Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Cortical Blindness

  • Steven Z. RapcsakEmail author
  • G. Alex Hishaw
  • Tanya P. Lin
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_1354

Synonyms

Anton’s syndrome; Blindsight

Short Description or Definition

Cortical blindness refers to severe visual loss produced by bilateral damage to the geniculostriate visual pathways. The underlying pathophysiological mechanism involves direct destruction and/or deafferentation of primary visual cortex (striate cortex, Brodmann area 17, or V1). The term Anton’s syndrome is applied to patients with cortical blindness who demonstrate explicit denial or unawareness (anosognosia) of their visual impairment. Some patients with cortical blindness retain the ability to process visual stimuli without conscious awareness, a phenomenon that has been coined “blindsight.”

Categorization

Although no universally agreed upon classification system exists, patients with cortical blindness differ in terms of the severity of the visual loss, the presence/absence of spared visual abilities (conscious or unconscious), the degree of awareness of the deficit, and the extent of functional recovery. There...

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References and Readings

  1. Adair, J. C., Schwartz, R. L., & Barrett, A. M. (2003). Anosognosia. In K. M. Heilman & E. Valenstein (Eds.), Clinical neuropsychology (pp. 193–197). New York: Oxford University Press.Google Scholar
  2. Ajina, S., Kennard, C., Rees, G., & Bridge, H. (2015a). Motion area V5/MT+ response to global motion in the absence of V1 resembles early visual cortex. Brain, 138, 164–178.PubMedCrossRefPubMedCentralGoogle Scholar
  3. Ajina, S., Pestilli, F., Rokem, A., Kennard, C., & Bridge, H. (2015b). Human blindsight is mediated by an intact geniculo-extrastriate pathway. eLife, 4, e08935.PubMedPubMedCentralCrossRefGoogle Scholar
  4. Bisiach, E., & Geminiani, G. (1991). Anosognosia related to hemiplegia and hemianopia. In G. P. Prigatano & D. L. Schacter (Eds.), Awareness of deficit after brain injury: Clinical and theoretical issues (pp. 17–39). New York: Oxford University Press.Google Scholar
  5. Block, N. (2005). Two neural correlates of consciousness. Trends in Cognitive Sciences, 9, 46–52.PubMedCrossRefPubMedCentralGoogle Scholar
  6. Bridge, H., Thomas, O., Jbabdi, S., & Cowey, A. (2008). Changes in connectivity after visual cortical brain damage underlie altered visual function. Brain, 131, 1433–1444.PubMedCrossRefPubMedCentralGoogle Scholar
  7. Bridge, H., Hicks, S. L., Xie, J., Okell, T. W., Mannan, S., Alexander, I., Cowey, A., & Kennard, C. (2010). Visual activation of extra-striate cortex in the absence of V1 activation. Neuropsychologia, 48, 4148–4154.PubMedPubMedCentralCrossRefGoogle Scholar
  8. Bridge, H., Harrold, S., Holmes, E. A., Stokes, M. S., & Kennard, C. (2012). Vivid visual mental imagery in the absence of the primary visual cortex. Journal of Neuroscience, 259, 1062–1070.Google Scholar
  9. Buetti, S., Tamietto, M., Hervais-Adelman, A., Kerzel, D., de Gelder, B., & Pegna, A. J. (2013). Dissociation between goal-directed and discrete response localization in a patient with bilateral cortical blindness. Journal of Cognitive Neuroscience, 25, 1769–1775.PubMedCrossRefPubMedCentralGoogle Scholar
