Back to Search Start Over

Nodal and paranodal antibody-associated neuropathies

Authors :
Stephen Keddie
Simon Rinaldi
Tom A Vale
Janev Fehmi
Source :
Practical Neurology. 21:284-291
Publication Year :
2021
Publisher :
BMJ, 2021.

Abstract

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.

Details

ISSN :
14747766 and 14747758
Volume :
21
Database :
OpenAIRE
Journal :
Practical Neurology
Accession number :
edsair.doi.dedup.....03541882de7e3119f5f410bc95c6d35c
Full Text :
https://doi.org/10.1136/practneurol-2021-002960