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.
How common is Locked-In Syndrome?
Locked-in syndrome is a rare neurological disorder that affects males and females in equal numbers. Locked-in syndrome can affect individuals of all ages including children, but most often is seen in adults who are more at risk for brain stroke and bleeding. Because cases of locked-in syndrome may go unrecognized or misdiagnosed, it is difficult to determine the actual number of individuals who have had the disorder in the general population. Please discuss with your doctor for further information.
What are the symptoms of Locked-In Syndrome?
The common symptoms of Locked-In Syndrome are:
- 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
There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.
When should I see my doctor?
If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.
What causes Locked-In Syndrome?
Locked-in syndrome is most often caused by damage to a specific part of the brainstem known as the pons. The pons contains important neuronal pathways between the cerebrum, spinal cord and cerebellum. In locked-in syndrome there is an interruption of all the motor fibers running from grey matter in the brain via the spinal cord to the body’s muscles and also damage to the centers in the brainstem important for facial control and speaking.
Damage to the pons most often results from tissue loss due to lack of blood flow (infarct) or bleeding (hemorrhage) – less frequently it can be caused by trauma. An infarct can be caused by several different conditions such as a blood clot (thrombosis) or stroke. Additional conditions that can cause locked-in syndrome include infection in certain portions of the brain, tumors, loss of the protective insulation (myelin) that surrounds nerve cells (myelinolysis), inflammation of the nerves (polymyositis), and certain disorders such as amyotrophic lateral sclerosis (ALS).
What increases my risk for Locked-In Syndrome?
Please discuss with your doctor for further information.
Diagnosis & treatment
The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.
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.
How is Locked-In Syndrome treated?
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.
Lifestyle changes & home remedies
What are some lifestyle changes or home remedies that can help me manage Locked-In Syndrome?
The following lifestyles and home remedies might help you cope with Locked-In Syndrome:
- To prevent contractures, physical therapists gently move the person’s joints in all directions (passive range-of-motion exercises) or splint joints in certain positions.
- Preventing blood clots includes use of drugs and compression or elevation of the person’s legs. Moving the limbs, as occurs in passive-range-of-motion exercises, may also help prevent blood clots.
- Because people are incontinent, care should be taken to keep the skin clean and dry. If the bladder is not functioning and urine is being retained, a tube (catheter) may be placed in the bladder to drain urine. Catheters are carefully cleaned and regularly examined to prevent urinary tract infections from developing.
- Pressure sores can be prevented by frequently repositioning the person and placing protective padding under parts of the body that are in contact with the bed, such as the heels, to protect them.
People with the locked-in syndrome can learn to communicate using a computer input device controlled by eye movements. Other devices can detect when people sniff slightly. These devices can also be connected to a computer and used to communicate. Speech therapists can help people develop a communication code using eye blinks or sniffs. If they recover use of another body part (such as a thumb or the neck), they can communicate in other ways.
However, these methods are tiring and slow. So other methods are being developed using electrodes that are attached to the scalp or implanted in the brain. The electrodes can detect electrical signals produced by nerve cells. These signals are sent to a computer and processed. They can be used to move cursors on a computer screen, operate a robotic arm, and produce computer-generated speech.
If you have any questions, please consult with your doctor to better understand the best solution for you.
Hello Health Group does not provide medical advice, diagnosis or treatment.
Locked-In Syndrome. https://www.msdmanuals.com/home/brain,-spinal-cord,-and-nerve-disorders/coma-and-impaired-consciousness/locked-in-syndrome. Accessed May 21, 2018.
Locked-in Syndrome. https://www.medicinenet.com/locked-in_syndrome/article.htm#locked-in_syndrome_facts. Accessed May 21, 2018.
Locked In Syndrome. https://rarediseases.org/rare-diseases/locked-in-syndrome/. Accessed May 21, 2018.
Review Date: May 22, 2018 | Last Modified: May 22, 2018