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Review
Multifocal motor neuropathy: controversies and priorities
  1. Wei Zhen Yeh1,2,
  2. P James Dyck3,
  3. Leonard H van den Berg4,
  4. Matthew C Kiernan5,6,
  5. Bruce V Taylor1,7
  1. 1Department of Neurology, Royal Hobart Hospital, Hobart, Tasmania, Australia
  2. 2Department of Neuroscience, Central Clinical School, Monash University, Melbourne, Victoria, Australia
  3. 3Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  4. 4UMC Utrecht Brain Center, Department of Neurology, University Medical Center Utrecht, Utrecht, Netherlands
  5. 5Bushell Chair of Neurology, Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia
  6. 6Department of Neurology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia
  7. 7Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia
  1. Correspondence to Professor Bruce V Taylor, Menzies Institute for Medical Research, University of Tasmania, Hobart, Tasmania, Australia; bruce.taylor{at}utas.edu.au

Abstract

Despite 30 years of research there are still significant unknowns and controversies associated with multifocal motor neuropathy (MMN) including disease pathophysiology, diagnostic criteria and treatment. Foremost relates to the underlying pathophysiology, specifically whether MMN represents an axonal or demyelinating neuropathy and whether the underlying pathophysiology is focused at the node of Ranvier. In turn, this discussion promotes consideration of therapeutic approaches, an issue that becomes more directed in this evolving era of precision medicine. It is generally accepted that MMN represents a chronic progressive immune-mediated motor neuropathy clinically characterised by progressive asymmetric weakness and electrophysiologically by partial motor conduction block. Anti-GM1 IgM antibodies are identified in at least 40% of patients. There have been recent developments in the use of neuromuscular ultrasound and MRI to aid in diagnosing MMN and in further elucidation of its pathophysiological mechanisms. The present Review will critically analyse the knowledge accumulated about MMN over the past 30 years, culminating in a state-of-the-art approach to therapy.

  • NEUROPATHY
  • NEUROIMMUNOLOGY
  • GANGLIOSIDE
  • NEUROPHYSIOLOGY
  • PERIPHERAL NEUROPATHOLOGY
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Footnotes

  • Contributors BVT, MCK, PJBD and LHvdB conceived the idea for the article. WZY drafted the manuscript. All authors revised the manuscript critically for important intellectual content and gave final approval of the version to be published.

  • Funding BVT was supported by a Macquarie Foundation MSRA Clinical Fellowship. This work was supported in part by funding to Forefront from the National Health and Medical Research Council of Australia (NHMRC) program grant (#1037746). MCK was supported by an NHMRC Practitioner Fellowship (#1156093).

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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