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

Journal of Neurology volume 267, pages 2353–2361 (2020)Cite this article

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.

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Acknowledgements

The authors wish to thank Salvatore Monaco for his critical review of the manuscript.

Author information

Author notes

  1. D. Marastoni and L. Africa have contributed equally to the manuscript.

  2. G. M. Fabrizi and F. Giannini have contributed equally to the manuscript.

Affiliations

  1. Department of Neurosciences, Biomedicine and Movement Sciences, University of Verona, Policlinico “G.B. Rossi”, Piazzale L.A. Scuro 10, 37134, Verona, Italy

    D. Marastoni, A. Peretti, S. Ferrari & G. M. Fabrizi

  2. Dipartimento Di Scienze Mediche, Chirurgiche e Neuroscienze, Policlinico Le Scotte, Azienda Ospedaliera Universitaria Senese, Università Di Siena, Viale Bracci 16, Siena, Italy

    L. Africa, S. Bocci, L. Insana, F. Ginanneschi & F. Giannini

  3. Neurology Division, Pederzoli Hospital, Peschiera del Garda, Verona, Italy

    G. Zanette

  4. Neurology Division, Department of Neuroscience AOUI Verona, Verona, Italy

    G. M. Fabrizi

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  1. D. Marastoni
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Contributions

LA, DM, AP, GZ, GMF, and FG: study conception and design. LA, DM, AP, GMF, FG, SI, LB, SF, FG, and GZ: data collection and literature search. All authors: draft and critical revision of the manuscript.

Corresponding author

Correspondence to G. M. Fabrizi.

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Conflicts of interest

The authors have no disclosures of any competing interest.

Ethical approval

All patients gave informed consent to the present report.

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Marastoni, D., Africa, L., Peretti, A. et al. 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 267, 2353–2361 (2020). https://doi.org/10.1007/s00415-020-09843-y

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  • DOI: https://doi.org/10.1007/s00415-020-09843-y

Keyword

  • Chronic ataxic neuropathy
  • CANDA
  • Anti-disialosyl antibodies
  • Subcutaneous immunoglobulins
  • B-cell lymphoma
  • Therapy