Locked-in syndrome (LiS) is a rare and serious neurological disorder that happens when a part of your brainstem is damaged, usually from a stroke. People with LiS have total paralysis but still have consciousness and their normal cognitive abilities. Most people with LiS can communicate with eye movements and lead meaningful lives.
Locked-in syndrome (LiS) is a rare neurological disorder characterized by paralysis of voluntary muscles, except for those that control your vertical eye (up and down) movements. People with locked-in syndrome are conscious, alert and have their usual cognitive abilities (thinking and reasoning), but they’re unable to show facial expressions, speak or move.
People with LiS can hear and typically communicate through purposeful eye movements, blinking or both. They can also use assistive technologies to communicate.
Locked-in syndrome is caused by damage to a specific part of your brainstem known as the pons.
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Locked-in syndrome (LiS) has three main types, or forms, including:
Anyone can develop locked-in syndrome (LiS), though it more commonly affects people who are 30 to 50 years old.
Because cases of locked-in syndrome (LiS) may go unrecognized or misdiagnosed, it’s difficult for researchers to determine the actual number of people who are affected by LiS each year. However, researchers agree that LiS is rare.
The effects of locked-in syndrome (LiS) on your body vary slightly depending on which type you have.
People with locked-in syndrome are often initially thought to be in a coma before recognition of consciousness.
Most people with locked-in syndrome cannot consciously or voluntarily:
Most people with locked-in syndrome can:
All people with locked-in syndrome can:
Depending on which form of locked-in syndrome (LiS) you have, you may or may not be able to feel physical pain.
If you have the total immobility form of LiS, you won’t be able to feel physical pain due to total paralysis of your body.
If you have the incomplete form of LiS, you may be able to feel physical pain and other sensations in certain areas of your body.
Locked-in syndrome (LiS) is caused by damage to a specific part of your brainstem called the pons.
The pons is a broad horseshoe-shaped mass of nerve fibers that connects the medulla oblongata (the lowest portion of your brainstem) with the cerebellum (a part of your brain that plays a vital role in virtually all physical movement, particularly coordination).
The pons contains important neuronal pathways between your cerebrum, spinal cord and cerebellum. In locked-in syndrome, damage to your pons results in interruption of all the nerves running from your brain, through your spinal cord and to your body’s muscles. This results in body paralysis. The damage to your pons also affects the centers in your brainstem that are important for facial control and speaking, which prevents you from making facial expressions, chewing, swallowing and talking.
Several specific conditions and situations can damage your pons, causing locked-in syndrome. The most common cause is ischemic or hemorrhagic stroke affecting the corticospinal, corticopontine and corticobulbar tracts in your brainstem.
An ischemic stroke happens when a blood vessel in your brain develops a blood clot and cuts off the blood supply to your brain. A hemorrhagic stroke happens when blood from an artery suddenly begins bleeding into your brain.
Other less common causes of damage to your pons that can lead to locked-in syndrome include:
As people with locked-in syndrome (LiS) lack motor responses (such as withdrawal from painful stimulation), which healthcare providers use to measure responsiveness, healthcare providers may mistakenly think people with LiS are unconscious. Because of this, LiS can be difficult to diagnose or take a while to correctly diagnose.
A significant aspect of an LiS diagnosis is ruling out other conditions that could be causing your symptoms. Healthcare providers also need to determine the cause of locked-in syndrome as a part of the diagnosis and treatment.
Healthcare providers use several tests to help diagnose locked-in syndrome, determine the cause and rule out other possible conditions.
These tests include:
There is no cure or specific treatments for locked-in syndrome other than treating the cause and preventing further complications, if possible.
Management of locked-in syndrome includes supportive therapy and communication training.
Supportive therapy for breathing and feeding is very important, especially early on. People with locked-in syndrome (LiS) often need an artificial aid for breathing and will have a tracheotomy (a tube going in their airway through a small hole in their throat).
Because eating and drinking using your mouth isn’t safe with LiS, people with LiS usually have a small tube inserted in their stomach called a gastrostomy tube (G-tube) to receive food and water.
Other supportive therapies include:
Speech therapists can help people with locked-in syndrome (LiS) communicate more clearly with eye movements and blinking. The method of communication is unique to each person. For example, looking up could mean “yes” and looking down could mean “no” or vice versa. People with LiS can also form words and sentences by signaling different letters in the alphabet while someone else verbally lists each letter.
In addition to coded eye movements for communication, electronic communication devices, such as infrared eye movement sensors and computer voice prosthetics, allow people with locked-in syndrome to communicate more freely and have access to the internet.
Depending on the cause of locked-in syndrome (LiS), you may recover motor abilities (movement), but a complete recovery is highly unlikely. Some people with locked-in syndrome have minor motor function and sensation in certain areas of their body.
The majority of people with LiS don’t recover their lost nerve functions, but they can learn to communicate using eye movements.
Unfortunately, most cases of locked-in syndrome aren’t preventable. But your risk of locked-in syndrome may increase if you have a higher risk of stroke.
Talk to your healthcare provider about your risk of stroke and ways you can lower your risk.
The prognosis (outlook) for people with locked-in syndrome (LiS) depends on the cause and form of the condition, as well as the level of support and care they’re able to receive.
A recent survey revealed that people with LiS reported having a happy and meaningful life, especially when they have proper social services and adaptive technology to help them have a normal role at home, as well as in their community.
Many people with LiS can use a motorized wheelchair and a computer with adaptive technology.
Some people with locked-in syndrome don’t live beyond the early stage of the condition due to medical complications. But others live for another 10 to 20 years and report a good quality of life.
If you have locked-in syndrome, it’s essential to ensure you’re getting quality medical care to prevent complications from immobilization, such as pneumonia and bedsores.
It’s also important to find ways to communicate — whether it’s through eye movements or assistive technology, such as eye-tracking technology. Consider joining a support group to meet others who can relate to your experiences, too.
If you’re taking care of someone with locked-in syndrome, it’s important to advocate for them to ensure they get the best medical care and access to technologies that can help them communicate and be more independent.
A note from Cleveland Clinic
Locked-in syndrome (LiS) is a rare but serious condition. It can be very overwhelming to receive this kind of diagnosis. Know that your healthcare team is there to support you and your loved ones. It’s essential to receive continuous quality medical care when living with LiS and to learn new ways of communicating. Due to supportive care and advancements in technology, many people with LiS live full and meaningful lives.
Last reviewed by a Cleveland Clinic medical professional on 03/03/2022.
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