INFORMATION >> LOCKED-IN SYNDROME

Locked-in syndrome

The locked-in syndrome (pseudocoma) describes patients who are awake and conscious but have no means of producing speech, limb, or face movements. Brainstem lesions are its most common cause.

People with such lesions often remain comatose for some days or weeks, needing artificial respiration and then gradually wake up, but remaining paralyzed and voiceless, superficially resembling patients in a vegetative state. In acute locked-in syndrome (LIS), eye-coded communication and evaluation of cognitive and emotional functioning is very limited because vigilance is fluctuating and eye movements may be inconsistent, very small, and easily exhausted [1].

The locked-in syndrome was already described in Alexandre Dumas's novel the Count of Monte Cristo (1844-45)[2]. Herein a character, Monsieur Noirtier de Villefort, was depicted as "a corpse with living eyes". Mr. Noirtier had been in this state for more than six years, and he could only communicate by blinking his eyes. His helper pointed at words in a dictionary and the monsignor indicated with his eyes the words he wanted.
Some years later, Emile Zola wrote in his novel Thérèse Raquin[3] (1868) about a paralyzed woman who “was buried alive in a dead body” and “had language only in her eyes”. Dumas and Zola highlighted the locked-in condition before the medical community did.

The term "locked-in" syndrome was first introduced in 1966 by Plum and Posner [4]. It is defined by

(i) the presence of sustained eye opening (bilateral ptosis should be ruled out as a complicating factor);
(ii) preserved awareness of the environment;
(iii) aphonia or hypophonia;
(iv) quadriplegia or quadriparesis;
(v) a primary mode of communication that uses vertical or lateral eye movement or blinking of the upper eyelid to signal yes/no responses [5].

The Locked-in syndrome can be divided into three categories [6]:
(a) classical LIS is characterized by quadriplegia and anarthria with preserved consciousness and vertical eye movement or blinking;
(b) incomplete LIS permits remnants of voluntary motion other than vertical eye movement; and
(c) total LIS consists of complete immobility including all eye movements combined with preserved consciousness.

Once a LIS patient becomes medically stable, and given appropriate medical care, life expectancy now is several decades. Even if the chances of good motor recovery are very limited, existing eye-controlled computer-based communication technology currently allow the patient to control his environment, use a word processor coupled to a speech synthesizer and access the world wide net.

In December 1995, Jean-Dominique Bauby, aged 43 and editor in chief of the fashion magazine ‘Elle’, had a brainstem stroke. He emerged from a coma several weeks later to find himself in a LIS only able to move his left eyelid and with very little hope of recovery. Bauby 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 in which he composed each passage mentally and then dictated it, letter by letter, to an amanuensis who painstakingly recited a frequency-ordered alphabet until Bauby chose a letter by blinking his left eyelid once to signify "yes". His book The Diving Bell and the Butterfly [7] became a best-seller only weeks after his death due to septic shock on March 9, 1997. Bauby created an Association of Locked-In Syndrome (ALIS) aimed to help patients with this condition and their families .

Computerized devices now allow the LIS patient and other patients with severe motor impairment to “speak” [8]. The preeminent physicist Stephen Hawking, author of the best sellers A Brief History of Time and The Universe in a Nutshell, is able to communicate solely through the use of a computerized voice synthesizer. With one finger, he selects words presented serially on a computer screen; the words are then stored and later presented as a synthesized and coherent message. The continuing brilliant productivity of Hawking despite his failure to move or speak illustrates that locked-in patients can be productive members of the society.

 

Practical info

Guide pratique pour la rééducation orthophonique du "locked in syndrome" en secteur libéral - PDF (French)

Communiquer sans la parole - PDF (French)

Aides techniques et locked-in syndrome - PDF (French)

 

Associations

French association of Locked-In Syndrome (ALIS)

Italian association of Locked-In Syndrome (LISA)

 

Books written by locked in syndrome survivors

 

English

Only the eyes say yes - Philippe & Stéphane Vigand

The Diving Bell and the Butterfly: A Memoir of Life in Death - Jean-Dominique Bauby

Look up for Yes - Julia Tavalaro

 

Français

Je parle : L'extraordinaire retour à la vie d'un Locked-in Syndrome - Laetitia Bohn-Derrien

Le Scaphandre et le papillon - Jean-Dominique Bauby

Putain de silence - Philippe et Stéphane Vigand

 

Nederlands

Eenzame stilte - Roland Boulangier

 