  10. Burra, N., Hervais-Adelman, A., Kerzel, D., Tamietto, M., de Gelder, B., & Pegna, A. J. (2013). Amygdala activation for eye contact despite complete cortical blindness. Journal of Neuroscience, 33, 10483–10489.PubMedCrossRefPubMedCentralGoogle Scholar
  11. Celesia, G. G., Brigell, M. G., & Vaphiades, M. S. (1997). Hemianopic anosognosia. Neurology, 49, 88–97.PubMedCrossRefPubMedCentralGoogle Scholar
  12. Cowey, A. (2010). The blindsight saga. Experimental Brain Research, 200, 3–24.PubMedCrossRefPubMedCentralGoogle Scholar
  13. Danckert, J., & Rossetti, Y. (2005). Blindsight in action: What can the different sub-types of blindsight tell us about the control of visually guided actions? Neuroscience and Biobehavioral Reviews, 29, 1035–1046.PubMedCrossRefPubMedCentralGoogle Scholar
  14. Dehaene, S., & Changeux, J.-P. (2011). Experimental and theoretical approaches to conscious processing. Neuron, 70, 200–227.PubMedCrossRefPubMedCentralGoogle Scholar
  15. Dehaene, S., Changeux, J.-P., Naccache, L., Sackur, J., & Sergent, C. (2006). Conscious, preconscious, and subliminal processing: A testable taxonomy. Trends in Cognitive Sciences, 10, 204–211.PubMedCrossRefPubMedCentralGoogle Scholar
  16. de Gelder, B., Tamietto, M., van Boxtel, G., Goebel, R., Sahraie, A., Van den Stock, J., Stienen, B. M. C., Weiskrantz, L., & Pegna, A. J. (2008). Intact navigation skills after bilateral loss of striate cortex. Current Biology, 18, R1128–R1129.PubMedCrossRefPubMedCentralGoogle Scholar
  17. de Gelder, B., Tamietto, M., Pegna, A. J., & Van den Stock, J. (2015). Visual imagery influences brain responses to visual stimulation in bilateral cortical blindness. Cortex, 72, 15–26.PubMedCrossRefPubMedCentralGoogle Scholar
  18. Goebel, R., Muckli, L., Zanella, F. E., Singer, W., & Stoerig, P. (2001). Sustained extrastriate cortical activation without visual awareness revealed by fMRI studies of hemianopic patients. Vision Research, 41, 1459–1474.PubMedCrossRefPubMedCentralGoogle Scholar
  19. Heilman, K. M. (1991). Anosognosia: Possible neuropsychological mechanisms. In G. P. Prigatano & D. L. Schacter (Eds.), Awareness of deficit after brain injury: Clinical and theoretical issues (pp. 53–62). New York: Oxford University Press.Google Scholar
  20. Hervais-Adelman, A., Legrande, B. L., Zhan, M., Tamietto, M., de Gelder, B., & Pegna, A. J. (2015). Looming sensitive cortical regions without V1 input: evidence from a patient with bilateral cortical blindness. Frontiers in Integrative Neuroscience, 9, 51.  https://doi.org/10.3389/fnint.2015.00051.CrossRefPubMedPubMedCentralGoogle Scholar
  21. Kerkhoff, G. (2000). Neurovisual rehabilitation: Recent developments and future directions. Journal of Neurology, Neurosurgery, and Psychiatry, 68, 691–706.PubMedPubMedCentralCrossRefGoogle Scholar
  22. Morris, J. S., DeGelder, B., Weiskrantz, L., & Dolan, R. J. (2001). Differential extrageniculostriate and amygdala responses to presentation of emotional faces in a cortically blind field. Brain, 124, 1241–1252.PubMedCrossRefPubMedCentralGoogle Scholar
  23. Pegna, A. J., Khateb, A., Lazeyras, F., & Seghier, M. L. (2005). Discriminating emotional faces without primary visual cortices involves right amygdala. Nature Neuroscience, 8, 24–25.PubMedCrossRefPubMedCentralGoogle Scholar
  24. Rees, G., Kreiman, G., & Koch, C. (2002). Neural correlates of consciousness in humans. Nature Reviews Neuroscience, 3, 261–270.PubMedCrossRefPubMedCentralGoogle Scholar
