| Locked-in syndrome | |
|---|---|
| Classification and external resources | |
Locked-in syndrome can be caused by stroke at the level of the basilar artery denying blood to the pons, among other causes. |
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| ICD-10 | G46.3 |
| ICD-9 | 344.81 |
| MeSH | D011782 |
Locked-in syndrome is a condition in which a patient is aware and awake but cannot move or communicate verbally due to complete paralysis of nearly all voluntary muscles in the body except for the eyes. Total locked-in syndrome is a version of locked-in syndrome where the eyes are paralyzed as well.[1] The term for this disorder was coined by Fred Plum and Jerome Posner in 1966.[2][3] Locked-in syndrome is also known as cerebromedullospinal disconnection,[4] de-efferented state, pseudocoma,[5] and ventral pontine syndrome.
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Locked-in syndrome usually results in quadriplegia and the inability to speak in otherwise cognitively intact individuals. Those with locked-in syndrome may be able to communicate with others through coded messages by blinking or moving their eyes, which are often not affected by the paralysis. The symptoms are similar to those of sleep paralysis. Patients who have locked-in syndrome are conscious and aware with no loss of cognitive function. They can sometimes retain proprioception and sensation throughout their body. Some patients may have the ability to move certain facial muscles, most often some or all of the extraocular eye muscles. Individuals with locked-in syndrome lack coordination between breathing and voice.[6] This restricts them from producing voluntary sounds, even though the vocal cords themselves are not paralysed.[6]
Unlike persistent vegetative state, in which the upper portions of the brain are damaged and the lower portions are spared, locked-in syndrome is caused by damage to specific portions of the lower brain and brainstem with no damage to the upper brain.
Possible causes of locked-in syndrome include:
There is neither a standard treatment for locked-in syndrome, nor is there a cure. Stimulation of muscle reflexes with electrodes (NMES) has been known to help patients regain some muscle function. Other courses of treatment are often symptomatic.[8] Assistive computer interface technologies, such as Dasher in combination with eye tracking, may be used to help patients communicate. New direct brain interface mechanisms may provide future remedies.[9][10] Israeli scientists have reported that they have developed a technique that allows locked-in patients to communicate via sniffing.[11]
It is extremely rare for any significant motor function to return. The majority of locked-in syndrome patients do not regain motor control, but devices are available to help patients communicate. Within the first four months after its onset, 90% of those with this condition die. However, some people with the condition continue to live much longer periods of time.[12]
Parisian journalist Jean-Dominique Bauby suffered a stroke in December 1995, and, when he awoke 20 days later, he found that his body was almost completely paralyzed: he could control only his left eyelid. By blinking this eye, he slowly dictated one alphabetic character at a time and, in so doing, was able over a great deal of time to write his memoir, The Diving Bell and the Butterfly. Three days after it was published in March 1997, Bauby died of pneumonia.[13] The 2007 film The Diving Bell and the Butterfly is a screen adaptation of Bauby's memoir. Jean-Dominique was instrumental in forming the Association du Locked-In Syndrome (ALIS) in France.[14]
In 1966, Julia Tavalaro, then aged 32, suffered two strokes and a brain hemorrhage and was sent to Goldwater Memorial Hospital on Roosevelt Island, New York. For six years, it was believed she was in a vegetative state. In 1972, a family member noticed her trying to smile after she heard a joke. After alerting doctors, a speech therapist, Arlene Kratt, discerned cognizance in her eye movements. Kratt and another therapist, Joyce Sabari, were eventually able to convince doctors that she was in a locked-in state. After learning to communicate with eye blinks in response to letters being pointed to on an alphabet board, she became a poet and author. Eventually, she gained the ability to move her head enough to touch a switch with her cheek, which operated a motorized wheelchair and a computer. She gained national attention in 1995 when the Los Angeles Times published her life story. It was republished by Newsday on Long Island and in other newspapers across the country. She died in 2003 at the age of 68.[12][15]
Gary Griffin was a veteran of the United States Air Force who became immobile due to amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig's disease). He was later equipped with a device called the NeuroSwitch, which allows him to control a computer and communicate with his family. Sensors are attached to the skin over a patient's muscles, and signals are sent to an interface that translates the slightest muscle contractions into usable code.
