This review summarises recent evidence supporting the involvement of the specialised nodal and perinodal domains (the paranode and juxtaparanode) of myelinated axons in the pathology of acquired, inflammatory, peripheral neuropathies.
The identification of new target antigens in the inflammatory neuropathies heralds a revolution in diagnosis, and has already begun to inform increasingly targeted and individualised therapies. Rapid progress in our basic understanding of the highly specialised nodal regions of peripheral nerves serves to strengthen the links between their unique microstructural identities, functions and pathologies. In this context, the detection of autoantibodies directed against nodal and perinodal targets is likely to be of increasing clinical importance. Antiganglioside antibodies have long been used in clinical practice as diagnostic serum biomarkers, and associate with specific clinical variants but not to the common forms of either acute or chronic demyelinating autoimmune neuropathy. It is now apparent that antibodies directed against several region-specific cell adhesion molecules, including neurofascin, contactin and contactin-associated protein, can be linked to phenotypically distinct peripheral neuropathies. Importantly, the immunological characteristics of these antibodies facilitate the prediction of treatment responsiveness.
- inflammatory neuropathy
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Contributors JF wrote the initial draft of the manuscript, created the figures, revised content following comments from other authors, contributed to the development of its intellectual content and approved the final version. HJW and SSS edited and commented on drafts of the manuscript, contributed to the development of its intellectual content and approved the final version. SR conceived the manuscript, edited and commented on drafts of the manuscript, contributed to the development of its intellectual content and approved the final version.
Competing interests None declared.
Provenance and peer review Commissioned; externally peer reviewed.
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