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.
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.
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.
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.
Schnakers, C., et al. (2008). Cognitive function in the locked-in syndrome. Journal of Neurology, 255(3), 323–330.
Smith, E., & Delargy, M. (2005). Locked-in syndrome. BMJ, 330, 406–409.
Bauby, J. D. (1997). The diving bell and the butterfly. New York: Vintage Books.
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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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DOI: https://doi.org/10.1007/978-3-319-57111-9_463
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