Isolated angiitis of the central nervous system: lack of inflammation after long-term treatment

Abstract

A case of isolated angiitis of the CNS was observed for 5 years. Initial response to cyclophosphamide was followed by relapse on therapy interruption. After renewed treatment, clinical stabilization was achieved despite progressive stenoses shown by angiography. The patient died of cyclophosphamide-induced myelodysplastic syndrome. Autopsy revealed lack of inflammation, vascular scarring, and amyloid angiopathy. We conclude that cure from isolated angiitis of the CNS is possible and that the risk of overtreatment should be minimized.