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CNS Involvement in Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare disease of the choroid and retina, of unknown etiology and mostly affecting young subjects, that can involve the central nervous system (CNS) due to possible associated vasculitis or granulomatous inflammation. Central nervous system manifestations can range from headache, to meningo-encephalitis, to stroke1,2. Less than 30 cases of APMPPE with CNS involvement have been reported in the literature so far. We report herein the case of a woman in her early 20’s who developed CNS manifestations within a month following the diagnosis of APMPPE.