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Acute posterior multifocal placoid pigment epitheliopathy: clinical presentation and risk of stroke and transient ischaemic attack
  1. Rachael L Niederer1,2,
  2. Priya D Samalia3,
  3. Yi-Hsing Chen4,5,
  4. Jonathan KS Goh3,
  5. Elisa Eleanor Cornish6,
  6. Stephen Guest7,
  7. Peter J McCluskey6,
  8. Lyndell L Lim3,8,
  9. Joanne L Sims1,
  10. Susan Lightman4,9,
  11. Oren Tomkins-Netzer4,10
  1. 1Ophthalmology, Auckland District Health Board, Auckland, New Zealand
  2. 2Department of Ophthalmology, The University of Auckland, Auckland, New Zealand
  3. 3Royal Victorian Eye and Ear Hospital, East Melbourne, Victoria, Australia
  4. 4Institute of Ophthalmology, University College London, London, UK
  5. 5Department of Ophthalmology, Chang Gung Memorial Hospital, Taoyuan, Taiwan
  6. 6Save Sight Institute, Faculty of Medicine & Health, University of Sydney, Sydney, New South Wales, Australia
  7. 7Ophthalmology, Waikato District Health Board, Hamilton, New Zealand
  8. 8Centre for Eye Research Australia, Melbourne, Victoria, Australia
  9. 9Moorfields Eye Hospital, London, England
  10. 10Department of Ophthalmology, Lady David Carmel Medical Center, Ruth and Bruch Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel
  1. Correspondence to Dr Priya D Samalia, Royal Victorian Eye and Ear Hospital, East Melbourne VIC 3002, Victoria, Australia; dr.priyasamalia{at}


Background/aims Acute posterior multifocal placoid pigment epitheliopathy is a rare but important disease that can be associated with life-threatening complications due to cerebral vasculitis. The primary objective was to determine the incidence of neurological complications and risk factors for stroke and transient ischaemic attack (TIA) associated with acute posterior multifocal placoid pigment epitheliopathy. Secondary objectives included the clinical presentation, visual outcomes and recurrence rates.

Methods This was a multicentre retrospective case series including 111 eyes from 60 subjects presenting from January 2009 to June 2020.

Results Median age at presentation was 29 years (IQR 24.7–35.1) and 36 subjects (60.0%) were male. 20 subjects (33.3%) reported a viral prodrome. Stroke and TIA were observed in seven subjects (11.7%). Older age was the only significant risk factor for stroke/TIA (p=0.042). Vision loss occurred in seven eyes, with four eyes (3.6%) having final visual acuity 6/15–6/60 and three eyes (2.7%) having visual acuity of 6/60 or worse. Recurrence occurred in 10 subjects (16.7%).

Conclusions The presence of headache cannot reliably predict those at risk of stroke/TIA. Individuals presenting with acute posterior multifocal pigment epitheliopathy should therefore undergo a clinical neurological review and work-up for cerebral vasculitis as deemed appropriate by the treating ophthalmologist and collaborating neurologist.

  • Immunology
  • Inflammation
  • Retina
  • Macula

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  • Contributors RLN and OT-N: study design. RLN and PDS: revision of the work. All authors: preparation and agreement on the final manuscript and agreement to be accountable for all aspects of the work. PDS: responsible for overall content as the guarantor

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.