disialosyl

CANOMAD (Chronic ataxic neuropathy with ophthalmoplegia, M-protein, cold agglutinins and disialosyl antibodies) is a rare disorder with specific clinical features consisting of severe sensory ataxia and cranial nerve involvement including ophthalmoplegia, dysphagia or dysarthria and only minimal weakness. 50 It occurs in around 2% of patients with IgM PDN. 51 CANOMAD is associated with antibodies to ganglioside disialosyl moieties. 50 CANOMAD typically progresses over years and peripheral neuropathy may precede the development of other features such as ophthalmoplegia. 52 Slightly less uncommon is the POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinology, Monoclonal gammopathy and Skin changes), which is usually associated with plasma cell dyscrasia of an IgA or IgG paraprotein and a cluster of multisystem clinical features. 42 It often presents with neuropathy 53 typified by sensory and motor involvement with demyelinating and axonal features. 42 The onset is subacute and progression leads to severe motor weakness. 54 Neuropathic pain may be prominent. 53 High levels of the cytokine vascular endothelial growth factor 55 are helpful in diagnosis.

2015 February 12, Emily K Mathey et al., “Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype”, in Journal of Neurology, Neurosurgery, and Psychiatry, volume 86, →DOI