In 1999, 16-year-old Erik Ramsey suffered a stroke after a car accident that left him in a locked-in state. His story was profiled in an edition of Esquire magazine in 2008. Erik is currently working with doctors to develop a new communication system that uses a computer that, through implants in his brain, reads the electronic signals produced when he thinks certain words and sounds. At present, Erik is only able to communicate short and basic sounds. However, doctors believe that within a few years, Erik will be able to use this system to communicate words and phrases and, eventually, to "talk" normally.[12][16]
In 1983, Rom Houben survived a near-fatal car crash and was diagnosed as being in a vegetative state. Twenty-three years later, using "modern brain imaging techniques and equipment", doctors revised his diagnosis to locked-in syndrome.[17] He was initially reported as communicating by typing into a keyboard with his right hand,[18] though the presence of a facilitator to move his hand attracted sharp criticism and strong doubts that Houben's communications were authentic.[19][20][21]
In early 2010, Dr. Steven Laureys, Houben's neurologist, admitted that subsequent tests had demonstrated that Houben had not actually been communicating via the facilitator, and Der Spiegel, which had originally "quoted" many of Houben's facilitated statements, retracted those quotes as being inauthentic.[22] Laureys maintained that the MRI data that had led him to diagnose Houben as locked-in still suggested that he was conscious.
Houben's case had been thought to call into question the current methods of diagnosing vegetative state and arguments against withholding care from such patients.[17][23][24]
In 1993, Graham Miles, originally from Sanderstead, Surrey, suffered a stroke after which he could not move any part of his body except his eyes. His condition improved gradually until in 2010, he could walk with two sticks and drive a car.[25][26]
in 2010, ex-premiership footballer Gary Parkinson suffered a massive stroke and was later diagnosed with Locked-in Syndrome. This has however not ended his career in football as he is now part of Middlesbrough F.C.'s scouting analysis team, watching potential players on DVD and relaying the verdict to the Middlesbrough manager Tony Mowbray solely through blinking.
In the French novel Thérèse Raquin, by Émile Zola, Thérèse Raquin and her second husband Laurent accidentally reveal to Thérèse's aunt, Madame Raquin (who suffered from locked-in syndrome after a stroke), that they killed Camille Raquin (Madame Raquin's son). One day, when some friends are over, Madame Raquin eventually musters an enormous amount of strength to move her finger on a table, tracing words that would reveal Thérèse's and Laurent's deed. However, she is interrupted, and her words are misinterpreted as Thérèse and Laurent have taken good care of me.
The character M. Noirtier de Villefort in Alexandre Dumas's novel The Count of Monte Cristo apparently suffers from locked-in syndrome. He is described as a "corpse with living eyes" who communicates with eye movements and expressions. His granddaughter Valentine helps him form sentences by reciting the alphabet and scanning dictionary pages with her finger until he indicates which letters and words he wants.
The television program House MD presented a case of locked-in syndrome, which later turned into a case of total locked in syndrome in the episode Locked In, the patient was portrayed by Mos Def.
On an episode of Scrubs entitled "His Story III", a patient is presented with locked-in syndrome. The character is played by Eric McNair.
Joe Bonham, protagonist of Dalton Trumbo's infamous 1938 anti-war novel, suffers from locked-in syndrome after being caught in the blast of an artillery shell and losing his face and all of his limbs. He is thus rendered incapable of communicating in any form other than tapping his head on his field hospital pillow in Morse Code to communicate to his nurse that he still has his full brain capacity. He asks first to die, then to be toured around the country to show people the true horrors of war. Both requests are denied by the chief medical staff, and it is implied that he lives the rest of his natural life in this state, unable to live or die.
The character Jean-Louis Martin (Bernard Werber) suffers from locked-in syndrome after being paralysed in a car accident. He could move just his one eye and hear just with his one ear. He could communicate blinking his eye. Blinking once he said "Yes" and twice he said "No". Later he started comunicated through computer and became very powerful using just his brain.
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