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Locked-In Syndrome

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  • First Online:
Encyclopedia of Clinical Neuropsychology
  • 23 Accesses

Synonyms

De-efferented state; Locked-in state

Definition

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.

Categorization

  • 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.

Epidemiology

Incidence and Prevalence

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

Etiology

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

  • Bauby, J.-D. (1996). The diving bell and the butterfly: A memoir of life in death. New York: Vintage.

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  • 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.

    Article  Google Scholar 

  • 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.

    Article  Google Scholar 

  • 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.

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  • Schnakers, C., et al. (2008). Cognitive function in the locked-in syndrome. Journal of Neurology, 255(3), 323–330.

    Article  PubMed  Google Scholar 

  • Smith, E., & Delargy, M. (2005). Locked-in syndrome. BMJ, 330, 406–409.

    Article  PubMed  PubMed Central  Google Scholar 

  • Bauby, J. D. (1997). The diving bell and the butterfly. New York: Vintage Books.

    Google Scholar 

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Correspondence to Theslee Joy DePiero .

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DePiero, T.J. (2018). Locked-In Syndrome. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_463

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