Abstract
Introduction: CANOMAD (an acronym for its full manifestation of chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies) is an uncommon paraproteinaemic neuropathy. Methods and Results: We present the case of a man of 58 years at presentation, with chronic ataxic neuropathy, ophthalmoplegia, and an IgM paraprotein who was found to have antibodies active against disialosylgangliosides. Despite treatment trials with a range of immunosuppressive therapies, intravenous immunoglobulin proved the only effective treatment strategy in his case. Conclusions: CANOMAD has been described in a growing number of individual reports and small series for which we have methodically searched the literature. In the absence of randomised trials of treatment for this disabling condition, we bring together these published clinical experiences, describing the degree of heterogeneity in the presentation/laboratory findings and demonstrating that intravenous immunoglobulin is the therapy found most frequently to be successful.
Highlights
A monoclonal IgM paraproteinaemic neuropathy in which IgM antibodies were found to react against disialosylgangliosides components of the human nervous system, was first described almost three decades ago [2]
Willison and colleagues in 1996 demonstrated in a rodent model that the antidisialosyl antibody itself exerted pathophysiological action on the peripheral nervous system and proposed the acronym CANOMAD to encompass the clinical features seen in this form of chronic neuropathy [3]
A case series of 18 patients published by Willison and colleagues provides one of the best descriptions available of the clinical and laboratory features of CANOMAD [4]
Summary
CANOMAD (an acronym for its full manifestation of chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies) is an uncommon paraproteinaemic neuropathy. Willison and colleagues in 1996 demonstrated in a rodent model that the antidisialosyl antibody itself exerted pathophysiological action on the peripheral nervous system and proposed the acronym CANOMAD to encompass the clinical features seen in this form of chronic neuropathy (chronic ataxic neuropathy, ophthalmoplegia, IgMparaprotein, cold agglutinins and disialosyl antibodies) [3]. We describe a case of this rare neuropathy and our experience of multiple trialled treatments over an 11 year period. This is supplemented by description of several case reports gathered from the published literature, refining the observed phenotype and collating experiences of treatment
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