Posterior Cortical Atrophy
Posterior cortical atrophy (PCA) is a relatively rare neurodegenerative syndrome marked by prominent disturbance in visuoperceptual and visuospatial functioning. Its prevalence and incidence rates are unknown (Crutch SJ, Lehmann M, Schott JM, Rabinovici GD, Rossor MN, Fox NC, Lancet Neurol. 11:170-178, 2012). There has been increased awareness of this condition among the general public, with the diagnosis made by British author Sir Terry Pratchett (1948–2015). He was quite open about his journey with the disease, making several documentaries and appearing on a number of radio programs. He was initially misdiagnosed, which is typical with the challenges of making this diagnosis. This condition was also written about by American neurologist/author Dr. Oliver Sacks in his 2010 book, The Mind’s Eye, in which he describes this condition in one of his case reports.
In clinical settings, presenting complaints from patients with differential diagnosis of PCA often involve disturbance in activities of daily living, such as difficulties recognizing familiar routes, reading maps, or locating and organizing objects in the environment. They may report recent history of fender benders or more serious car accidents. Reading may have become challenging because of its visuospatial component, but verbal comprehension likely remains intact. In a recent study, patients with PCA were found to be more severely impaired in everyday skills and self-care (majority associated with visuoperceptual and visuospatial impairments) than their Alzheimer’s disease (AD) counterparts, while more AD patients showed high levels of impairment in stereotypic and motor behaviors, but they maintained motivation to keep in contact with friends or family (Shakespeare TJ, Yong KX, Foxe D, Hodges J, Crutch SJ. Journal of Alzheimer’s Disease 43:381–384, 2015).
KeywordsPosterior cortical atrophy Visuospatial functioning Occipital lobes Parietal lobes Atypical Alzheimer’s disease Neuro-ophthalmology Neurology Neuropsychology
- 9.Ropper AH, Samuels MA, Klein J. Adams and Victor’s principles of neurology. 10th ed. New York: McGraw-Hill Education Medical; 2014.Google Scholar
- 19.IG MK, Dickson DW, Lowe J, Emre M, O’Brien JT, Feldman H, Cummings J, Duda JE, Lippa C, Perry EK, Aarsland D, Arai H, Ballard CG, Boeve B, Burn DJ, Costa D, Del Ser T, Dubois B, Galasko D, Gauthier S, Goetz CG, Gomez-Tortosa E, Halliday G, Hansen LA, Hardy J, Iwatsubo T, Kalaria RN, Kaufer D, Kenny RA, Korczyn A, Kosaka K, Lee VM, Lees A, Litvan I, Londos E, Lopez OL, Minoshima S, Mizuno Y, Molina JA, Mukaetova-Ladinska EB, Pasquier F, Perry RH, Schulz JB, Trojanowski JQ, Yamada M, Consortium on DLB. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology. 2005;65:1863–72.CrossRefGoogle Scholar
- 20.Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA, Kertesz A, Lee SE, Miller BL, Reich SG, Riley DE, Tolosa E, Tröster AI, Vidailhet M, Weiner WJ. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013;80:496–503.CrossRefGoogle Scholar
- 21.Dubois B, Feldman HH, Jacova C, Hampel H, Molinuevo JL, Blennow K, DeKosky ST, Gauthier S, Selkoe D, Bateman R, Cappa S, Crutch S, Engelborghs S, Frisoni GB, Fox NC, Galasko D, Habert MO, Jicha GA, Nordberg A, Pasquier F, Rabinovici G, Robert P, Rowe C, Salloway S, Sarazin M, Epelbaum S, de Souza LC, Vellas B, Visser PJ, Schneider L, Stern Y, Scheltens P, Cummings JL. Advancing research diagnostic criteria for Alzheimer’s disease: the IWG-2 criteria. Lancet Neurol. 2014;13:614–29.CrossRefGoogle Scholar
- 24.McGuire LI, Peden AH, Orrú CD, Wilham JM, Appleford NE, Cbiol GM, Andrews M, Head MW, Caughey B, Will RG, Knight RSG, Green AJE. RT-QuIC analysis of cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease. Ann Neurol. 2012;72(2):278–85.Google Scholar