Acute Posterior Multifocal Placoid Pigment Epitheliopathy Associated with Stroke: A Case Report and Review of the Literature

Abstract

Background: Present a case report of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with stroke along with the serologic findings, diagnostic findings, and treatment in addition to presenting a review of other case reports to date in literature. With this information, we are attempting to identify key diagnostic features and appropriate treatment options for patients with this disease.

Methods: Presentation of case report, background on APMPPE, review of the literature and previous case reports, and discussion of diagnostic workup and treatment options for these patients.

Results: There are 28 well-described case reports in the literature to date, not including our case report; 23 had documented radiographic findings consistent with parenchymal injury, ischemic stroke, hemorrhagic stroke, or cerebral venous thrombosis. We report a 23-year-old right-handed man with left hemiparesis, ischemic strokes, and angiographic findings characteristic of cerebral vasculitis 4 months after he was diagnosed with APMPPE.

Conclusions: Based on our present case and previously documented cases to date, corticosteroid therapy with potential escalation of care to immunomodulatory treatment appears to benefit clinical outcomes.

Keywords: Vasculitis; cerebral angiography; chorioretinal disease; inflammatory CNS disease; neuroradiology; stroke.