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{{short description|Condition in which a patient is aware but completely paralysed}}
{{short description|Condition in which a patient is aware but completely paralysed}}
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<!---This info is horribly inaccurate and needs a full revision.-->
{{Infobox medical condition (new)
{{Infobox medical condition (new)
| name = Locked-in syndrome
| 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}}</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=Dtsch. Med. Wochenschr. |volume=102 |issue=13 |pages=465–70 |year=1977 |pmid=844425 |doi=10.1055/s-0028-1104912}}</ref> ventral pontine 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
| image = CerebellumArteries.svg
| 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.
| 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.
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| deaths =
| deaths =
}}
}}
'''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 the body except for vertical eye movements and blinking. 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>
'''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.
⚫
'''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>
[[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.<ref name= "bauer1979varieties">{{cite journal | doi = 10.1007/BF00313105 | author =Bauer, G. | author2 =Gerstenbrand, F. | author3 =Rumpl, E. | name-list-style =amp | 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 | 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==
==Signs and symptoms==
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. 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 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}}</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==
{{More citations needed section|date=June 2024}}
[[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>]]
[[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.{{cn|date=December 2020}}
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.


Possible causes of locked-in syndrome include:
Possible causes of locked-in syndrome include:
* [[Poisoning]] cases – More frequently from a [[krait]] bite and other [[neurotoxic]] [[venom]]s, as they cannot usually cross the [[blood–brain barrier]]{{cn|date=December 2020}}
* [[Poisoning]] cases – More frequently from a [[Bungarus|krait]] bite and other [[neurotoxic]] [[venom]]s, as they cannot usually cross the [[blood–brain barrier]]
* [[Brainstem stroke syndrome|Brainstem stroke]]{{cn|date=December 2020}}
* [[Brainstem stroke syndrome|Brainstem stroke]]
* Diseases of the [[circulatory system]]
* Diseases of the [[circulatory system]]
* Medication overdose{{Example needed |s|date=December 2018}}
* 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>
* 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>
* A stroke or brain hemorrhage, usually of the [[basilar artery]]{{cn|date=December 2020}}
* A stroke or brain hemorrhage, usually of the [[basilar artery]]
* [[Traumatic brain injury]]{{cn|date=December 2020}}
* [[Traumatic brain injury]]
* Result from [[lesion]] of the [[Brainstem|brain-stem]]
* Result from [[lesion]] of the brainstem


[[Curare poisoning]] mimics 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]].
[[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]].


==Diagnosis==
==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}}</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.{{cn|date=December 2020}}
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}}


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>
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>
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* [[Amyotrophic lateral sclerosis]] (ALS)
* [[Amyotrophic lateral sclerosis]] (ALS)
* [[Brain tumor|Bilateral brainstem tumor]]s
* [[Brain tumor|Bilateral brainstem tumor]]s
* [[Brain death]] (of the whole brain or the brain stem or other part)
* [[Brain death]] (of the whole brain or the brainstem or other part)
* [[Coma]] (deep or irreversible)
* [[Coma]] (deep or irreversible)
* [[Guillain–Barré syndrome]]
* [[Guillain–Barré syndrome]]
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==Treatment==
==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|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.{{cn|date=December 2020}}
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}}


==Prognosis==
==Prognosis==
It is extremely rare for any significant motor function to return. The majority of locked-in syndrome patients do not regain motor control. 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><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> [[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> and [[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> a full spontaneous recovery may be achieved.
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> 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.


==Research==
==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 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 |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 feet.<ref>{{cite web|url=http://www.sciencemag.org/news/2010/07/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>
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]]
* [[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
* ''[[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|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}}
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==
==Further reading==
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| MeshID = D011782
| MeshID = D011782
}}
}}

{{disorders of consciousness}}
{{disorders of consciousness}}
{{Lesions of spinal cord and brainstem}}
{{Lesions of spinal cord and brainstem}}
{{Authority control}}


{{DEFAULTSORT:Locked-In Syndrome}}
{{DEFAULTSORT:Locked-In Syndrome}}

Latest revision as of 08:05, 15 November 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

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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

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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

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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

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Treatment

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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

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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] Jacob Haendel,[19] Kate Allatt,[20] and Jessica Wegbrans,[21] a near-full recovery may be achieved with intensive physical therapy.

Research

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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

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References

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  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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