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{{short description|Condition in which a patient is aware but completely paralysed}}
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{{Infobox disease
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| Name = Locked-in syndrome
{{Infobox medical condition (new)
| Image = CerebellumArteries.jpg
| Caption = Locked-in syndrome can be caused by stroke at the level of the [[basilar artery]] denying blood to the [[pons]], among other causes.
| name = Locked-in syndrome
| synonyms = Cerebromedullospinal disconnection,<ref name="pmid5166219">{{cite journal |vauthors=Nordgren RE, Markesbery WR, Fukuda K, Reeves AG | title= Seven cases of cerebromedullospinal disconnection: the "locked-in" syndrome |journal=Neurology |volume=21 |issue=11 |pages=1140–8 |year=1971 |pmid=5166219 |doi=10.1212/wnl.21.11.1140| s2cid= 32398246 }}</ref> de-efferented state, pseudocoma,<ref name="pmid844425">{{cite journal |vauthors=Flügel KA, Fuchs HH, Druschky KF |title=The "locked-in" syndrome: pseudocoma in thrombosis of the basilar artery (author's trans.) |language=de |journal=Deutsche Medizinische Wochenschrift |volume=102 |issue=13 |pages=465–70 |year=1977 |pmid=844425 |doi=10.1055/s-0028-1104912}}</ref> ventral pontine syndrome
| DiseasesDB =
| ICD10 = {{ICD10|G|93|8|g|40}}
| image = CerebellumArteries.svg
| caption = Locked-in syndrome can be caused by a stroke at the level of the [[basilar artery]] denying blood to the [[pons]], among other causes.
| ICD9 = {{ICD9|344.81}}
| ICDO =
| field = [[Neurology]], [[Psychiatry]]
| OMIM =
| symptoms =
| MedlinePlus =
| complications =
| eMedicineSubj =
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| duration =
| eMedicineTopic =
| MeshID = D011782
| types =
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'''Locked-in syndrome''' ('''LIS'''), also known as '''pseudocoma''', 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 their body except for vertical eye movements and blinking.<ref>{{cite journal|url=https://www.ncbi.nlm.nih.gov/books/NBK559026|title=Locked-in Syndrome|first1=Joe|last1=Das|first2=Kingsley|last2=Anosike|first3=Ria Monica|last3=Asuncion|date=2022|access-date=10 June 2023|website=[[National Center for Biotechnology Information]]|pmid=32644452 }}</ref> The individual is conscious and sufficiently intact cognitively to be able to communicate with eye movements.<ref>{{Cite book|title=motor speech disorders substrates, differential diagnosis, and management|last=Duffy|first=Joseph|publisher=Elsevier|pages=295}}</ref> [[Electroencephalography]] results are normal in locked-in syndrome.
'''Locked-in syndrome''', or '''LIS''' for short, 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 wherein the eyes are paralyzed, as well.<ref name= "bauer1979varieties">{{cite journal | doi = 10.1007/BF00313105 | author =Bauer, G. and Gerstenbrand, F. and Rumpl, E. | title= Varieties of the locked-in syndrome | journal= Journal of Neurology | volume =221 |issue=2 |pages=77–91 |year=1979 |pmid=92545}}</ref> [[Fred Plum]] and [[Jerome B. Posner|Jerome Posner]] coined the term for this disorder in 1966.<ref name="titleeMedicine - Stroke Motor Impairment : Article by Adam B Agranoff, MD">{{cite web |url=http://www.emedicine.com/pmr/topic189.htm |title= Stroke Motor Impairment | first = Adam B | last = Agranoff | accessdate = 2007-11-29 | work = eMedicine}}</ref><ref>{{Citation | last1 = Plum | last2 = F | last2 = Posner | first2 = JB | year = 1966 | title = The diagnosis of stupor and coma | publisher = FA Davis | place = Philadelphia, PA, USA}}, 197 pp.</ref> Locked-in syndrome is also known as '''cerebromedullospinal disconnection''',<ref name="pmid5166219">{{cite journal | author =Nordgren RE, Markesbery WR, Fukuda K, Reeves AG | title= Seven cases of cerebromedullospinal disconnection: the "locked-in" syndrome |journal=Neurology |volume=21 |issue=11 |pages=1140–8 |year=1971 |pmid=5166219 |doi=}}</ref> '''de-efferented state''', '''pseudocoma''',<ref name="pmid844425">{{cite journal |author=Flügel KA, Fuchs HH, Druschky KF |title=The "locked-in" syndrome: pseudocoma in thrombosis of the basilar artery (author's trans.) |language=German |journal=Dtsch. Med. Wochenschr. |volume=102 |issue=13 |pages=465–70 |year=1977 |pmid=844425 |doi=10.1055/s-0028-1104912}}</ref> and '''ventral pontine syndrome'''.
'''Total locked-in syndrome''', or '''completely locked-in state''' ('''CLIS'''), is a version of locked-in syndrome wherein the eyes are paralyzed as well.<ref name= "bauer1979varieties">{{cite journal | doi = 10.1007/BF00313105 | author =Bauer, G. | author2 =Gerstenbrand, F. | author3 =Rumpl, E. | title= Varieties of the locked-in syndrome | journal= Journal of Neurology | volume =221 |issue=2 |pages=77–91 |year=1979 |pmid=92545| s2cid =10984425 }}</ref> [[Fred Plum]] and [[Jerome B. Posner]] coined the term for this disorder in 1966.<ref name="titleeMedicine - Stroke Motor Impairment : Article by Adam B Agranoff, MD">{{cite web |url=http://www.emedicine.com/pmr/topic189.htm |title= Stroke Motor Impairment | first = Adam B | last = Agranoff | access-date = 2007-11-29 | work = eMedicine}}</ref><ref>{{Citation | last1 = Plum | first1 = F | last2 = Posner | first2 = JB | year = 1966 | title = The diagnosis of stupor and coma | publisher = FA Davis | place = Philadelphia, PA, USA}}, 197 pp.</ref>


