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Neurodegeneration in multiple sclerosis and neuromyelitis optica
  1. Izumi Kawachi1,
  2. Hans Lassmann2
  1. 1Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan
  2. 2Center for Brain Research, Medical University of Vienna, Vienna, Austria
  1. Correspondence to Dr Izumi Kawachi, Department of Neurology, Brain Research Institute, Niigata University, 757 Asahimachi 1, Chuo-ku, Niigata 951-8585, Japan; ikawachi{at}bri.niigata-u.ac.jp

Abstract

Multiple sclerosis (MS) and neuromyelitis optica (NMO) are autoimmune demyelinating diseases of the central nervous system (CNS), having distinct immunological and pathological features. They have two pathogenic components, ‘inflammation’ and ‘neurodegeneration’, with different degrees of severity and pathogenetic mechanisms. The target antigen of autoimmunity in NMO is the water channel aquaporin-4 (AQP4), and antibodies directed against this antigen result in astrocyte damage. MS is a disease primarily affecting myelin and oligodendrocytes, but thus far, no MS-specific autoantigen has been identified. The distinct inflammatory processes in these diseases may trigger cascades of events leading to disease-specific neurodegeneration. Damage of the CNS tissue appears to be amplified by mechanisms that are in part shared by the two conditions and involve oxidative burst activation in microglia/macrophages, mitochondrial damage and axonal energy failure, Wallerian degeneration and meningeal inflammation. However, they appear to differ regarding the nature of the inflammatory response, the type and extent of cortical injury, and the type of astrocyte reaction and damage. Here, we provide a detailed comparison of the pathology between MS and NMO, which may help to define shared and disease-specific mechanisms of neurodegeneration in these diseases.

  • MULTIPLE SCLEROSIS
  • NEUROPATHOLOGY
  • NEUROIMMUNOLOGY
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Footnotes

  • Contributors IK and HL wrote and edited the manuscript.

  • Funding This work was supported in part by JSPS KAKENHI grant number 26461289 (IK).

  • Competing interests IK has received funding for travel/speaker honoraria or for serving on scientific advisory boards from Novartis, Biogen, Bayer, Mitsubishi Tanabe, Takeda and Astellas. HL received honoraria for lectures from Novartis, Biogen, TEVA and Sanofi-Aventis.

  • Provenance and peer review Commissioned; externally peer reviewed.

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