8/1/2015

LockedinSyndromeNeurologicDisordersMerckManualsProfessionalEdition

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LockedinSyndrome
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by KennethMaiese,MD

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-Barr 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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Diagnosis). Brain imaging with CT or MRI is done and helps identify the pontine
abnormality. PET, SPECT, or functional MRI may be done to further assess cerebral
function 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, GuillainBarr 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 March 2014 by Kenneth Maiese, MD

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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
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Brain Death

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