Locked-in Syndrome (LIS)
Definitions
The term ‘locked-in syndrome’ was first introduced by Plum and Posner in 1966 in the first edition of their monograph (The Diagnosis of Stupor and Coma). In the third edition of their book, locked-in syndrome (LIS) is described as “a state in which selective supranuclear motor deeferentation produces paralysis of all four extremities and the lower cranial nerves without interfering with consciousness.“ (p9)
Characteristics of LIS:
- presence of sustained eye opening
- preserved awareness of self and the environment
- aphonia or hypophonia;
- quadriplegia or quadriparesis
- usually, but not always, capacity to use vertical eye movements and blinking to communicate
Locked-in syndrome can be divided into three categories (Bauer et al 1979):
- classical LIS: where the only movement is the vertical eye movement or blinking
- incomplete LIS: nearly complete paralysis though there may be some very limited movement other than the vertical movement
- total LIS: consists of complete immobility including all eye movements
In all three categories consciousness remains intact.
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Prevalence
There has been no study looking at the prevalence of locked-in syndrome. Like vegetative state and minimally conscious state, LIS is not recognised by the International Classification of Diseases which means that no data is collected about the prevalence or incidence of the syndrome. The most accurate and up-to-date dataset on LIS is the survey carried by the Association du Locked-in Syndrome (ALIS), France. The association has surveyed over 500 people in LIS in France since 1997.
For more up-to-date information see ALIS website in English: http://www.alis-asso.fr/ewb_pages/e/eng_alis.php
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Facts
With appropriate medical care, a patient’s life expectancy can be several decades. In contrast to the beliefs of many healthy people, LIS patients report a meaningful quality of life and the demand for euthanasia is uncommon: based on a survey of 70 to 78 individuals with LIS by ALIS in France: 71% never thought of suicide, 26% thought of it occasionally and only 3% often; 53% had never considered euthanasia, 39% had considered it at some stage but not anymore and 8% would demand it now.
Electronic assistive technologies such as the ones offered by our Compass clinic enable individuals with LIS to interact with their environment, to use a computer (emails, word processing, games) and to navigate the internet. This access to technology may be one of the factors contributing to the level of quality of life reported by patients in LIS. (Laureys et al. 2005)
LIS in literature
In his novel The Count of Monte Cristo (1844-45), Alexandre Dumas depicts Monsieur Noirtier de Villefort as "a corpse with living eyes" who was “perfectly clear in his mind but still incapable of moving or speaking”. Mr Noirtier could only communicate by blinking his eyes. His granddaughter would go through the alphabet, when a letter was selected, she will then open the dictionary to that letter and point at words until he indicated with his eyes the words he wanted.
Emile Zola mentioned in his novel Thérèse Raquin (1868) a paralysed woman who “was buried alive in a dead body” and “had language only in her eyes”.
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Links
Association du Locked-in syndrome
http://alis-asso.fr/ewb_pages/e/eng_the_locked_in_syndrome.php
ALIS was created in March 1997. The idea and implementation are due to Jean-Dominique Bauby. He himself was diagnosed with locked-in syndrome in December 1995, and wanted to show the world that this pathology, which impedes movement and speech, does not prevent patients from living. He has proven it in an extraordinary book which he wrote with his left eyelid alone, "Le Scaphandre et le papillon" (Ed. Robert Laffont) which became a best-seller only weeks after his sudden death on March 9, 1997.
Information on LIS by the National Institute of Neurological Disorders and Stroke (USA)
http://www.ninds.nih.gov/disorders/lockedinsyndrome/lockedinsyndrome.htm
Coma Science group
http://www.coma.ulg.ac.be
Impaired Consciousness Research Group
http://www.wbic.cam.ac.uk/~mrc30/index.html
The Impaired Consciousness Research Group consists of a small group of healthcare professionals and academics with a common interest and desire to improve the care of persons with impaired consciousness (coma, vegetative state, minimally conscious state) after severe brain injury.
The Brain and Spine Foundation
http://www.brainandspine.org.uk
Founded in 1992 to help the 10 million people affected by brain and spine conditions in the UK.
Association for the scientific study of consciousness
http://www.theassc.org/about_assc
The ASSC is an academic society that promotes rigorous research directed toward understanding the nature, function, and underlying mechanisms of consciousness. The ASSC includes members working in the fields of cognitive science, medicine, neuroscience, philosophy, and other relevant disciplines in the sciences and humanities.
