Sustained response to subcutaneous immunoglobulins in chronic ataxic neuropathy with anti-disialosyl IgM antibodies (CANDA): report of two cases and review of the literature

J Neurol. 2020 Aug;267(8):2353-2361. doi: 10.1007/s00415-020-09843-y. Epub 2020 Apr 28.

Abstract

Introduction: Chronic ataxic neuropathy with anti-disialosyl IgM antibodies (CANDA) is a rare disorder for which the pathological, neurophysiological, and therapeutic evidence remains anecdotal and controversial.

Methods: This report on CANDA focuses on the neurophysiological patterns and treatment responses shared by two cases. One patient underwent nerve ultrasound follow-up. A comprehensive review of the literature highlighted the diverse experiences with different treatment options.

Results: Response to different therapies was similar in both patients: intravenous immunoglobulins achieved a favorable response albeit with significant wearing-off fluctuations; treatment with subcutaneous immunoglobulins (SCIg) was an effective alternative leading to a clinical response for at least 2 years. Rituximab, which was trialed in both patients, was not continued long enough to determine its efficacy in modifying the disease course and/or modulating responsiveness to immunoglobulins. Steroids caused clinical worsening in both patients.

Conclusions: Immunoglobulin therapy appeared as the most effective in the treatment of these two patients. SCIg provided an effective treatment option for the long-term management of CANDA.

Keywords: Anti-disialosyl antibodies; B-cell lymphoma; CANDA; Chronic ataxic neuropathy; Subcutaneous immunoglobulins; Therapy.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Ataxia
  • Gangliosides*
  • Humans
  • Immunoglobulin M
  • Immunoglobulins
  • Immunoglobulins, Intravenous / therapeutic use
  • Peripheral Nervous System Diseases*

Substances

  • Gangliosides
  • Immunoglobulin M
  • Immunoglobulins
  • Immunoglobulins, Intravenous