Diagnosis and Treatment of Neuromyelitis Optica
Diagnosis and Treatment of Neuromyelitis Optica
US Neurology, 2009;5(1):56-8
Abstract
Neuromyelitis optica (NMO), an autoimmune inflammatory disease of the central nervous system (CNS), is characterized by severe attacks of optic neuritis and myelitis. A specific immunoglobulin G1 (IgG1) autoantibody, NMO-IgG, is present in NMO patients. Its discovery facilitates the early recognition of NMO, differentiation of NMO from multiple sclerosis (MS), and recognition of a broader spectrum of manifestations of NMO. Following an attack of NMO, high-dose intravenous methylprednisolone is the treatment of choice. Plasmapheresis is recommended for attacks that do not respond to first-line treatment. For long-term relapse prevention, immunosuppressive drugs such as azathioprine, mycophenolate mofetil, rituximab, and mitoxantrone are recommended rather than the immunomodulatory agents used for MS. The study of NMO has rapidly progressed due to the successful translation of the discovery of a specific biomarker into clinical practice and basic research. The discovery of the antigenic target of NMO-IgG, the water channel aquaporin-4, improved understanding of the physiopathology of NMO and may lead to the development of new treatments.
Keywords
Neuromyelitis optica, Devic’s disease, NMO-IgG, aquaporin-4, demyelinating disease
Disclosure: Marcelo Matiello, MD, has no conflicts of interest to declare. Brian G Weinshenker, MD, has intellectual property associated with the discovery of NMO-IgG that has been licensed to a commercial entity. The NMO-IgG test is offered on a service basis by Mayo Collaborative Service Inc., an agency of the Mayo Foundation.
Received: February 18, 2009 Accepted: August 28, 2009
Correspondence: Brian G Weinshenker, MD, Department of Neurology, Mayo Clinic College of Medicine, 200 First Avenue SW, Rochester, MN 55905. E: weinb@mayo.edu
Neuromyelitis optica (NMO, or Devic’s disease) is an autoimmune inflammatory disease of the central nervous system (CNS) that is typically associated with severe attacks of optic neuritis and myelitis and characteristically spares the brain early in the disease course. Although historically believed to be distinct from multiple sclerosis (MS) in that it presented with simultaneous or rapidly sequential optic neuritis and myelitis with a short interval, it is now believed that it usually begins with unilateral optic neuritis or myelitis and follows a relapsing course.1 In the past, the relapsing form of NMO was typically diagnosed as MS, based on the principle that relapsing CNS inflammatory disease was by definition MS. Over the last decade, however, studies of the clinical aspects, natural history, neuroimaging, pathology and immunology of NMO have established that it is distinct from prototypic MS.1,2 A specific immunoglobulin G (IgG1) autoantibody, NMO-IgG, was discovered in NMO patients3 that subsequently was shown to target the astrocytic water channel aquaporin-4 (AQP4).4 This advance has facilitated early recognition of NMO5,6 and its differentiation from MS, appreciation of a broader spectrum of manifestation, including limited forms of NMO (e.g. recurrent optic neuritis and recurrent transverse myelitis), and, perhaps most importantly, has facilitated breakthrough research in understanding NMO pathogenesis.
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