Yes, Locked In Syndrome (LIS) is a real medical condition. It is a rare neurological disorder that results in complete paralysis of the voluntary muscles in the body, including those that control speech and facial expressions.
However, individuals with LIS retain full cognitive ability, including the ability to see, hear, and think.
LIS is caused by a variety of conditions, mainly brainstem strokes and traumatic brain injuries. It can also be caused by diseases such as Amyotrophic Lateral Sclerosis (ALS), Multiple System Atrophy (MSA), progressive supranuclear palsy (PSP), or some cases of Guillain-Barre Syndrome.
Due to its rarity, diagnosis of LIS can be difficult because there is no specific test to detect the condition. To determine if an individual has LIS, physicians take into account their medical history, physical examination, laboratory tests, imaging studies, and clinical experience.
Because of the extreme paralysis, those with Locked In Syndrome cannot move or speak and they lack the ability to consciously control their facial expressions. However, they are often able to communicate by using eye-movement, facial expression, and communication devices.
Although Locked In Syndrome is a debilitating condition, many individuals live with it for years, often with improved motor function as time goes on. Research is ongoing to further understand how to diagnose, treat, and manage LIS.
What triggers locked-in syndrome?
Locked-in syndrome is a neurological condition caused by damage to a person’s brain stem. It is typically caused by damage to the pons, a part of the brain stem that is responsible for sending and receiving signals from the lower part of the brain to the rest of the body.
This damage prevents someone from controlling any voluntary movement beyond their eyes, meaning they are conscious but completely paralyzed. The most common cause of damage to the pons is a stroke, although it can be caused by traumatic head injury, brain stem tumor, genetic disorder, infection, or auto-immune disease.
Other neurological diseases, such as multiple sclerosis, amyotrophic lateral sclerosis, and botulism, can also cause locked-in syndrome.
Does locked-in syndrome happen suddenly?
Locked-in syndrome, which is a type of paralysis that affects all voluntary muscle actions except those of the eyes, does not typically happen suddenly. Most cases of locked-in syndrome are the result of brain stem stroke, trauma to the brain stem or spine, or brain stem tumor.
Therefore, the condition can take days, or even weeks, for symptoms to develop and for an accurate diagnosis to be made. The condition can also be caused by neurodegenerative diseases or other conditions like myasthenia gravis, which can cause the onset of symptoms to occur gradually over a period of months or even years.
In either case, the slow progression of symptoms often results in a situation in which the onset is difficult to pinpoint because patient may not experience the sudden onset of symptoms that other neurological conditions may cause.
Can you still feel pain with locked-in syndrome?
Yes, individuals with locked-in syndrome can still feel pain. However, since they are paralyzed and unable to express their pain, it often goes unnoticed or unreported. For this reason, it is important to be aware of pain behaviors that may indicate discomfort, such as changes in heart rate, body temperature, or facial expressions.
It is also important to regularly monitor the health of loved ones with locked-in syndrome. Other potential signs of pain include increased muscle tone, restlessness, and an agitated response when touched.
Providing regular pain relief such as medication, massage, physical therapy, and relaxation strategies can also be helpful in managing pain for those with locked-in syndrome. Additionally, engaging individuals with locked-in syndrome in communication, whether verbal or non-verbal, can often help determine the presence of pain.
Are people with locked-in syndrome conscious?
Yes, people with locked-in syndrome are conscious, meaning they’re aware of both their environment and themselves. Locked-in syndrome is defined as a state of total paralysis in which the patient is conscious and able to think, but unable to move or express himself in any manner—including speaking or blinking.
Unfortunately, no cure exists for locked-in syndrome, which often results from a stroke or other neurological issue. While those with locked-in syndrome are conscious and aware of their environment, their communication with the outside world is extremely limited.
While there have been some cases where patients have been able to communicate using spelling boards, motorized wheelchairs, or eye-tracking technology, for the most part, doctors and family members must rely on non-verbal cues and difficult to detect changes in facial expressions to assess the patient’s condition.
For individuals with locked-in syndrome, their families must play an incredibly important role in their lives, capitalizing on any and all communication techniques to build a bond with the patient.
Is locked-in syndrome Guillain Barre?
No, locked-in syndrome is not the same as Guillain Barre. Locked-in syndrome is a rare neurological disorder that occurs when almost all voluntary muscle activity is inhibited due to severe damage to the brain stem.
It is caused by stroke, brainstem hemorrhage, brainstem trauma or encephalitis. Symptoms of locked-in syndrome include paralysis of all voluntary muscles except those of the eye, inability to speak, and lack of a gag reflex.
People who suffer from locked-in syndrome are considered to be mentally alert.
Guillain Barre is an autoimmune disorder in which the body’s immune system attacks the peripheral nervous system. It usually begins with weakness and tingling in the legs and can cause paralysis of the face, arms and legs.
Symptoms of Guillain Barre vary from person to person, but may include muscle weakness, numbness and tingling, loss of feeling in the extremities and/or facial paralysis. In more severe cases, Guillain Barre can cause respiratory failure.
Treatment for Guillain Barre includes plasmapheresis, intravenous immunoglobulin, and steroids.
How long can someone live with locked-in syndrome?
The prognosis for someone with locked-in syndrome depends on the cause and severity of the condition. In some cases, individuals can live for many years, as long as 20 years or more. Other cases, however, may not be as long-lasting.
In general, individuals with locked-in syndrome have a reduced life expectancy, and some deaths may occur within weeks or months of diagnosis. Treatment options and supportive care can help to maximize quality of life and, in some cases, improve prognosis.
An outlook is always individualized and dependent on factors such as the individual’s age, overall health, and response to treatment.
How often do people recover from locked-in syndrome?
The recovery rate of people with locked-in syndrome is variable, and there is no definitive answer as to how often people recover. Studies have shown that a number of factors, such as the severity of the condition, the patient’s mental and physical condition before the onset of locked-in syndrome, and the type of treatment received, can all influence recovery.
Some patients may have a relatively good recovery, such as being able to speak and move certain parts of their body, while others may not make as much progress. Some studies have suggested that up to 40% of people with locked-in syndrome make some meaningful progress, but it may not be enough for them to return to a normal lifestyle.
In most cases, however, progress does continue over a long period of time.
The best way to determine a person’s exact recovery rate is to get a detailed evaluation from a doctor or specialist that is familiar with locked-in syndrome. In some cases, special therapies such as physical therapy, communication aids, and assisted living may help improve a patient’s quality of life, while in other cases a permanent disability may be unavoidable.
How do locked-in syndrome patients breathe?
Locked-in syndrome patients are generally able to breathe on their own, however the process can be more challenging than for those without any functional paralysis. To help with this, many patients benefit from additional respiratory support such as ventilators and/or diaphragmatic (breathing) pacing.
Ventilators can be used to provide total support for breathing or to assist the patient with additional breaths at night or during times of increased exertion. Diaphragmatic pacing is a type of electrical stimulation that is applied to the diaphragm to help the patient with their respiration efforts.
It can be used in conjunction with ventilators, or on its own as a form of assisted ventilation. In terms of self-respiration, patients are able to voluntarily control their breathing as much as possible, improving their ability to take in oxygen and expel carbon dioxide.
As with any medical condition, it is important to speak with a medical professional to determine the best course of action to support the breathing of a locked-in syndrome patient.