Locked-in Syndrome

  • Medical Author:
    Charles Patrick Davis, MD, PhD

    Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.

  • Medical Editor: Melissa Conrad Stöppler, MD
    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD

    Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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Locked-in syndrome facts

  • Locked-in syndrome occurs when a person is quadriplegic and also has no way to produce speech or facial movements. The affected person cannot communicate but is aware of their surroundings; the patient can hear and see and has normal intelligence and reasoning ability but may only communicate with eye movements.
  • The main causes of locked-in syndrome are brainstem hemorrhage or infarct; rarely other causes such as trauma, tumors, or infection, for example, may cause the syndrome.
  • The symptoms and signs of locked-in syndrome include quadriplegia and the inability to speak or make facial movements, but those affected are aware and may communicate by eye movements.
  • The syndrome is often (about 50% of the time) discovered by the patient's family members that recognize awareness in their family member; MRI and eye-movement tests can help diagnose locked-in syndrome.
  • Although there is no specific treatment for locked-in syndrome, supportive care and communication by eye movements can help the patient survive and improve their quality of life.
  • Most locked-in syndrome patients do not recover lost functions; however, in rare instances, significant improvement in the condition may occur.
  • Statistics suggest that patients diagnosed with locked-in syndrome and given good supportive care that includes communication via eye movements may have a 80% chance of 10-year survival; the majority of patients who develop locked-in syndrome are adults that have increased risk for strokes.
  • For most patients with locked-in syndrome, the prognosis is fair to poor.
  • Although locked-in syndrome is usually not preventable, the risk of locked-in syndrome may increase in individuals with higher risk of stroke.
  • Other sources for more detailed information about locked-in syndrome are presented.

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Locked-in Syndrome Causes: Brain Hemorrhage

A brain hemorrhage is bleeding in or around the brain. Causes of brain hemorrhage include high blood pressure, abnormally weak blood vessels that leak, drug abuse, and trauma. Many people who experience a brain hemorrhage have symptoms as though they are having a stroke, and can develop:

  • weakness on one side of their body,
  • difficulty speaking, or
  • a sense of numbness.

Difficulty performing usual activities, including problems with walking or even falling, are not uncommon symptoms. About 10% of all strokes are hemorrhagic, or caused by bleeding into the brain.

What is locked-in syndrome?

Locked-in syndrome is a rare situation in which a person is wakeful and aware but has quadriplegia and paralysis of the lower cranial nerves that does not allow the person to show facial expressions or make muscular movements such as moving limbs, swallowing, speaking, or breathing. The affected person cannot communicate except by eye movements (blinking, and some patients may possess vertical eye movements). The patients, however, have the ability to see and hear and have normal intelligence and reasoning capabilities. Total locked-in syndrome occurs when the eyes are also paralyzed. Locked-in syndrome has also been termed cerebromedullospinal disconnection, de-efferented state, pseudocoma, and ventral pontine syndrome.

What causes locked-in syndrome?

Locked-in syndrome usually results from a brainstem hemorrhage or infarct resulting in quadriplegia and disruption of the lower cranial nerves. Other potential causes that can affect this part of the brainstem can include trauma, tumors, infection, loss of myelin (protective insulation of nerves), polymyositis (inflammation of the nerves), and other disorders such as ALS (amyotrophic lateral sclerosis) or medication overdose.

What are the symptoms and signs of locked-in syndrome?

The signs and symptoms of locked-in syndrome are as follows:

  • Quadriplegia and paralysis that is global except for vertical eye movements and blinking
  • A coma-like condition (pseudocoma) where the patient only can respond or communicate with others by eye movements
  • Unresponsiveness to painful stimuli (inability to withdraw an extremity from painful stimuli)
  • No horizontal eye movements
  • Cannot consciously chew food, swallow, breathe, speak, or move voluntary muscles
  • Must rely on caregivers for most basic functions (body movements and hygiene, for example)
  • Fully alert and aware of the environment
  • Ability to see, hear, and have normal sleep-wake cycles
  • Ability to think and reason normally

How is locked-in syndrome diagnosed?

