MMN: From Immunological Cross-Talk to Conduction Block

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• 作者：Oliver Harschnitz (1) (2)
  Bas A. Jongbloed (1) (3)
  Hessel Franssen (1)
  Dirk C. G Straver (1)
  W. Ludo van der Pol (1)
  Leonard H. van den Berg (1)
  
  1. Department of Neurology and Neurosurgery ; UMC Utrecht Brain Center Rudolf Magnus ; Utrecht ; 3584 CG ; The Netherlands
  2. Department of Translational Neuroscience ; UMC Utrecht Brain Center Rudolf Magnus ; Utrecht ; 3584 CG ; The Netherlands
  3. Department of Neurology ; St. Elisabeth Hospital ; Tilburg ; 5000 LC ; The Netherlands
  
• 关键词：MMN ; multifocal motor neuropathy ; gangliosides ; anti ; GM1 antibodies ; immune pathogenesis ; conduction block ; intravenous immunoglobulin
• 刊名：Journal of Clinical Immunology
• 出版年：2014
• 出版时间：July 2014
• 年：2014
• 卷：34
• 期：1-supp
• 页码：112-119
• 全文大小：540 KB
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• 刊物类别：Biomedical and Life Sciences
• 刊物主题：Biomedicine
  Immunology
  Infectious Diseases
  Internal Medicine
  Medical Microbiology
  
• 出版者：Springer Netherlands
• ISSN：1573-2592

文摘

Multifocal motor neuropathy (MMN) is a rare inflammatory neuropathy characterized by progressive, asymmetric distal limb weakness and conduction block (CB). Clinically MMN is a pure motor neuropathy, which as such can mimic motor neuron disease. GM1-specific IgM antibodies are present in the serum of approximately half of all MMN patients, and are thought to play a key role in the immune pathophysiology. Intravenous immunoglobulin (IVIg) treatment has been shown to be effective in MMN in five randomized placebo-controlled trials. Despite long-term treatment with intravenous immunoglobulin (IVIg), which is efficient in the majority of patients, slowly progressive axonal degeneration and subsequent muscle weakness cannot be fully prevented. In this review, we will discuss the current understanding of the immune pathogenesis underlying MMN and how this may cause CB, available treatment strategies and future therapeutic targets.