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8/1/2015

Locked­in Syndrome ­ Neurologic Disorders ­ Merck Manuals Professional Edition

PROFESSIONAL VERSION
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Consumer Version

Locked­in Syndrome
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by Kenneth Maiese, MD

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ocked-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 sleepwake 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 vegetative state, neuroimaging is indicated to rule out treatable disorders (see
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recovery can occur over months but is seldom complete. Speech therapists may help establish a communication code using eye blinks or movements. MD http://www. PET. pneumonia.8/1/2015 Locked­in Syndrome ­ Neurologic Disorders ­ Merck Manuals Professional Edition Diagnosis). In patients with locked-in syndrome. GuillainBarré syndrome) is partly reversible. cancers that involve the posterior fossa and the pons) are usually fatal. When the cause (eg. EEG shows normal sleep-wake patterns. Prognosis Prognosis depends on the cause and the subsequent level of support provided. locked-in syndrome due to transient ischemia or a small stroke in the vertebrobasilar artery distribution may resolve completely. UTI. patients should make their own health care decisions. Because cognitive function is intact and communication is possible. or functional MRI may be done to further assess cerebral function if the diagnosis is in doubt. thromboembolic disease) Providing good nutrition Preventing pressure ulcers Providing physical therapy to prevent limb contractures There is no specific treatment. For example.com/professional/neurologic­disorders/coma­and­impaired­consciousness/locked­in­syndrome 2/3 . SPECT. Favorable prognostic features include early recovery of lateral eye movements and of evoked potentials in response to magnetic stimulation of the motor cortex.merckmanuals. Some patients with locked-in syndrome communicate with each other via the Internet using a computer terminal controlled by eye movements and other means. Treatment Supportive care Supportive care is the mainstay of treatment and should include the following: Preventing systemic complications due to immobilization (eg. Brain imaging with CT or MRI is done and helps identify the pontine abnormality. Last full review/revision March 2014 by Kenneth Maiese. Irreversible or progressive disorders (eg.

..merckmanuals. NJ. Kenilworth.8/1/2015 Locked­in Syndrome ­ Neurologic Disorders ­ Merck Manuals Professional Edition Coma and Impaired Consciousness Overview of Coma and Impaired Consciousness Vegetative State and Minimally Conscious State Locked-in Syndrome Brain Death Vegetative State and Minimally Conscious State Was This Page Helpful? Brain Death * This is a professional Version * Consumers: Tap here forthe Consumer Version © 2015 Merck Sharp & Dohme Corp.com/professional/neurologic­disorders/coma­and­impaired­consciousness/locked­in­syndrome 3/3 . USA http://www.. a subsidiary of Merck & Co. Inc..