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 result in severe widespread motor paralysis (eg, Guillain-Barré syndrome) and cancers that involve the posterior fossa and the pons are less common causes.

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

• Clinical evaluation

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 by requesting eye blinking or vertical eye movements.

As in persistent vegetative state, neuroimaging is indicated to rule out treatable disorders (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 depends on the cause and the subsequent level of support provided. For example, locked-in syndrome due to transient ischemia or a small stroke in the vertebrobasilar artery distribution may resolve completely. When the cause (eg, Guillain-Barré syndrome) is partly reversible, 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

• Supportive care

Supportive care is the mainstay of treatment and should include the following:

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

There is no specific treatment.

Speech therapists may help establish a communication code using eye blinks or movements. Because cognitive function is intact and communication is possible, patients should make their own health care decisions. Some patients with locked-in syndrome communicate with each other via the Internet using a computer terminal controlled by eye movements and other means.

Last full review/revision September 2012 by Kenneth Maiese, MD

Content last modified November 2012