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A patient with chronic inflammatory demyelinating polyneuropathy is reported on who never had spontaneous remissions for 10 years, but who is in remission for 2 years after an autologous stem cell transplantation (ASCT). Before ASCT he needed at least 20 mg prednisone/day and in addition intravenous immunoglobulin (IVIg) treatment at regular intervals. An ASCT was considered in this patient because of serious side effects of immunosuppressive treatment.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterised by weakness with sensory impairments in the arms and legs. The reflexes in the arms and legs disappear. The onset is insidious. There may be progressive deterioration or a course with remissions and exacerbations. Routine blood examination is usually normal. Protein in CSF is increased in most patients. Electrophysiological studies may show slow nerve conduction velocities, conduction blocks, or dispersion.1 Inflammatory demyelinating polyneuropathy is considered to be an autoimmune disease which is supported by the presence of inflammatory cells in sural nerve biopsies and the beneficial response to immunosuppressive treatment in most patients.2 We describe a patient with CIDP who has had this disease for 10 years without spontaneous remissions. He responded to immunosuppressive therapy, but needed high doses and had severe side effects. Therefore, we decided to try to induce a long lasting remission by autologous stem cell transplantation (ASCT).
This patient was 38 years old when, in 1988, he began to have tingling and numbness in his fingers. The sensory symptoms progressed to his arms and legs, followed by weakness. Initially his symptoms were attributed to too much stress but at the end of 1990 when weakness had worsened to such an extent that he was no longer independent in his daily activities, it …