==Signs and symptoms==
==Presentation==
Locked-in syndrome usually results in [[quadriplegia]] and the inability to [[Manner of articulation|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.<ref name= "fager" /> This restricts them from producing voluntary sounds, though the [[vocal cords]] are not paralysed.<ref name =fager>{{cite journal|doi=10.1080/07434610600650318|last=Fager|first=Susan|coauthors=Beukelman, Karantounis, Jakobs| year= 2006| title = Use of safe-laser access technology to increase head movements in persons with severe motor impairments: a series of case reports| journal= Augmentative and Alternative Communication|volume= 22| issue= 3| pages= 222–29 | pmid= 17114165}}</ref>
Locked-in syndrome is usually characterized by [[quadriplegia]] (loss of limb function) and the inability to [[Manner of articulation|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. 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 muscles]]. Individuals with the syndrome lack coordination between breathing and voice.<ref name= "fager" /> This prevents them from producing voluntary sounds, though the [[vocal cords]] themselves may not be paralysed.<ref name ="fager">{{cite journal|doi=10.1080/07434610600650318| last1=Fager| first1=Susan| last2=Beukelman| first2=Dave| last3=Karantounis| first3=Renee| last4=Jakobs| first4=Tom| year= 2006| title = Use of safe-laser access technology to increase head movements in persons with severe motor impairments: a series of case reports| journal= Augmentative and Alternative Communication|volume= 22| issue= 3| pages= 222–29 | pmid= 17114165| s2cid=36840057}}</ref>


==Causes==
==Causes==
{{Unreferenced section|date=October 2011}}
{{More citations needed section|date=June 2024}}
[[Image:Gray760.png|thumb|In children, the most common cause is a stroke of the ventral [[pons]].<ref name="pmid19748042">{{cite journal | author= Bruno MA, Schnakers C, Damas F, ''et al.'' |title= Locked-in syndrome in children: report of five cases and review of the literature |journal=Pediatr. Neurol. |volume=41 |issue=4 |pages=237–46 |date=October 2009 |pmid= 19748042 | doi = 10.1016/j.pediatrneurol.2009.05.001 |url=http://linkinghub.elsevier.com/retrieve/pii/S0887-8994(09)00266-5}}</ref>]]
[[Image:Gray760.png|thumb|upright=1.3|In children, the most common cause is a stroke of the ventral [[pons]].<ref name="pmid19748042">{{cite journal |vauthors=Bruno MA, Schnakers C, Damas F, etal |title= Locked-in syndrome in children: report of five cases and review of the literature |journal=Pediatr. Neurol. |volume=41 |issue=4 |pages=237–46 |date=October 2009 |pmid= 19748042 |doi=10.1016/j.pediatrneurol.2009.05.001 }}</ref>]]
Unlike [[persistent vegetative state]], in which the upper portions of the brain are damaged and the lower portions are spared, locked-in syndrome is essentially the opposite, caused by damage to specific portions of the lower brain and [[brainstem]], with no damage to the upper brain.{{citation needed|date=December 2020}} Injuries to the pons are the most common cause of locked-in syndrome.

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:
Possible causes of locked-in syndrome include:
* [[Poisoning]] cases – More frequently from a [[Bungarus|krait]] bite and other [[neurotoxic]] [[venom]]s, as they cannot usually cross the [[blood–brain barrier]]
* [[Amyotrophic lateral sclerosis]] (aka Lou Gehrig's disease)
* [[Brainstem stroke syndrome|Brainstem stroke]]
* Diseases of the [[circulatory system]]
* Diseases of the [[circulatory system]]
* Medication overdose
* Medication overdose{{Example needed |s|date=December 2018}}
* Damage to nerve cells, particularly destruction of the [[myelin sheath]], caused by disease or ''osmotic demyelination syndrome'' (formerly designated [[central pontine myelinolysis]]) secondary to excessively rapid correction of [[hyponatremia]] [>1 mEq/L/h])<ref>{{cite book |language=en |last=Aminoff |first=Michael |title=Clinical Neurology |edition=9nth |year=2015 |page=76 |publisher=Lange |isbn=978-0-07-184142-9}}</ref>
* [[Multiple Sclerosis]]
* Damage to nerve cells, particularly destruction of the [[myelin sheath]], caused by disease (e.g. [[central pontine myelinolysis]] secondary to rapid correction of [[hyponatremia]])
* A stroke or brain hemorrhage, usually of the [[basilar artery]]
* A stroke or brain hemorrhage, usually of the [[basilar artery]]
* [[Traumatic brain injury]]
* [[Traumatic brain injury]]
* Result from [[lesion]] of the brainstem


[[Curare poisoning]] and [[paralytic shellfish poisoning]] mimic a total locked-in syndrome by causing [[paralysis]] of all voluntarily controlled [[skeletal muscle]]s.<ref>[https://books.google.com/books?id=RSOPDHP9QekC&pg=PA357 Page 357] in: {{cite book |author=Damasio, Antonio R. |title=The feeling of what happens: body and emotion in the making of consciousness |publisher=Harcourt Brace |location=San Diego |year=1999 |isbn=978-0-15-601075-7 |url-access=registration |url=https://archive.org/details/feelingofwhathap00dama_0 }}</ref> The respiratory muscles are also paralyzed, but the victim can be kept alive by [[artificial respiration]].
==Treatment==
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]].<ref>{{NINDS|lockedinsyndrome}}</ref> Assistive computer interface technologies, such as [[Dasher (software)|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.<ref>Parker, I., "Reading Minds," The New Yorker, January 20, 2003, 52–63</ref><ref>{{Citation | url = http://www.bu.edu/today/2008/10/14/turning-thoughts-words | contribution = Turning Thoughts into Words | first = Chris | last = Berdik | format = Research | publisher = Boston University | title = BU Today | date = October 15, 2008}}.</ref><ref name="Keiper 2006 4–41">{{cite web|last=Keiper|first=Adam|title=The Age of Neuroelectronics|url=http://www.thenewatlantis.com/publications/the-age-of-neuroelectronics|publisher=The New Atlantis|pages=4–41|date=Winter 2006}}</ref> Some scientists have reported that they have developed a technique that allows locked-in patients to communicate via sniffing.<ref>{{cite web|title='Locked-In' Patients Can Follow Their Noses | url=http://news.sciencemag.org/sciencenow/2010/07/locked-in-patients-can-follow-th.html | publisher= Science Mag | date=26 Jul 2010 | accessdate=27 Jul 2010}}</ref>


