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Locked-in syndrome

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Locked-in syndrome
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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] It is the result of a brain stem lesion in which the ventral (anterior) part of the pons is damaged. The term for this disorder was coined by Fred Plum and Posner in 1966.[2][3] In French, the common term is maladie de l'emmuré vivant, literally translated as "walled-in-alive disease"; in German, it is sometimes called Eingeschlossensein.[4] Locked-in syndrome is also known as cerebromedullospinal disconnection,[5] de-efferented state, pseudocoma,[6] and ventral pontine syndrome.

Presentation

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.[7] This restricts them from producing voluntary sounds, even though the vocal cords themselves are not paralysed.[7]

Causes

In children, the most common cause is a stroke of the ventral pons.[8]

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

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.[9] 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.[10][11] Israeli scientists have reported that they have developed a technique that allows locked-in patients to communicate via sniffing.[12]

Prognosis

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.[13]

Notable cases

Ahmed Deedat

Sheikh Ahmed Deedat was a popular muslim scholar of the bible and public speaker. After a long and prolific career as a public speaker, Ahmed Deedat suffered from a stroke which paralysed everry voluntary muscle of the body except for his eyes. He survived with locked-in syndrome for 9 years before dieing on August 8, 2005, at the age of 87.

Jean-Dominique Bauby

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. Two days after it was published in March 1997, Bauby died of pneumonia.[14] 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) "Association du Locked In Syndrome".

Julia Tavalaro

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.[13][15]

Gary Griffin

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. A video of Griffin and his use of the NeuroSwitch has been posted on YouTube.[16]

Erik Ramsey

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.[13][17]

Rom Houben

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.[18] He was initially reported as communicating by typing into a keyboard with his right hand,[19] though the presence of a facilitator to move his hand attracted sharp criticism and strong doubts that Houben's communications were authentic.[20][21][22]

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.[23] 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.[18][24][25]

Graham Miles

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.[26][27]

Johnny Got His Gun

The main character of Dalton Trumbo's novel Johnny Got His Gun is a soldier whose body is so badly damaged by a shell blast that he is essentially in locked-in syndrome (although the term had not been coined when the book was written). He eventually learns to communicate using Morse code by banging on the pillow with the back of his head. The song "One" by heavy metal band Metallica was based on the novel.

Thérèse Raquin

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 Count of Monte Cristo

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.

House M.D

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

References

  1. ^ Bauer, G. and Gerstenbrand, F. and Rumpl, E. (1979). "Varieties of the locked-in syndrome". Journal of Neurology. 221 (2): 77–91. doi:10.1007/BF00313105. PMID 92545.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. ^ "eMedicine - Stroke Motor Impairment : Article by Adam B Agranoff, MD". Retrieved 2007-11-29.
  3. ^ Plum F. and Posner J.B. 1966. The diagnosis of stupor and coma. F.A. Davis Co. Philadelphia, Pennsylvania, USA. 197 pp.
  4. ^ "Scientists seek to help 'locked-in' man speak", CNN, 14 December 2007
  5. ^ Nordgren RE, Markesbery WR, Fukuda K, Reeves AG (1971). "Seven cases of cerebromedullospinal disconnection: the "locked-in" syndrome". Neurology. 21 (11): 1140–8. PMID 5166219.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  6. ^ 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.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  7. ^ a b Fager, Susan (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): 222–229. doi:10.1080/07434610600650318. PMID 17114165. {{cite journal}}: Unknown parameter |coauthors= ignored (|author= suggested) (help)
  8. ^ Bruno MA, Schnakers C, Damas F; et al. (2009). "Locked-in syndrome in children: report of five cases and review of the literature". Pediatr. Neurol. 41 (4): 237–46. doi:10.1016/j.pediatrneurol.2009.05.001. PMID 19748042. {{cite journal}}: Explicit use of et al. in: |author= (help); Unknown parameter |month= ignored (help)CS1 maint: multiple names: authors list (link)
  9. ^ lockedinsyndrome at NINDS
  10. ^ Parker, I., "Reading Minds," The New Yorker, January 20, 2003, 52-63
  11. ^ Turning Thoughts into Words By Chris Berdik, Research at Boston University 2008 magazine (reprinted in BU Today), October 15, 2008.
  12. ^ "'Locked-In' Patients Can Follow Their Noses". Science Mag.org. 26 Jul 2010. Retrieved 27 Jul 2010.
  13. ^ a b c The Unspeakable Odyssey of the Motionless Boy by Joshua Foer, Esquire Magazine, October 2, 2008.
  14. ^ "The Diving Bell And The Butterfly". The A.V. Club. Retrieved 2007-11-29.
  15. ^ "Julia Tavalaro, 68; Poet and Author Noted for Defying Severe Paralysis". Los Angeles Times. December 21, 2003. p. B16.
  16. ^ NeuroSwitch Enables Veteran with Locked in Syndrome at Youtube.com
  17. ^ S.I. Rosenbaum (July 27, 2008). "Out of silence, the sounds of hope". The Boston Globe.
  18. ^ a b Connolly, Kate (23 November 2009). "Trapped in his own body for 23 years - the coma victim who screamed unheard". The Guardian. Berlin.
  19. ^ Mark Memmott (November 23, 2009). "For 23 Years Man Thought To Be In Coma Tried To 'Scream', And No One Heard". NPR.
  20. ^ PZ Myers (November 24, 2009). "Really? This guy is conscious?". Pharyngula.
  21. ^ Raf Casert (November 24, 2009). "Comatose for 23 years Belgian feels reborn".
  22. ^ Appel, Jacob M. (Nov. 24, 2009). "The Rom Houben Tragedy And The Case For Active Euthanasia". The Huffington Post. {{cite news}}: Check date values in: |date= (help)
  23. ^ Manfred Dworschak (February 13, 2010). "Communicating with Those Trapped within Their Brains". Der Spiegel.
  24. ^ Caroline Schnakers, Audrey Vanhaudenhuyse, Joseph Giacino, Manfredi Ventura, Melanie Boly, Steve Majerus, Gustave Moonen, Steven Laureys (2009). "Diagnostic accuracy of the vegetative and minimally conscious state: Clinical consensus versus standardized neurobehavioral assessment". BMC Neurology. 9: 35. doi:10.1186/1471-2377-9-35. PMC 2718857. PMID 19622138.{{cite journal}}: CS1 maint: multiple names: authors list (link) CS1 maint: unflagged free DOI (link)
  25. ^ Diagnostic accuracy of the vegetative and minimally conscious state: Clinical consensus versus standardized neurobehavioral assessment Steven Laureys, Caroline Schnakers, Audrey Vanhaudenhuyse, et al
  26. ^ Alleyne, Richard (August 26, 2010). "Pensioner tells how he beat "locked in" syndrome after massive stroke - Telegraph". The Daily Telegraph. London. Retrieved 2010-09-03.
  27. ^ "BBC News - Locked-in syndrome man learns to walk again". August 26, 2010. Retrieved 2010-09-03.

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