Locked-in syndrome

From Wikipedia, the free encyclopedia

Jump to navigationJump to search

Locked-in syndrome

Other Cerebromedullospinal disconnection,[1] de-efferented

names 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.

Specialty Neurology, 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 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.
[3]
 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. [4] Fred
Plum and Jerome Posner coined the term for this disorder in 1966.[5][6]

Contents

 1Signs and symptoms

 2Causes
 3Diagnosis
o 3.1Similar conditions
 4Treatment
 5Prognosis
 6Research
 7See also
 8References
 9Further reading
 10External links

Signs and symptoms[edit]

Locked-in syndrome is usually characterized by quadriplegia 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. 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. [7] This prevents them from producing
voluntary sounds, though the vocal cords may not be paralysed.[7]

Causes[edit]

In children, the most common cause is a stroke of the ventral pons.[8]

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.[citation needed]
Possible causes of locked-in syndrome include:

Poisoning cases – More frequently from a krait bite and
other neurotoxic venoms, as they cannot usually cross the blood–brain
barrier[citation needed]

Brainstem stroke[citation needed]

Diseases of the circulatory system

Medication overdose[examples needed]

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])[9]

A stroke or brain hemorrhage, usually of the basilar artery[citation needed]

Traumatic brain injury[citation needed]

Result from lesion of the brain-stem

Curare poisoning mimics a total locked-in syndrome by causing paralysis of all

voluntarily controlled skeletal muscles.[10] 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 29 army volunteers who were paralyzed with
curare, artificial respiration kept oxygen saturation above 85%,[11] a level at which
there is no evidence of altered state of consciousness.[12] Spontaneous breathing
is resumed after the end of the duration of action of curare, which is generally
between 30 minutes[13] and eight hours,[14] depending on the variant of the toxin
and dosage.

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.[15] 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. [16]
Similar conditions[edit]
 Amyotrophic lateral sclerosis (ALS)
 Bilateral brainstem tumors
 Brain death (of the whole brain or the brain stem or other part)
 Coma (deep or irreversible)
 Guillain–Barré syndrome
 Myasthenia gravis
 Poliomyelitis
 Polyneuritis
 Vegetative state (chronic or otherwise)

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.[17] 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. The majority of
locked-in syndrome patients do not regain motor control. However, some people
with the condition continue to live much longer, [18][19] while in exceptional cases, like
that of Kerry Pink,[20] Gareth Shepherd,[21] Jacob Haendel[22] and Kate Allatt[23] a full
spontaneous recovery may be achieved.

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. [24][25] In 2006,
researchers created and successfully tested a neural interface which allowed
someone with locked-in syndrome to operate a web browser. [26] Some scientists
have reported that they have developed a technique that allows locked-in
patients to communicate via sniffing.[27]

See also[edit]
 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

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.
2. ^ Flügel KA, Fuchs HH, Druschky KF (1977). "The "locked-in" syndrome:
pseudocoma in thrombosis of the basilar artery (author's trans.)". Dtsch. Med.
Wochenschr. (in German). 102 (13): 465–70. doi:10.1055/s-0028-1104912.  PMID  844425.
3. ^ Duffy, Joseph. motor speech disorders substrates, differential diagnosis, and
management. Elsevier. p. 295.
4. ^ 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.
 5. ^ Agranoff, Adam B. "Stroke Motor Impairment". eMedicine. Retrieved 2007-11-29.
6. ^ Plum, F; Posner, JB (1966), The diagnosis of stupor and coma, Philadelphia, PA,
USA: FA Davis, 197 pp.
7. ^ Jump up to:a b Fager, Susan; Beukelman, Dave; Karantounis, Renee; Jakobs, Tom
(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.
8. ^ 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.
9. ^ Aminoff, Michael (2015).  Clinical Neurology  (9nth  ed.). Lange. p. 76.  ISBN  978-0-
07-184142-9.
10. ^ 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.
11. ^ Page 520 in: Paradis, Norman A. (2007).  Cardiac arrest: the science and practice
of resuscitation medicine. Cambridge, UK: Cambridge University Press. ISBN 978-0-521-
84700-1.
12. ^ Oxymoron: Our Love-Hate Relationship with Oxygen, By Mike McEvoy at Albany
Medical College, New York. 10/12/2010
13. ^ For therapeutic dose of tubocurarine by shorter limit as given at page 151 in: Rang,
H. P. (2003). Pharmacology. Edinburgh: Churchill Livingstone.  ISBN  978-0-443-07145-
4. OCLC  51622037.
14. ^ For 20-fold paralytic dose of toxiferine ("calebas curare"), according to: Page
330 in: The Alkaloids: v. 1: A Review of Chemical Literature (Specialist Periodical Reports).
Cambridge, Eng: Royal Society of Chemistry. 1971. ISBN 978-0-85186-257-6.
15. ^ León-Carrión, J.; van Eeckhout, P.; Domínguez-Morales Mdel, R.; Pérez-
Santamaría, F. J. (2002). "The locked-in syndrome: a syndrome looking for a therapy".  Brain
Inj.  16  (7): 571–82.  doi:10.1080/02699050110119781. PMID 12119076.
16. ^ Maiese, Kenneth (March 2014).  "Locked-in Syndrome".
17. ^ Locked-in syndrome at NINDS
18. ^ Joshua Foer (October 2, 2008). "The Unspeakable Odyssey of the Motionless
Boy".  Esquire.
19. ^ Piotr Kniecicki "An art of graceful dying". Clitheroe: Łukasz Świderski, 2014, s.
73. ISBN 978-0-9928486-0-6
20. ^ Stephen Nolan (August 16, 2010). "I recovered from locked-in syndrome".  BBC
Radio 5 Live.
21. ^ "He crashed his motorbike and had a stroke - but Hampshire man Gareth
Shepherd is back on his feet".  Daily Echo. November 8, 2016.
22. ^ "Jacob Haendel Recovery Channel". Jacob Handel Recovery. June 29, 2020.
23. ^ "Woman's recovery from 'locked-in' syndrome".  BBC News. March 14, 2012.
24. ^ Parker, I., "Reading Minds," The New Yorker, January 20, 2003, 52–63
25. ^ Keiper, Adam (Winter 2006).  "The Age of Neuroelectronics". The New Atlantis.
pp.  4–41. Archived from  the original on 2016-02-12.
26. ^ 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.
27. ^ "'Locked-In' Patients Can Follow Their Noses". Science Mag. 26 Jul 2010.
Retrieved 27 Dec  2016.

Further reading[edit]
 Piotr Kniecicki (2014). An Art of Graceful Dying. Lukasz
Swiderski ISBN 978-0-9928486-0-6 (Autobiography, written while residual
wrist movements and specially adapted computer)