Cyclosporin A in treatment of refractory patients with chronic inflammatory demyelinating polyradiculoneuropathy

To investigate the therapeutic efficacy of cyclosporin A (CyA) in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a microemulsion form of this drug (Neoral(R)) was orally given to seven patients with the disease who were unresponsive or resistant to conventional therapies. The daily dose of CyA was carefully controlled in order to keep the plasma trough concentration between 100 and 150 ng/ml. Within 1 month of initiation of CyA, all patients subjectively showed improvement of clinical symptoms, while both modified Rankin and INCAT disability scores were significantly decreased (p<0.05) and grip strength was significantly increased (p<0.05) 3 months after initiation compared with before. Total protein in the cerebrospinal fluid was significantly decreased 3 and 6 months after starting CyA (p<0.05). Although the maximal motor nerve conduction velocity showed a significant improvement in the median nerve 1 to 1.5 years after commencement of CyA (p<0.05), there were no significant changes in any other neurophysiological parameters. One patient with anti-sulphoglucuronyl paragloboside IGM antibodies gradually became resistant to CyA. but the rest have since been in good neurological condition without complications ascribable to this drug. These results suggest that oral CyA may be effective even for refractory cases with CIDP. CyA should be actively considered as a therapeutic option when patients with CIDP are resistant to conventional treatment. (C) 2004 Elsevier B.V. All rights reserved.