Locked-in syndrome

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Locked-in syndrome
Classification and external resources
CerebellumArteries.jpg
Locked-in syndrome can be caused by stroke at the level of the basilar artery denying blood to the pons, among other causes.
ICD-10 G93.8
ICD-9 344.81
MeSH D011782

Locked-in syndrome (LIS) is a condition in which a patient is aware 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 wherein the eyes are paralyzed, as well.[1] Fred Plum and Jerome Posner coined the term for this disorder in 1966.[2][3] Locked-in syndrome is also known as cerebromedullospinal disconnection,[4] de-efferented state, pseudocoma,[5] and ventral pontine syndrome.

Presentation[edit]

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 bodies. Some patients may have the ability to move certain facial muscles, and most often some or all of the extraocular eye muscles. Individuals with the syndrome lack coordination between breathing and voice.[6] This restricts them from producing voluntary sounds, though the vocal cords are not paralysed.[6]

Causes[edit]

In children, the most common cause is a stroke of the ventral pons.[7]

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:

Treatment[edit]

Neither a standard treatment nor a cure is available. 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, combined with eye tracking, may be used to help patients communicate. New direct brain interface mechanisms may provide future remedies; one effort in 2002 allowed a fully locked-in patient to answer yes-or-no questions.[9][10][11] Some scientists have reported that they have developed a technique that allows locked-in patients to communicate via sniffing.[12]

Prognosis[edit]

Extremely rarely does any significant motor function 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,[13] while in exceptional cases, like that of Kerry Pink[14] and Kate Allatt,[15] a full spontaneous recovery may be achieved.

Notable cases[edit]

Kate Allatt[edit]

Kate Allatt is a mother-of-three from Sheffield, South Yorkshire who has successfully recovered from locked-in syndrome. She now runs Fighting Strokes and devotes her life to assisting those who have locked-in syndrome.[16]

Jean-Dominique Bauby[edit]

Parisian journalist Jean-Dominique Bauby suffered a stroke in December 1995, and when he awoke 20 days later, he found his body was almost completely paralyzed; he could control only his left eyelid (as the other was sewn shut to prevent an infection). 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; the memoir was adapted to the screen in an eponymous 2007 movie. Three days after the book was published in March 1997, Bauby died of pneumonia.[17] He was instrumental in forming the Association du Locked-In Syndrome (ALIS) in France.[18]

Rabbi Ronnie Cahana[edit]

In the summer of 2011, Rabbi Ronnie Cahana of Congregation Beth-El in Montreal suffered a severe brainstem stroke that left him in a locked-in state, able to communicate only with his eyes. With his family's help he continued to write poems and sermons for his congregation, letter by letter, by blinking. He has since regained his ability to breathe by himself and speak. He describes his experiences as a blessing and a spiritual revelation of body and mind.[19] He is the son of painter Alice Lok Cahana.[citation needed]

Lynsey Cribbin[edit]

Lynsey Cribbin, from Cavan, Ireland, woke up with several headaches in January 2012. She suffered multiple strokes which left her on life support and eventually with one of the most severe cases of locked-in syndrome. Her brain works but she cannot move.[20]

Stephen Hawking[edit]

Stephen Hawking has had a progressive motor neuron disease related to amyotrophic lateral sclerosis (ALS) since the early 1960s. He is almost entirely paralyzed and communicates through a speech generating device. Since 2005, as the disease progressed, he began to control his communication device with movements of his cheek muscles,[21][22][23] with a rate of about one word per minute.[22] Hawking is collaborating with researchers on systems that could translate his brain patterns or facial expressions into switch activations.[23][24][25]

Rom Houben[edit]

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.[26] He was initially reported as communicating by typing into a keyboard with his right hand,[27] though the presence of a facilitator to move his hand attracted sharp criticism and strong doubts that Houben's communications were authentic.[28][29][30]

In early 2010, Dr. Steven Laureys, Houben's neurologist, admitted that subsequent tests had demonstrated 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.[31] Laureys maintained the MRI data that had led him to diagnose Houben as locked-in still suggested 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.[26][32][33]

Graham Miles[edit]

