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An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes.

ABSTRACT: Purpose:To report an atypical case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with central nervous system (CNS) vasculitis and recurrent strokes. Observations:A 57 year-old female presented with APMPPE after a febrile illness and rash. She developed an acute infarct on magnetic resonance imaging. Computed tomography angiography of the cerebral vasculature was normal. Cerebrospinal fluid (CSF) analysis and an extensive serum lab workup were also unremarkable. She was treated with high-dose corticosteroids and eventually transitioned to methotrexate. A month after being on treatment she developed a second stroke. A cerebral angiogram was obtained and did not show evidence of CNS vasculitis. The methotrexate was eventually stopped and the prednisone was tapered. Approximately 3 months later she developed a third stroke and worsening APMPPE-associated maculopathy in both eyes. She was eventually started on oral cyclophosphamide. Conclusions & importance:Although rare, CNS vasculitis is a known complication of APMPPE. This case is atypical given the development of multiple recurrent strokes, lack of inflammatory evidence on CSF analysis, and normal imaging of the cerebral vasculature. This report highlights the need for a high level of clinical suspicion for CNS vasculitis with APMPPE despite noncontributory cerebral angiographic imaging and normal CSF analysis.

SUBMITTER: Berger E

PROVIDER: S-EPMC6872853 | biostudies-literature | 2019 Dec

REPOSITORIES: biostudies-literature

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An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes.

Berger Evan E Ghorayeb Ghassan G Hogg Jeffery J

American journal of ophthalmology case reports 20191109

<h4>Purpose</h4>To report an atypical case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with central nervous system (CNS) vasculitis and recurrent strokes.<h4>Observations</h4>A 57 year-old female presented with APMPPE after a febrile illness and rash. She developed an acute infarct on magnetic resonance imaging. Computed tomography angiography of the cerebral vasculature was normal. Cerebrospinal fluid (CSF) analysis and an extensive serum lab workup were also unremarka ...[more]

PMID: 31768473

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Project description:The purpose of this study was to evaluate potential insights into the pathogenesis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) using multimodal diagnostic imaging and laboratory evaluation in long-term follow-up. A retrospective, single-center case series was conducted on seven consecutive patients (14 eyes) who were given a diagnosis of APMPPE from March 1, 2011, through June 30, 2019 with at least three months of follow-up. Clinical characteristics (age, symptoms, visual acuity [VA]), laboratory testing including coxsackievirus titers, and multimodal imaging from fundus photography, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICG) were analyzed for each patient. The initial median VA was 20/71 and final median VA was 20/22. Coxsackievirus B (CVB) titers were elevated (≥ 1:80) in six of seven patients, with a four-fold increase in convalescent titers seen in two patients suggestive of recent infection. All patients were treated with oral corticosteroids, and five patients underwent corticosteroid-sparing immunomodulatory therapy. Initially, multifocal deep choroidal lesions were observed in the posterior pole corresponding to patches of hypocyanescence on ICG. Overlying retinal pigment epithelium (RPE) disease was observed on FAF, although this finding was not universally observed, suggesting that RPE disease may occur as a sequelae to unchecked choroidal inflammation. SD-OCT architectural changes confirmed outer retina and ellipsoid zone disruption. FA of active lesions showed early hypofluorescence and late hyperfluorescence with surrounding leakage while inactive disease showed areas of staining. Long-term follow-up of multimodal diagnostic imaging in APMPPE revealed that choroidal inflammation likely precedes RPE change and photoreceptor damage. Elevation of coxsackievirus titers with seroconversion may be associated with an infectious trigger in concert with immune-mediated disease in this posterior uveitis syndrome.

Project description:PurposeTo investigate the natural course of pachychoroid pigment epitheliopathy (PPE).DesignA retrospective cohort study.SubjectsFrom the Kyoto central serous chorioretinopathy (CSC) cohort consisting of 548 patients with CSC as of September 2020, we included consecutive unilateral patients with acute or chronic CSC between January 2013 and December 2016.MethodsAll patients underwent complete ophthalmic examination, including multimodal imaging such as fundus autofluorescence, spectral-domain optical coherence tomography, and fluorescein angiography/indocyanine green angiography and/or optimal coherence tomography angiography. The fellow eyes of eyes diagnosed with CSC were screened for PPE, and their natural course was evaluated. We also evaluated the association of ARMS2 rs10490924, CFH rs800292, TNFRSF10A rs13278062, and GATA5 rs6061548 genotypes with the natural course.Main outcome measuresIncidence of CSC, pachychoroid neovasculopathy, and pachychoroid geographic atrophy (GA).ResultsIn total, 165 patients with unilateral CSC (mean age, 55.7 ± 12.6 years; female, 22.4%) were included from the Kyoto CSC cohort. Among them, 148 (89.7%) were diagnosed as having PPE in their non-CSC eye. Survival analysis revealed that 16.8% of PPE eyes developed CSC during the 6-year follow up, whereas non-PPE eyes did not. Although genetic factors did not have significant association with CSC development (P > 0.05, log-rank test), choroidal vascular hyperpermeability (CVH) and subfoveal choroidal thickness (SFCT) were significantly associated with CSC incidence (P = 0.001, log-rank test). Survival analysis showed that eyes without CVH and eyes with SFCT < 300 μm did not develop CSC during the 6-year follow-up. Pachychoroid neovasculopathy developed in only 1 eye with PPE during a follow-up of 46.4 months. Pachychoroid GA did not develop in any of the studied eyes.ConclusionsThis study revealed a natural history of PPE in a relatively large Japanese cohort. Choroidal vascular hyperpermeability and SFCT were significant risk factors for the development of CSC in PPE eyes. Although the current results cannot be generalized for all eyes with PPE, these findings present an important clinical implication.

Dataset Information

An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes.

Publications

An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes.

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