Severe CIDP-MGUS responsive to Rituximab

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a relatively rare disease with progressive limb weakness and sensory loss. A few patients show a severely progressing course without major response to intravenous immunoglobulin or plasma exchange therapy. CIDP-MGUS (monoclonal gammopathy of undetermined significance) is a seldom CIDP variant that has been rarely addressed in therapeutic studies. In the presented CIDP-MGUS case, B cell depletion with rituximab had a favourable effect on the disease course, clinically and in nerve conduction studies.

Keywords: Anatomy; B-cell dyscrasia; Chronic Inflammatory Demyelinating Polyneuropathy (CIDP); Monoclonal Gammopathy of Undetermined Significance (MGUS); Musculoskeletal system; Nerve Conduction Sudies (NCS); Nervous system; Neurology; Physiology; Rituximab.

Figure 1

Timeline of therapy, clinical effect, INCAT-score and nerve conduction studies; d, day; g, gram; I-Adsorp, immunadsorption; INCAT-Score, inflammatory neuropathy cause and treatment disability score, IVIG, intravenous immunoglobulin; kg, kilogram; M-Predni, methylprednisolone; mg, milligram; ms, milliseconds; mV, millivolt; NCS, nerve conduction studie; P-Pheresis, plasmapheresis; Predni, prednisolone.