[Polyneuropathy associated with monoclonal gammopathy of undetermined significance]. / Forekomst, kausalitet og behandling af polyneuropati associeret til monoklonal gammopati af ukendt signifikans.

The prevalence of polyneuropathy in patients with monoclonal gammopathy of undetermined significance (MGUS) has been reported to be 10-50%. The majority of patients have a chronic, slowly progressive, distal, symmetric and predominantly sensory polyneuropathy. A caused relationship between polyneuropathy and immunoglobulin (Ig)M MGUS is better established than the relationship between polyneuropathy and IgG/IgA MGUS because of the observed binding of IgM to myelin sheaths and widening of myelin lamellae. In randomized controlled trials plasma exchange, immunosuppressive, rituximab and intravenous Ig have been found to have a clinical meaningful effect.

[A syndrome of chronic ataxic polyneuropathy, ophtalmoplegia, IgM paraprotein, cold agglutinins and anti-disialosyl antibodies can cause diplopia in patients with chronic sensory polyneuropathy]. / Monoklonal gammopati af ukendt signifikans kan medføre polyneuropati og oftalmoplegi.

CANOMAD is a rare syndrome of chronic ataxic polyneuropathy, ophtalmoplegia, IgM paraprotein, cold agglutinins and anti-disialosyl antibodies. We present a case of a 65-year-old woman with clinical and electrophysiological features of chronic sensory polyneuropathy and diplopia. Serum samples from the patient contained IgM paraprotein and anti-GM2-antibodies. Treatment with intravenous immunoglobulins resulted in an improvement of the patient's diplopia and polyneuropathy. The case shows the importance of considering CANOMAD as a cause of diplopia in patients with chronic sensory polyneuropathy.