Neuropathies with antibodies against disialylated gangliosides and MAG
Targets . | Gangliosides GD1b, GQ1b, GT1a . | Gangliosides GD1b, GD3, GT1b, GQ1b . | MAG + SGPG or SGLPG . |
---|---|---|---|
Isotype | IgG | IgM | IgM |
Kappa = lambda | Kappa > lambda | ||
Mono/polyclonal | Polyclonal | Monoclonal | Monoclonal |
Evolution | Acute, reversible | Chronic | Chronic |
Syndrome | Miller Fisher | CANDA | Anti-MAG neuropathy |
Clinical signs | Ophthalmoplegia, ataxia, areflexia | Sensory ataxia, paresthesia, hypoesthesia, areflexia | Distal demyelinating symmetric sensory and motor neuropathy, ataxia, tremor |
Acute ataxic neuropathy | CANOMAD | ||
Idem, no ophthalmoplegia | + ophthalmoplegia and cold agglutinin | ||
Underlying clonal hematologic disease | No | MGUS IgM 66% | MGUS IgM 80% |
Waldenström disease | Waldenström disease | ||
Low-grade B-cell lymphoma | Low-grade B-cell lymphoma | ||
Treatment | IVIG | IVIG, rituximab-based regimens | Rituximab-based regimens |
Targets . | Gangliosides GD1b, GQ1b, GT1a . | Gangliosides GD1b, GD3, GT1b, GQ1b . | MAG + SGPG or SGLPG . |
---|---|---|---|
Isotype | IgG | IgM | IgM |
Kappa = lambda | Kappa > lambda | ||
Mono/polyclonal | Polyclonal | Monoclonal | Monoclonal |
Evolution | Acute, reversible | Chronic | Chronic |
Syndrome | Miller Fisher | CANDA | Anti-MAG neuropathy |
Clinical signs | Ophthalmoplegia, ataxia, areflexia | Sensory ataxia, paresthesia, hypoesthesia, areflexia | Distal demyelinating symmetric sensory and motor neuropathy, ataxia, tremor |
Acute ataxic neuropathy | CANOMAD | ||
Idem, no ophthalmoplegia | + ophthalmoplegia and cold agglutinin | ||
Underlying clonal hematologic disease | No | MGUS IgM 66% | MGUS IgM 80% |
Waldenström disease | Waldenström disease | ||
Low-grade B-cell lymphoma | Low-grade B-cell lymphoma | ||
Treatment | IVIG | IVIG, rituximab-based regimens | Rituximab-based regimens |