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Pseudoexfoliation syndrome is one of the most common causes of glaucoma worldwide.1 This complex disease can present a management challenge for ophthalmologists as the presence of pseudoexfoliation material can represent a spectrum of intraocular diseases including glaucomatous optic neuropathy, cataract formation, phacodonesis, lens subluxation, iris atrophy, poor mydriasis and a Fuchs’ like keratopathy.2 This syndrome was first described in 1917 by its appearance of bluish-grey flakes on the anterior lens capsule and was historically known solely as a disease of the eye.3–5 Evidence, however, indicates that pseudoexfoliation material can be found systemically within visceral organs, dermis and even the brain.6 ,7 Thus, pseudoexfoliation is a general disorder of extracellular matrix, but it is still unclear whether the changes within the extracellular matrix can lead to any systemic disease.
Several small-scale retrospective case–control studies have linked pseudoexfoliation syndrome to various maladies of ageing. These studies have shown associations with cardiovascular and cerebrovascular diseases,8–11 sensory neural hearing loss12 ,13 and Alzheimer's disease,14 as described later in this edition of BJO.
The connection of Alzheimer's disease with pseudoexfoliation syndrome was first considered after the discovery of Αβ crystallines in the amyloid plaques in Alzheimer's patients.15 These crystallines are the major protein found in lens capsules. Brain and eye produce amyloid peptide Aβ and these amyloids are found to occur in direct association with pseudoexfoliation material.16 ,17 Although these findings provide compelling evidence of an association between these two diseases, …
Contributors RED took part in conception and design, drafting the article and final approval of the version to be published. JSS took part in conception and design, revising the article critically for important intellectual content and final approval of the version to be published.
Funding Supported in part by National Institutes of Health contract P30-EY08098 (Bethesda, MD, USA), The Eye and Ear Foundation (Pittsburgh, PA, USA) and an unrestricted grant from Research to Prevent Blindness (New York, NY, USA).
Competing interests JSS receives royalties for intellectual property licensed by Massachusetts Institute of Technology and Massachusetts Eye and Ear Infirmary to Carl Zeiss Meditec, Inc.
Provenance and peer review Commissioned; internally peer reviewed.