Within the last decade, antibodies targeting the node and paranode of myelinated peripheral nerves have been increasingly identified in patients with acquired immune-mediated neuropathies, commonly termed ‘nodo-paranodopathies’. Crucially, these patients often present with additional clinical features not usually seen with the most common immune-mediated neuropathies, Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, and respond poorly to conventionally used immunomodulatory therapies. Emerging evidence that these are pathologically distinct diseases has further prompted the use of more targeted treatment, such as the B cell depleting monoclonal antibody rituximab, which has been reported to significantly improve functional outcomes in this subset of patients. We provide an overview of the emerging clinical and serological phenotypes in patients with specific nodal/paranodal antibodies, the practicalities of antibody testing and current evidence supporting the use of non-standard therapies.
- Guillain-Barre syndrome
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Contributors JF and SR wrote the original manuscript. TV and SK reviewed and contributed to subsequent revisions of the manuscript.
Funding The funding was provided by the Medical Research Council (Clinician Scientist Fellowship awarded to Simon Rinaldi, MR/P008399/1) and the GBS/CIDP Foundation International Benson Fellowship awarded to Janev Fehmi (1709HM001/SB17).
Competing interests None declared.
Provenance and peer review Provenance and peer review. Commissioned. Externally peer reviewed by Hugh Willison, Glasgow, UK.