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34 - ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY

from PART III: - VASCULAR CONDITIONS OF THE EYES, EARS, AND BRAIN

Published online by Cambridge University Press:  06 January 2010

Louis R. Caplan
Affiliation:
Beth Israel Deaconess Medical Center, Boston
Julien Bogousslavsky
Affiliation:
Valmont Clinique, Glion, Switzerland
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Summary

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an ophthalmologic syndrome rather than a specific entity, characterized by multiple cream-colored placoid lesions located in the posterior pole "lying at the level of the pigment epithelium and choroids". The ophthalmoscopic hallmarks of APMPPE consist of creamcolored, flat, and discrete placoid, without clear-cut marginal lesions at the level of the retinal pigment epithelium, masking the fundus view of the underlying choroids, which typically involve the macula but are never seen anterior to the equator. The fact that cardiovascular diseases (CVDs) occur in patients with APMPPE strongly supports the thesis that it represents a particular "uveo-cerebral vasculitic syndrome". Various etiologies have been found (infectious/postinfectious; vaccinations; inflammations; autoimmune diseases; vasculitis; paraneoplastic syndrome). The neurological complications of APMPPE are headache, aseptic meningitis, encephalitis, multiple sclerosis-like disease, and pseudo tumor cerebri. CVDs associated with APMPPE consist of ischemic cortical strokes and deep infarcts with striatocapsular infarctions.
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Publisher: Cambridge University Press
Print publication year: 2008

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