Paraproteinemic neuropathies

Abstract

The term paraproteinemic neuropathy refers to a heterogeneous group of neuropathies, which share the common feature of a homogeneous immunoglobulin in the serum. The presence of a monoclonal gammopathy indicates an underlying clonal B-cell expansion, which may appear in the context of a lymphoproliferative disorder. If a neoplastic origin of the gammopathy is identified, the treatment should be targeted to the neoplasm. In most patients, however, the monoclonal gammopathy is not associated with malignant haematological disorders, and is defined as monoclonal gammopathy of undetermined significance.

Clinical assessment of the sensory ataxias; diagnostic algorithm with illustrative cases

“…Chronic sensory ataxia can result from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP),12 paraproteinaemia,13 14 diabetic neuropathy,15 coeliac disease,16 vitamin E deficiency17 and mitochondrial dysfunction 40. Chronic sensory ataxia can uncommonly be associated with anti-GD1b IgM antibody; it is worthwhile measuring antiganglioside antibodies in all patients with otherwise idiopathic sensory ataxic neuropathies, as intravenous immunoglobulin treatment is often effective 41…”

“…A range of paraproteinaemic neuropathies can cause sensory ataxia 13 42. Antimyelin-associated glycoprotein neuropathy is a distinct syndrome of late onset, a slowly progressive, demyelinating neuropathy characterised by distal and symmetrical, mainly sensory neuropathy.…”

“…Antimyelin-associated glycoprotein neuropathy is a distinct syndrome of late onset, a slowly progressive, demyelinating neuropathy characterised by distal and symmetrical, mainly sensory neuropathy. It results from monoclonal IgM antibodies against myelin-associated glycoprotein 13 42. There is predominant impairment of joint position and vibration sense.…”

“…Note that IgM paraproteinaemic neuropathies may cause sensory ataxia in the absence of antimyelin-associated glycoprotein antibodies 13 42…”

Cellular targets and mechanistic strategies of remyelination-promoting IgMs as part of the naturally occurring autoantibody repertoire

“…However, the efficacy of current immune therapies such as rituximab, prednisolone and cyclophosphamide in neuropathies with anti-MAG IgM antibodies remains unproven [137]. Plasma exchange (PE) seems to be effective in patients with paraproteinemic neuropathies associated with high IgG or IgA levels but not IgM levels [138].…”