In 1993, Graham Miles, originally from Sanderstead, London Borough of Croydon, suffered a stroke after which he could not move any part of his body except his eyes. His condition improved gradually, to the point that in 2010 he was able to walk with two sticks and drive a car.[34][35]

Elias Musiris[edit]

In 2002, Elias Musiris made headlines as the first fully locked-in patient to regain some measure of communication through EEG. Though ALS had left Musiris unable even to move his eyes or blink, training from neurological researcher Niels Birbaumer taught him to use an EEG brain-machine interface to answer yes-or-no questions and spell his name.[11]

Tony Nicklinson[edit]

Tony Nicklinson, of Melksham, Wiltshire, England, was left paralyzed after suffering a stroke in June 2005,[36] at age 51. In the years that followed, he started a legal battle for a right to assisted death. On 16 August 2012, his request was turned down by the High Court of Justice.[37] Upon learning the outcome of his appeal, he refused to eat, contracted pneumonia, deteriorated rapidly, and died a week later on 22 August 2012, aged 58.[38] Nicklinson was cremated and his ashes were scattered over his local rugby pitch.[citation needed]

Gary Parkinson[edit]

In 2010, ex-Premiership footballer Gary Parkinson suffered a massive stroke and was later diagnosed with locked-in syndrome. This, however, has 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.[citation needed]

Tony Quan AKA Tempt One[edit]

Tony Quan, a popular graffiti artist, was diagnosed with the nerve disorder ALS in 2003, which eventually left him fully paralyzed except for his eyes. Quan uses the technology called EyeWriter to communicate his art and has since had his work displayed in numerous art shows nationally.[39]

Erik Ramsey[edit]

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.[40] 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, within a few years, Erik will be able to use this system to communicate words and phrases, and eventually, to "talk" normally.[13][41][42]

Julia Tavalaro[edit]

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, she was believed to be 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 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.[13][43]

Christine Waddell[edit]

Christine Waddell is Britain's longest survivor of locked-in syndrome, which left her in a state of constant paralysis, but awareness. At the age of 26 in April 1997, she fell in her bathroom. She tried to get up but ultimately fell again and lay there for three days – until a colleague noticed her absence from work and her father broke into her flat. Seventeen months in the hospital followed before she moved in with her parents. After years of suffering she was given a grant for a computer which allows her to communicate. She is now able to use the Internet and to communicate with old friends and others who have locked-in syndrome. She also listens to music and audiobooks, is able to swallow melted chocolate, and sometimes has occasional vodka via her feeding tube. She misses most the ability to talk and regrets being unable to eat burgers. In 2013 she ate for the first time in 17 years. She now stands with less support, reaching on a bench, holding her head up alone, and can ride a stationary bike in physiotherapy.[16]

Cases in literature[edit]

The Count of Monte Cristo[edit]

The character of M. Noirtier de Villefort in Alexandre Dumas' novel The Count of Monte Cristo (1844) 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.

Thérèse Raquin[edit]

In Émile Zola's novel Thérèse Raquin (1867), Thérèse Raquin and her second husband Laurent accidentally reveal to Thérèse's aunt, Madame Raquin (who has suffered from locked-in syndrome after a stroke), that they have murdered 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 and Laurent's deed. However, she is interrupted, and her words are misinterpreted as "Thérèse and Laurent have taken good care of me".

Johnny Got His Gun[edit]

Johnny Got His Gun (1938) is a novel by American author and screenwriter Dalton Trumbo, which describes a young American soldier who loses both his arms, his legs, and his face in World War I. This novel portrays "how it might feel to be totally locked-in", but the issue has nothing to do with the "locked-in syndrome", according to the WHO definition.[citation needed]

The Ultimate Secret[edit]

The character of Jean-Louis Martin in Bernard Werber's sci-fi novel L'Ultime Secret (2001),[44] suffers from locked-in syndrome after being paralyzed in a car accident. Able at first only communicate by blinking – once for "Yes" and twice for "No" – with the use of high tech, he eventually gains control not only over his own mind, but that of others.[45]

Locked In[edit]

Sharon McCone, the protagonist of Marcia Muller's suspense novel Locked In (2009), is the founder of a successful San Francisco detective agency. On returning to her office late one night, she is shot in the head. She wakes up in a hospital able to move only her eyes, forced to struggle to rehabilitate herself while finding the attacker.[citation needed]

Sleepyhead[edit]

Mark Billingham's novel Sleepyhead (2013)[46] addresses a criminal who purposely manipulates pressure points on each victim's head and neck with the intention of inducing locked-in syndrome.[47]

The Diving Bell and the Butterfly[edit]

Is a memoir by journalist Jean-Dominique Bauby. It describes what his life is like after suffering a massive stroke that left him with locked-in syndrome. It also details what his life was like before the stroke.

