Introduction
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease causing acute visual symptoms predominantly in young adults. Central nervous system involvement is a rare manifestation, and CNS vasculitis is associated with increased mortality and morbidity. Patients with APMPPE who develop CNS manifestations should begin immunosuppressive therapy immediately to prevent further neurological complications. Here we discuss an interesting presentation of APMPPE in a young woman who developed acute ischemic stroke as a CNS complication.
Case Presentation
A 28-year-old woman developed progressive binocular blurry vision over the course of several months and was diagnosed with APMPPE on color fundus photography (Figure 1). A few months later, she presented to the ED with acute onset of right-sided weakness, sensory symptoms, and expressive aphasia. CT angiogram showed multifocal stenoses concerning for cerebral vasculitis (Figure 2). MRI showed multi-territorial infarcts and multiple enhancing leptomeningeal lesions (Figure 3). Lumbar puncture showed a lymphocytic pleocytosis. Leptomeningeal biopsy showed lymphocytic infiltrates. She received pulse steroids followed by long-term azathioprine treatment and recovered neurologically.
Clinical Impacts/Relevance
This case adds to the literature of rare but serious neurological complications of APMPPE, such as cerebral vasculitis. It highlights the need for a high level of suspicion as early treatment with immunosuppressive therapy can improve the outcomes.
Discussion
APMPPE is a rare immune-mediated chorioretinal disease that can present with fatal CNS complications. Physicians should carry a high index of suspicion for such CNS complications including cerebral vasculitis because it is imperative to begin early therapy with immunosuppressive