Advertisement

Posterior Cortical Atrophy

  • Ernest Y. S. FungEmail author
  • Mark O. Herring
Chapter

Abstract

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).

Keywords

Posterior cortical atrophy Visuospatial functioning Occipital lobes Parietal lobes Atypical Alzheimer’s disease Neuro-ophthalmology Neurology Neuropsychology 

References

  1. 1.
    Crutch SJ, Lehmann M, Schott JM, Rabinovici GD, Rossor MN, Fox NC. Posterior cortical atrophy. Lancet Neurol. 2012;11(2):170–8.CrossRefGoogle Scholar
  2. 2.
    Shakespeare TJ, Yong KX, Foxe D, Hodges J, Crutch SJ. Pronounced impairment of everyday skills and self-care in posterior cortical atrophy. J Alzheimers Dis. 2015;43(2):381–4.CrossRefGoogle Scholar
  3. 3.
    Mendez MF, Ghajarania M, Perryman KM. Posterior cortical atrophy: clinical characteristics and differences compared to Alzheimer’s disease. Dement Geriatr Cogn Disord. 2002;14(1):33–40.CrossRefGoogle Scholar
  4. 4.
    Tang-Wai DF, Graff-Radford NR, Boeve BF, et al. Clinical, genetic, and neuropathologic characteristics of posterior cortical atrophy. Neurology. 2004;63(7):1168–74.CrossRefGoogle Scholar
  5. 5.
    Crutch SJ, Schott JM, Rabinovici GD, et al. Shining a light on posterior cortical atrophy. Alzheimers Dement. 2013;9(4):463–5.CrossRefGoogle Scholar
  6. 6.
    Crutch SJ, Schott JM, Rabinovici GD, et al. Consensus classification of posterior cortical atrophy. Alzheimers Dement. 2017;13(8):870–84.CrossRefGoogle Scholar
  7. 7.
    Renner JA, Burns JM, Hou CE, McKeel DW, Storandt M, Morris JC. Progressive posterior cortical dysfunction: a clinicopathologic series. Neurology. 2004;63(7):1175–80.CrossRefGoogle Scholar
  8. 8.
    Tang-Wai DF, Josephs KA, Boeve BF, Dickson DW, Parisi JE, Petersen RC. Pathologically confirmed corticobasal degeneration presenting with visuospatial dysfunction. Neurology. 2003;61(8):1134–5.CrossRefGoogle Scholar
  9. 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
  10. 10.
    Lehmann M, Crutch SJ, Ridgway GR, et al. Cortical thickness and voxel-based morphometry in posterior cortical atrophy and typical Alzheimer’s disease. Neurobiol Aging. 2011;32(8):1466–76.CrossRefGoogle Scholar
  11. 11.
    Migliaccio R, Agosta F, Rascovsky K, et al. Clinical syndromes associated with posterior atrophy: early age at onset AD spectrum. Neurology. 2009;73(19):1571–8.CrossRefGoogle Scholar
  12. 12.
    Nestor PJ, Caine D, Fryer TD, Clarke J, Hodges JR. The topography of metabolic deficits in posterior cortical atrophy (the visual variant of Alzheimer’s disease) with FDG-PET. J Neurol Neurosurg Psychiatry. 2003;74(11):1521–9.CrossRefGoogle Scholar
  13. 13.
    Whitwell JL, Jack CR, Kantarci K, et al. Imaging correlates of posterior cortical atrophy. Neurobiol Aging. 2007;28(7):1051–61.CrossRefGoogle Scholar
  14. 14.
    Hof PR, Vogt BA, Bouras C, Morrison JH. Atypical form of Alzheimer’s disease with prominent posterior cortical atrophy: a review of lesion distribution and circuit disconnection in cortical visual pathways. Vision Res. 1997;37(24):3609–25.CrossRefGoogle Scholar
  15. 15.
    Caso F, Agosta F, Mattavelli D, et al. White Matter Degeneration in Atypical Alzheimer Disease. Radiology. 2015;277(1):162–72.CrossRefGoogle Scholar
  16. 16.
    Schott JM, Crutch SJ, Carrasquillo MM, et al. Genetic risk factors for the posterior cortical atrophy variant of Alzheimer’s disease. Alzheimers Dement. 2016;12(8):862–71.CrossRefGoogle Scholar
  17. 17.
    Yong KX, Rajdev K, Shakespeare TJ, Leff AP, Crutch SJ. Facilitating text reading in posterior cortical atrophy. Neurology. 2015;85(4):339–48.CrossRefGoogle Scholar
  18. 18.
    Westervelt HJ, McCaffrey RJ. Neuropsychological functioning in chronic Lyme disease. Neuropsychol Rev. 2002;12(3):153–77.CrossRefGoogle Scholar
  19. 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. 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. 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
  22. 22.
    CD1 O, Bongianni M, Tonoli G, Ferrari S, Hughson AG, Groveman BR, Fiorini M, Pocchiari M, Monaco S, Caughey B, Zanusso G. A test for Creutzfeldt-Jakob disease using nasal brushings. N Engl J Med. 2014;371(6):519–29.CrossRefGoogle Scholar
  23. 23.
    Jackson GS, Burk-Rafel J, Edgeworth JA, Sicilia A, Abdilahi S, Korteweg J, Mackey J, Thomas C, Wang G, Mead S, Collinge J. A highly specific blood test for vCJD. Blood. 2014;123(3):452–3.CrossRefGoogle Scholar
  24. 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

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Atlanta Neuropsychology, LLC, NP Neuropsychology, LLCAtlantaUSA
  2. 2.Oregon Neurology AssociatesSpringfieldUSA

Personalised recommendations