Progressive polyradiculoneuropathy: treatment with Azathioprine.

A case is presented of a 64-year-old male with chronic inflammatory polyradiculoneuropathy (CIP) relentlessly progressing (despite steroids) to virtually complete quadriplegia over seven months. Once commenced on Azathioprine he dramatically improved over a six week period. The truly progressive form of CIP is rare. It is, however, 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 weeks. 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 only 5%. Although only isolated case reports are available, it would seem that if more aggressive immunosuppressive therapy was used more frequently, the prognosis might be considerably improved.