List of people with locked-in syndrome

This is a list of notable people with locked-in syndrome, a syndrome where a patient is conscious and self-aware but is completely paralyzed.

This is a dynamic list and may never be able to satisfy particular standards for completeness. You can help by adding missing items with reliable sources.

Gil Avni

Israeli citizen Gil Avni, then 33, experienced complete locked-in syndrome (CLIS) at Meir Hospital, Kefar Sava, Israel, after experiencing an unknown health impacting incident at his home while watching TV. When Avni's wife, Orit, a nurse, arrived home, she found him bearing a pulse rate of about 160, making 50 breaths per minute, with symptoms of cyanosis, and called an ambulance. During transport, a dangerously low oxygen saturation of 50% was detected. When reaching the hospital, Avni was anesthetized and ventilated, later (after CT) diagnosed with cerebral edema. Avni, since arriving at the hospital, consciously experienced and remembered most if not all of his surroundings audio-visually, later auditory (with eye lids closed), including after anesthesia.

Avni remained in hospital in critical condition due to continuing low oxygen saturation and pulse rates now reaching 180-200. After almost two days, when his family had already given him a last goodbye, his sedatives were reduced. This allowed him to regain the ability for eye movement, later full body control. At the same time, his oxygen saturation returned to normal, and lung ventilation could finally be removed. He has since fully recovered - apart from a PTSD stemming from this experience.

Avni remembered and reported most, if not all, the conversations taking place during the hospital period, both days and nights, and was able to precisely cite conversations, and people encountered. The documentary film 44 hours^[1] covers his unique experience in his own words, as well as the reactions of his wife and friends, and those treating him in hospital, some of whom are first confronted with details of what Gil Avni reports occurred during the hospital visit - and confirm it.^[2]

Jean-Dominique Bauby

French journalist Jean-Dominique Bauby had a stroke in December 1995. 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 length of time to write his memoir, The Diving Bell and the Butterfly; the memoir was adapted to the screen in a namesake 2007 movie. Two days after the book was published in March 1997, Bauby died from pneumonia.^[3] He was instrumental in forming the Association du Locked in Syndrome (ALIS) in France.^[4]

Nick Chisholm

Nick Chisholm (born 1973 in Dunedin, New Zealand), the brother of Survivor NZ host Matt Chisholm, had a series of mini-strokes, culminating in a massive brainstem stroke during a rugby game at the age of 27 on 29 July 2000. He has since recovered some muscle usage, and has become a bodybuilder and a personal trainer for other disabled people. He can't speak, but communicates via pointedly moving his eyes around a clear plastic board with letters and number on it to spell out what he wants to say. On March 26, 2020, his wife Nicola gave birth to triplets, conceived with Nick via IVF.^[5][6]

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.^[7] He was initially reported as communicating by typing into a keyboard with his right hand,^[8] though the presence of a facilitator to move his hand attracted sharp criticism and strong doubts that Houben's communications were authentic.^[9][10][11]

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.^[12] 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.^[7][13]

Tony Nicklinson

Tony Nicklinson was born on 2 April 1954. Nicklinson was a rugby union player and a successful civil engineer. The 58 year old was paralyzed from the neck down after having a stroke in 2005. He was not able to speak or move any parts of his body apart from his head and eyes. He had spent two-and-a-half years undergoing therapy in a hospital before moving home in a wheelchair to be cared for by his wife, Jane, and his two teenage daughters Lauren and Beth. He described his life as a "living nightmare".

Nicklinson attempted to seek a landmark ruling in the British courts which would have allowed him the right to an assisted death, but he lost the case in the High Court.^[14] He died on 20 August 2012 at his home in Melksham, Wiltshire by refusing food. His family continued his case after his death, before it was ultimately rejected in the Supreme Court.^[15]

See also: R (Nicklinson) v Ministry of Justice

Gary Parkinson

In 2010, former Premiership footballer Gary Parkinson had a massive stroke and was later diagnosed with locked-in syndrome.^[16] 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.^[17]

Martin Pistorius

Martin Pistorius began developing locked-in syndrome when he was 12 years old. He went into a coma for two to three years, after which point he slowly regained consciousness but was unable to communicate this to others until he was around 19 years of age. Now capable of some movement and able to communicate with a speech synthesizer, Pistorius currently works as a freelance web designer/developer and has published a book about his life entitled Ghost Boy.^[18][19][20]

Tony Quan, aka Tempt One

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 used the technology called EyeWriter to communicate his art and has since had his work displayed in numerous art shows nationally.^[21][22]

Cases in literature

The Count of Monte Cristo

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

A Song of Ice and Fire

In the first novel A Game of Thrones, the character Khal Drogo enters into a vegetative state after suffering sepsis countered by blood magic, resulting in complete paralysis. Though he is able to move his eyes along the orbit of the sun, he is implied to be blind and only able to sense it because of the heat. His wife, Daenerys, ultimately suffocates him out of pity.