==Prognosis==
==Diagnosis==
Locked-in syndrome can be difficult to diagnose. In a 2002 survey of 44 people with LIS, it took almost three months to recognize and diagnose the condition after it had begun.<ref>{{cite journal|last1=León-Carrión|first1=J.|last2=van Eeckhout|first2=P.|last3=Domínguez-Morales Mdel|first3=R.|last4=Pérez-Santamaría|first4=F. J.|title=The locked-in syndrome: a syndrome looking for a therapy|journal=Brain Inj.|date=2002|volume=16|issue=7|pages=571–82|doi=10.1080/02699050110119781|pmid=12119076|s2cid=20970974}}</ref> Locked-in syndrome may mimic [[Unconsciousness|loss of consciousness]] in patients, or, in the case that respiratory control is lost, may even resemble death. People are also unable to actuate standard motor responses such as [[Withdrawal reflex|withdrawal from pain]]; as a result, testing often requires making requests of the patient such as blinking or vertical eye movement.{{citation needed|date=December 2020}}
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,<ref name=esquire1>{{cite news|url=http://www.esquire.com/features/unspeakable-odyssey-motionless-boy-1008 |title=The Unspeakable Odyssey of the Motionless Boy|author= Joshua Foer|work= Esquire |date= October 2, 2008}}</ref> while in exceptional cases, like that of Kerry Pink<ref name=BBCnews>{{cite news|url=http://www.bbc.co.uk/news/health-10985836 |title=I recovered from locked-in syndrome|work= BBC Radio 5 Live|author= Stephen Nolan|date= August 16, 2010}}</ref> and Kate Allatt,<ref name=BBCnews2>{{cite news|url=http://www.bbc.co.uk/news/health-17363584 |title=Woman's recovery from 'locked-in' syndrome|work= BBC News|date= March 14, 2012}}</ref> a full spontaneous recovery may be achieved.


Brain imaging may provide additional indicators of locked-in syndrome, as brain imaging provides clues as to whether or not brain function has been lost. Additionally, an [[Electroencephalography|EEG]] can allow the observation of sleep-wake patterns indicating that the patient is not unconscious but simply unable to move.<ref name="Merck Manual">{{cite web|last=Maiese|first=Kenneth|url=https://www.merckmanuals.com/professional/neurologic-disorders/coma-and-impaired-consciousness/locked-in-syndrome|title = Locked-in Syndrome|date=March 2014}}</ref>
==Notable cases==


===Kate Allatt===
=== Similar conditions ===
* [[Amyotrophic lateral sclerosis]] (ALS)
Kate Allatt is a mother-of-three from [[Sheffield]], [[South Yorkshire]] who has successfully recovered from locked-in syndrome. Now, she runs Fighting Strokes and devotes her life to assisting those who have locked-in syndrome.<ref name=amazing_spirit/>
* [[Brain tumor|Bilateral brainstem tumor]]s
* [[Brain death]] (of the whole brain or the brainstem or other part)
* [[Coma]] (deep or irreversible)
* [[Guillain–Barré syndrome]]
* [[Myasthenia gravis]]
* [[Poliomyelitis]]
* [[Peripheral neuropathy|Polyneuritis]]
* [[Vegetative state]] (chronic or otherwise)


==Treatment==
===Jean-Dominique Bauby===
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]].<ref>{{NINDS|Locked-Syndrome|Locked-in syndrome}}</ref> [[Assistive technology|Assistive computer interface technologies]] such as [[Dasher (software)|Dasher]], combined with [[eye tracking]], may be used to help people with LIS communicate with their environment.{{citation needed|date=December 2020}}
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 [[The Diving Bell and the Butterfly (film)|eponymous 2007 movie]]. Three days after the book was published in March 1997, Bauby died of [[pneumonia]].<ref name= "titleThe Diving Bell And The Butterfly | The A.V. Club">{{cite web |url= http://www.avclub.com/content/cinema/the_diving_bell_and_the | title= The Diving Bell And The Butterfly | work=The A.V. Club |accessdate=2007-11-29}}</ref> He was instrumental in forming the ''Association du Locked-In Syndrome'' (ALIS) in [[France]].<ref>{{cite web | url = http://www.alis-asso.fr/ | title = Association du Locked In Syndrome | language = French | location = FR}}</ref>


