Autoimmune nodopathy: pathology
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Autoimmune nodopathy: pathology. They result from autoimmune damage to the node of Ranvier and paranodal structures. Fully referenced neurology checklist.
Pathogenesis
- They result from autoimmune damage to the node of Ranvier and paranodal structures
- They are caused by antibodies to gangliosides and paranodal axo-glial proteins
- They are characterised by conduction block and progression to axonal degeneration
Pathology
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Pathogenic paranodal antibodies (PNAbs)
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Synonym
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References
- Fehmi J, Vale T, Keddie S, Rinaldi S. Nodal and paranodal antibody-associated neuropathies. Pract Neurol 2021 (Online ahead of print).
- Uncini A, Vallat JM. Autoimmune nodo-paranodopathies of peripheral nerve: the concept is gaining ground. JNNP 2018; 89:627-635.
- Uncini A, Susuki K, Yuki N. Nodo-paranodopathy: beyond the demyelinating and axonal classification in anti-ganglioside antibody-mediated neuropathies. Clin Neurophysiol 2013; 124:1928-1934.
- Uncini A, Mathis S, Vallat JM. New classification of autoimmune neuropathies based on target antigens and involved domains of myelinated fibres. JNNP 2021 (Online ahead of print).
- Cortese A, Lombardi R, Briani C, et al. Antibodies to neurofascin, contactin-1, and contactin-associated protein 1 in CIDP: Clinical relevance of IgG isotype. Neurol Neuroimmunol Neuroinflamm 2019; 7:e639.
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