Thérèse Raquin

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 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

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 it is not a true case of "locked-in syndrome", according to the WHO definition. Johnny attempts to communicate with the outside world using Morse code through banging his head on his pillow and weakly chanting (in his mind) "SOS Help me".

The Ultimate Secret

The character of Jean-Louis Martin in Bernard Werber's sci-fi novel L'Ultime Secret (2001),^[23] has 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 those of others.^[24]

Locked In

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.^[25]

Sleepyhead

Mark Billingham's novel Sleepyhead (2013)^[26] addresses a criminal who purposely manipulates pressure points on each victim's head and neck with the intention of inducing locked-in syndrome.^[27]

The Diving Bell and the Butterfly

The book The Diving Bell and the Butterfly is a memoir by journalist Jean-Dominique Bauby. It describes what his life is like following a massive stroke that left him with locked-in syndrome. It also details what his life was like before the stroke.^[28]

Lock In

John Scalzi's science fiction police procedural Lock In is based on a society where large numbers have the locked-in-like Haden's Syndrome due to a pandemic, and are able to interact with the world through BCI-controlled bodies.^[29]

Lamikorda

In D. R. Merrill's 2014 science-fiction novel, the Alplai virologist and epidemiologist Gihuunak appears to have a form of locked-in syndrome, being confined to a motorized wheelchair and using a speech synthesizer to communicate.^[30]

Cases in popular culture

The 100

In season 6, episode 4 of The 100, titled "The Face Behind the Glass", the main character (Clarke Griffin) is shot in the neck with a paralytic dart, resulting in a locked-in state in which she was able to move only her eyes voluntarily.^[31]

Alfred Hitchcock Presents

In season 1, episode 7 of Alfred Hitchcock Presents, titled "Breakdown" (November 13, 1955, on CBS),^[32] 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.^[33]

Breaking Bad

Hector Salamanca, a character on Breaking Bad and Better Call Saul, was left paralyzed after having a stroke. Initially unable to move any part of his body, he later gained use of his right index finger and rang a bell attached to his wheelchair to communicate.^[34]

Criminal Minds

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. The episode "To Bear Witness" also uses locked-in syndrome; in it a man falls into locked-in syndrome after surviving a botched lobotomy and communicates to Derek (Shemar Moore) through blinks.^[35]

CSI: New York

The CSI: NY episode "Blink" 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.

Forever

In the Forever episode "The Last Death of Henry Morgan," Henry confronts his immortal stalker, Adam, and lures Adam close enough to plunge a needle into his neck and inject a full syringe of air. We later see Adam now has Locked-In Syndrome from an air embolism to his brainstem. The scene is from Adam's point of view, the image out of focus and voices mildly distorted. The doctor tells Henry Adam's condition could last a lifetime - the only way to stop Adam killing mortals and tormenting Henry will be to keep him alive in this state.

House M.D.

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.^[36]

Scrubs

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

Star Trek

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.

TED Talks

On the TED Talk website a talk was posted about the story of one family's journey with a brainstem stroke called "My Father, Locked-in his Body but Soaring Free".^[37] Another talk was given about graffiti artist TEMPT and the open source eye tracking device that was developed for him by his friends: "The Invention That Unlocked A Locked In Artist".^[38]

Calvary

In Calvary Dr. Frank Harte (Aidan Gillen) tells Father James (Brendan Gleeson) a story about a small child rendered deaf, mute, paralyzed and blind after botched anaesthesia, and contemplates the ineffable terror of such sensory isolation.

