Encyclopedia of Clinical Neuropsychology

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

Locked-In Syndrome

  • Theslee Joy DePieroEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_463


De-efferented state; Locked-in state


Locked-in syndrome refers to a clinical condition consisting of:
  • Paralysis of all four limbs.

  • Paralysis of bilateral facial musculature.

  • Paralysis of oral and pharyngeal musculature.

  • Paralysis of horizontal eye movements.

  • Voluntary control of eyelids and vertical eye movements are usually preserved.

  • Consciousness is preserved.


  • Total: No voluntary control of limbs, tongue, pharynx, face, or eye movements, with preserved consciousness.

  • Classic: As described in definition.

  • Incomplete: Some preserved horizontal eye movements or limb movements.


Incidence and Prevalence

Locked-in syndrome is a rare disorder. There is no good data regarding the incidence or prevalence of locked-in syndrome.


The most common cause of locked-in syndrome is stroke, either an infarction or hemorrhage in the pons. Other causes are trauma, tumors of the brain stem, central pontine myelinolysis (CPM), Guillain-Barre syndrome...
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References and Readings

  1. Bauby, J.-D. (1996). The diving bell and the butterfly: A memoir of life in death. New York: Vintage.Google Scholar
  2. Devuysy, G., et al. (2002). Stroke or transient ischemic attacks with basilar artery stenosis or occlusion: Clinical patterns and outcome. Archives of Neurology, 59, 567–573.CrossRefGoogle Scholar
  3. Jorgenson, H. S., et al. (1999). What determines good recovery in patients with the most severe strokes? The Copenhagen stroke study. Stroke, 30, 2008–2012.CrossRefGoogle Scholar
  4. Posner, J. B., Saper, C. B., Schiff, N. D., & Plum, F. (2007). Plum and Posner’s diagnosis of stupor and coma (4th ed.). New York: Oxford University Press.Google Scholar
  5. Schnakers, C., et al. (2008). Cognitive function in the locked-in syndrome. Journal of Neurology, 255(3), 323–330.PubMedCrossRefGoogle Scholar
  6. Smith, E., & Delargy, M. (2005). Locked-in syndrome. BMJ, 330, 406–409.PubMedPubMedCentralCrossRefGoogle Scholar
  7. Bauby, J. D. (1997). The diving bell and the butterfly. New York: Vintage Books.Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Braintree Rehabilitation Hospital, Boston University School of MedicineBostonUSA