Asymptomatic oligoclonal CSF IgG and progressive increase of intrathecal IgG synthesis in a patient with myasthenia gravis treated with thymectomy — A 4-years follow-up

Abstract

A young woman is described who had myasthenia gravis which was favourably influenced by thymectomy. Although there was no evidence for another neurological disease over 7.5 years of clinical observation and computed tomography as well as visual evoked response were normal, CSF studies over 0.5 years prior to and 3.5 years after thymectomy revealed persistent mononuclear pleocytosis, high CSF IgG index, oligoclonal IgG bands in CSF, and increasing IgG synthesis rate within the CNS, reflecting a continuous local humoral immune response. CSF/serum ratios of antibodies to acetylcholine receptors (AChR) were continuously lower than CSF/serum IgG ratios, contradicting intrathecal AChR antibody production. Proportion of total T cells was slightly higher in CSF than peripheral blood, while active T cells were lower in CSF. CSF lymphocytes did not proliferate on PHA stimulation but responded in allogeneic mixed lymphocyte culture. B cells were 4% in CSF and 4.5% in peripheral blood, but 227 IgG, 0 IgA and 0 IgM producing cells were detected among 20 × 10 3 CSF lymphocytes, compared to 5, 4 and 0 in 20 × 10 3 peripheral blood lymphocytes. This patient represents an in vivo ‘experiment’ regarding influence of thymectomy on CSF compartment constituents. The present study also shows that an individual can be clinically healthy despite continuous and pronounced intrathecal immune response.