of the patient with neuromyelitis optica (T2 weighted imaging). (A) Longitudinally extensive cervico- tho-racic myelopathy in the sagittal view. (B) Central gray matter is preferentially involved in the axial view.


Reference

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