Progressive Polyradiculoneuropathy: Treatment with Azathioprine

Abstract

A 64 yr old male with chronic inflammatory polyradiculoneuropathy (CIP) relentlessly progressing (despite steroids) to virtually complete quadriplegia over 7 mo. was presented. On azathioprine he dramatically improved over a 6 wk period. The truly progressive form of CIP is rare. It is immunologically similar to relapsing CIP and to the more common acute inflammatory polyradiculoneuropathy (AIP). Although the place of steroid therapy is still in doubt it would seem that cytotoxic immunosuppressives have something definite to offer in these conditions where there is progression beyond 4-5 wk. In the largest series of CIP available (where the patients were untreated, or treated with steroids alone) the mortality rate was 11%, and the complete recovery rate was only 5%. Although only isolated case reports were available, if more aggressive immunosuppressive therapy was used more frequently, the prognosis might be considerably improved.