Conditions encompassed by ‘nodo-paranodopathies,’ their associated clinical features and antigen targets (strongest associations emboldened)
| Localisation | Antigen | Associated neuropathy | Notable phenotypic features |
| Node | Gangliosides | AMAN (GM1a/b, GD1a, GalNac-GD1a) AMSAN (GM1, GM1b, GD1a) MMN (IgM GM1) CANOMAD/sensory ataxic (GD1b and/or other disialosyl gangliosides) | Anti-GM1 Abs are linked with: • RCF • AMAN (IgG) and MMN (IgM) |
| Gliomedin | EAN (AIDP), MMN | NF186 and gliomedin Abs are associated with an animal model of AIDP, and are found in patients with MMN | |
| NF186 | AMAN, AIDP, CIDP, CCPD | ||
| Paranode | Gangliosides | MFS (GQ1b, GT1a) PCB (GT1a, GQ1b) | Anti-GQ1b Abs suggest good treatment response/prognosis |
| NF155 | CCPD, AIDP, CIDP | • Younger onset CIDP • Particularly raised CSF protein levels • Distal, motor predominant • Ataxia and tremor • Good response to rituximab but not IVIg | |
| CNTN1 | AIDP, CIDP | • Older age • Aggressive disease onset • Motor predominant • Early axonal loss • Poor IVIg responsiveness | |
| Caspr | CIDP | Caspr Abs share similar features to NF155/CNTN1 Ab cohort, and associated specifically with neuropathic pain | |
| Juxtaparanode | CNTN2/TAG1 | None specific | SNPs in TAG-1 gene may favour IVIg responsiveness in CIDP |
| Caspr2 | None specific, but clear association with acquired neuromyotonia |
Case studies link Caspr2 Abs to: • neuropathic pain • favourable response to IVIg in GBS |
Gangliosides: GQ1b, GT1a, GM1a/b, GD1a.
Ganglioside complex: GalNac-GD1a.
Variants of GBS: AIDP, acute inflammatory demyelinating polyneuropathy; AMAN, acute motor axonal neuropathy; AMSAN, acute motor-sensory axonal neuropathy; CANOMAD, chronic ataxic neuropathy, ophthalmoplegia, monoclonal IgM paraprotein, cold agglutinins and disialosyl antibodies; CCPD, combined central and peripheral demyelination; CIDP, chronic inflammatory demyelinating polyneuropathy; CSF, cerebrospinal fluid; EAN, experimental autoimmune neuritis; GBS, Guillain-Barré syndrome; MFS, Miller Fisher syndrome; MMN, multifocal motor neuropathy; PCB, pharyngeal cervical brachial; RCF, reversible conduction failure; SNP, single nucleotide polymorphism.