List of people with locked-in syndrome

Kate Allatt

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.^[1]

Jean-Dominique Bauby

French 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 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. Three days after the book was published in March 1997, Bauby died from pneumonia.^[2] He was instrumental in forming the Association du Locked-In Syndrome (ALIS) in France.^[3]

Rabbi Ronnie Cahana

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.^[4] His story was told in a Ted Talk given by his daughter called: "My Father, Locked-in his Body but Soaring Free" He is the son of painter Alice Lok Cahana.

Lynsey Cribbin

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.^[5]

Stephen Hawking

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,^[6][7][8] with a rate of about one word per minute.^[7] Hawking is collaborating with researchers on systems that could translate his brain patterns or facial expressions into switch activations.^[8][9][10]

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.^[11] He was initially reported as communicating by typing into a keyboard with his right hand,^[12] though the presence of a facilitator to move his hand attracted sharp criticism and strong doubts that Houben's communications were authentic.^[13][14][15]

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.^[16] 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.^[11][17][18]

Graham Miles

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.^[19][20]

Elias Musiris

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.^[21]

Tony Nicklinson

Tony Nicklinson, of Melksham, Wiltshire, England, was left paralyzed after suffering a stroke in June 2005,^[22] 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.^[23] 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.^[24] Nicklinson was cremated and his ashes were scattered over his local rugby pitch.^[25]

Eoin O'Mahony

Eoin O'Mahony of Blarney, Ireland developed headaches while studying for his Leaving Certificate in 2001. Brain surgery was carried out at Cork University Hospital. Then O'Mahony entered a coma-like state. He cannot even use a finger to press a button for assistance. The High Court has been told that he "essentially suffers from locked-in syndrome."^[26]

Gary Parkinson

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.

Martin Pistorius

Martin Pistorius began developing locked-in syndrome when he was 12 years old. He went into a coma for 2–3 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 computer, Pistorius currently works as a freelance web designer/developer and has published a book about his life entitled Ghost Boy.^[27][28][29]

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 uses the technology called EyeWriter to communicate his art and has since had his work displayed in numerous art shows nationally.^[30]

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.^[31] 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.^[32][33][34]

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, 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 Richard E. Meyer of the Los Angeles Times published a cover story about Tavalaro. In 1997, Erika Duncan's profile of Julia and her co-author Richard Tayson, "Decades After Silence, a Voice Is Recognized," ran in the Long Island edition of The New York Times and in April 1997, "The Long Road Home" appeared in Newsday. Julia Tavalaro appears with Richard Tayson on Dateline NBC and Melissa Etheridge's Beyond Chance (Lifetime). Their book was published by Viking-Penguin in 1998 and was translated into German, where it was published as Bis auf den Grund des Ozeans by Verlag Herder. Tavalaro's story became a bestseller in Germany. She died on December 19, 2003 at the age of 68.^[32][35]

Christine Waddell

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.^[1]

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 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.

A Song of Ice and Fire

In the first novel A Game of Thrones, the character Khal Drogo succumbs into a vegetative state after suffering septicemia 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 that he can sense it only 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 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

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 his eyelids and weakly chanting "SOS Help me".

The Ultimate Secret

The character of Jean-Louis Martin in Bernard Werber's sci-fi novel L'Ultime Secret (2001),^[36] 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.^[37]

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.

Sleepyhead

Mark Billingham's novel Sleepyhead (2013)^[38] addresses a criminal who purposely manipulates pressure points on each victim's head and neck with the intention of inducing locked-in syndrome.^[39]

The Diving Bell and the Butterfly

Bauby on "dictating" the book through blinking

The book 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.

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.

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 computer to communicate.

Cases in popular culture

Alfred Hitchcock Presents

In season 1, episode 7 of Alfred Hitchcock Presents, titled "Breakdown" (November 13, 1955 on CBS),^[40] 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.^[41]

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 use locked-in syndrome after a man falls into locked-in syndrome after surviving a botched lobotomy and communicates to Derek (Shemar Moore) through blinks.

CSI: New York

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.

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.

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". 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"

References