CANOMAD syndrome with respiratory failure

András Salamon, Lívia Dézsi, Bence Radics, Edina Tímea Varga, Tibor Hortobágyi, Adrienn Tömösvári, László Vécsei, Péter Klivényi, Cecília Rajda

Research output: Contribution to journalArticle

Abstract

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient's disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients' quality of life.

Original languageEnglish
Pages (from-to)141-144
Number of pages4
JournalIdeggyogyaszati szemle
Volume73
Issue number3-4
DOIs
Publication statusPublished - Mar 30 2020

Keywords

  • ataxic neuropathy
  • CANOMAD syndrome
  • respiratory failure
  • rituximab

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Salamon, A., Dézsi, L., Radics, B., Varga, E. T., Hortobágyi, T., Tömösvári, A., Vécsei, L., Klivényi, P., & Rajda, C. (2020). CANOMAD syndrome with respiratory failure. Ideggyogyaszati szemle, 73(3-4), 141-144. https://doi.org/10.18071/isz.73.0141