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Berger E, Ghorayeb G, Hogg J. An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes. Am J Ophthalmol Case Rep. 2019;16:100574doi: 10.1016/j.ajoc.2019.100574.
Berger, E., Ghorayeb, G., & Hogg, J. (2019). An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes. American journal of ophthalmology case reports, 16100574. https://doi.org/10.1016/j.ajoc.2019.100574
Berger, Evan, et al. "An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes." American journal of ophthalmology case reports vol. 16 (2019): 100574. doi: https://doi.org/10.1016/j.ajoc.2019.100574
Berger E, Ghorayeb G, Hogg J. An atypical case of acute posterior multifocal placoid pigment epitheliopathy with recurrent strokes. Am J Ophthalmol Case Rep. 2019 Nov 09;16:100574. doi: 10.1016/j.ajoc.2019.100574. eCollection 2019 Dec. PMID: 31768473; PMCID: PMC6872853.
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Am J Ophthalmol Case Rep. 2019 Nov 09;16:100574. doi: 10.1016/j.ajoc.2019.100574. eCollection 2019 Dec.

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

American journal of ophthalmology case reports

Evan Berger, Ghassan Ghorayeb, Jeffery Hogg

Affiliations

  1. West Virginia University, Department of Ophthalmology & Visual Sciences, 1 Medical Center Drive, Morgantown, WV, United States.
  2. West Virginia University, Department of Radiology, 1 Medical Center Drive, Morgantown, WV, United States.

PMID: 31768473 PMCID: PMC6872853 DOI: 10.1016/j.ajoc.2019.100574

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.

© 2019 The Authors.

Keywords: APMPPE; Acute posterior multifocal placoid pigment epitheliopathy; Cerebral vasculitis; Stroke

Conflict of interest statement

The following authors have no financial disclosures: EB, GG, JH.

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