The natural history of chronic demyelinating neuropathy associated with benign IgM paraproteinaemia. A clinical and neurophysiological study

Abstract

Eighteen patients are described, all of whom had chronic demyelinating peripheral neuropathy and benign IgM paraproteinaemia. All patients had serum antibodies against peripheral nerve myelin or myelin-associated glycoprotein. Seventeen were followed up clinically and electrophysiologically for between 1 and 14 years (mean 7.4 years). The presenting symptoms and signs were almost always those of a distal sensory disturbance in the limbs followed by distal weakness. All patients developed tremor or ataxia in the arms, and gait ataxia. The severity of the neuropathy varied greatly between patients at similar stages. Some had a predominantly sensory deficit and others a predominantly motor deficit. All patients eventually developed both motor and sensory signs. The neuropathy became slowly worse over the first 2-5 years and then appeared to stabilize, although long-term follow-up did reveal a very slow progression in the group as a whole. No patient developed evidence of haematological malignancy but two patients died of malignancy involving other systems. On reviewing 75 patients from the literature, non-haematological malignancy was found to be the commonest cause of death.