Refractory Form of Chronic Idiopathic Demyelinating Polyneuropathy with Rituximab Effect: A Case Report

The basic characteristic of chronic idiopathic demyelinating polyneuropathy (CIDP) is demyelination of peripheral nerves of autoimmune origin. Antibodies against various myelin sheath proteins play an important role in the pathogenesis of the disease. Cell-mediated immunity, characterised by T-cell and macrophage infiltration in peripheral nerves and spinal roots, is also significantly involved (1). Treatment of CIDP is aimed at suppressing inflammation, but also at removing autoantibodies, cytokines and other pro-inflammatory molecules from the blood. Treatment options include corticosteroids, (intravenous or subcutaneous) immunoglobulins, and plasma exchange (plasmapheresis). If a patient with CIDP does not show an adequate clinical response to these three treatment modalities, it is then refractory CIDP. Based on various studies, this affects up to 10% of patients. We present here a patient with CIDP whose condition gradually stopped improving after all three commonly used treatment modalities (corticosteroids, immunoglobulins, and plasmapheresis). His neurological findings were very severe. The case was ultimately diagnosed as a refractory form of CIDP, which is discussed here in the light of current knowledge of this issue.