Posterior cortical atrophy (PCA), also called Benson's syndrome, is a rare form of dementia which is considered a visual variant or an atypical variant of Alzheimer's disease (AD).[1][2][3] The disease causes atrophy of the posterior part of the cerebral cortex, resulting in the progressive disruption of complex visual processing.[4] PCA was first described by D. Frank Benson in 1988.[5][6]
PCA usually affects people at an earlier age than typical cases of Alzheimer's disease, with initial symptoms often experienced in people in their mid-fifties or early sixties.[4] This was the case with writer Terry Pratchett (1948–2015), who went public in 2007 about being diagnosed with PCA.[7] In rare cases, PCA can be caused by dementia with Lewy bodies and Creutzfeldt–Jakob disease.[6][4]
^"Visual variant of Alzheimer's disease - EyeWiki". eyewiki.aao.org. Retrieved 2017-11-07.
^Nestor PJ, Caine D, Fryer TD, Clarke J, Hodges JR (2003). "The topography of metabolic deficits in posterior cortical atrophy (the visual variant of Alzheimer's disease) with FDG-PET". J Neurol Neurosurg Psychiatry. 74 (11): 1521–1529. doi:10.1136/jnnp.74.11.1521. PMC 1738241. PMID 14617709.
^ abc"Posterior Cortical Atrophy". UCSF Memory and Aging Center. University of California, San Francisco. Retrieved 2011-10-22.
^Benson DF, Davis RJ, Snyder BD (July 1988). "Posterior Cortical Atrophy". Archives of Neurology. 45 (7): 789–793. doi:10.1001/archneur.1988.00520310107024. PMID 3390033.
^ ab"Posterior Cortical Atrophy". Martinos Center for Biomedical Imaging. Harvard University. 2009-01-19. Retrieved 2011-10-22.
^"Terry Pratchett pledges $1 million to Alzheimer's research". Alzheimer's Research Trust. 2011-07-29. Archived from the original on 2013-01-14. Retrieved 2011-07-29.
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