Acute Posterior Multifocal Placoid Pigment Epitheliopathy Complicated by Fatal Cerebral Vasculitis

Robi Maamari, Leanne Stunkel, Nathan Kung, Cole Ferguson, Jody Tanabe, Robert Schmidt, Sonika Dahiya, Amar Dhand, Gregory Van Stavern, Rithwick Rajagopal, George Harocopos. Journal of Neuro-Ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society, Volume 39, Issue 2, 1 June 2019, Pages 260-267 Read More


A 21-year-old man experienced unilateral vision loss associated with multiple atrophic chorioretinal lesions. He was treated for a presumptive diagnosis of acute retinal necrosis, but his vision did not improve with antiviral therapy. Over the course of several weeks, his symptoms progressed to involve both eyes. The fellow eye showed characteristic yellow-white placoid lesions, prompting treatment with oral corticosteroids for acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Despite high-dose therapy with prednisone 80 mg daily, the patient developed the acute onset of mental status changes within the next several days. Neuroimaging revealed multifocal large-vessel strokes associated with cerebral edema; these infarcts led to herniation and death. Postmortem histopathologic examination confirmed granulomatous inflammation in both ocular and cerebral vasculatures. Together with findings from multimodal imaging obtained throughout this patient’s clinical course, our findings support the notion that granulomatous choroiditis is the mechanism of the ocular lesions seen in APMPPE. This granulomatous inflammation can also affect cerebral vessels, leading to strokes.

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Posted on June 20, 2019
Posted in: Axon Injury & Repair, Publications Authors: