Skip to main content

Quality of Life in Locked-in Syndrome Survivors

  • Conference paper
Intensive Care Medicine

Abstract

Pseudocoma or locked-in syndrome was first described by Plum and Posner in 1966 [2]. The patient with locked-in syndrome is fully conscious but interaction with the external world is very limited due to anarthria, lower cranial nerve paralysis, and quadriplegia. Usually, but not always, the anatomy of the responsible lesion in the brainstem is such that locked-in syndrome patients are left with the capacity to use vertical eye movements and blinking to communicate. The earliest example of a ‘locked-in patient’ was described in 1854 in Alexandre Dumas’s novel “The Count of Monte Cristo”. Some years later, Zola described a woman who was paralyzed and “buried alive in a dead body” but could communicate via eye movements in his book “Therese Raquin”. Dumas and Zola thus described the locked-in syndrome before the medical community did.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Tavalaro J, Tayson R (1997) Look Up for Yes. Kodansha America, Inc, New York

    Google Scholar 

  2. Plum F, Posner JB (1966) The Diagnosis of Stupor and Coma, 1st ed. FA Davis, Philadelphia

    Google Scholar 

  3. Bauby JD (1998) The Diving bell and the Butterfly: A Memoir of Life in Death (original title: Le scaphandre et le papillon). Vintage, New York

    Google Scholar 

  4. American Congress of Rehabilitation Medicine (1995) Recommendations for use of uniform nomenclature pertinent to patients with severe alterations of consciousness. Arch Phys Med Rehabil 76:205–209

    Article  Google Scholar 

  5. Bauer G, Gerstenbrand F, Rumpl E (1979) Varieties of the locked-in syndrome. J Neurol 221: 77–91

    Article  PubMed  CAS  Google Scholar 

  6. Plum F, Posner JB (1983) The Diagnosis of Stupor and Coma, 3rd ed. FA Davis, Philadelphia

    Google Scholar 

  7. Patterson JR, Grabois M (1986) Locked-in syndrome: a review of 139 cases. Stroke 17:758–764

    PubMed  CAS  Google Scholar 

  8. Dehaene I, Dom R (1982) A mesencephalic locked-in syndrome. J Neurol 227:255–259

    Article  PubMed  CAS  Google Scholar 

  9. Golubovic V, Muhvic D, Golubovic S (2004) Posttraumatic locked-in syndrome with an unusual three day delay in the appearance. Coll Antropol 28:923–926

    PubMed  Google Scholar 

  10. Keane JR (1986) Locked-in syndrome after head and neck trauma. Neurology 36:80–82

    PubMed  CAS  Google Scholar 

  11. Laureys S, Pellas F, Van Eeckhout P, et al (2005) The locked-in syndrome: what is it like to be conscious but paralyzed and voiceless? Prog Brain Res 150:495–511

    Article  PubMed  Google Scholar 

  12. Ragazzoni A, Grippo A, Tozzi F, Zaccara G (2000) Event-related potentials in patients with total locked-in state due to fulminant Guillain-Barre syndrome. Int J Psychophysiol 37:99–109

    Article  PubMed  CAS  Google Scholar 

  13. O’Donnell PP (1979) ‘Locked-in syndrome’ in postinfective polyneuropathy. Arch Neurol 36:860

    CAS  Google Scholar 

  14. Kotchoubey B, Lang S, Winter S, Birbaumer N (2003) Cognitive processing in completely paralyzed patients with amyotrophic lateral sclerosis. Eur J Neurol 10:551–558

    Article  PubMed  CAS  Google Scholar 

  15. Sandin RH, Enlund G, Samuelsson P, Lennmarken C (2000) Awareness during anaesthesia: a prospective case study. Lancet 355:707–711

    Article  PubMed  CAS  Google Scholar 

  16. Majerus S, Gill-Thwaites H, Andrews K, Laureys S (2005) Behavioral evaluation of consciousness in severe brain damage. Prog Brain Res 150:397–413

    Article  PubMed  Google Scholar 

  17. Leon-Carrion J, van Eeckhout P, Dominguez-Morales Mdel R, Perez-Santamaria FJ (2002) The locked-in syndrome: a syndrome looking for a therapy. Brain Inj 16:571–582

    Google Scholar 

  18. Leon-Carrion J, van Eeckhout P, Dominguez-Morales Mdel R (2002) The locked-in syndrome: a syndrome looking for a therapy. Brain Inj 16:555–569

    Google Scholar 

  19. Bassetti C, Mathis J, Hess CW (1994) Multimodal electrophysiological studies including motor evoked potentials in patients with locked-in syndrome: report of six patients. J Neurol Neurosurg Psychiatry 57:1403–1406

    Article  PubMed  CAS  Google Scholar 

  20. Gutling E, Isenmann S, Wichmann W (1996) Electrophysiology in the locked-in-syndrome. Neurology 46:1092–1101

    PubMed  CAS  Google Scholar 

  21. Jacome DE, Morilla-Pastor D (1990) Unreactive EEG: pattern in locked-in syndrome. Clin Electroencephalogr 21:31–36

    PubMed  CAS  Google Scholar 

  22. The Multi-Society Task Force on PVS (1994) Medical aspects of the persistent vegetative state (1). N Engl J Med 330:1499–1508

    Article  Google Scholar 

  23. Levy DE, Sidtis JJ, Rottenberg DA, et al (1987) Differences in cerebral blood flow and glucose utilization in vegetative versus locked-in patients. Ann Neurol 22:673–682

    Article  PubMed  CAS  Google Scholar 

  24. Laureys S, Owen AM, Schiff ND (2004) Brain function in coma, vegetative state, and related disorders. Lancet Neurol 3:537–546

    Article  PubMed  Google Scholar 

  25. Doble JE, Haig AJ, Anderson C, Katz R (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. J Head Trauma Rehabil 18:435–444

    Article  PubMed  Google Scholar 

  26. Casanova E, Lazzari RE, Lotta S, Mazzucchi A (2003) Locked-in syndrome: improvement in the prognosis after an early intensive multidisciplinary rehabilitation. Arch Phys Med Rehabil 84:862–867

    Article  PubMed  Google Scholar 

  27. Carr AJ, Gibson B, Robinson PG (2001) Measuring quality of life: Is quality of life determined by expectations or experience? BMJ 322:1240–1243

    Article  PubMed  CAS  Google Scholar 

  28. Murrell R (1999) Quality of life and neurological illness: a review of the literature. Neuropsychol Rev 9:209–229

    Article  PubMed  CAS  Google Scholar 

  29. Addington-Hall J, Kalra L (2001) Who should measure quality of life? BMJ 322:1417–1420

    Article  PubMed  CAS  Google Scholar 

  30. Wood-Dauphinee S, Williams JI (1987) Reintegration to normal living as a proxy to quality of life. J Chronic Dis 40:491–502

    Article  PubMed  CAS  Google Scholar 

  31. Ware JE, Snow KK, Kosinski M (1993) SF-36 Health Survey Manual and Interpretation Guide. The Health Institute, New England Medical Center, Boston

    Google Scholar 

  32. Ghorbel S (2002) Statut fonctionnel et qualité de vie chez le locked-in syndrome a domicile. Université Jean Monnet Saint-Etienne, Montpellier

    Google Scholar 

  33. Bernheim JL (1999) How to get serious answers to the serious question: “How have you been?”: subjective quality of life (QOL) as an individual experiential emergent construct. Bioethics 13:272–287

    Article  PubMed  Google Scholar 

  34. Ethics and Humanities Subcommittee of the AAN (1993) Position statement: certain aspects of the care and management of profoundly and irreversibly paralyzed patients with retained consciousness and cognition. Report of the Ethics and Humanities Subcommittee of the American Academy of Neurology. Neurology 43:222–223

    Google Scholar 

  35. Anderson C, Dillon C, Burns R (1993) Life-sustaining treatment and locked-in syndrome. Lancet 342:867–868

    Article  PubMed  CAS  Google Scholar 

  36. Lakerveld J, Kotchoubey B, Kubier A (2007) Cognitive function in late stage ALS patients. J Neurol Neurosurg Psychiatry (epub ahead of print)

    Google Scholar 

  37. Bruno MA, Bernheim JL, Schnakers C, Laureys S (2008) Locked-in: don’t judge a book by its cover. J Neurol Neurosurg Psychiatry 79:2

    Article  PubMed  CAS  Google Scholar 

  38. Kubier A, Neumann N (2005) Brain-computer interfaces — the key for the conscious brain locked into a paralyzed body. Prog Brain Res 150:513–525

    Article  Google Scholar 

  39. Wilhelm B, Jordan M, Birbaumer N (2006) Communication in locked-in syndrome: effects of imagery on salivary pH. Neurology 67:534–535

    Article  PubMed  CAS  Google Scholar 

  40. Birbaumer N, Ghanayim N, Hinterberger T, et al (1999) A spelling device for the paralysed. Nature 398:297–298

    Article  PubMed  CAS  Google Scholar 

  41. Vanhaudenhuyse A, Bruno MA, Bredart S, Pleneveux A, Laureys S (2007) The challenge of disentangling reportability and phenomenal consciousness in post-comatose states. Behavioral and Brain Sciences (in press)

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Springer Science + Business Media Inc.

About this paper

Cite this paper

Bruno, M.A., Pellas, F., Laureys, S. (2008). Quality of Life in Locked-in Syndrome Survivors. In: Vincent, JL. (eds) Intensive Care Medicine. Springer, New York, NY. https://doi.org/10.1007/978-0-387-77383-4_80

Download citation

  • DOI: https://doi.org/10.1007/978-0-387-77383-4_80

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-0-387-77382-7

  • Online ISBN: 978-0-387-77383-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics