Autoimmune nodopathy: pathology

Evidence-based neurology checklist on autoimmune nodopathy: pathology: 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…

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

Pathogenic paranodal antibodies (PNAbs)

Synonym

References

  1. Fehmi J, Vale T, Keddie S, Rinaldi S. Nodal and paranodal antibody-associated neuropathies. Pract Neurol 2021 (Online ahead of print).
  2. Uncini A, Vallat JM. Autoimmune nodo-paranodopathies of peripheral nerve: the concept is gaining ground. JNNP 2018; 89:627-635.
  3. 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. 
  4. 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).
  5. 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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