Cases in popular culture[edit]

Alfred Hitchcock Presents[edit]

In season 1, episode 7 of Alfred Hitchcock Presents, titled "Breakdown" (November 13, 1955 on CBS),[48] the sole survivor of a violent collision (Joseph Cotten) finds himself in a locked-in state, unable even to move an eyelid. The viewer experiences the victim's point of view, "hearing" his thoughts and feelings as they run from shock to anger to frustration to the realization that he may be put in his grave alive.[49]

Criminal Minds[edit]

In the Criminal Minds episode "The Uncanny Valley", the unsub Samantha Malcolm induces locked-in syndrome using a series of drugs in three women. Her reason is she is trying to complete a series of dolls she lost as a young girl. Every two months, a woman will die as the stress wreaks havoc on the body. Only one woman, who has diabetes, is able to counteract the drugs and fight off her locked-in syndrome.

CSI: New York[edit]

The pilot of CSI: New York presented an instance of locked-in syndrome wherein a woman (portrayed by Jewel Christian) was sedated by the killer, who applied pressure to certain points on her head, resulting in her paralysis. The killer's previous attempts resulted in his victims' dying.[citation needed]

House M.D.[edit]

The House M.D. episode "Locked In" presented a case of locked-in syndrome, which later turned into a case of total locked-in syndrome; the patient was portrayed by Mos Def.

Scrubs[edit]

In the Scrubs episode "His Story III", a patient (played by Henry LeBlanc) is presented with locked-in syndrome.[citation needed]

Star Trek[edit]

In the Star Trek episode "The Menagerie", Star Fleet captain Christopher Pike (played by Jeffrey Hunter when healthy, and Sean Kenney when injured) is severely burned, completely paralyzed, and can communicate only by brain waves; he can operate an electrical wheelchair and can answer yes/no questions by "one flash for yes, two flashes for no". This episode aired in November 1966; the first actual such interface was done by Fetz at the University of Washington in 1969, as noted in brain–computer interface.

Mimicking conditions[edit]

Curare poisoning mimics a total locked-in syndrome by causing paralysis of all voluntarily controlled skeletal muscles.[50] The respiratory muscles are also paralyzed, but the victim can be kept alive by artificial respiration, such as mouth-to-mouth resuscitation. In a study of 27 army volunteers who were paralyzed with curare, artificial respiration managed to keep an oxygen saturation of always above 85%,[51] a level at which there is no evidence of altered state of consciousness.[52] Spontaneous breathing is resumed after the end of the duration of action of curare, which is generally between 30 minutes[53] and eight hours,[54] depending on the variant of the toxin and dosage.

See also[edit]

References[edit]