  25. Sahraie, A., Weiskrantz, L., Barbur, J. L., Simmons, A., Williams, S. C. R., & Brammer, M. J. (1997). Pattern of neural activity associated with conscious and unconscious processing of visual signals. Proceedings of the National Academy of Sciences, 94, 9406–9411.CrossRefGoogle Scholar
  26. Sahraie, A., Trevethan, C. T., MacLeod, M. J., Murray, A. D., Olson, J. A., & Weiskrantz, L. (2006). Increased sensitivity after repeated stimulation of residual spatial channels in blindsight. Proceedings of the National Academy of Sciences, 103, 14971–14976.CrossRefGoogle Scholar
  27. Sahraie, A., Trevethan, C. T., McLeod, M.-J., Weiskrantz, L., & Hunt, A. R. (2013). The continuum of detection and awareness of visual stimuli within the blindfield: from blindsight to sighted-sight. Investigative Ophthalmology and Visual Science, 54, 3579–3585.PubMedCrossRefPubMedCentralGoogle Scholar
  28. Solca, M., Guggisberg, A. G., Schnider, A., & Leemann, B. (2015). Facial blindsight. Frontiers in Human Neuroscience., 9, 522.  https://doi.org/10.3389/fnhum.2015.00522.CrossRefPubMedPubMedCentralGoogle Scholar
  29. Stoerig, P. (2006). Blindsight, conscious vision, and the role of primary visual cortex. Progress in Brain Research, 155, 217–234.PubMedCrossRefPubMedCentralGoogle Scholar
  30. Stoerig, P., & Cowey, A. (1997). Blindsight in man and monkey. Brain, 120, 535–559.PubMedCrossRefPubMedCentralGoogle Scholar
  31. Stoerig, P., & Cowey, A. (2007). Blindsight. Current Biology, 17, R822–R824.PubMedCrossRefPubMedCentralGoogle Scholar
  32. Tamietto, M., Cauda, F., Corazzini, L. L., Savazzi, S., Marzi, C. A., Goebel, R., Weiskrantz, L., & de Gelder, B. (2010). Collicular vision guides nonconscious behavior. Journal of Cognitive Neuroscience, 22, 888–902.PubMedCrossRefPubMedCentralGoogle Scholar
  33. Tamietto, M., Pullens, P., de Gelder, B., Weiskrantz, L., & Goebel, R. (2012). Subcortical connections to human amygdala and changes following destruction of the visual cortex. Current Biology, 22, 1449–1455.PubMedCrossRefPubMedCentralGoogle Scholar
  34. Trevethan, C. T., Sahraie, A., & Weiskrantz, L. (2007). Form discrimination in a case of blindsight. Neuropsychologia, 45, 2092–2103.PubMedCrossRefPubMedCentralGoogle Scholar
  35. Van den Stock, J., Tamietto, M., Sorger, B., Pichon, S., Grezes, J., & de Gelder, B. (2011). Cortico-subcortical visual, somatosensory, and motor activations for perceiving dynamic whole-body emotional expressions with and without striate cortex (V1). Proceedings of the National Academy of Science, 108, 16188–16193.CrossRefGoogle Scholar
  36. Van den Stock, J., Tamietto, M., Zhan, M., Heinecke, A., Hervais-Adelman, A., Legrand, L. B., Pegna, A. J., & de Gelder, B. (2014). Neural correlates of body and face perception following bilateral destruction of the primary visual cortices. Frontiers in Behavioral Neuroscience, 8, 30.  https://doi.org/10.3389/fnbeh.2014.00030.CrossRefPubMedPubMedCentralGoogle Scholar
  37. Weiskrantz, L. (1986). Blindsight: A case study and implications. Oxford: Clarendon Press.Google Scholar
  38. Weiskrantz, L. (1997). Consciousness lost and found. Oxford: Oxford University Press.Google Scholar

Copyright information

© Springer International Publishing AG (outside the USA) 2018

Authors and Affiliations

  • Steven Z. Rapcsak
    • 1
    • 2
    Email author
  • G. Alex Hishaw
    • 1
    • 2
  • Tanya P. Lin
    • 1
    • 2
  1. 1.Neurology SectionSouthern Arizona VA Healthcare SystemTucsonUSA
  2. 2.Department of NeurologyUniversity of ArizonaTucsonUSA