Central nervous system vasculitis as a complication of refractory dermatomyositis

We describe a 47-year-old woman with refractory dermatomyositis (DM) who developed progressive cognitive dysfunction. Magnetic resonance imaging showed a large cerebral infarction, and the diagnosis of central nervous system (CNS) vasculitis was confirmed by both angiogram and brain biopsy. Her DM and CNS vasculitis responded promptly to the institution of daily cyclophosphamide, and her previously refractory disease entered remission.