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Locked-in Syndrome: A Rare Neurological Disorder

Author: Thomas C. Weiss : Contact: Disabled World

Published: 2014-05-05 : (Rev. 2015-12-22)

Synopsis and Key Points:

Information regarding Locked-in syndrome (LIS) a condition in which a person is aware and awake but cannot move or communicate verbally due to complete paralysis of nearly all voluntary muscles except for the eyes.

Main Digest

'Locked-in syndrome,' is a rare form of neurological disorder, one that involves a state of awareness and wakefulness with quadriplegia and paralysis of the person's lower cranial nerves, resulting in an inability to show facial expressions, to move or speak, or to communicate with the exception of by coded eye movements.

Locked-in syndrome commonly results from a, 'pontine,' hemorrhage or infarct that causes quadriplegia and disrupts and damages a person's lower cranial nerves and the centers that control their horizontal gaze. Additional disorders that result in severe widespread motor paralysis, such as Guillain-Barre' syndrome, or cancers that involve the posterior fossa and the pons, are less common causes.

People with locked-in syndrome have intact cognitive function and are awake. They can open their eyes and have usual sleeping and waking cycles. They have the ability to see and hear. Unfortunately, they do not have the ability to:

Chart presenting inabilities of people with locked-in syndrome
Chart presenting inabilities of people with locked-in syndrome

A person with locked-in syndrome may have the ability to move their eyes vertically. They can open and close their eyes, or blink a specific number of times in order to answer questions posed to them.

Achieving a Diagnosis of Locked-in syndrome

Working to achieve a diagnosis of locked-in syndrome is mainly a clinical effort. Due to the fact that people with locked-in syndrome lack the motor responses, such as withdrawal from painful stimuli, that are usually used to measure responsiveness, they might be mistakenly thought to be unconscious. Therefore, every person who does not have the ability to move should have their comprehension tested by asking them to blink their eyes, or move their eyes vertically.

As with people who are in a vegetative state, neuroimaging is indicated in order to rule out disorders that are treatable. Brain imaging with CT or MRI is performed and assists with identifying the pontine abnormality. A SPECT, PET, or functional MRI might be performed to further assess the person's cerebral function if a diagnosis of locked-in syndrome is in doubt. In people who experience locked-in syndrome, an EEG shows usual sleeping and waking patterns.

The Prognosis for People with Locked-in syndrome

The prognosis for people with locked-in syndrome depends on the cause and the subsequent level of support they receive. For example; locked-in syndrome due to transient ischemia, or a small stroke in their vertebrobasilar distribution, might resolve completely. When the cause, such as Guillain-Barre' syndrome, is partly reversible, their recovery might happen over a period of months, yet is rarely complete. Favorable prognostic features include early recovery of lateral eye movements and of evoked potentials in response to magnetic stimulation of their motor cortex. Irreversible or progressive disorders, such as cancers that involve the person's posterior fossa and the pons, are sadly usually fatal.

Treating Locked-in syndrome

Supportive care is the mainstay of treatment for people with locked-in syndrome. Supportive care for people with locked-in syndrome should include the following:

Supportive care for people with locked-in syndrome should include:

Unfortunately, there is no specific treatment for locked-in syndrome. Speech therapists might help to establish a communication code using eye blinks or movements. Because the person's cognitive function remains intact and communication is possible, people with locked-in syndrome should make their own health care decisions. Some people with locked-in syndrome communicate with one another through the Internet using a computer terminal controlled by their eye movements and additional means.

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