Delayed Recurrent Stroke in a Young Patient with Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Abstract

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a chorioretinal disease that causes acute binocular visual disturbance with characteristic funduscopic lesions at the level of the retinal pigment epithelium. APMPPE has been associated with multiple neurologic complications, including cerebrovascular diseases. We report a 15-year-old patient who had bilateral APMPPE, which was successfully treated with corticosteroids. One year later he presented with transient dysarthria and right hemiparesis. Brain magnetic resonance imaging (MRI) showed bilateral ischemic lesions in both lenticular nuclei and corona radiata. Brain MRI performed 3 months later revealed a new asymptomatic ischemic lesion. Cerebral angiography showed diffuse multifocal segmental vessel narrowing. The cerebrospinal fluid showed mononuclear pleocytosis in keeping with vasculitis. We started corticosteroid treatment, which lasted 10 months. Currently, after 2 years of clinical and neuroradiologic follow-up, the patient is asymptomatic and shows no worsening of the cerebrovascular lesions.