Locked-In syndrome
Encyclopedia
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. The term for this disorder was coined by Fred Plum
Fred Plum
Fred Plum was an American neurologist who developed the terms"persistent vegetative state" and "locked-in syndrome" as part of his continuing research on consciousness and comas and care of the comatose....
and Jerome Posner
Jerome B. Posner
Jerome B. Posner is an American neurologist and co-author of Plum and Posner's Diagnosis of Stupor and Coma. Dr. Posner has served as chief of the Neuro-Psychiatry Service of the Department of Medicine, chair of the Department of Neurology and currently occupies the George C...
in 1966. Locked-in syndrome is also known as cerebromedullospinal disconnection, de-efferented state, pseudocoma, and ventral pontine syndrome.
Presentation
Locked-in syndrome usually results in quadriplegiaQuadriplegia
Tetraplegia, also known as quadriplegia, is paralysis caused by illness or injury to a human that results in the partial or total loss of use of all their limbs and torso; paraplegia is similar but does not affect the arms...
and the inability to speak
Manner of articulation
In linguistics, manner of articulation describes how the tongue, lips, jaw, and other speech organs are involved in making a sound. Often the concept is only used for the production of consonants, even though the movement of the articulars will also greatly alter the resonant properties of the...
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
Sleep paralysis
Sleep paralysis is paralysis associated with sleep that may occur in healthy persons or may be associated with narcolepsy, cataplexy, and hypnagogic hallucinations. The pathophysiology of this condition is closely related to the normal hypotonia that occurs during REM sleep. When considered to be a...
. Patients who have locked-in syndrome are conscious and aware with no loss of cognitive function. They can sometimes retain proprioception
Proprioception
Proprioception , from Latin proprius, meaning "one's own" and perception, is the sense of the relative position of neighbouring parts of the body and strength of effort being employed in movement...
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. This restricts them from producing voluntary sounds, even though the vocal cords themselves are not paralysed.
Causes
Persistent vegetative state
A persistent vegetative state is a disorder of consciousness in which patients with severe brain damage are in a state of partial arousal rather than true awareness. It is a diagnosis of some uncertainty in that it deals with a syndrome. After four weeks in a vegetative state , the patient is...
, 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:
- Traumatic brain injuryTraumatic brain injuryTraumatic brain injury , also known as intracranial injury, occurs when an external force traumatically injures the brain. TBI can be classified based on severity, mechanism , or other features...
- Diseases of the circulatory systemCirculatory systemThe circulatory system is an organ system that passes nutrients , gases, hormones, blood cells, etc...
- Medication overdose
- Damage to nerve cells, particularly destruction of the myelin sheath, caused by disease (e.g. central pontine myelinolysisCentral pontine myelinolysisCentral pontine myelinolysis is neurological disease caused by severe damage of the myelin sheath of nerve cells in the brainstem, more precisely in the area termed the pons, predominately of iatrogenic etiology...
secondary to rapid correction of hyponatremiaHyponatremiaHyponatremia is an electrolyte disturbance in which the sodium concentration in the serum is lower than normal. In the vast majority of cases, hyponatremia occurs as a result of excess body water diluting the serum sodium and is not due to sodium deficiency. Sodium is the dominant extracellular...
) - A stroke or brain hemorrhage, usually of the basilar arteryBasilar arteryIn human anatomy, the basilar artery is one of the arteries that supplies the brain with oxygen-rich blood.The two vertebral arteries and the basilar artery are sometimes together called the vertebrobasilar system, which supplies blood to the posterior part of circle of Willis and anastomoses with...
- A bite from a snake most commonly found in India (Common Krait snake)
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. Assistive computer interface technologies, such as DasherDasher
Dasher is a computer accessibility tool which enables users to write without using a keyboard, by entering text on a screen using a pointing device such as a mouse, a touchpad, a touch screen, a roller ball, a joystick, a Push-button, a Wii Remote, or even mice operated by the foot or head...
in combination with eye tracking
Eye tracking
Eye tracking is the process of measuring either the point of gaze or the motion of an eye relative to the head. An eye tracker is a device for measuring eye positions and eye movement. Eye trackers are used in research on the visual system, in psychology, in cognitive linguistics and in product...
