AP Photo/Yves Logghe
Rom Houben uses his touch screen with
his speech therapist Linda Wouters.

Locked-In Syndrome: When a Coma’s Not a Coma

December 01, 2009 05:00 PM
by Shannon Firth
A scientist in Belgium found that a patient believed to be in a coma has actually been conscious for more than 23 years. Journalists debate the veracity of his claim, and whether the findings hurt or help opponents of right-to-die legislation.

The Impact of a 23-Year Coma Mistake

Following a car accident in 1983, Rom Houben’s heart stopped beating for several minutes, cutting off the oxygen supply to his brain. Houben survived the trauma, but for 23 years afterward remained in what appeared to be a vegetative state. Experts now say he may have been conscious the entire time. For Houben, a former engineering student, now 46, the discovery of his consciousness has been a “second birth.”

Three years ago, Steven Laureys, a neurologist at the University of Liege in Belgium, began a study of misidentified coma cases, and found that 18 of 44 patients believed to be in a vegetative state “responded to communication.” According to Laureys, Houben suffered from total paralysis, despite the fact that “his brain was functioning almost normally,” reported the Guardian.

The medical term for Houben’s condition is appropriately dubbed “locked-in syndrome,” and described by the National Institute of Neurological Disorders and Strokes as “a rare neurological disorder characterized by complete paralysis of voluntary muscles in all parts of the body except for those that control eye movement.”

During the last three years, Houben has undergone “intense physiotherapy” which enabled him to use his finger and a touch screen to communicate. While he felt both angry and powerless at first, Houben said he meditated to help him through his ordeal. He told the Guardian “[I] travelled with my thoughts into the past, or into another existence altogether.”

The causes of LIS include brain injury, diseases of the circulatory system, an overdose of medication, and diseases that attack the coating of nerve cells (or myelin sheath) leaving victims speechless and paralyzed, according to NINDS. The BBC notes that the most common trigger for LIS is a stroke.

Dr. Mark DeLargy, director of the brain injury program at the National Rehabilitation Hospital in Dublin, has experience working with locked-in patients. “It’s often family members who believe that their nearest and dearest have some intellect. They are not believed for a while,” he told the BBC.

According to Laureys, Houben’s case “put a human face to the very important problem of assessing consciousness, the importance of using a standardised scale and the power of neuro-imaging,” reported New Scientist.

Background: How can you tell if a patient is conscious?

Laureys told the Guardian that before diagnosing a patient with a “non-reversible coma,” he or she should be “tested 10 times,” because similar to sleep, comas have certain cycles.

He also explained to New Scientist how he first discovered Houben was conscious. First he used the revised Coma Recovery Scale, which includes items such as eye movement commands (“look away from me”) and oral movement commands (“stick out your tongue”). He repeated the test several times and found minimal signs of consciousness. Then he conducted several brain scans, such as a PET (Positron Emission Tomography) scan which, according to Laureys, “measures the brain energy used by injecting radioactively labelled glucose.” Many of the patients Laureys examined for his study showed minimal signs of consciousness, but Houben’s brain scans showed “near-normal brain function.”

Opinion & Analysis: Facilitated communication and end-of-life treatment

Recently critics in the media have questioned not Houben’s consciousness, but his ability to communicate using a touch screen, because his finger is guided with help from an aid.
American bioethicist Arthur Caplan “described facilitated communication as ‘Ouija board stuff’ that has ‘been discredited time and time again,’” according to Jacob M. Appel, a bioethicist and medical historian, writing for The Huffington Post.

Such criticism frustrates Laureys, who says some people still think “it is impossible, he [Houben] cannot be a cognitive being.” But he argues, “I am a scientist. I am a skeptic … And I don’t think one can say, based on videos on the internet, something meaningful about the use of the touch screen.”

Houben’s case also calls to mind legal battles over end-of-life decisions, such as the “right-to-die” cases of Terry Schiavo in the U.S. and Eluana Englaro in Italy, both of which garnered strong media attention.

Though Appel predicts that conservative activists may use Houben’s case to protest the withdrawal of care in “right to die” battles, he argues, “While we might ultimately decide to let these patients live, even at the risk of allowing them to suffer, we should recognize that such a policy is neither obvious nor intuitive.”

Historical Context: Exploring comas in Literature and the Arts

The Journal of the Royal Society of Medicine analyzes two cases of cerebrovascular disease—one of which is locked-in syndrome—as illustrated by characters in Alexandre Dumas’ “The Count of Monte Cristo,” published between 1844 and 1845.

The journal spotlights Dumas’ description of Monsieur Noirtier de Villeforte, the father of the central character’s enemy: “He was a corpse with living eyes, and at times, nothing could be more terrifying than this marble face out of which anger burned or joy shone.”

The article’s author A.N. Williams explained that Dumas likely learned of locked-in syndrome from Dr. Thibaut, whom he sometimes accompanied on patient visits. “We can speculate that, as with medical students, [the] experience of walking the wards had a profound influence on him, though it is noteworthy that he had an ‘insurmountable repugnance for operations and dead bodies,’” writes Williams.

More than a century later, Richard Selzer, a surgeon and author of “Raising the Dead,” described falling into a 23-day coma, from a third person point-of-view: “The sounds, smells, sights, perceptions of the outside world are disappearing ... At last his fall is broken by a soft cushion and he is suspended in a viscous, pearly matrix.”

Like Houben, Jean-Dominique Bauby, a French magazine editor, was also a victim of LIS. He authored the “The Diving Bell and the Butterfly,” using only his left eye. The book was later adapted into a film, directed by Julian Schnabel in 2006. 

According to the Guardian, Bauby’s speech therapist developed a special alphabet that others would recite; Bauby would blink to select a letter--the letters were ordered based on frequency of use.

Claude Mendibil the book’s ghostwriter explained how difficult this alphabet was at first, and how initially she forgot to look at his eye. “He could tell I was struggling because the first thing he dictated to me was ‘Pas de panique’ [‘Don’t be scared’],” she told the Guardian.
Thomas Mallon, a writer for The New York Times, explained, “The diving bell of Bauby’s title is his corporeal trap, the butterfly his imagination.”

Bauby died two days after his book was published.

Related Topic: Longest Coma

According to Time magazine, which cites the Guinness Book of World Records, Elaine Esposito, “the sleeping beauty” was put under general anesthesia when she was 6 years old in order for surgeons to complete an appendectomy. She never recovered from the effects of the anesthesia and endured the longest coma in history, 37 years, 111 days.

Reference: coma research


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