This paper reflects the research and thoughts of a student at the time the paper was written for a course at Bryn Mawr College. Like other materials on Serendip, it is not intended to be "authoritative" but rather to help others further develop their own explorations. Web links were active as of the time the paper was posted but are not updated.
2004 First Web Paper
"Twelfth Night", "Freaky Friday"--we are all familiar with the many scenarios that depict a common fear—being trapped in another's body. But there exists a bigger nightmare. Imagine the horror of being trapped in one's own body. For those with locked-in syndrome (LIS), that fear is a reality.
LIS describes one of the most debilitating conditions in which a person retains consciousness. The result of head injury, brain-stem strokes, or neurological diseases like ALS, locked-in syndrome is caused by a lesion in the nerve centers that control muscle contraction or a blood clot that blocks circulation of oxygen to the brain stem(6),. First introduced in 1966 by Plum and Posner, the term has since then been redefined as "quadriplegia and anarthia, with preservation of consciousness".(1). (Anarthia refers to the neurologic inability to speak, as opposed to an unwillingness to speak.) Unable to either move, or speak, yet fully cognizant of the world around them, these individuals are virtually locked in. An accurate diagnosis of LIS depends on the recognition that the patient can open his eyes voluntarily rather than spontaneously in the vegetative state.(4). Although horizontal eye movements are usually lost, the ability to open their eyes and blink is retained.(4) Therein lies the key to communication with the outside world.
I first learned about this extremely rare condition while helping a friend with a French paper. The subject, Jean-Dominique Bauby's, "The Diving Bell and the Butterfly", piqued my interest. On Dec. 8th, 1995, Bauby, a 42-year-old father of two, was test-driving a new car when he suffered a massive stroke. He awoke from a coma two months later to find himself paralyzed and speechless, but able to move one muscle: his left eyelid.(3) Due to his privileged position as an author and editor of a popular French magazine, he was afforded the opportunity to do the unimaginable—share his experience with the outside world. With the aid of a secretary and an elaborate alphabet in which each letter was recited to him in the frequency with which it occurs in the French language, he was able to blink his novel.(3)
It was Alexandre Dumas who in 1820 first described LIS when he created Monsieur Noirtier de Villefort in his novel, The Count of Monte Cristo. He described his character as a "corpse with living eyes"(1), but Bauby's tale contradicts this commonly held notion. He recounts his struggle with the realization that he is trapped within a paralyzed body—the diving bell—in which his mind flies like a butterfly:
"I am fading away, slowly, but surely. Like the sailor who watches his home shore gradually disappear, I watch my past recede. My own life still burns within me, but more of it is reduced to the ashes of memory. Since taking up life in my cocoon, I have made two brief trips to the world of Paris medicine to hear the verdict pronounced on me from medical heights. I shed a few tears as we passed the corner café where I used to drop in for a bite. I can weep discreetly, yet the professionals think my eye is watering." (3).
In his memoir, Bauby continually addresses the very sense of alienation and exclusion from society that is shared by all who are severely handicapped. How worthy are these individuals to our society? Those with profound neurological disabilities such as LIS, tetraplegia, or who are in persistent vegeatative state, have been the subject of substantial medical and ethical debate. Many feel that the allocation of resources to maintain their lives is too much of a high-stakes game. After reading Bauby's book, I sought to better inform myself about this rare condition. But the paucity of information or research available was disheartening. An interview with Roger Rees, Director of the Institute for the Study of Learning Difficulties at Flinders University in Adelaide, explains that "from an economic rationalist's view of rehabilitation or of a simplistic absolute view that a person is either cured or not cured people in the locked-in state are considered of no account." (3) Although there are no statistics available on the number of patients with LIS, the locked-in populating is growing due to advances in artificial respiration.(2) How then to convince those responsible that the benefits to sustaining these individuals far outweigh the monetary sacrifices?
Niels Birbaumer, a German neuroscientist and leading expert in the field, works on brain-computer interface (BCI) research in an attempt to give those who are locked in a voice so that they might be involved in the decisions that affect their lives. One of his "patients", Elias Musiris, a wealthy Peruvian owner of a casino, suffers from Lou Gehrig's disease which has induced a locked-in state. Using BCI, electrodes were attached to his scalp producing a moving white dot across a screen—Musiris was looking at his EEG, who's up and down motion represents his brain activity. His task—to willingly change the electricity of his brain by changing his thoughts, and in doing so, to control the white dot by keeping it in one half of the screen. Birbaumer had previously developed a similar technique to train epileptics to fend off impending seizures.(2) He hoped that teaching Musiris to influence his EEG would then enable him to "respond" to simple yes-no questions by moving the dot to a certain half of the screen. The results ? After a week of intensive practice, Musiris was able to produce answers that, through repetition, had reached a statistical safety level of more than ninety per cent.(2) Through this new method his family learned that he wished to buy new pool tables and keep the old slot machines in his casino which they were about to sell. For the first time in five years Musiris began to have a deliberate impact on his world and his business—without having to move a single muscle.
The stories of Bauby and Musiris not only put a human face on locked-in syndrome, they offer insights into the question of a mind/body dualism discussed in class. The body is inextricably linked to one's sense of self, however, physical suffering need not steal one's sense of self. There remains a wealth of thoughts, feelings, memories and dreams to be generated and recalled. Bauby's tale is a poignant testimony of human resilience in the face of adversity; it demonstrates that the loss of one's last faint muscle movement does not somehow eliminate the will to be heard.
1. http://web5.infotrac.galegroup.com/itw/infomark/301/818/45683206w5/; very comprehensive research on "Impairment, activity, participation, life satisfaction , and survival in persons with LIS"(Jennifer Doble)
2. http://web5.infotrac.galegroup.com/itw/infomark/301/818/; article on brain-computer interface research; (Ian Parker).
3. http://www.abc.net.au/rn/science/ockham/stories/s10275.htm ; interview with Prof. Roger Rees on LIS
4. http://www.jnnp.com/cgi/content/full/71/suppl_1/i18?RESULTFORMAT=1&eaf; contrasts LIS with coma
5. http://jnnp.bmjjournals.com/cgi/content/full/63/6/759 ; a look at ERP's in patients with LIS
6. http://www.questdiagnostics.com/kbase/nord/nord472.htm; gives the basics of LIS
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