Treatment of Neuromyelitis Optica Spectrum Disorder: Acute, Preventive, and Symptomatic

Abstract

Opinion statement Neuromyelitis optica spectrum disorder (NMOSD) is a rare, autoimmune disease of the central nervous system that primarily attacks the optic nerves and spinal cord leading to blindness and paralysis. The spectrum of the disease has expanded based on the specificity of the autoimmune response to the aquaporin-4 water channel on astrocytes. With wider recognition of NMOSD, a standard of care for treatment of this condition has condition based on a growing series of retrospective and prospective studies. This review covers the present state of the field in the treatment of acute relapses, preventive approaches, and therapies for symptoms of NMOSD.

Vaccines and the association with relapses in patients with neuromyelitis optica spectrum disorder

“…Relapse severity was characterized by net change in the Expanded Disability Status Scale (EDSS) from baseline to nadir. Preventive immunotherapy included immunosuppressive medications for which there is evidence of effectiveness in NMOSD (Kessler et al, 2016). Because interferon beta has been shown to worsen NMOSD disease (Kimbrough et al, 2012) and use of glatiramer acetate has not been shown to affect disease severity (Ayzenberg et al, 2016), patients on these medications were not considered to be treated for their disease.…”

Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorder

“…In general, patients with NMO and NMOSD do not present differential clinical behaviours, and patients with either disorder should receive the same treatment. Without appropriate treatment, the prognosis of NMO is poor, as many patients usually experience a relapsing course, which most often leads to blindness and paralysis; many patients with NMO die within weeks or months, whereas patients with MS typically experience milder attacks with good recovery (only 15% of patients with MS have a primary‐progressive disease) . Early therapy should be initiated immediately after the diagnosis .…”

Plasma exchange as a first line therapy in acute attacks of neuromyelitis optica spectrum disorders

“…Maintenance therapy for NMOSD are aimed to prevent the relapses with the administration of different immunosuppressive drugs. [456] Due to the low incidence and prevalence of NMO, interventional studies with level I or II evidence are not currently available. Therefore, treatment strategies are mostly based on small case series and reports.…”

“…[457] This widely used steroid treatment usually fails to control severe attacks of NMOSD, and so generally the next step is the use of PLEX as an add-on or a rescue therapy in order to halt the stepwise progression of residual impairment. [8] In the indications given by American Society for Apheresis (ASFA), PLEX for NMOSD comes under category II i.e disorders for which apheresis is accepted as second-line therapy, either as a standalone treatment or in conjunction with other modes of treatment.…”