National Institute of Neurological Disorders and Stroke (NINDS) (USA)
http://www.ninds.nih.gov/disorders/lockedinsyndrome/lockedinsyndrome.htm
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Key references
Laureys S, Pellas F, Van Eeckhout P, Ghorbel S, Schnakers C, Perrin F, Berré J, Faymonville ME, Pantke KH, Damas F, Lamy M, Moonen G, Goldman S. The locked-in syndrome: what is it like to be conscious but paralyzed and voiceless. Progress in Brain Research 150 (2005) 495-511
Bauer G, Gerstenbrand F, Rumpl E. Varieties of the locked-in syndrome. J Neurol 1979; 221(2):77-91.
Lulé D, Zickler C, Häcker S, Bruno MA, Demertzi A, Pellas F, Laureys S, Kübler A. Life can be worth living in locked-in syndrome. Prog Brain Res. 2009;177:339-51.
Soddu A, Boly M, Nir Y, Noirhomme Q, Vanhaudenhuyse A, Demertzi A, Arzi A, Ovadia S, Stanziano M, Papa M, Laureys S, Malach R. Reaching across the abyss:recent advances in functional magnetic resonance imaging and their potential relevance to disorders of consciousness. Prog Brain Res. 2009;177:261-74.
Palmieri RL. Unlocking the secrets of locked-in syndrome. Nursing. 2009 Jul;39(7):22-9; quiz 29-30.
Schnakers C, Majerus S, Goldman S, Boly M, Van Eeckhout P, Gay S, Pellas F, Bartsch V, Peigneux P, Moonen G, Laureys S. Cognitive function in the locked-in syndrome. J Neurol. 2008 Mar;255(3):323-30. Epub 2008 Mar 20.
Books written by or for people in Locked-in-Syndrome:
The Diving-bell and the Butterfly (Paperback) by Jean-Dominique Bauby (1997) New York: Vintage International, 1997.
-So What? Poems by Piotr Kniecicki (2007) United Press ltd
-Blah, blah, blah Poems by Piotr Kniecicki (2008) United Press ltd
I’m writing because I want to prove to myself and others, that even in such a miserable physical condition, one can ignite something positive out of one’s brain, if it still remains active….Regarding the satirical drawings, I have read somewhere that the greatest artistic ability is to be able to laugh at oneself, despite the circumstances. Hence, here’s what I’m doing now. However, a careful observer will notice some tears…Piotr K
Speaking with the eyes: Locked-in syndrome (Jan 09)
http://www.eurordis.org/article.php3?id_article=1891
Howell, Patton and James Hall. Locked in to Life. Boise, ID: Tea Road Press,2002. James Hall became a victim of 'locked-in syndrome', unable to talk or move. He found an extraordinary way to tell the doctors he wanted to live.
The Official Patient’s Sourcebook on Locked-In Syndrome: A Revised and Updated Directory for the Internet Age. San Diego, CA: Icon Health Publications, 2004. This book has been created for patients who have decided to make education and research an integral part of the treatment process. Although it also gives information useful to doctors, caregivers and other health professionals, it tells patients where and how to look for information covering virtually all topics related to locked-in syndrome.
Tavalaro, Julia and Richard Tayson. Look Up for Yes. New York: Kodansha International, 1997.
When Julia Tavalaro opened her eyes after spending seven months in a coma, she awoke to a nightmare. Nobody in the hospital ward to which she had been consigned even noticed that she was alert. Paralyzed and unable to speak, Tavalaro had no way of making them take notice. She spent the next six years languishing in her bed, and although able to hear everything around her, she was unable to communicate. Finally, a young speech therapist broke through Tavalaro's isolation by composing a method by which Tavalaro could spell out words with her eyes. After mastering the technique, Tavalaro went on to write poetry about her life both before and after the stroke that crippled her.
Vigand, Philippe and Stephane. Only the Eyes Say Yes: A Love Story. New York: Arcade Publishing, 1997.
In 1990 Philippe Vigand collapsed while walking to work in Paris and slowly developed locked-in syndrome, losing all control of his body. The problem was caused by the occlusion of a vital artery in the brain. He and his wife spell out the condition's physical, mental, and psychological results as they became aware of them and learned how to live with them. At first, Philippe could move only his eyelids to indicate yes or no and somewhat more for those who learned a personal code he developed. Thanks to Stephane and his sister, who became "tigresses" on his behalf, to his circle of friends, and to his own remarkable inventiveness, he acquired electronic aids he called "Eyegaze" and "James" to assist in communication, reading, and basic activities.
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