Neuromyelitis Optica Spectrum Disorders.

PURPOSE OF REVIEW: This article provides a practical approach for providers caring for patients with neuromyelitis optica (NMO) spectrum disorders. Clinical and imaging features, diagnostic criteria, treatment of acute exacerbations, chronic preventive therapy, and symptom management in NMO spectrum disorders are discussed.

RECENT FINDINGS: The rapid pace of research in NMO spectrum disorders has led to many recent advances. A broader understanding of the clinical spectrum of the disease as well as improvements in anti-aquaporin-4 antibody assays have led to recent revision of the diagnostic criteria. Several recent studies have expanded the knowledge base regarding the efficacy and safety of current therapies for NMO spectrum disorders.

SUMMARY: An NMO spectrum disorder is an inflammatory disorder affecting the central nervous system, previously thought to be closely related to multiple sclerosis but more recently demonstrated to represent a distinct clinical and pathophysiologic entity. As NMO spectrum disorders carry significant morbidity and, at times, mortality, prompt and accurate diagnosis followed by swift initiation of therapy for both treatment of acute exacerbations and prevention of further relapses is critical. This article provides a practical approach to the diagnosis and management of NMO spectrum disorders.