References

1. "44h". Neue Celluloid Fabrik – Film Production and Documentaries. 2022-11-28. Retrieved 2024-02-13.
2. "Gefangen im eigenen Körper - Dokumentation & Reportage - ARD" [ARD documentary: Trapped in your own body]. Das Erste (in German). Retrieved 2024-02-13.
3. Robinson, Tasha (November 29, 2007). "The Diving Bell And The Butterfly". The A.V. Club. Retrieved November 22, 2018.
4. "Association du Locked in Syndrome" (in French). FR. Retrieved November 22, 2018.
5. Caldwell, Olivia (June 9, 2019). "A stroke on the rugby field left Nick Chisholm with the life-changing syndrome that would have killed most". Stuff.co.nz.
6. Holden, Joanne (June 14, 2020). "Brother of Survivor NZ host Matt Chisholm's defies locked-in syndrome to become parent". Stuff.co.nz.
7. Connolly, Kate (November 23, 2009). "Trapped in his own body for 23 years – the coma victim who screamed unheard". The Guardian. UK. Retrieved November 22, 2018.
8. Memmott, Mark (November 23, 2009). "For 23 Years Man Thought To Be In Coma Tried To 'Scream', And No One Heard". NPR. Retrieved November 22, 2018.
9. Myers, PZ (November 24, 2009). . Pharyngula. Archived from the original on November 27, 2009. Retrieved November 22, 2018.
10. Casert, Raf (November 25, 2009). "Comatose for 23 years, Belgian feels reborn". The Seattle Times. Retrieved November 22, 2018.
11. Appel, Jacob M. (November 24, 2009). "The Rom Houben Tragedy And The Case For Active Euthanasia". The Huffington Post. Retrieved November 22, 2018.
12. Dworschak, Manfred (February 13, 2010). "Communicating with Those Trapped within Their Brains". Der Spiegel. DE. Retrieved November 22, 2018.
13. Schnakers, Caroline; Vanhaudenhuyse, Audrey; Giacino, Joseph; Ventura, Manfredi; Boly, Melanie; Majerus, Steve; Moonen, Gustave; Laureys, Steven (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.
14. Gallagher, James (August 16, 2012). "Tony Nicklinson loses High Court right-to-die case". BBC News. Retrieved November 22, 2018.
15. "Campaigners lose 'right to die' case". BBC News. July 25, 2014. Retrieved November 22, 2018.
16. "Desperate fight for Pool coach". The Gazette. September 18, 2010. Retrieved November 22, 2018.
17. "Middlesbrough hand Gary Parkinson role despite crippling condition". The Guardian. November 22, 2011. Retrieved November 22, 2018.
18. Deveney, Catherine (July 17, 2011). . Scotland on Sunday. Archived from the original on March 29, 2015. Retrieved November 22, 2018.(subscription required)
19. "Martin Pistorius and Rebecca Grant". BBC Radio London. Retrieved November 22, 2018.
20. Hager, Emily B. (July 26, 2012). "For Children Who Cannot Speak, a True Voice via Technology". The New York Times. Retrieved November 22, 2018.
21. Golijan, Rosa (November 13, 2009). "EyeWriter Allows Man To Paint Despite Paralysis". Gizmodo.
22. . November 11, 2009. Archived from the original on July 4, 2017. Retrieved November 22, 2018.
23. Werber, Bernard (2001). L'Ultime Secret [The Ultimate Secret]. Le Grand livre du mois. ISBN 2-226-12740-2.
24. . The French Publisher's Agency. Archived from the original on July 2, 2012. Retrieved November 22, 2018.
25. Muller, Marcia (2009). Locked In. Grand Central Publishing. ISBN 9780446581059.
26. Billingham, Mark (2013). Sleepyhead. Tom Thorne Series, #1. Grove/Atlantic, Inc. ISBN 9780802193261.
27. "Publisher's description of Sleepyhead". BN.com. Retrieved November 22, 2018.
28. Bauby, Jean-Dominique (1997). The Diving Bell and the Butterfly (in French). Éditions Robert Laffont. ISBN 978-0-375-40115-2.
29. Scalzi, John (2014). Lock In. Tor Books. ISBN 978-0-7653-7586-5.
30. Merrill, D. R. (2014). Lamikorda. CreateSpace Independent Publishing Platform. ISBN 9781499346350.
31. "The Face Behind the Glass". The 100. Season 6. Episode 4. May 21, 2019.
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34. "Something Stupid". Better Call Saul. Season 4. Episode 7. September 17, 2018.
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37. Cahana, Kitra (2014-10-17). "My father, locked in his body but soaring free". TED.com. Retrieved 2024-02-13.
38. Ebeling, Mick (2011-04-07). "The invention that unlocked a locked-in artist". TED.com. Retrieved 2024-02-13.