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 except for the eyes. Total locked-in syndrome is a version of locked-in syndrome where the eyes are paralyzed as well.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; in German it is sometimes called “Eingeschlossensein”.

Locked-in syndrome is also known as cerebromedullospinal disconnection, de-efferented state, pseudocoma,and ventral pontine syndrome.

The term for this disorder was coined by Plum and Posner in 1966.

Locked-in syndrome is a state of wakefulness and awareness with quadriplegia and paralysis of the lower cranial nerves, resulting in inability to show facial expression, move, speak, or communicate, except by coded eye movements.

Locked-in syndrome typically results from a pontine hemorrhage or infarct that causes quadriplegia and disrupts and damages the lower cranial nerves and the centers that control horizontal gaze. Other disorders that produce severe widespread motor paralysis (eg, Guillain-Barré syndrome) are a less common cause.

Patients have intact cognitive function and are awake, with eye opening and normal sleep-wake cycles. They can hear and see. However, they cannot move their lower face, chew, swallow, speak, breathe, move their limbs, or move their eyes laterally. Vertical eye movement is possible; patients can open and close their eyes or blink a specific number of times to answer questions.

Diagnosis

Diagnosis is primarily clinical. Because patients lack the motor responses (eg, withdrawal from painful stimuli) usually used to measure responsiveness, they may be mistakenly thought to be unconscious. Thus, all patients who cannot move should have their comprehension tested through requesting eye blinking or vertical eye movements.

Tests are chosen for the same indications as persistent vegetative state (see Coma and Impaired Consciousness: Diagnosis). Brain imaging with CT or MRI is done and helps identify the pontine abnormality. PET or SPECT may be done if the diagnosis is in doubt. In patients with locked-in syndrome, EEG shows normal sleep-wake patterns.

Prognosis

Prognosis is usually dire. However, locked-in syndrome due to transient ischemia or a small stroke in the vertebrobasilar artery distribution may resolve completely. When the cause is partly reversible (eg, Guillain-Barré syndrome), recovery can occur over months but is seldom complete. Favorable prognostic features include early recovery of lateral eye movements and of evoked potentials in response to magnetic stimulation of the motor cortex. Irreversible or progressive disorders (eg, cancers that involve the posterior fossa and the pons) are usually fatal.

Treatment

There is no specific treatment, but supportive care should include the following:

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Preventing systemic complications due to immobilization (eg, pneumonia, UTI, thromboembolic disease) Providing good nutrition
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Preventing pressure ulcers
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Providing physical therapy to prevent limb contractures

Speech therapists may help establish a communication code using eye blinks or movements. Because cognitive function is intact, patients should make their own health care decisions if communication can be established.