Journal
THERAPEUTIC ADVANCES IN NEUROLOGICAL DISORDERS
Volume 8, Issue 3, Pages 109-122Publisher
SAGE PUBLICATIONS LTD
DOI: 10.1177/1756285615575269
Keywords
multifocal motor neuropathy; anti-ganglioside antibodies; intravenous immuno-globulin; immunosuppressive treatments
Categories
Funding
- Biogen-Idec
- Baxter
- CSL-Behring
- Kedrion
- LFB
- Novartis
- Octapharma
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Multifocal motor neuropathy (MMN) is a rare and disabling disease. Several experimental studies and clinical data are strongly suggestive of an immune-mediated pathogenesis, although underlying mechanisms in MMN seem to be very specific, mainly because of the presence of IgM anti-GM1 serum antibodies and the dramatic response to intravenous immunoglobulins (IVIg). The origin of antiganglioside antibodies and the way in which they act at the molecular level remain unclear. Several studies have demonstrated the key role of complement activation in the underlying mechanisms of MMN, as well as in animal models of acute motor axonal neuropathy (AMAN). Deposition of the membrane attack complex may disrupt the architecture of the nodes of Ranvier and paranodal areas, causing local disruption of nodal sodium-channel clusters. In patients with MMN, muscle weakness is the consequence of conduction blocks (CB), which leads to secondary axonal degeneration, consequently the aim of the treatment is to reverse CB at early stages of the disease. High-dose immunoglobulin is to date the only therapy which has proven efficacy in MMN patients in providing transient improvement of muscle strength, but long-term follow-up studies show a progressive motor decline. Therefore, other therapies are needed to improve the conduction nerve properties in long-term design. The reduction of complement activation and more generally the gain in paranodal stabilization could be directions for future therapeutic strategies.
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