==Prognosis==
===Rabbi Ronnie Cahana===
It is extremely rare for any significant motor function to return, with the majority of locked-in syndrome patients never regaining motor control. However, some people with the condition continue to live for extended periods of time,<ref name=esquire1>{{cite news|url=http://www.esquire.com/features/unspeakable-odyssey-motionless-boy-1008 |title=The Unspeakable Odyssey of the Motionless Boy|author= Joshua Foer|work= Esquire |date= October 2, 2008}}</ref><ref>Piotr Kniecicki "An art of graceful dying". Clitheroe: Łukasz Świderski, 2014, s. 73. {{ISBN|978-0-9928486-0-6}}</ref> while in exceptional cases, like that of Kerry Pink,<ref name=BBCnews>{{cite news|url=https://www.bbc.co.uk/news/health-10985836 |title=I recovered from locked-in syndrome|work= BBC Radio 5 Live|author= Stephen Nolan|date= August 16, 2010}}</ref> Gareth Shepherd,<ref name=Dailyecho>{{cite news|url=https://www.dailyecho.co.uk/news/14866880.he-crashed-his-motorbike-and-had-a-stroke-but-hampshire-man-gareth-shepherd-is-back-on-his-feet |title=He crashed his motorbike and had a stroke - but Hampshire man Gareth Shepherd is back on his feet|work=Daily Echo|date=November 8, 2016}}</ref>{{Failed verification|date=December 2024|talk=Gareth Shepherd|reason=Article references Shepherd being comatose, but not having Locked-in syndrome}} Jacob Haendel,<ref name=YouTube>{{cite news|url=https://www.youtube.com/channel/UCpoJxQNKSybRuSXEEuQ0cNA |title=Jacob Haendel Recovery Channel|work= Jacob Handel Recovery|date= June 29, 2020}}</ref> Kate Allatt,<ref name=BBCnews2>{{cite news|url=https://www.bbc.co.uk/news/health-17363584 |title=Woman's recovery from 'locked-in' syndrome|work= BBC News|date= March 14, 2012}}</ref> and Jessica Wegbrans,<ref name=Flinkberoerd>{{cite news|url=https://www.flinkberoerd.nl |title=Het gevecht tegen locked-in|work= Flinkberoerd|date=April 23, 2022}}</ref> a near-full recovery may be achieved with intensive physical therapy.
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.<ref>''[http://www.rabbicahana.com Rabbi Ronnie Cahana]'', Rabbi Ronnie Cahana's poetry and sermons</ref> He is the son of painter [[Alice Lok Cahana]].{{citation needed|date=February 2014}}

===Lynsey Cribbin===
Lynsey Cribbin, from [[County Cavan|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.<ref>{{cite news|first=Nick|last=Bramhill|url=http://www.thesun.co.uk/sol/homepage/irishsun/irishsunnews/4506279/Locked-in-Lynseys-care-woe-Parents-anger-at-denial-of-specialist-help.html|title=Locked-in Lynseys care woe: Parents anger at denial of specialist help|newspaper=The Sun|date=27 August 2012|accessdate=27 August 2012}}</ref>

===Stephen Hawking===
[[Stephen William Hawking]] has a [[motor neuron disease]] related to [[amyotrophic lateral sclerosis]] (ALS), a condition that has progressed over the years. 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,{{sfn|Ferguson|2011|p=224}}<ref name="tech" /><ref name=hacked>{{cite web|url=http://cosmiclog.nbcnews.com/_news/2012/06/25/12401493-how-researchers-hacked-into-stephen-hawkings-brain?lite|title=How researchers hacked into Stephen Hawking's brain|date=25 June 2012|first=Alan|last=Boyle|publisher=NBC News|accessdate=29 September 2012}}</ref> with a rate of about one word per minute.<ref name="tech">{{cite news
|url=http://www.newscientist.com/article/dn21323-the-man-who-saves-stephen-hawkings-voice.html
|title=The man who saves Stephen Hawking's voice
|date=30 December 2011
|first=Catherine|last=de Lange
|work=New Scientist
|accessdate=18 June 2012}}</ref> Hawking is collaborating with researchers on systems that could translate his brain patterns or facial expressions into switch activations.<ref name=hacked/><ref name=dt2012>{{cite news |url= http://www.telegraph.co.uk/science/stephen-hawking/8989060/Stephen-Hawking-driven-by-a-cosmic-force-of-will.html |title=Stephen Hawking: driven by a cosmic force of will – Telegraph |first= Roger|last=Highfield |work=[[The Daily Telegraph]] |date= 3 January 2012 |publisher=[[Telegraph Media Group|TMG]] |location=London |accessdate=7 December 2012}}</ref><ref name=start>{{cite web|url=http://www.bbc.co.uk/news/technology-18749963|title=Start-up attempts to convert Prof Hawking's brainwaves into speech|date=7 July 2012|publisher=BBC|accessdate=29 September 2012}}</ref>

===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.<ref name= "guardian_houben">{{cite news| url= http://www.guardian.co.uk/world/2009/nov/23/man-trapped-coma-23-years|title= Trapped in his own body for 23 years – the coma victim who screamed unheard| last= Connolly| first= Kate| date= 23 November 2009| location= Berlin | work = The Guardian | location = UK}}</ref> He was initially reported as communicating by typing into a keyboard with his right hand,<ref>{{cite news| url = http://www.npr.org/blogs/thetwo-way/2009/11/for_23_years_man_thought_to_be.html| title = For 23 Years Man Thought To Be In Coma Tried To 'Scream', And No One Heard| author= Mark Memmott|work= NPR|date= November 23, 2009}}</ref> though the presence of a [[Facilitated communication|facilitator]] to move his hand attracted sharp criticism and strong doubts that Houben's communications were authentic.<ref>{{cite web|url=http://scienceblogs.com/pharyngula/2009/11/really_this_guy_is_conscious.php | title= Really? This guy is conscious?| author= PZ Myers| work = Pharyngula| date = November 24, 2009}}</ref><ref name="urlHealth | Comatose for 23 years, Belgian feels reborn | Seattle Times Newspaper">{{cite web |url=http://seattletimes.nwsource.com/html/health/2010344060_apeubelgiumcomarecovery.html |title=Health &#124; Comatose for 23 years, Belgian feels reborn &#124; Seattle Times Newspaper |work= |accessdate=2012-03-12}}</ref><ref name="active_euthanasia">{{cite news|url= http://www.huffingtonpost.com/jacob-m-appel/the-rom-houben-tragedy-an_b_370032.html | title= The Rom Houben Tragedy And The Case For Active Euthanasia|last=Appel|first=Jacob M.|date=Nov 24, 2009|work=[[The Huffington Post]]}}</ref>