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  2. ^ Agranoff, Adam B. "Stroke Motor Impairment". eMedicine. Retrieved 2007-11-29. 
  3. ^ Plum; Posner, JB (1966), The diagnosis of stupor and coma, Philadelphia, PA, USA: FA Davis , 197 pp.
  4. ^ Nordgren RE, Markesbery WR, Fukuda K, Reeves AG (1971). "Seven cases of cerebromedullospinal disconnection: the "locked-in" syndrome". Neurology 21 (11): 1140–8. doi:10.1212/wnl.21.11.1140. PMID 5166219. 
  5. ^ Flügel KA, Fuchs HH, Druschky KF (1977). "The "locked-in" syndrome: pseudocoma in thrombosis of the basilar artery (author's trans.)". Dtsch. Med. Wochenschr. (in German) 102 (13): 465–70. doi:10.1055/s-0028-1104912. PMID 844425. 
  6. ^ a b Fager, Susan; Beukelman, Karantounis, Jakobs (2006). "Use of safe-laser access technology to increase head movements in persons with severe motor impairments: a series of case reports". Augmentative and Alternative Communication 22 (3): 222–29. doi:10.1080/07434610600650318. PMID 17114165. 
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  8. ^ lockedinsyndrome at NINDS
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  17. ^ "The Diving Bell And The Butterfly". The A.V. Club. Retrieved 2007-11-29. 
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  19. ^ Rabbi Ronnie Cahana, Rabbi Ronnie Cahana's poetry and sermons
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  21. ^ Ferguson 2011, p. 224.
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  27. ^ Mark Memmott (November 23, 2009). "For 23 Years Man Thought To Be In Coma Tried To 'Scream', And No One Heard". NPR. 
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  29. ^ "Health | Comatose for 23 years, Belgian feels reborn | Seattle Times Newspaper". Retrieved 2012-03-12. 
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  33. ^ Diagnostic accuracy of the vegetative and minimally conscious state: Clinical consensus versus standardized neurobehavioral assessment Steven Laureys, Caroline Schnakers, Audrey Vanhaudenhuyse, et al
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  38. ^ "Right-to-die man Tony Nicklinson dead after refusing food". BBC News. 22 August 2012. Retrieved 22 August 2012. 
  39. ^ http://gizmodo.com/5403741/eyewriter-allows-man-to-paint-despite-paralysis http://infosthetics.com/archives/2009/11/eyewriter_graffiti_physically_paralyzed.html
  40. ^ http://www.esquire.com/features/unspeakable-odyssey-motionless-boy-1008
  41. ^ Guenther, F.H., Brumberg, J.S., Wright, E.J., Nieto-Castanon, A., Tourville, J.A., Panko, M., Law, R., Siebert, S.A., Bartels, J.L., Andreasen, D.S., Ehirim, P., Mao, H., and Kennedy, P.R. (2009). A wireless brain-machine interface for real-time speech synthesis. PLoS ONE, 4(12), pp. e8218+.
  42. ^ S.I. Rosenbaum (July 27, 2008). "Out of silence, the sounds of hope". The Boston Globe. 
  43. ^ "Julia Tavalaro, 68; Poet and Author Noted for Defying Severe Paralysis". Los Angeles Times. December 21, 2003. p. B16. 
  44. ^ Bernard Werber (2001). L'Ultime Secret [The Ultimate Secret]. ISBN 2-226-12740-2. 
  45. ^ "L'Ultime Secret". The French Publisher's Agency. 2012. Retrieved 5 February 2012. 
  46. ^ Mark Billingham (2013). Sleepyhead. Tom Thorne Series , #1. Grove/Atlantic, Inc. ISBN 9780802193261. 
  47. ^ "Publisher's description of Sleepyhead". BN.com. Retrieved February 7, 2014. 
  48. ^ "Breakdown". TV.com. Retrieved February 7, 2014. 
  49. ^ "Alfred Hitchcock Presents - Breakdown". hitchcockwiki.com. Retrieved 2 April 2014. 
  50. ^ Page 357 in: Damasio, Antonio R. (1999). The feeling of what happens: body and emotion in the making of consciousness. San Diego: Harcourt Brace. ISBN 0-15-601075-5. 
  51. ^ Page 520 in: Paradis, Norman A. (2007). Cardiac arrest: the science and practice of resuscitation medicine. Cambridge, UK: Cambridge University Press. ISBN 0-521-84700-1. 
  52. ^ Oxymoron: Our Love-Hate Relationship with Oxygen, By Mike McEvoy at Albany Medical College, New York. 10/12/2010
  53. ^ For therapeutic dose of tubocurarine by shorter limit as given at page 151 in: Rang, H. P. (2003). Pharmacology. Edinburgh: Churchill Livingstone. ISBN 0-443-07145-4. OCLC 51622037. 
  54. ^ For 20-fold paralytic dose of toxiferine ("calebas curare"), according to: Page 330 in: The Alkaloids: v. 1: A Review of Chemical Literature (Specialist Periodical Reports). Cambridge, Eng: Royal Society of Chemistry. 1971. ISBN 0-85186-257-8. 

External links[edit]