Deutsch

Locked-in. Gefangen im eigenen Körper - Karl-Heinz Pantke

 

Movies & documentaries

 

2007 movie on locked in syndrome (Bauby's life)

The Diving Bell and the Butterfly - read and watch NY times movie rieview

Le scaphandre et le papillon - un film de Julian Schnabel basé sur le livre de Jean-Dominique BAUBY et sa vie en locked-in syndrome

 

2007 documentary on coma by Liz Garbus COMA goes inside the Center for Head Injuries at JFK Medical Center in Edison, New Jersey and follows four coma survivors over the course of one year

 

Quotes

Thirty years ago a stroke left me in a coma. When I awoke I found myself completely paralyzed and unable to speak… I didn’t know what paralysis was until I could move nothing but my eyes. I didn’t know what loneliness was until I had to wait all night in the dark, in pain from head to foot, vainly hoping for someone to come with a teardrop of comfort. I didn’t know what silence was until the only sound I could make was that of my own breath issuing from a hole drilled into my throat.

Julia Tavalaro [9]

 

Io non mori’, e non rimasi vivo;
Pensa omai per te, s’hai fior d’ingegno, Qual io divenni, d’uno e d’altro privo.

Neither did I die, nor did I remain alive;
Imagine yourself, if your spirit is fine,
what I came to be, deprived of both.

Alighieri Dante, 1265-1321, Divina Comedia, Inferno XXXIV, 25–27

 

An old laborer, bent double with age and toil, was gathering sticks in a forest.
At last he grew so tired and hopeless that he threw down the bundle of sticks, and cried out:
"I cannot bear this life any longer. Ah, I wish Death would only come and take me!"
As he spoke, Death, a grisly skeleton, appeared and said to him:
"What wouldst thou, Mortal? I heard thee call me."
"Please, sir," replied the woodcutter,
"would you kindly help me to lift this faggot of sticks on to my shoulder?"

Aesop, approximately 620-560 B.C., The Old Man and Death (translated in verse by Jean de La Fontaine, 1621-1695, La Mort et le Bucheron)

 

References

Le locked-in syndrome - view PDF
Laureys S, Pellas F, Van Eeckhout P
La lettre du neurologue, 10 (2006) 216-218

Renouer avec les consciences emmurées
Nouvelles Clés 37 (2003) 46-50

1.         Laureys, S., et al. (2005). The locked-in syndrome : what is it like to be conscious but paralyzed and voiceless? Prog Brain Res  150, 495-511.
2.         Dumas, A., The Count of Monte Cristo. 1997, London: Wordworth Editions Limited.
3.         Zola, E., Thérère Raquin. 1979, Paris: Ed. Gallimard. 352.
4.         Posner, J.B., Saper, C.B., Schiff, N.D., and Plum, F., The diagnosis of stupor and coma. 4th ed. 2007: Oxford University Press.
5.         American Congress of Rehabilitation Medicine (1995). Recommendations for use of uniform nomenclature pertinent to patients with severe alterations of consciousness. Arch. Phys. Med. Rehabil.  76, 205-209.
6.         Bauer, G., Gerstenbrand, F., and Rumpl, E. (1979). Varieties of the locked-in syndrome. J Neurol  221, 77-91.
7.         Bauby, J.-D., The diving bell and the butterfly (original title: Le scaphandre et le papillon), ed. E.R. Laffont. 1997.
8.         Kubler, A. and Neumann, N., Brain-computer interfaces - the key for the conscious brain locked into a paralyzed body, in The boundaries of cosnciousness - paperback edition. 2006, Elsevier. p. 513-525.
9.         Tavalaro, J. and Tayson, R., Look up for yes. 1997, New York, NY: Kodansha America, Inc.

links

Français

Association Locked-in Syndrome (ALIS)

Association des Paralysés de France

Handiaccess

Handicap International

Suppléance à la communication

Technologies de l'Information et de la Communication (TIC) pour les personnes handicapées - brochure en pdf

Nederlands

Bieke Wittebolsfonds

Eenzame stilte - LIS Roland Boulangier

Ondersteunde Communicatie: Adressen in België en Nederland

Informatie- en communicatietechnologie (ICT) voor personen met een handicap - download pdf brochure

English

International Society for Augmentative and Alternative Communication

Deutsch

Gestützte Kommunikation

Unterstützte Kommunikation


Italiano

Italian association of Locked-In Syndrome (LISA)