Objective: To determine the responses to treatment of patients with chronic sensory ataxic neuropathy associated with anti- GD1b IgM antibodies.
Methods: Patients with chronic sensory ataxic neuropathy associated with anti- GD1b IgM antibodies followed in our department for at least 12 months between 2001 and 2008 were identified and studied retrospectively. Patients were tested at regular intervals using the INCAT disability score. Patients whose disability scores improved by at least one point were taken to have responded to the treatment. Intravenous immunoglobulin (IVIg; 2 g/kg) was administered for 3 to 5 days once every 6 weeks or corticosteroids at an initial daily dose of 1 mg/kg.
Results: 13 patients treated during the 8-year period of interest were included in this study. 7 of 13 patients displayed IgM anti-GQ1b, GT1b and GD3 antibodies suggesting reactivity against disialosyl epitope. IgM gammopathy was detected in 4 of 6 of serum with anti-disialosyl antibodies and 2 of the 7 other sera. 9 of the 13 patients improved in response to IVIg. Oral corticosteroid treatment was attempted on 4 patients prior to IVIg treatment, and partial recovery occurred in one, who became steroid-dependent and showed little benefit in the long term.
Conclusions: Screening for anti-GD1b IgM antibodies should be carried out on all patients with chronic ataxic sensory neuropathies. In 69% of the cases studied, the patients’ condition improved in response to IVIg. This study shows the short term efficiency of this treatment. Sustained responses were obtained in the long term by continuing the infusions.