[Pulse-dose cyclophosphamide therapy for steroid-refractory autoimmune neurological diseases]

Abstract

Eight patients with neuroimmunological disorders refractory to conventional immunotherapies were given totally 17 courses of pulse-dose cyclophosphamide. The regimen consisted of 600 mg/m2 of intravenous cyclophosphamide at days 1,2,4,6 and 8 and infusion of 2,500-3,000 ml of fluids in order to avoid hemorrhagic cystitis. Some cases were followed by a monthly intravenous dose of cyclophosphamide as a booster treatment. As a result, all but one case of neuro-Behçet disease have shown neurological improvements. In particular, a complete remission was obtained in two cases of vasculitic neuropathy and a case of chronic inflammatory demyelinating polyneuropathy. The side effects, notably, granulocytopenia, alopecia, hemorrhagic cystitis and anorexia, are major problems. Granulocytopenia started 10 days after the first dose and the number of leucocytes was the lowest at day 15. However, the number steadily recovered thereafter without the use of granulocyte colony stimulating factor. There was no serious infections during the course. For neuroimmunological disorders, the pulse-dose cyclophosphamide therapy would serve a useful alternative when the conventional therapies are not efficacious or difficult to continue because of the adverse side effects.