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Locked-in syndrome

Author
Louis R Caplan, MD
Section Editor
Jose Biller, MD, FACP, FAAN, FAHA
Deputy Editor
John F Dashe, MD, PhD

INTRODUCTION

The locked-in syndrome (LIS) is a catastrophic condition caused most often by ischemic stroke or hemorrhage, affecting the corticospinal, corticopontine, and corticobulbar tracts in the brainstem. Because consciousness and higher cortical functions are spared, patients can sometimes communicate through eye movements.

Alexandre Dumas provided one of the earliest descriptions of LIS in "The Count of Monte Cristo" by vividly depicting a character who was "a corpse with living eyes." Following a stroke, Monsignor Noirtier de Villefort could only communicate by raising, closing, or winking his eyes [1].

Issues related to LIS will be reviewed here. Conditions superficially similar to LIS, such as coma, minimally conscious state, and persistent vegetative state, are discussed separately. (See "Stupor and coma in adults".)

DEFINITION AND ANATOMY

In 1966, Plum and Posner coined the term "locked-in" to describe the state of quadriplegia and anarthria (speechlessness due to severe dysarthria) with preserved consciousness [2]. Synonymous with locked-in syndrome (LIS) are "de-efferented state," "pseudocoma," and "coma vigilante."

There are two requisites for the diagnosis of locked-in syndrome:

                

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Literature review current through: Nov 2016. | This topic last updated: Mon Aug 25 00:00:00 GMT 2014.
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References
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