, may be used to help patients communicate. New direct brain interface mechanisms may provide future remedies. Israeli scientists have reported that they have developed a technique that allows locked-in patients to communicate via sniffing.
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.Jean-Dominique Bauby
Parisian journalist Jean-Dominique BaubyJean-Dominique Bauby
Jean-Dominique Bauby was a well-known French journalist, author and editor of the French fashion magazine ELLE.On 8 December 1995 at the age of 43, Bauby suffered a massive stroke. When he woke up twenty days later, he found he was entirely speechless; he could only blink his left eyelid...
suffered a stroke
Stroke
A stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...
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
The Diving Bell and the Butterfly
The Diving Bell and the Butterfly is a translation of the French memoir Le scaphandre et le papillon by journalist Jean-Dominique Bauby. It describes what his life is like after suffering a massive stroke that left him with a condition called locked-in syndrome...
. Three days after it was published in March 1997, Bauby died of pneumonia
Pneumonia
Pneumonia is an inflammatory condition of the lung—especially affecting the microscopic air sacs —associated with fever, chest symptoms, and a lack of air space on a chest X-ray. Pneumonia is typically caused by an infection but there are a number of other causes...
. The 2007 film The Diving Bell and the Butterfly
The Diving Bell and the Butterfly (film)
The Diving Bell and the Butterfly is a 2007 biographical drama film based on Jean-Dominique Bauby's memoir of the same name. The film depicts Bauby's life after suffering a massive stroke, on December 8, 1995, at the age of 42, which left him with a condition known as locked-in syndrome. The...
is a screen adaptation of Bauby's memoir. Jean-Dominique was instrumental in forming the Association du Locked-In Syndrome (ALIS) in France
France
The French Republic , The French Republic , The French Republic , (commonly known as France , is a unitary semi-presidential republic in Western Europe with several overseas territories and islands located on other continents and in the Indian, Pacific, and Atlantic oceans. Metropolitan France...
.
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 TimesLos Angeles Times
The Los Angeles Times is a daily newspaper published in Los Angeles, California, since 1881. It was the second-largest metropolitan newspaper in circulation in the United States in 2008 and the fourth most widely distributed newspaper in the country....
published her life story. It was republished by Newsday
Newsday
Newsday is a daily American newspaper that primarily serves Nassau and Suffolk counties and the New York City borough of Queens on Long Island, although it is sold throughout the New York metropolitan area...
on Long Island and in other newspapers across the country. She died in 2003 at the age of 68.
Gary Griffin
Gary Griffin was a veteran of the United States Air Force who became immobile due to amyotrophic lateral sclerosisAmyotrophic lateral sclerosis
Amyotrophic lateral sclerosis , also referred to as Lou Gehrig's disease, is a form of motor neuron disease caused by the degeneration of upper and lower neurons, located in the ventral horn of the spinal cord and the cortical neurons that provide their efferent input...
(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
YouTube
YouTube is a video-sharing website, created by three former PayPal employees in February 2005, on which users can upload, view and share videos....
.
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 EsquireEsquire (magazine)
Esquire is a men's magazine, published in the U.S. by the Hearst Corporation. Founded in 1932, it flourished during the Great Depression under the guidance of founder and editor Arnold Gingrich.-History:...
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.
Rom Houben
In 1983, Rom HoubenRom Houben
Rom Houben is a 48-year-old Belgian man presumed comatose and in a vegetative state for 23 years after a near-fatal automobile accident, but according to several of his caregivers, was conscious and paralyzed during the entirety of his hospital stay...
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. He was initially reported as communicating by typing into a keyboard with his right hand, though the presence of a facilitator
Facilitated communication
Facilitated communication is a process by which a facilitator supports the hand or arm of a communicatively impaired individual while using a keyboard or other devices with the aim of helping the individual to develop pointing skills and to communicate...
to move his hand attracted sharp criticism and strong doubts that Houben's communications were authentic.
In early 2010, Dr. Steven Laureys
Steven Laureys
Steven Laureys is a Belgian neurologist.-Career:Laureys graduated as a Medical Doctor from the Vrije Universiteit Brussel, Belgium, in 1993. While specializing in neurology he entered a research career and obtained his M.Sc. in Pharmaceutical Medicine working on pain and stroke using in vivo...
