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      Neurodegeneration in multiple sclerosis and neuromyelitis optica.

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          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.

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          Author and article information

          Journal
          J. Neurol. Neurosurg. Psychiatr.
          Journal of neurology, neurosurgery, and psychiatry
          BMJ
          1468-330X
          0022-3050
          Feb 2017
          : 88
          : 2
          Affiliations
          [1 ] Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan.
          [2 ] Center for Brain Research, Medical University of Vienna, Vienna, Austria.
          Article
          jnnp-2016-313300
          10.1136/jnnp-2016-313300
          27671902
          9584bb88-4158-4a77-8098-c78d4a45edfa
          History

          MULTIPLE SCLEROSIS,NEUROIMMUNOLOGY,NEUROPATHOLOGY
          MULTIPLE SCLEROSIS, NEUROIMMUNOLOGY, NEUROPATHOLOGY

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