CANOMAD and other chronic ataxic neuropathies with disialosyl antibodies (CANDA)

J Neurol. 2018 Jun;265(6):1402-1409. doi: 10.1007/s00415-018-8853-4. Epub 2018 Apr 9.


Introduction: CANOMAD/CANDA are syndromes characterized by ataxic neuropathy, ophthalmoplegia, monoclonal gammopathy, cold agglutinins and disialosyl antibodies.

Methods: A retrospective review of our neuromuscular autoantibody panel database was performed. Anti-GD1b seropositive patients with ataxia were included.

Results: Eleven patients were identified. Median age at onset was 56 years. Median disease duration was 6 years. All patients had gait disorders. Nine had ocular motility abnormalities. Most had a monoclonal protein and all had elevated serum IgM. Electrodiagnostic studies showed a mixed axonal/demyelinating pattern (6), an axonal pattern (4), or a pure demyelinating pattern (1). Ultrasounds showed nerve enlargement patterns consistent with acquired demyelination. A nerve biopsy showed near complete loss of myelinated axons with preservation of smaller axons. Rituximab was the most effective immunotherapy.

Conclusion: CANOMAD/CANDA are rare and debilitating disorders with characteristic clinical and diagnostic findings. In our cohort, nerve ultrasound showed regional nerve enlargement and rituximab was the most effective immunomodulatory therapy.

Keywords: Ataxic neuropathies; CANDA; CANOMAD; Disialosyl antibodies; Immune therapy; Ultrasound.

MeSH terms

  • Adult
  • Aged
  • Ataxia / diagnostic imaging
  • Ataxia / immunology*
  • Ataxia / pathology
  • Ataxia / therapy*
  • Autoantibodies / blood*
  • Chronic Disease
  • Female
  • Follow-Up Studies
  • Gangliosides / immunology*
  • Humans
  • Immunologic Factors / therapeutic use
  • Immunomodulation
  • Male
  • Middle Aged
  • Neural Conduction
  • Peripheral Nerves / diagnostic imaging
  • Peripheral Nerves / pathology
  • Peripheral Nerves / physiopathology
  • Retrospective Studies
  • Rituximab / therapeutic use
  • Ultrasonography


  • Autoantibodies
  • Gangliosides
  • Immunologic Factors
  • ganglioside, GD1b
  • Rituximab