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.<ref>{{cite news| url= http://www.spiegel.de/international/world/0,1518,677537,00.html |title= Communicating with Those Trapped within Their Brains| author= Manfred Dworschak| location = DE | work= Der Spiegel | date= February 13, 2010}}</ref> Laureys maintained the [[Magnetic resonance imaging|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.<ref name="guardian_houben"/><ref>{{Cite journal
| title = Diagnostic accuracy of the vegetative and minimally conscious state: Clinical consensus versus standardized neurobehavioral assessment
| author = Caroline Schnakers, Audrey Vanhaudenhuyse, Joseph Giacino, Manfredi Ventura, Melanie Boly, Steve Majerus, Gustave Moonen, [[Steven Laureys]]
| journal = [[BMC Neurology]]
| year = 2009
| volume = 9
| page = 35
| doi = 10.1186/1471-2377-9-35
| url = http://www.biomedcentral.com/1471-2377/9/35/
| pmid=19622138
| pmc=2718857
}}</ref><ref>[http://en.scientificcommons.org/48392734 Diagnostic accuracy of the vegetative and minimally conscious state: Clinical consensus versus standardized neurobehavioral assessment] Steven Laureys, Caroline Schnakers, Audrey Vanhaudenhuyse, ''et al''</ref>

===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.<ref name = "urlPensioner tells how he beat locked in syndrome after massive stroke - Telegraph">{{cite news | url = http://www.telegraph.co.uk/health/healthnews/7966357/Pensioner-tells-how-he-beat-locked-in-syndrome-after-massive-stroke.html | title = Pensioner tells how he beat "locked in" syndrome after massive stroke | work = The Daily Telegraph | accessdate = 2010-09-03 | location = London | first= Richard | last= Alleyne | date = August 26, 2010}}</ref><ref name = "urlBBC News - Locked-in syndrome man learns to walk again">{{cite news |url=http://www.bbc.co.uk/news/uk-england-dorset-11103527 |title= Locked-in syndrome man learns to walk again | work= News | publisher = The BBC | location = UK |accessdate= 2010-09-03 | date= August 26, 2010}}</ref>

===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.<ref name="Keiper 2006 4–41"/>

===Tony Nicklinson ===
Tony Nicklinson, of [[Melksham]], [[Wiltshire]], [[England]], was left paralyzed after suffering a stroke in June 2005,<ref>{{cite news|url=http://www.bbc.co.uk/news/uk-england-wiltshire-19341571|title=Tony Nicklinson's legal fight for right to die|date=22 August 2012|publisher=[[BBC News]]|accessdate=22 August 2012}}</ref> 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]].<ref>{{cite web|url=http://uk.news.yahoo.com/locked-man-loses-aided-death-bid-132622494.html|title=Locked-in man devastated at ruling|date=18 August 2012|accessdate=18 August 2012}}</ref> 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.<ref>{{cite news|url=http://www.bbc.co.uk/news/uk-england-19341722|title=Right-to-die man Tony Nicklinson dead after refusing food|date=22 August 2012|publisher=[[BBC News]]|accessdate=22 August 2012}}</ref> Nicklinson was cremated and his ashes were scattered over his local rugby pitch.{{citation needed|date=February 2014}}

===Gary Parkinson===
In 2010, ex-[[Premier League|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|date=February 2014}}

===Tony Quan AKA Tempt One===
[[Tempt One|Tony Quan]], a popular graffiti artist, was diagnosed with the nerve disorder [[Amyotrophic lateral sclerosis|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.<ref>http://gizmodo.com/5403741/eyewriter-allows-man-to-paint-despite-paralysis http://infosthetics.com/archives/2009/11/eyewriter_graffiti_physically_paralyzed.html</ref>

===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)|Esquire]]'' magazine in 2008.{{citation needed|date=February 2014}} 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.<ref name=esquire1/><ref>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). [http://www.ncbi.nlm.nih.gov/pubmed/20011034 A wireless brain-machine interface for real-time speech synthesis. PLoS ONE, 4(12), pp. e8218+.]</ref><ref>{{cite news|url=http://www.boston.com/news/local/articles/2008/07/27/out_of_silence_the_sounds_of_hope/?page=full|title=Out of silence, the sounds of hope|author=S.I. Rosenbaum|work=The Boston Globe|date=July 27, 2008}}</ref>

===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 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.<ref name=esquire1/><ref>{{cite news|url=http://articles.latimes.com/2003/dec/21/local/me-tavalaro21|title=Julia Tavalaro, 68; Poet and Author Noted for Defying Severe Paralysis|work=Los Angeles Times|page=B16|date=December 21, 2003}}</ref>

===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.<ref name=amazing_spirit>{{cite news|first=Jenna|last=Sloan|url=http://www.thesun.co.uk/sol/homepage/woman/real_life/4529462/Locked-in-Christine-Waddell-Im-mad-on-Facebook-shopping-50-Shades-of-Grey-and-vodka.html|title=I’m mad on Facebook, shopping, 50 Shades of Grey and I like vodka: Amazing spirit of 'locked-in' Christine Waddell|newspaper=The Sun|date=11 September 2012|accessdate=11 September 2012}}</ref>

===Chris Bloom===
Chris Bloom suffered an ischemic brain stem stroke in January 2013 at the age of 38. After regaining consciousness, he was diagnosed with Locked-In Syndrome. His only voluntary movement is up and down eye movement. He now uses an infrared switch mounted in his glasses to control an iPad. He is able to update blogs, use Facebook, Twitter, read and write emails, and play online board games and apps with his children. His public Facebook page is "Prayers for Chris Bloom & Family." There, you can find links to the blogs he writes, as well as updates on his progress in his fight to come out of being locked in.

