Abstract
Introduction: We present a patient with fulminant chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) refractory to conventional interventions successfully treated with cyclophosphamide. Case Report: 54year old male with history of GBS presented to a tertiary care center with bilateral upper and lower extremity weakness, double vision, decreased sensation with bowel movements and micturation, dysphagia, CHF with hypotension, and dyspnea. On examination he demonstrated generalized weakness, areflexia and diminished sensation in bilateral lower extremities. The patient underwent workup, and his laboratory and electrodiagnostic presentation were consistent with CIDP. After he had no response to conventional immunosuppressive treatment, the decision was made to administer cyclophosphamide. The patient demonstrated progressive improvement in his symptoms within one week. He was transferred to the rehabilitation unit and received two additional dosages of cyclophosphamide. He was discharged at an independent level. Conclusion: CIDP is an autoimmune disease driven by an immune response involving T cell activation. It is estimated that 20% of CIDP patients do not respond to the conventional treatments of prednisone, IVIG, and plasma exchange. Cyclophosphamide is an alkylating agent that prevents cell division by crosslinking DNA strands and decreasing DNA synthesis. Previous cases have shown that pulse cyclophosphamide and high dose cyclophosphamide are effective for treating select patients with refractory CIDP, but has never been reported for treatment in patients with fulminant CIDP. Our patient tolerated the medication with few complications. Cyclophosphamide is an option for treatment for fulminant CIDP when conventional immunosuppressive treatment is not effective in the acute and rehabilitation setting
Published Version
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