Cerebral complications in acute posterior multifocal placoid pigment epitheliopathy

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a self-limited ocular disease with a favorable visual outcome. Regarding its pathogenesis, there is increasing evidence of a vascular disorder of the precapillary choroidal arterioles that causes ischemic edema of the overlying retinal pigment epithelium. Systemic and neurological abnormalities associated with APMPPE have been documented. The development of severe cerebral vasculitis simultaneously or after a period of months or even years has been described in only a few cases. We present two new well-documented cases that developed several months after manifestation of the characteristic findings of APMPPE. A thorough medical and neurological workup is recommended, as is careful information of the patients and their families about the signs and symptoms of potential associated cerebral complications so as to facilitate early and adequate treatment.