==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.

===''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 the issue has nothing to do with the "locked-in syndrome", according to the [[World Health Organization|WHO]] definition.{{citation needed|date=February 2014}}

===''The Ultimate Secret''===
The character of Jean-Louis Martin in [[Bernard Werber]]'s sci-fi novel ''L'Ultime Secret'' (2001),<ref>{{cite book|author=Bernard Werber|title=L'Ultime Secret|year=2001| ISBN =2-226-12740-2 |trans-title= The Ultimate Secret}}</ref> 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.<ref>{{cite web |url= http://frenchpubagency.com/Title-199045-Fiction-Literature/L-Ultime-Secret.html |title=L'Ultime Secret |work=The French Publisher's Agency |year=2012 |accessdate=5 February 2012}}</ref>

===''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.{{citation needed|date=February 2014}}

===''Sleepyhead''===
[[Mark Billingham]]'s novel ''Sleepyhead'' (2013)<ref>{{cite book|title=Sleepyhead|author=Mark Billingham|isbn=9780802193261|publisher=Grove/Atlantic, Inc|year=2013|series=Tom Thorne Series , #1}}</ref> addresses a criminal who purposely manipulates pressure points on each victim's head and neck with the intention of inducing locked-in syndrome.<ref>{{cite web|website=BN.com|title=Publisher's description of ''Sleepyhead''|url=http://www.barnesandnoble.com/enwiki/w/sleepyhead-mark-billingham/1103371749?ean=9780802193261&itm=1&usri=sleepyhead|accessdate=February 7, 2014}}</ref>

==Cases in popular culture==

===''Alfred Hitchcock Presents''===
In season 1, episode 7 of ''[[Alfred Hitchcock Presents]]'', titled "Breakdown" (November 13, 1955 on CBS),<ref>{{cite web|url=http://www.tv.com/shows/alfred-hitchcock-presents/breakdown-40906/|title=Breakdown|accessdate=February 7, 2014|website=TV.com}}</ref> 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.{{citation needed|date=February 2014}}

===''Criminal Minds''===
In the ''Criminal Minds'' episode "The Uncanny Valley", the [[Person of interest|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''===
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|date=February 2014}}

===''House M.D.''===
The ''[[House (TV series)|House M.D.]]'' episode "[[Locked In (House)|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 (TV series)|Scrubs]]'' episode "His Story III", a patient (played by Henry LeBlanc) is presented with locked-in syndrome.{{citation needed|date=February 2014}}

===''Star Trek''===
In the ''[[Star Trek]]'' episode "[[The Menagerie (Star Trek: The Original Series)|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==
[[Curare poisoning]] mimics a total locked-in syndrome by causing [[paralysis]] of all voluntarily controlled [[skeletal muscle]]s.<ref>[http://books.google.com/books?id=RSOPDHP9QekC&pg=PA357 Page 357] in: {{cite book |author=Damasio, Antonio R. |title=The feeling of what happens: body and emotion in the making of consciousness |publisher=Harcourt Brace |location=San Diego |year=1999 |pages= |isbn=0-15-601075-5 |oclc= |doi= |accessdate=}}</ref> 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 [[Oxygenation (medical)|oxygen saturation]] of always above 85%,<ref>[http://books.google.com/books?id=inud-udaRFwC&pg=PA520 Page 520] in: {{cite book |author=Paradis, Norman A. |title=Cardiac arrest: the science and practice of resuscitation medicine |publisher=Cambridge University Press |location=Cambridge, UK |year=2007 |pages= |isbn=0-521-84700-1 |oclc= |doi= |accessdate=}}</ref> a level at which there is no evidence of [[altered state of consciousness]].<ref>[http://www.dshs.state.tx.us/emstraumasystems/Conference10Handouts/McEvoy_Oxymoron.pdf Oxymoron: Our Love-Hate Relationship with Oxygen], By Mike McEvoy at Albany Medical College, New York. 10/12/2010</ref> Spontaneous breathing is resumed after the end of the [[duration of action]] of curare, which is generally between 30 minutes<ref name=Rang151>For therapeutic dose of tubocurarine by shorter limit as given at page 151 in: {{cite book |author=Rang, H. P. |title=Pharmacology |publisher=Churchill Livingstone |location=Edinburgh |year=2003 |pages= |isbn=0-443-07145-4 |oclc= 51622037|doi=}}</ref> and eight hours,<ref>For 20-fold paralytic dose of toxiferine ("calebas curare"), according to: [http://books.google.com/books?id=R8BlpSvvSfwC&pg=PA330 Page 330] in: {{cite book |author= |title=The Alkaloids: v. 1: A Review of Chemical Literature (Specialist Periodical Reports) |publisher=Royal Society of Chemistry |location=Cambridge, Eng |year=1971 |pages= |isbn=0-85186-257-8 |oclc= |doi= |accessdate=}}</ref> depending on the variant of the toxin and dosage.


