pp 1–10 | Cite as

Medical Decision Making by Patients in the Locked-in Syndrome

Original Paper


The locked-in syndrome (LIS) is a state of profound paralysis with preserved awareness of self and environment who typically results from a brain stem stroke. Although patients in LIS have great difficulty communicating, their consciousness, cognition, and language usually remain intact. Medical decision-making by LIS patients is compromised, not by cognitive impairment, but by severe communication impairment. Former systems of communication that permitted LIS patients to make only “yes” or “no” responses to questions was sufficient to validate their consent for simple medical decisions but not for consequential medical decisions such as whether to refuse further life-sustaining therapy. Emerging technologies including computer-tracking assistance of retained partial motor function and brain-computer interfaces promise to improve the communication ability of LIS patients and, when better developed, will allow them to fully exercise their right to actively participate in consequential medical decisions about their own medical care. Although so-called “covert cognition” patients clinically diagnosed as vegetative state that are shown to be aware by functional neuroimaging maybe metaphorically considered locked-in, it is nosologically more coherent not to categorize them formally as LIS.


Locked-in syndrome Communication Medical decision-making Vegetative state Minimally conscious state fMRI 


  1. 1.
    Plum, Fred and Jerome B. Posner. 1966. The diagnosis of stupor and coma. Philadelphia: F. A. Davis Co.Google Scholar
  2. 2.
    Hayashi, H., and S. Kato. 1989. Total manifestations of amyotrophic lateral sclerosis. ALS in the totally locked-in state. Journal of the Neurological Sciences 93: 19–35.CrossRefGoogle Scholar
  3. 3.
    American Congress of Rehabilitation Medicine. 1995. Recommendations for use of uniform nomenclature pertinent to patients with severe alterations in consciousness. Archives of Physical Medicine and Rehabilitation 76: 205–209.CrossRefGoogle Scholar
  4. 4.
    Bauby, Jean-Dominique. 1997. The diving bell and the butterfly. New York: Alfred A. Knopf.Google Scholar
  5. 5.
    Mozersky, Judy. 1996. Locked-in: A young Woman’s battle with stroke. Ottawa: The Golden Dog Press.Google Scholar
  6. 6.
    Bernat, James L. 1990. Ethical considerations in the locked-in syndrome. In Ethics at the Bedside, ed. Charles M. Culver, 87–98. Hanover: University Press of New England.Google Scholar
  7. 7.
    Chisholm, Nick, and Grant Gillett. 2005. The patient’s journey: Living with locked-in syndrome. British Medical Journal 331: 94–97.CrossRefGoogle Scholar
  8. 8.
    Laureys, Steven, Frédérick Pellas, Phillipe Van Eeckhout, et al. 2005. The locked-in syndrome: What is it like to be conscious but paralyzed and voiceless? Progress in Brain Research 150: 495–512.CrossRefGoogle Scholar
  9. 9.
    Lulé, D., C. Zickler, S. Häcker, et al. 2009. Life can be worth living in locked-in syndrome. Progress in Brain Research 177: 339–351.CrossRefGoogle Scholar
  10. 10.
    Maiser, Samuel, Ashish Kabir, and David Sabsevitz. 2016. Locked-in syndrome: Case report and discussion of decisional capacity. Journal of Pain and Symptom Management. 51: 789–793.CrossRefGoogle Scholar
  11. 11.
    Dumas, Alexandre. 1909. The Count of Monte Cristo. New York: Dutton, vol. 2: p. 43.Google Scholar
  12. 12.
    Schnakers, Caroline, S. Majerus, S. Goldman, et al. 2008. Cognitive function in the locked-in syndrome. Journal of Neurology 255: 323–330.CrossRefGoogle Scholar
  13. 13.
    Smith, Eimear, and Mark Delargy. 2005. Locked-in syndrome. British Medical Journal 330: 406–409.CrossRefGoogle Scholar
  14. 14.
    Posner, Jerome B., Clifford B. Saper, Nicholas D. Schiff, and Fred Plum. 2007. Plum and Posner’s diagnosis of stupor and coma. 4th ed, 363. New York: Oxford University Press.Google Scholar
  15. 15.
    Bernat, James L. 2006. Chronic disorders of consciousness. Lancet 367: 1181–1192.CrossRefGoogle Scholar
  16. 16.
    León-Carrión, José., Phillippe Van Eeckhout, and Maria del Rosario Dominguez-Morales. 2002. Review of subject: The locked-in syndrome: A syndrome looking for a therapy. Brain Injury 16: 555–569.CrossRefGoogle Scholar
  17. 17.
    McCusker, Elizabeth A., Richard A. Rudick, Gerald W. Honch, and Robert C. Griggs. 1982. Recovery from the ‘locked-in’ syndrome. Archives of Neurology 39: 145–147.CrossRefGoogle Scholar
