Ponsford, Sawanthana; (2000) Long term clinical and neurophysiological follow up of patients with peripheral neuropathy associated with benign monoclonal gammopathy. Doctoral thesis (M.D), UCL (University College London).

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Abstract

The primary aim of this study was to determine the long term clinical outcome including the incidence of haematological malignancy in a group of patients initially diagnosed as having neuropathy associated with a benign monoclonal gammopathy. The evolution of the neurophysiological findings over time was also examined. In addition an attempt was made to delineate individual nosological subgroups based on the clinical course and the immunological and neurophysiological findings. Fifty patients with peripheral neuropathy associated with benign monoclonal gammopathy were studied. The duration of the follow-up period was 5-35 years. 74.5% had an IgM paraprotein (κ light chain in 64%). The remainder either had an IgG or (1 case only) an IgA paraprotein. Fifteen patients had died. Only 6% of patients developed haematological malignancy in comparison with the reported 17-25% in patients ascertained by the presence of a paraprotein in the absence of neuropathy. None had multiple myeloma. All had been on immunosuppressive therapy for at least 2 years before the diagnosis of the malignancy. The highest incidence of malignancy of all types (44%) was for the gastrointestinal tract. Of 24 survivors with benign IgM paraproteinaemic neuropathy, 29% had only myelin associated glycoprotein antibodies with typical male predominance, late onset, and a progressive distal sensorimotor polyneuropathy. Evoked distal muscle amplitudes were significantly smaller and the relative prolongation of distal motor latency observable in the early cases was no longer apparent. This suggested progressive axonal degeneration indicating a less favourable prognosis. Two patients (8.3%) with IgM antidisialosyl antibodies and cold agglutinin activity had a large fibre neuropathy with intermittent oculofacial involvement responding to intravenous immunoglobulin treatment. The above two categories thus formed separate clinical and immunological entities. Findings in the remaining patients were varied. Some were not dissimilar to patients with chronic inflammatory demyelinating polyneuropathy. Recognition of IgM subgroups is important both for prognosis and possible response to treatment.

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