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Acute posterior multifocal placoid pigment epitheliopathy associated with cerebral vasculitis

J M Weinstein1, G H Bresnick, C L Bell

  • 1Department of Ophthalmology, University of Wisconsin, School of Medicine, Madison.

Journal of Clinical Neuro-Ophthalmology
|September 1, 1988
PubMed

Insights

Acute multifocal posterior placoid pigment epitheliopathy (APMPPE) can involve the brain. This case highlights APMPPE associated with cerebral vasculitis and two ischemic events, emphasizing the need for early CNS evaluation.

Area of Science:

  • Ophthalmology
  • Neurology
  • Immunology

Background:

  • Acute multifocal posterior placoid pigment epitheliopathy (APMPPE) is a rare, self-limiting retinal condition.
  • APMPPE has been linked to systemic complications, including cerebral vasculitis in a few reported cases.

Observation:

  • This report details a patient with APMPPE and confirmed cerebral vasculitis.
  • The patient experienced two distinct cerebral ischemic events separated by one month.
  • A significant three-month delay was noted between initial ocular symptoms and the second ischemic event.

Findings:

  • The case demonstrates a prolonged latency period between ocular manifestation of APMPPE and subsequent neurological events.
  • Angiographic evidence confirmed cerebral vasculitis in conjunction with APMPPE.
  • The occurrence of two separate ischemic events suggests a potentially aggressive or complex vasculitic process.

Implications:

  • Early recognition of the APMPPE-cerebral vasculitis association is crucial for timely intervention.
  • Prompt diagnosis and treatment of central nervous system (CNS) involvement may prevent severe neurological morbidity.
  • This case underscores the importance of a comprehensive systemic evaluation in patients presenting with APMPPE.

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