Neuromyelitis Optica

Bruce A.C. Cree, MD, PhD; Douglas S. Goodin, MD; Stephen L. Hauser, MD


Semin Neurol. 2002;22(2) 

In This Article

Abstract and Introduction

Whether neuromyelitis optica (NMO), the co-occurrence of myelitis and optic neuritis, is a variant of multiple sclerosis (MS) or a unique disease is controversial. Distinct neuropathological features and a fulminant clinical course argue in favor of NMO as a distinct disease. However, the combination of neurological impairments of myelitis and optic neuritis occurs in patients with several inflammatory disorders, including multiple sclerosis and collagen vascular diseases. NMO is also associated with certain infectious diseases. The fact that the NMO phenotype occurs in a variety of disease states suggests that NMO does not represent a specific clinical entity. To better understand NMO and its associations with recognized diseases, a systematic review of the literature using MEDLINE was conducted. The history of NMO, its nosology, associations with other diseases, and current concepts of its pathogenesis and treatment is reviewed in this article.

Objectives: On completion of this article the reader will be able to recognize the clinical presentation, summarize the state-of-the-art diagnostic workup and differential diagnosis, and outline the contemporary views on management of neuromyelitis optica.
Accreditation: The Indiana University School of Medicine is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
Credit: The Indiana University School of Medicine designates this educational activity for a maximum of 1.0 hours in category one credit toward the AMA Physicians Recognition Award. Each physician should claim only those hours of credit that he/she actually spent in the educational activity.
Disclosure: Statements have been obtained regarding the authors' relationships with financial supporters of this activity. There is no apparent conflict of interest related to the context of participation of the authors of this article. Dr. Goodin has (or currently is) participating in industry-sponsored clinical trials in multiple sclerosis with the following pharmaceutical companies: Ares-Serono, Berlex Laboratories, Biogen, Immunex, and Teva-Marion Partners. This article discusses the use of DMTs in multiple sclerosis not limited to labeled use.

The syndrome of neuromyelitis optica (NMO) is defined as the co-occurrence of optic neuritis with myelitis. This combination of neurological impairments occurs in patients with multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), systemic lupus erythematosus (SLE), and Sjögren syndrome. It also occurs in association with viral and bacterial infections. However, most often, no underlying cause can be found. The clinical course of NMO is variable. It may occur as a monophasic illness that is either fulminant and fatal or associated with varying degrees of recovery. Polyphasic courses characterized by relapses and remissions also occur. Over the last century much debate has revolved around whether NMO is a distinct disease, at least in a subset of patients, and what its relationship is to MS and other inflammatory disorders. This review focuses on the history of NMO, its nosology, reported associations with other disorders, and current concepts of pathogenesis. Whether or not the NMO phenotype corresponds to a unique biologic process will await the identification of a disease-specific marker and, ultimately, the elucidation of the syndrome's pathogenesis.


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