Relapsing neuromyelitis optica spectrum disorder

Abstract

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a devastating inflammatory disorder of the central nervous system. Relapsing NMOSD (RNMOSD) is being reported frequently, leading to severe and permanent relapse related disability. Aims and Objectives: To study the clinical profile and imaging features of the NMOSD cases at the index time and addressing the long-term clinical spectrum of relapsing type of NMOSD who presented to the tertiary care hospital. Materials and Methods: All patients who attended the King George Hospital, Visakhapatnam and fulfilled the diagnostic criteria of NMOSD were studied for the epidemiological details, type of clinical spectrum on relapses, imaging features and treatment prescribed, and were studied between April, 2011 and March, 2018. Results: Total diagnosed cases were 28, out of which relapsing type were seen in 11 patients and all tested positive for antibodies. Female to male ratio is 3:1, and in RNMOSD group all were females. Most common clinical presentation was myelitis followed by optic neuritis. Noted clinical spectrum in the RNMOSD was unusual presentations like one each patient of Area-Postrema syndrome, diencephalic syndrome, cerebral syndrome, acute brainstem syndrome, and frequent relapses within a month in two. Five cases had also associated systemic autoimmune disorders. Conclusion: RNMOSD is very rapidly evolving disease, affecting primarily young women with wide spectrum of neurological presentations and also other non-neurological systemic features. Early diagnosis and aggressive immune therapy in the early phase might be warranted in NMOSD for relapse prevention and improving the quality of life.