Locked-in syndrome

Douglas J Lanska MD FAAN MS MSPH (Dr. Lanska of the Great Lakes VA Healthcare System and the University of Wisconsin School of Medicine and Public Health has no relevant financial relationships to disclose.)
Originally released May 19, 2004; last updated April 20, 2017; expires April 20, 2020

This article includes discussion of locked-in syndrome, cerebromedullospinal disconnection, de-efferented state, pseudocoma, disorders of consciousness, classic locked-in syndrome, and complete and incomplete locked-in syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

“Locked-in” syndrome describes patients who are awake and conscious, but selectively “de-efferented,” with no means of producing speech or of moving their facial muscles or limbs. They are aware and perceive normally but are effectively locked in their own bodies, unable to communicate except possibly by blinking or eye movements. Locked-in syndrome typically results from a lesion occurring in the brainstem, classically in the ventral pons. In this article, the author discusses the presentation, etiology, prognosis, diagnostic evaluation, and management of locked-in syndrome. Updates focus on the progress in brain-computer interfaces for communication and functionality of these patients.

Key points

 

• Locked-in syndrome is a de-efferented state characterized by quadriplegia and paralysis of the lower cranial nerves.

 

• Patients retain consciousness and can classically communicate by vertical eye movements and eye blinking.

 

• The most common etiology is atherothrombotic occlusion of the basilar artery resulting in ischemia of the ventral pons.

 

• Advances in technology, such as brain-computer interfaces, are allowing for improved communication for locked-in syndrome patients.

Historical note and terminology

New York neurologists Fred Plum (1924-2010) and Jerome Posner (1932-) first introduced the term in 1983, and they described the syndrome as “a state in which selective supranuclear motor de-efferentation produces paralysis of all 4 limbs and the last cranial nerves without interfering with consciousness” (Plum and Posner 1982).

The famous case of Jean-Dominique Bauby (1952-1997) provides a patient's perspective of locked-in syndrome following a devastating brainstem stroke. Despite being locked in, Bauby “dictated” his memoir to Claude Mendibil by the laborious method of blinking when the correct letter was reached by a person slowly reciting the alphabet over and over again. Included in the book are vivid depictions of the insensitivity of some health care providers toward a severely disabled person (as well as his outspoken reactions to such treatment) (Bauby 1997; Lanska 1998). Bauby died suddenly 2 days after the publication of his book, The Diving Bell and the Butterfly (Bauby 1997). The book was later adapted for a critically acclaimed film of the same name.

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