==Research==
New [[brain–computer interface]]s (BCIs) may provide future remedies. One effort in 2002 allowed a fully locked-in patient to answer yes-or-no questions.<ref>[http://www.newyorker.com/magazine/2003/01/20/reading-minds Parker, I., "Reading Minds," The New Yorker, January 20, 2003, 52–63]</ref><ref name="Keiper 2006 4–41">{{cite journal|last=Keiper |first=Adam |title=The Age of Neuroelectronics |journal=New Atlantis (Washington, D.c.) |url=http://www.thenewatlantis.com/publications/the-age-of-neuroelectronics |publisher=The New Atlantis |pages=4–41 |date=Winter 2006 |volume=11 |pmid=16789311 |url-status=dead |archive-url=https://web.archive.org/web/20160212222927/http://www.thenewatlantis.com/publications/the-age-of-neuroelectronics |archive-date=2016-02-12 }}</ref> In 2006, researchers created and successfully tested a neural interface which allowed someone with locked-in syndrome to operate a web browser.<ref>{{cite journal |vauthors=Karim AA, Hinterberger T, Richter J, Mellinger J, Neumann N, Flor H, Kübler A, Birbaumer N |title=Neural internet: Web surfing with brain potentials for the completely paralyzed |journal=Neurorehabilitation & Neural Repair |volume=40 |issue=4 |pages=508–515}}</ref> Some scientists have reported that they have developed a technique that allows locked-in patients to communicate via sniffing.<ref>{{cite web|url=https://www.science.org/content/article/locked-patients-can-follow-their-noses|title='Locked-In' Patients Can Follow Their Noses|date=26 Jul 2010|publisher=Science Mag|access-date=27 Dec 2016}}</ref>
For the first time in 2020, a 34-year-old German patient, paralyzed since 2015 (later also the eyeballs) managed to communicate through an implant capable of reading brain activity.<ref>{{cite web|url=https://www.technologyreview.com/2022/03/22/1047664/locked-in-patient-bci-communicate-in-sentences/|title=A locked-in man state with ALS has been able to communicate thought alone / MIT Technology Review by Jessica Hamzelou / March 26, 2022}}</ref>
==See also==
==See also==
*[[Akinetic mutism]]
* [[Akinetic mutism]]
* [[List of people with locked-in syndrome]]
* ''[[The Diving Bell and the Butterfly]]'': memoirs of journalist [[Jean-Dominique Bauby]] about his life with the condition
* ''[[Johnny Got His Gun]]'', novel about a soldier who loses his limbs and senses after being wounded fighting in WWI
* [[One (Metallica song)]], song interpretation of ''[[Johnny Got His Gun (film)|Johnny Got His Gun]]''


==References==
==References==
{{Reflist|refs=Locked In Syndrome - NORD (National Organization for Rare Disorders). (n.d.). Retrieved June 28, 2016, from http://rarediseases.org/rare-diseases/locked-in-syndrome/|colwidth=30em}}
{{Reflist|colwidth=30em}}
25. Injuries to the pons are the most common cause of locked-in syndrome,Harrison’s principles of internal medicine 21st edition vol 2 page 3332.


==External links==
==Further reading==
* Piotr Kniecicki (2014). ''An Art of Graceful Dying''. Lukasz Swiderski {{ISBN|978-0-9928486-0-6}} (Autobiography, written with residual wrist movements and specially adapted computer)
* [http://unlockproject.org/ The Unlock Project]

* [http://alis-asso.fr/ewb_pages/c/communiquer_sans_parole.php Locked-in Syndrome Association's guide to communicating without language] {{fr}} {{Dead link|date=October 2012}}
== External links ==
* Rachel's [https://skydrive.live.com/redir?resid=EB0220FB135D614B!1717 Communication boards] – for when you need to communicate with someone with LIS.
{{Medical resources
| DiseasesDB =
| ICD10 = {{ICD10|G|93|8|g|40}}
| ICD9 = {{ICD9|344.81}}
| ICDO =
| OMIM =
| MedlinePlus =
| eMedicineSubj =
| eMedicineTopic =
| MeshID = D011782
}}


{{disorders of consciousness}}
{{disorders of consciousness}}
{{Cerebral palsy and other paralytic syndromes}}
{{Lesions of spinal cord and brainstem}}
{{Lesions of spinal cord and brainstem}}
{{Authority control}}


{{DEFAULTSORT:Locked-In Syndrome}}
{{DEFAULTSORT:Locked-In Syndrome}}
[[Category:Neurotrauma]]
[[Category:Neurotrauma]]
[[Category:Syndromes]]
[[Category:Syndromes]]
[[Category:Medical ethics]]

Latest revision as of 21:04, 29 December 2024

Locked-in syndrome
Other namesCerebromedullospinal disconnection,[1] de-efferented state, pseudocoma,[2] ventral pontine syndrome
Locked-in syndrome can be caused by a stroke at the level of the basilar artery denying blood to the pons, among other causes.
SpecialtyNeurology, Psychiatry

Locked-in syndrome (LIS), also known as pseudocoma, 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 their body except for vertical eye movements and blinking.[3] The individual is conscious and sufficiently intact cognitively to be able to communicate with eye movements.[4] Electroencephalography results are normal in locked-in syndrome. Total locked-in syndrome, or completely locked-in state (CLIS), is a version of locked-in syndrome wherein the eyes are paralyzed as well.[5] Fred Plum and Jerome B. Posner coined the term for this disorder in 1966.[6][7]

Signs and symptoms

[edit]

Locked-in syndrome is usually characterized by quadriplegia (loss of limb function) 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. 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 muscles. Individuals with the syndrome lack coordination between breathing and voice.[8] This prevents them from producing voluntary sounds, though the vocal cords themselves may not be paralysed.[8]

Causes

[edit]
In children, the most common cause is a stroke of the ventral pons.[9]

Unlike persistent vegetative state, in which the upper portions of the brain are damaged and the lower portions are spared, locked-in syndrome is essentially the opposite, caused by damage to specific portions of the lower brain and brainstem, with no damage to the upper brain.[citation needed] Injuries to the pons are the most common cause of locked-in syndrome.

Possible causes of locked-in syndrome include:

Curare poisoning and paralytic shellfish poisoning mimic a total locked-in syndrome by causing paralysis of all voluntarily controlled skeletal muscles.[11] The respiratory muscles are also paralyzed, but the victim can be kept alive by artificial respiration.