  18. 18.
    Hocker, Sarah, and Eelco F.M. Wijdicks. 2015. Recovery from locked-in syndrome. JAMA Neurology 72: 832–833.CrossRefGoogle Scholar
  19. 19.
    Patterson, James R., and Martin Grabois. 1986. Locked-in syndrome: A review of 139 cases. Stroke 17: 758–764.CrossRefGoogle Scholar
  20. 20.
    Richard, I., Y. Péreon, P. Guiheneu, B. Nogues, B. Perrouin-Verbe, and J.F. Mathe. 1995. Persistence of distal motor control in the locked-in syndrome: A review of 11 patients. Paraplegia 33: 640–646.Google Scholar
  21. 21.
    Thadani, V.M., D.L. Rimm, L. Urquhart, L. Fisher, P.D. Williamson, R. Enriquez, J.H. Kim, and L.L. Levy. 1991. Locked-in syndrome for 27 years following a viral illness: Clinical and pathologic findings. Neurology 41: 498–500.CrossRefGoogle Scholar
  22. 22. (Accessed March 12, 2017).
  23. 23.
    Hochberg, Leigh R., and Merit E. Cudkowicz. 2014. Locked in, but not out? Neurology 82: 1852–1853.CrossRefGoogle Scholar
  24. 24.
    Combaz, A., C. Chatelle, A. Robben, et al. 2013. A comparison of two spelling brain-computer interfaces based on visual P3 and SSVEP in locked-in syndrome. PLoS One Sep 25;8(9):e73691.Google Scholar
  25. 25.
    Stoll, Josef, Camille Chatelle, Olivia Carter, Christof Koch, Steven Laureys, and Wolfgang Einhäuser. 2013. Pupil responses allow communication in locked-in syndrome patients. Current Biology 23: R647–R648.CrossRefGoogle Scholar
  26. 26.
    Wilhelm, B., M. Jordan, and N. Birbaumer. 2006. Communication in locked-in syndrome: Effects of imagery on salivary pH. Neurology 67: 534–535.CrossRefGoogle Scholar
  27. 27.
    Gallegos-Ayala, Guillermo, Adrian Furdea, Kouji Takano, Carolin A. Ruf, Herta Flor, and Niels Birbaumer. 2014. Brain communication is a completely locked-in patient using bedside near-infrared spectroscopy. Neurology 82: 1930–1932.CrossRefGoogle Scholar
  28. 28.
    Lugo, Zulay R., Marie-Aurélie Bruno, Olivia Gosseries, et al. 2015. Beyond the gaze: Communicating in locked-in syndrome. Brain Injury 29: 1056–1961.CrossRefGoogle Scholar
  29. 29.
    Doble, Jennifer E., Andrew J. Haig, Christopher Anderson, and Richard Katz. 2003. Impairment, activity, participation, life satisfaction, and survival in persons with locked-in syndrome for over a decade: Follow-up on a previously reported cohort. Journal of Head Trauma Rehabilitation 18: 435–444.CrossRefGoogle Scholar
  30. 30.
    McDonald, Evelyn R., Hillel Al, and Sue A. Wiedenfeld. 1996. Evaluation of the psychological status of ventilatory-supported patients with ALS/MND. Palliative Medicine 10: 35–41.CrossRefGoogle Scholar
  31. 31.
    Albrecht, Gary L., and Patrick J. Devlieger. 1999. The disability paradox: High quality of life against all odds. Social Science & Medicine 48: 977–988.CrossRefGoogle Scholar
  32. 32.
    Bruno, Marie-Aurélie, Jan L. Bernheim, Didier Ledoux, Frédéric Pellas, Athena Demertzi, and Steven Laureys. 2011. A survey on self-assessed well-being in a cohort of chronic locked-in syndrome patients: Happy majority, miserable minority. BMJ Open 1 (e000039): 1–9.Google Scholar
  33. 33.
    Demertzi, Athena, Ralf J. Jox, Eric Racine, and Steven Laureys. 2014. A European survey on attitudes towards pain and end-of-life issues in locked-in syndrome. Brain Injury 28: 1209–1215.CrossRefGoogle Scholar
  34. 34.
    Bernat, James L. 2008. Ethical issues in neurology, 3rd ed. Philadelphia: Lippincott Williams & Wilkins, pp. 24–36.Google Scholar
  35. 35.
    Rousseaux, M., E. Castelnot, P. Rigaux, O. Kozlowski, and F. Danzé. 2009. Evidence of persisting cognitive impairment in a case series of patients with locked-in syndrome. Journal of Neurology, Neurosurgery and Psychiatry 80: 166–170.CrossRefGoogle Scholar
  36. 36.
    Connolly, Sheelah, Miriam Glavin, and Orla Hardiman. 2015. End-of-life management in patients with amyotrophic lateral sclerosis. Lancet Neurology 14: 435–442.CrossRefGoogle Scholar
  37. 37.
    Applebaum Paul, S., and T. Grisso. 1988. Assessing patients' capacities to consent to treatment. New England Journal of Medicine 319: 1635–1638.CrossRefGoogle Scholar
  38. 38.
    Laureys, Steven, Gastone G. Celesia, Francois Cohadon, et al. 2010. Unresponsive wakefulness syndrome: A new name for the vegetative state or apallic syndrome. BMC Medicine 8 (68): 1–4.Google Scholar
  39. 39.
