chorioiditis. 16,17 The granulomas in our patient contained no characteristic asteroid bodies or Schaumann bodies, which can be seen in sarcoidosis. Furthermore, the occurrence of stroke in sarcoidosis is extremely rare and the fluorescein angiogram shows a different pattern. 18 The absence of any signs of previous or present vasculitis in the choriocapillaris does not support the hypothesis that APMPPE is caused by a choroidal vasculitis of the lamina choriocapillaris. Instead, our findings indicate that APMPPE is caused by choroidal granulomas and can be part of a generalized granulomatous disease. The granulomas resemble those seen in sarcoidosis. However, its clinical presentation and the occurrence of a cerebral granulomatous vasculitis of large and medium arteries instead suggests that it may be a distinct multisystem granulomatous disease. Recognition of this syndrome is important and our case illustrates that it can be rapidly fatal. Because cerebral vasculitis associated with APMPPE usually responds well to corticosteroid therapy, 4,7,19 we propose that patients with APMPPE complicated by central nervous system manifestations should be treated immediately with intravenous corticosteroids.