Diagnosis

[edit]

Locked-in syndrome can be difficult to diagnose. In a 2002 survey of 44 people with LIS, it took almost three months to recognize and diagnose the condition after it had begun.[12] Locked-in syndrome may mimic loss of consciousness in patients, or, in the case that respiratory control is lost, may even resemble death. People are also unable to actuate standard motor responses such as withdrawal from pain; as a result, testing often requires making requests of the patient such as blinking or vertical eye movement.[citation needed]

Brain imaging may provide additional indicators of locked-in syndrome, as brain imaging provides clues as to whether or not brain function has been lost. Additionally, an EEG can allow the observation of sleep-wake patterns indicating that the patient is not unconscious but simply unable to move.[13]

Similar conditions

[edit]

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.[14] Assistive computer interface technologies such as Dasher, combined with eye tracking, may be used to help people with LIS communicate with their environment.[citation needed]

Prognosis

[edit]

It is extremely rare for any significant motor function to return, with the majority of locked-in syndrome patients never regaining motor control. However, some people with the condition continue to live for extended periods of time,[15][16] while in exceptional cases, like that of Kerry Pink,[17] Gareth Shepherd,[18][failed verification – see discussion] Jacob Haendel,[19] Kate Allatt,[20] and Jessica Wegbrans,[21] a near-full recovery may be achieved with intensive physical therapy.

Research

[edit]

New brain–computer interfaces (BCIs) may provide future remedies. One effort in 2002 allowed a fully locked-in patient to answer yes-or-no questions.[22][23] In 2006, researchers created and successfully tested a neural interface which allowed someone with locked-in syndrome to operate a web browser.[24] Some scientists have reported that they have developed a technique that allows locked-in patients to communicate via sniffing.[25] For the first time in 2020, a 34-year-old German patient, paralyzed since 2015 (later also the eyeballs) managed to communicate through an implant capable of reading brain activity.[26]

See also

[edit]

References

[edit]
  1. ^ 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. S2CID 32398246.
  2. ^ Flügel KA, Fuchs HH, Druschky KF (1977). "The "locked-in" syndrome: pseudocoma in thrombosis of the basilar artery (author's trans.)". Deutsche Medizinische Wochenschrift (in German). 102 (13): 465–70. doi:10.1055/s-0028-1104912. PMID 844425.
  3. ^ Das J, Anosike K, Asuncion RM (2022). "Locked-in Syndrome". National Center for Biotechnology Information. PMID 32644452. Retrieved 10 June 2023.
  4. ^ Duffy J. motor speech disorders substrates, differential diagnosis, and management. Elsevier. p. 295.
  5. ^ Bauer, G., Gerstenbrand, F., Rumpl, E. (1979). "Varieties of the locked-in syndrome". Journal of Neurology. 221 (2): 77–91. doi:10.1007/BF00313105. PMID 92545. S2CID 10984425.
  6. ^ Agranoff AB. "Stroke Motor Impairment". eMedicine. Retrieved 2007-11-29.
  7. ^ Plum F, Posner JB (1966), The diagnosis of stupor and coma, Philadelphia, PA, USA: FA Davis, 197 pp.
  8. ^ a b Fager S, Beukelman D, Karantounis R, Jakobs T (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. S2CID 36840057.
  9. ^ Bruno MA, Schnakers C, Damas F, et al. (October 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.
  10. ^ Aminoff M (2015). Clinical Neurology (9nth ed.). Lange. p. 76. ISBN 978-0-07-184142-9.
  11. ^ 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 978-0-15-601075-7.
  12. ^ León-Carrión J, van Eeckhout P, Domínguez-Morales Mdel R, Pérez-Santamaría FJ (2002). "The locked-in syndrome: a syndrome looking for a therapy". Brain Inj. 16 (7): 571–82. doi:10.1080/02699050110119781. PMID 12119076. S2CID 20970974.
  13. ^ Maiese K (March 2014). "Locked-in Syndrome".
  14. ^ Locked-in syndrome at NINDS
  15. ^ Joshua Foer (October 2, 2008). "The Unspeakable Odyssey of the Motionless Boy". Esquire.
  16. ^ Piotr Kniecicki "An art of graceful dying". Clitheroe: Łukasz Świderski, 2014, s. 73. ISBN 978-0-9928486-0-6
  17. ^ Stephen Nolan (August 16, 2010). "I recovered from locked-in syndrome". BBC Radio 5 Live.
  18. ^ "He crashed his motorbike and had a stroke - but Hampshire man Gareth Shepherd is back on his feet". Daily Echo. November 8, 2016.
  19. ^ "Jacob Haendel Recovery Channel". Jacob Handel Recovery. June 29, 2020.
  20. ^ "Woman's recovery from 'locked-in' syndrome". BBC News. March 14, 2012.
  21. ^ "Het gevecht tegen locked-in". Flinkberoerd. April 23, 2022.
  22. ^ Parker, I., "Reading Minds," The New Yorker, January 20, 2003, 52–63
  23. ^ Keiper A (Winter 2006). "The Age of Neuroelectronics". New Atlantis (Washington, D.c.). 11. The New Atlantis: 4–41. PMID 16789311. Archived from the original on 2016-02-12.
  24. ^ Karim AA, Hinterberger T, Richter J, Mellinger J, Neumann N, Flor H, Kübler A, Birbaumer N. "Neural internet: Web surfing with brain potentials for the completely paralyzed". Neurorehabilitation & Neural Repair. 40 (4): 508–515.
  25. ^ "'Locked-In' Patients Can Follow Their Noses". Science Mag. 26 Jul 2010. Retrieved 27 Dec 2016.
  26. ^ "A locked-in man state with ALS has been able to communicate thought alone / MIT Technology Review by Jessica Hamzelou / March 26, 2022".

25. Injuries to the pons are the most common cause of locked-in syndrome,Harrison’s principles of internal medicine 21st edition vol 2 page 3332.

Further reading

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  • Piotr Kniecicki (2014). An Art of Graceful Dying. Lukasz Swiderski ISBN 978-0-9928486-0-6 (Autobiography, written with residual wrist movements and specially adapted computer)
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