    Formisano, R., F. Pistoia, and M. Sarà. 2011. Disorders of consciousness: A taxonomy to be changed? Brain Injury 25: 638–639.CrossRefGoogle Scholar
  40. 40.
    Giacino, Joseph T., Joseph J. Fins, Steven Laureys, and Nicholas D. Schiff. 2014. Disorders of consciousness after acquired brain injury: The state of the science. Nature Reviews Neurology 10: 99–114.CrossRefGoogle Scholar
  41. 41.
    Stender, Johan, Olivia Gosseries, Marie-Aurélie Bruno, et al. 2014. Diagnostic precision of PET imaging and functional MRI in disorders of consciousness: A clinical validation study. Lancet 384: 514–522.CrossRefGoogle Scholar
  42. 42.
    Owen, Adrian M., Martin R. Coleman, Melanie Boly, Matthew H. Davis, Steven Laureys, and John D. Pickard. 2006. Detecting awareness in the vegetative state. Science 313: 1402.CrossRefGoogle Scholar
  43. 43.
    Monti, Martin M., Audrey Vanhaudenhuyse, Martin R. Coleman, Melanie Boly, John D. Pickard, Luaba Tshibanda, Adrian M. Owen, and Steven Laureys. 2010. Willful modulation of brain activity in disorders of consciousness. New England Journal of Medicine 362: 579–589.CrossRefGoogle Scholar
  44. 44.
    Cruse, D., S. Chennu, C. Chatelle, D. Fernandez-Espejo, T.A. Bekinschtein, J.D. Pickard, S. Laureys, and A.M. Owen. 2012. Relationship between etiology and covert cognition in the minimally conscious state. Neurology 78: 816–822.CrossRefGoogle Scholar
  45. 45.
    Ropper, Allan H. 2010. Cogito ergo sum by MRI. New England Journal of Medicine 362: 648–649.CrossRefGoogle Scholar
  46. 46.
    Celesia, Gastone G. 2013. Conscious awareness in patients in vegetative states: Myth or reality? Current Neurology and Neuroscience Report 13 (11): 395.CrossRefGoogle Scholar
  47. 47.
    Bernat, James L. 2013. Increasing awareness in unawareness. JAMA Neurology 70: 1231–1232.Google Scholar
  48. 48.
    Coleman, M.R., M.H. Davis, J.M. Rodd, T. Robson, A. Ali, A.M. Owen, and J.D. Pickard. 2009. Toward the routine use of brain imaging to aid the clinical diagnosis of disorders of consciousness. Brain 132: 2541–2552.CrossRefGoogle Scholar
  49. 49.
    Bernat, James L. 2017. Nosologic considerations in disorders of consciousness. Annals of Neurology 82: 863–865.CrossRefGoogle Scholar
  50. 50.
    Bernat, James L. 2016. The medical practice impact of functional neuroimaging studies in patients with disorders of consciousness. In Brain function and responsiveness in disorders of consciousness, ed. Martin M. Monti and Walter G. Sannita, 157–169. Heidelberg: Springer.Google Scholar
  51. 51.
    Bardin, Jonathan C., Joseph J. Fins, Douglas I. Katz, Jennifer Hersh, Linda A. Heier, Karsten Tabelow, Jonathan P. Dyke, Douglas J. Ballon, Nicholas D. Schiff, and Henning U. Voss. 2011. Dissociations between behavioral and functional magnetic resonance imaging-based evaluations of cognitive function after brain injury. Brain 134: 769–782.CrossRefGoogle Scholar
  52. 52.
    Chaudhary, Ujwal, Bin Xia, Stefano Silvoni, Leonardo G. Cohen, and Niels Birbaumer. 2017. Brain-computer interface-based communication in the completely locked-in syndrome. PLoS Biology Jan 31;15(1):e1002593.Google Scholar
  53. 53.
    Vansteensel, Mariska J., Elmar G.M. Pels, Martin G. Bleichner, Mariana P. Branco, Timothy Denison, Zachary V. Freudenburg, Peter Gosselaar, Sacha Leinders, Thomas H. Ottens, Max A. van den Boom, Peter C. van Rijen, Erik J. Aarnoutse, and Nick F. Ramsey. 2016. Fully implanted brain-computer interface in a locked-in patient with ALS. New England Journal of Medicine 375: 2060–2066.CrossRefGoogle Scholar
  54. 54.
    Naci, Lorina, and Adrian M. Owen. 2013. Making every word count for nonresponsive patients. JAMA Neurology 70: 1235–1241.Google Scholar
  55. 55.
    Placidi, Giuseppe, Andrea Petracca, Mateo Spezialetti, and Daniela Iacoviello. 2016. A modular framework for EEG web-based binary brain-computer interfaces to recover communication abilities in impaired people. Journal of Medical Systems 40 (1): 34.CrossRefGoogle Scholar
  56. 56.
    United Nations Division for Social Policy and Development Disability: Convention on the Rights of Persons with Disabilities and Optional Protocol. and (accessed March 2, 2018).

Copyright information

© Springer Science+Business Media B.V., part of Springer Nature 2018

Authors and Affiliations

  1. 1.Geisel School of Medicine at DartmouthHanoverUSA

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