Presentation of Neuromyelitis Optica Spectrum Disorder After More Than Twenty Years of Systemic Sclerosis

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.