Table 1

Conditions encompassed by ‘nodo-paranodopathies,’ their associated clinical features and antigen targets (strongest associations emboldened)

LocalisationAntigenAssociated neuropathyNotable phenotypic features
Node Gangliosides AMAN (GM1a/b, GD1a, GalNac-GD1a)
AMSAN (GM1, GM1b, GD1a)
CANOMAD/sensory ataxic (GD1b and/or other disialosyl gangliosides)
Anti-GM1 Abs are linked with:
• AMAN (IgG) and MMN (IgM)
Gliomedin EAN (AIDP), MMNNF186 and gliomedin Abs are associated with an animal model of AIDP, and are found in patients with MMN
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 CIDPCaspr Abs share similar features to NF155/CNTN1 Ab cohort, and associated specifically with neuropathic pain
Juxtaparanode CNTN2/TAG1 None specificSNPs 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.