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Multifocal motor neuropathy: controversies and priorities

Yeh, WZ, Dyck, PJ, van den Berg, LH, Kiernan, MC and Taylor, BV 2020 , 'Multifocal motor neuropathy: controversies and priorities' , Journal of Neurology, Neurosurgery and Psychiatry, vol. 91, no. 2 , pp. 140-148 , doi: 10.1136/jnnp-2019-321532.

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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.

Item Type: Article
Authors/Creators:Yeh, WZ and Dyck, PJ and van den Berg, LH and Kiernan, MC and Taylor, BV
Keywords: ganglioside, neuroimmunology, neuropathy, neurophysiology, peripheral neuropathology
Journal or Publication Title: Journal of Neurology, Neurosurgery and Psychiatry
Publisher: B M J Publishing Group
ISSN: 0022-3050
DOI / ID Number: 10.1136/jnnp-2019-321532
Copyright Information:

© Author(s) (or their employer(s)) 2020.

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