%0 Journal Article
%T Immunotherapy of Neuromyelitis Optica
%A Benjamin Bienia
%A Roumen Balabanov
%J Autoimmune Diseases
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/741490
%X Neuromyelitis optica (NMO) is a chronic inflammatory disease of the central nervous system that affects the optic nerves and spinal cord resulting in visual impairment and myelopathy. There is a growing body of evidence that immunotherapeutic agents targeting T and B cell functions, as well as active elimination of proinflammatory molecules from the peripheral blood circulation, can attenuate disease progression. In this review, we discuss the immunotherapeutic options and the treatment strategies in NMO. We also analyze the pathogenic mechanisms of the disease in order to provide recommendations regarding treatments. 1. Introduction Neuromyelitis optica (NMO), also known as Devic¡¯s disease, is a chronic inflammatory disease of the central nervous system (CNS) that preferentially targets the optic nerves and spinal cord [1]. The overall disease incidence has been estimated at 1£¿:£¿100,000 and that it has a predilection for middle-aged, non-Caucasian females [2]. NMO spectrum disorders (NMOSD) encompass a variation of this classical picture in that patients may have brain involvement or a more limited presentation such as isolated transverse myelitis or an optic neuritis [3]. Historically, many thought of NMO as a rare variant of multiple sclerosis (MS). Given the identification of unique clinical and radiological differences and the discovery of the NMO-IgG, an autoantibody against aquaporin-4 (aqp4), it is now understood to be its own entity with distinct pathogenesis, diagnostic criteria, prognosis, and treatment [1¨C5]. Until recently, NMO was considered a disease of limited therapeutic options and poor prognosis. Research over the last decade brought new understanding of the disease pathogenesis that translated into immunotherapy directed against this disease. Moreover, there is a growing body of evidence that NMO can be controlled by immunotherapeutics targeting its cellular and humoral immune mechanisms. We review the immunotherapy of NMO, the various treatment options, and the clinical strategies that are typically encountered in practice. 2. Neuromyelitis Optica: An Overview NMO is a neurological disorder that classically presents as a case of severe bilateral optic neuritis associated with a transverse myelitis [1¨C5]. The typical disease onset is either acute or subacute, and the symptoms are likely to persist without treatment. Optic neuritis results in decreased or a complete loss of vision. Transverse myelitis is usually extensive and spans more than 3 consecutive vertebral segments. Deficits related to myelitis include paralysis and sensory
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