Locked-in syndrome may be difficult to diagnose in some patients initially because some patients may be comatose for a while and then develop locked-in syndrome; some patients with a new onset stroke may resemble individuals with locked-in syndrome. The diagnosis can be missed if eye movement (vertical and blinking) is not assessed in seemingly unresponsive patients. Evidence for locked-in syndrome can be seen with MRI imaging of the specific brain area that shows damage. In addition, PET and SPECT brain scans can further assess the patient's abnormality. About half of patients with locked-in syndrome are discovered (diagnosed) by family members that realize the patient is aware and able to respond (communicate), usually with their eye movements. Other tests such as EEGs show normal sleep-wake patterns.

What is the treatment for locked-in syndrome?

There is no specific treatment for locked-in syndrome. Supportive care is the main treatment for locked-in syndrome. Supportive care includes the following:

  • Breathing support
  • Good nutrition
  • Preventing complications of immobilization such as lung infections, urinary tract infections, and blood clot formation
  • Preventing pressure ulcers
  • Physical therapy to prevent contractures
  • Speech therapy to help in developing communication via eye blinks and/or eye vertical movements
  • Possibly, computer terminal control linked to the patient's eye movements

Infrequently, treatment of the underlying cause such as shrinking a tumor or rapidly treating a medical overdose may improve the patient's condition.

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Is recovery from locked-in syndrome possible?

Depending upon the cause (for example, transient blood loss to the brainstem), rarely, a person may recover, although complete recovery is highly unusual. The majority of patients with this syndrome do not recover although they may learn to communicate using eye movements.

What statistics are associated with locked-in syndrome?

About half of all patients with locked-in syndrome are initially found to be aware and able to communicate with others by family members who visit the patient regularly and communicate to the patient's physicians that they suspect the patient is conscious and aware of their surroundings. The other half of patients are diagnosed by their physicians. Although locked-in syndrome can affect anyone at any age, it is most often seen in adults that are at higher risk for brain strokes and bleeding problems. The number of those affected by locked-in syndrome is unknown, because it is rare and often either misdiagnosed or not recognized.

What is the prognosis with locked-in syndrome?

The prognosis of patients with locked-in syndrome varies from fair to poor. Those patients that are diagnosed early and get supportive care that includes development of ways to communicate with other people usually do better than those diagnosed at a later time (sometimes months to years later). Patients that learn to communicate may have a survival rate of 80% after 10 years.

The majority of patients with locked-in syndrome do not recover functionality; most will be entirely dependent upon caregivers for most of their basic functions (feeding, cleaning, changing positions in bed, for example). Rarely, the patient may recover some basic functionality.

Can locked-in syndrome be prevented?

Prevention of trauma that leads to locked-in syndrome is possible, but difficult to predict. Avoiding lifestyles that lead to coronary artery disease which, in turn, can result in strokes may prevent locked-in syndrome in some patients; but because this syndrome is rare, there are little or no data to support this speculation.

Additional information on locked-in syndrome

The family and friends of a patient with locked-in syndrome may want to better understand the patient's condition. Some physicians and families suggest reading a book written by Jean-Dominique Bauby, a patient with locked-in syndrome called, The Diving Bell and the Butterfly: A Memoir of Life in Death. The book has also been made into a movie by the same name.

Additional information may be obtained by contacting the following:

NIH National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
Phone: (301) 496-5751
Toll-free: (800) 352-9424

United States Society for Augmentative and Alternative Communication
100 E. Pennsylvania Avenue, Courtyard
Towson, MD 21286 USA
Toll-free: (877) 887-7222

REFERENCE:

"Locked In Syndrome." National Organization for Rare Diseases (NORD). 2015.

Quick GuideConcussions & Brain Injuries: Symptoms, Tests, Treatment

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Reviewed on 3/18/2016
References
REFERENCE:

"Locked In Syndrome." National Organization for Rare Diseases (NORD). 2015.

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