Locked-In syndrome

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Locked-In syndrome Classification and external resources
Locked-In syndrome can be caused by stroke at the level of the basilar artery denying blood to the pons, among other causes.)
ICD-10	G46.3
ICD-9	344.81
MeSH	D011782

Locked-In syndrome is a condition in which a patient is aware and awake, but cannot move or communicate due to complete paralysis of nearly all voluntary muscles in the body. It is the result of a brain stem lesion in which the ventral part of the pons is damaged. The condition has been described as "the closest thing to being buried alive". In French, the common term is "maladie de l'emmuré vivant", literally translated as walled-in alive disease. ^[1]

Locked-in syndrome is also known as Cerebromedullospinal Disconnection,^[1] De-Efferented State, Pseudocoma,^[1] and ventral pontine syndrome.

The phrase "Locked-In syndrome" was created by Plum and Posner in 1966.^[1][1]

Contents 1 Presentation 2 Causes 3 Treatment 4 Prognosis 5 Assistive technology 6 Notable case 7 Cultural references 8 References

Presentation

Locked-in syndrome results in quadriplegia and inability to speak in otherwise cognitively-intact individuals. Those with locked-in syndrome may be able to communicate with others by coding 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 body. Some patients may have the ability to move certain facial muscles, most often some or all of the extraocular eye muscles.

Causes

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:

• Traumatic brain injury
• Diseases of the circulatory system
• Medication overdose
• 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

Treatment

There is no standard treatment for Locked-In syndrome, nor is there a cure. Stimulation of muscle reflexes with electrodes (Neuromuscular stimulation) has been known to help patients regain some muscle function. Other courses of treatment are often symptomatic.^[1]

New direct brain interface mechanisms may provide future remedies.^[1]

Prognosis

It is extremely rare for any significant motor function to return. The majority of locked-in syndrome patients do not regain motor control, but devices are available to help patients communicate.

Assistive technology

See:

• Dasher

Notable case

Parisian journalist Jean-Dominique Bauby had a stroke in 1995, and when he awoke 20 days later he found that his body had stopped working: he could only control his left eyelid. By blinking his eye he dictated a letter at a time and in this way he wrote his memoir The Diving Bell and the Butterfly.^[1]

Cultural references

• (1844) In Dumas's The Count of Monte Cristo, Monsieur Noirtier de Villeforte becomes locked-in after suffering a stroke.
• (2001) Bernard Werber - L'ultime Secret (The Ultimate Secret): one side of two parallel stories is about broken life of a modest bank clerk in Nice, Jean-Louis Martin, victim of a car accident to Locked-In Syndrome ("Immured alive", whose brain continues to function, the rest of the nerve system being paralysed) (list of works)
• (2003) In the first of Mark Billingham's novels featuring London detective Tom Thorne, Sleepyhead, a murderer is able to induce locked-in syndrome by manipulating cranial pressure points.
• (2003) German electronic-industrial band SITD, on their album Stronghold, released a song entitled "Locked In Syndrom," discussing the condition and its effects.
• (2004) In the first episode of CSI: NY, the only witness to a series of murders is a woman with locked-in syndrome.
• (2006) In the episode "His Story III" of Scrubs, a patient with locked-in syndrome is featured.
• (2007) In episode #315 "The Little Things You Do Together" of Desperate Housewives, Gloria Hodge suffers a massive stroke that leaves her paralyzed and unable to speak but otherwise cognitively aware.
• (2003-2008) Series of novels by Jim Kelly featuring journalist Philip Dryden, whose wife Laura suffers from locked-in-syndrome following automobile accident and subsequent coma. Titles to date are "The Water Clock" (2003); "The Fire Baby" (2004); "The Moon Tunnel" (2005); "The Coldest Blood" (2007); "The Skeleton Man" (2008).

References

v • d • e Cerebral palsy and other paralytic syndromes (G80-G83, 342-344)
Paresis and plegia NOS	Paralysis - Quadriplegia - Triplegia - Hemiplegia/Hemiparesis - Paraplegia/Diplegia - Monoplegia
Flaccid vs. spastic	Flaccid paralysis - Spastic diplegia - Spastic paraplegia
Specific types	Cerebral palsy - Cauda equina syndrome - Locked-In syndrome

v • d • e Lesions of spinal cord, brainstem, and cortex
Spinal cord	Anterior cord syndrome - Brown-Séquard syndrome - Central cord syndrome
Medulla (CN 8, 9, 10, 12)	Lateral medullary syndrome/Wallenberg (PICA) - Medial medullary syndrome (ASA)
Pons (CN 5, 6, 7, 8)	Lateral pontine syndrome (AICA) - Medial pontine syndrome (Basilar) - Locked-In syndrome (ventral)
Midbrain (CN 3, 4)	Weber's syndrome (medial, PCA) - Parinaud's syndrome (dorsal)
Cortex	Anterior cerebral artery syndrome - Middle cerebral artery syndrome - Posterior cerebral artery syndrome

da:Locked-in-syndrom

de:Locked-in-Syndrom fr:Locked-in syndrome it:Sindrome locked-in nl:Locked-in-syndroom