, Houben's neurologist, admitted that subsequent tests had demonstrated that Houben had not actually been communicating via the facilitator, and Der Spiegel
Der Spiegel
Der Spiegel is a German weekly news magazine published in Hamburg. It is one of Europe's largest publications of its kind, with a weekly circulation of more than one million.-Overview:...
, which had originally "quoted" many of Houben's facilitated statements, retracted those quotes as being inauthentic. Laureys maintained that the MRI
Magnetic resonance imaging
Magnetic resonance imaging , nuclear magnetic resonance imaging , or magnetic resonance tomography is a medical imaging technique used in radiology to visualize detailed internal structures...
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.
Graham Miles
In 1993, Graham Miles, originally from SandersteadSanderstead
Sanderstead is a village in London Borough of Croydon, located on high ground at the edge of the built-up area of Greater London. From 1915 to 1965 it formed a parish in the Coulsdon and Purley Urban District of Surrey. Having been a farming community in previous centuries, Sanderstead is now...
, Surrey
Surrey
Surrey is a county in the South East of England and is one of the Home Counties. The county borders Greater London, Kent, East Sussex, West Sussex, Hampshire and Berkshire. The historic county town is Guildford. Surrey County Council sits at Kingston upon Thames, although this has been part of...
, suffered a stroke
Stroke
A stroke, previously known medically as a cerebrovascular accident , is the rapidly developing loss of brain function due to disturbance in the blood supply to the brain. This can be due to ischemia caused by blockage , or a hemorrhage...
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.
Thérèse Raquin
In the French novel Thérèse RaquinThérèse Raquin
Thérèse Raquin is the title of a novel and a play by the French writer Émile Zola. The novel was originally published in serial format in the journal L'Artiste and in book format in December of the same year.-Plot introduction:Thérèse Raquin tells the story of a young woman, unhappily married to...
, by Émile Zola
Émile Zola
Émile François Zola was a French writer, the most important exemplar of the literary school of naturalism and an important contributor to the development of theatrical naturalism...
, 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 CristoThe Count of Monte Cristo
The Count of Monte Cristo is an adventure novel by Alexandre Dumas. It is often considered to be, along with The Three Musketeers, Dumas's most popular work. He completed the work in 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.
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 InLocked In (House)
"Locked In" is the nineteenth episode of the fifth season of House. It aired on March 30, 2009.- Plot :Gregory House is injured in a motorcycle accident in Middletown, NY and finds himself in bed next to a patient suffering from locked-in syndrome after a bicycling accident...
, the patient was portrayed by Mos Def
Mos Def
Dante Terrell Smith is an American actor and Emcee known by the stage names Mos Def and Yasiin Bey. He started his hip hop career in a group called Urban Thermo Dynamics, after which he appeared on albums by Da Bush Babees and De La Soul. With Talib Kweli, he formed the duo Black Star, which...
.
Scrubs
On an episode of ScrubsScrubs (TV series)
Scrubs is an American medical comedy-drama television series created in 2001 by Bill Lawrence and produced by ABC Studios. The show follows the lives of several employees of the fictional Sacred Heart, a teaching hospital. It features fast-paced screenplay, slapstick, and surreal vignettes...
entitled "His Story III", a patient is presented with locked-in syndrome. The character is played by Eric McNair.
Johnny Got His Gun
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 Ultimate Secret
The character Jean-Louis Martin (Bernard Werber) suffers from locked-in syndrome after being paralysed in a car incident. 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.See also
- Akinetic mutismAkinetic mutismAkinetic mutism is a medical term describing patients who tend neither to speak nor move . It is the result of severe frontal lobe injury in which the pattern of inhibitory control is one of increasing passivity and gradually decreasing speech and motion.An example of a cause of this disorder...
- Gary ParkinsonGary ParkinsonGary Parkinson is a retired England|English footballer who played as a right-back.In 2006 he was appointed Head of Youth at his former club Blackpool, a role he occupied until he suffered a severe stroke in September 2010....
- Association football scout who continued in his job by watching DVDs of players from his bed, and then communicating his analysis to his wife by blinking.