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Review
Current concept of neuromyelitis optica (NMO) and NMO spectrum disorders
  1. Anu Jacob1,
  2. Andrew McKeon2,
  3. Ichiro Nakashima3,
  4. Douglas Kazutoshi Sato3,
  5. Liene Elsone1,
  6. Kazuo Fujihara3,
  7. Jerome de Seze4
  1. 1Department of Neurology, The Walton Centre for Neurology and Neurosurgery, Liverpool, UK
  2. 2Department of Neurology, and Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Mayo Clinic, Rochester, Minnesota, USA
  3. 3Department of Neurology, Tohoku University School of Medicine, Sendai, Japan
  4. 4Department of Neurology, Strasbourg University and Clinical Investigation Center, Strasbourg Hospital, France
  1. Correspondence to Dr Jerome de Seze, Department of Neurology and Clinical Investigation Center, CHU de Strasbourg, 1 Avenue Molière, 67098 Strasbourg Cedex, France; jerome.de.seze{at}chru-strasbourg.fr

Abstract

Neuromyelitis optica (NMO) has been described as a disease clinically characterised by severe optic neuritis (ON) and transverse myelitis (TM). Other features of NMO include female preponderance, longitudinally extensive spinal cord lesions (>3 vertebral segments), and absence of oligoclonal IgG bands . In spite of these differences from multiple sclerosis (MS), the relationship between NMO and MS has long been controversial. However, since the discovery of NMO-IgG or aquaporin-4 (AQP4) antibody (AQP4-antibody), an NMO-specific autoantibody to AQP4, the dominant water channel in the central nervous system densely expressed on end-feet of astrocytes, unique clinical features, MRI and other laboratory findings in NMO have been clarified further. AQP4-antibody is now the most important laboratory finding for the diagnosis of NMO. Apart from NMO, some patients with recurrent ON or recurrent longitudinally extensive myelitis alone are also often positive for AQP4-antibody. Moreover, studies of AQP4-antibody-positive patients have revealed that brain lesions are not uncommon in NMO, and some patterns appear to be unique to NMO. Thus, the spectrum of NMO is wider than mere ON and TM. Pathological analyses of autopsied cases strongly suggest that unlike MS, astrocytic damage is the primary pathology in NMO, and experimental studies confirm the pathogenicity of AQP4-antibody. Importantly, therapeutic outcomes of some immunological treatments are different between NMO and MS, making early differential diagnosis of these two disorders crucial. We provide an overview of the epidemiology, clinical and neuroimaging features, immunopathology and therapy of NMO and NMO spectrum disorders.

  • Neuroimmunology
  • Multiple Sclerosis
  • Myelopathy
  • Neuroradiology
  • Neuroophthalmology

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