Neurologic Complications of Acute Posterior Multifocal Placoid Pigment Epitheliopathy: A Case Series of 4 Patients

Abstract

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a self-limited idiopathic inflammatory ophthalmologic condition with characteristic funduscopic and fluorescein angiography findings. It is typically characterized by a flu-like prodrome followed by monocular or binocular vision loss. Often, prognosis is excellent with complete or near-complete recovery of vision. Rarely, however, APMPPE is associated with neurologic complications, including meningitis, cerebral vasculitis, and stroke. Treatment in patients with central nervous system (CNS) involvement involves steroids and ultimately other immunosuppressive therapy, as there can be significant resulting morbidity and mortality otherwise. Evidence or guidelines regarding duration of treatment are lacking. We present 4 patients diagnosed with APMPPE who demonstrate the spectrum of neurologic sequelae associated with APMPPE. The first 2 cases highlight cerebrospinal fluid lymphocytic pleocytosis as an indicator of active CNS inflammation and the potential utility of serial lumbar punctures (LPs) to guide treatment duration. Cases 3 and 4 demonstrate the neurovascular complications seen in CNS vasculitis. Case 4 also highlights the potential use of magnetic resonance vessel wall imaging (VWI) as a noninvasive means for disease surveillance and treatment guidance. This case series emphasizes the importance of recognition by neurologists of APMPPE as an entity associated with strokes and cerebral vasculitis in order to provide appropriate and timely treatment. Active CNS inflammation warrants continued aggressive immunosuppressant treatment, and we propose that serial LPs and/or magnetic resonance VWIs may be effective tools to guide disease surveillance and subsequent treatment duration.

Keywords: cerebral vascular disease; headache; meningitis; uveitis; vasculitis.

Figure 1.

Ophthalmologic findings for case 1 (A-C) and case 2 (D-F). A, Fundus photograph of the left eye showing multiple coalescing creamy white lesions at the level of the retinal pigmented epithelium (RPE). B, Fluorescein angiography of the left eye in early arteriovenous phase showing hypofluorescence. C, Fluorescein angiography of the left eye in late arteriovenous phase showing hyperfluorescence. D, Fundus photograph of the right eye showing multiple coalescing creamy white lesions at the level of the RPE. E, Fluorescein angiography of the right eye in early arteriovenous phase showing hypofluorescence. F, Fluorescein angiography of the right eye in late arteriovenous phase showing hyperfluorescence.

Figure 2.

Neuroradiographic findings in case 3 (A-C) and case 4 (D-G). A, Conventional cerebral angiography reveals decreased flow and vascular beading of the right posterior cerebral artery (yellow arrows). B, Coronal reformatted computed tomography angiography shows diminutive basilar circulation and dilation of collateral vascular channels (yellow circle). C, Magnetic resonance diffusion-weighted imaging (DWI) shows ischemic infarctions throughout the posterior circulation consistent with catastrophic vasculitis (yellow arrows). D, Magnetic resonance vessel wall imaging (VWI) shows concentric perivascular enhancement consistent with vasculitis (orange arrow). E, Follow-up VWI shows resolution of perivascular enhancement with immunosuppressive therapy at 5-month follow-up. F, Vessel wall imaging shows persistent perivascular enhancement of the left middle cerebral artery after 5 months of immunosuppression (orange arrow). G, Diffusion-weighted imaging shows ischemic infarction in the left middle cerebral artery consistent with a vasculitic infarct (orange arrow).