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From the Departments of Neurology (D.F. Tang-Wai and N.R. Graff-Radford, and Drs. Boeve, Caselli, Knopman, and Petersen), Pathology (Dr. Dickson), Laboratory Medicine and Pathology (Dr. Parisi), and Neuroscience and Pharmacology (R. Crook), Mayo Alzheimer’s Disease Research Center, Mayo Clinic Rochester (Drs. Boeve, Parisi, Knopman, and Petersen), MN; Mayo Clinic Jacksonville (D.F. Tang-Wai, N.R. Graff-Radford, and R. Crook, and Dr. Dickson), FL; and Mayo Clinic Scottsdale (Dr. Caselli), AZ.
Address correspondence and reprint requests to David F. Tang-Wai, University of Toronto, University Health Network, Department of Medicine (Neurology), Toronto Western Hospital, 399 Bathurst Street, Suite 5WW-441, Toronto, Ontario, Canada M5T 2S3; e-mail: david.tang-wai{at}uhn.on.ca
Objective: To examine the clinical, genetic, and neuropathologic features of posterior cortical atrophy (PCA). Design/Methods: Using a broad definition of PCA as a syndrome with the insidious onset of visual dysfunction in the absence of primary ophthalmologic causes, the authors identified and then reviewed the presenting signs and symptoms, ApoE genotypes, tau haplotypes, and neuropathologic findings when available of PCA cases from two dementia research centers collected over the past 14 years.
Results: The authors identified 40 PCA cases. Their mean age at symptom onset was 60.5 ± 8.9 years. There were twice as many women as men in the series. The principal types of visual impairment were simultanagnosia (82%) and visual field defect (47.5%). Acalculia, alexia, and anomia were also common. Insight was preserved in almost all (95%) early in the disorder. Neither apoE 
4 nor tau haplotype frequencies were different from typical Alzheimer disease (AD). Nine patients had died and underwent postmortem examination. Seven autopsied cases had AD pathology but when compared to typical AD, the neurofibrillary tangle (NFT) densities were significantly higher in Brodmann areas 17 and 18 (p < 0.05) and significantly lower in the hippocampus (p < 0.05). Two cases had corticobasal degeneration with maximal involvement of tau positive glial pathology in the posterior parietal lobe and Brodmann areas 17 and 18.
Conclusions: PCA is a distinctive dementia syndrome in which the most pronounced pathologic involvement is in the occipitoparietal regions independent of the specific underlying pathology. AD was the most common pathologic cause, but its regional distribution differed from typical AD.
Received December 4, 2003. Accepted in final form June 7, 2004.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the October 12 issue to find the title link for this article.
Presented at the annual meeting of the American Academy of Neurology; Honolulu, HI; April 2003.
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