Presentation of neuromyelitis optica spectrum disorder after more than twenty years of systemic sclerosis

Zulma M Hernández; Bruce A Cohen; Joy Derwenskus

doi:10.1016/j.msard.2012.04.002

Presentation of neuromyelitis optica spectrum disorder after more than twenty years of systemic sclerosis

Mult Scler Relat Disord. 2012 Oct;1(4):202-3. doi: 10.1016/j.msard.2012.04.002. Epub 2012 Jun 20.

Authors

Zulma M Hernández¹, Bruce A Cohen², Joy Derwenskus³

Affiliations

¹ Department of Neurology, Feinberg School of Medicine, Northwestern University, 710N Lake Shore Drive, #1105, Chicago, IL 60611, USA. Electronic address: z-hernandez-peraza@northwestern.edu.
² Department of Neurology, Feinberg School of Medicine, Northwestern University, 710N Lake Shore Drive, #1105, Chicago, IL 60611, USA. Electronic address: bac106@northwestern.edu.
³ Department of Neurology, Feinberg School of Medicine, Northwestern University, 710N Lake Shore Drive, #1105, Chicago, IL 60611, USA. Electronic address: jderwenskus@northwestern.edu.

PMID: 25877268
DOI: 10.1016/j.msard.2012.04.002

Abstract

Neuromyelitis optica is an autoimmune disorder characterized by optic neuritis, transverse myelitis, and aquaporin-4 autoantibodies. The term "neuromyelitis optica spectrum disorder" refers neuromyelitis optica features occurring in association with other systemic rheumatologic conditions. We present a case of a 51-year-old woman with long-standing systemic sclerosis who developed transverse myelitis in association with the presence aquaporin-4 autoantibodies. This is the first report case of neuromyelitis optica spectrum disorder associated with systemic sclerosis. Given that patients with neuromyelitis optica spectrum disorder with aquaporin-4 autoantibodies have a high risk for clinical relapse, this report underscores the need for recognition of this disorder in patients with systemic sclerosis and neurological dysfunction.

Keywords: Aquaporin-4 antibodies; Devic's disease; Neuromyelitis optica; Neuromyelitis optica spectrum disorder; Systemic sclerosis; Transverse myelitis.