Monoclonal gammopathy and neuropathy

Purpose of review To provide clinically useful guidelines in the management of neuropathy associated with monoclonal gammopathy from a review of the most recent literature and our own experience. Recent findings Recent data on neuropathy associated with monoclonal gammopathy come from better descriptions of subgroups, and from new treatment compounds that have shown encouraging results in different entities. Summary Neuropathies associated with monoclonal gammopathy are relatively rare and most often the neuropathy reveals the monoclonal gammopathy. These conditions require combined neurological and haematological assessments. Their clinical presentations are highly heterogeneous but most have an electrophysiological demyelinating pattern. The main described subgroup is IgM anti-(myelin-associated glycoprotein) neuropathy, which presents as a relatively benign, slowly progressive sensory neuropathy. Nerve biopsy should be considered in patients with progressive and disabling axonal neuropathy. Neuropathies associated with monoclonal gammopathy have various neurological and general outcomes, including life-threatening entities such as light-chain amyloid neuropathy and POEMS syndrome. Treatment choice is wide and depends both on the underlying haematological disorder and severity of the neuropathy. Intravenous immunoglobulin should be assessed in demyelinating monoclonal gammopathy of undetermined significance neuropathy. Malignant haematological disorders should be treated per se. The possibility of a malignant evolution of monoclonal gammopathy of undetermined significance warrants regular haematological monitoring.