Imagine living trapped in a completely soundproof prison, from which all the information comes to you from the outside but without your voice or your actions being seen through the walls. You can’t do anything or talk to anyone, or have any interaction with the environment. The world knows that you exist, but apart from that it can practically not know how you are, how you feel, or what you think.
Now imagine that that prison is nothing other than your own body. This is what happens to people who They suffer from the so-called locked-in syndrome a medical condition so disturbing that there is already at least one movie whose plot revolves around it: The Diving Bell and the Butterfly.
Locked-in syndrome is a neurological syndrome in which the subject is not able to perform any motor activity despite maintaining consciousness. The person is able to perceive the environment normally and is conscious, but cannot participate or respond to stimulation. In general, The only exception to this is eye and possibly upper eyelid movement which remain preserved.
People who suffer from this syndrome maintain practically all of their cognitive functions, with only motor impairment. Although your muscles do have the capacity to move, motor commands are not transmitted to them. The same goes for the voice.
The subject is quadriplegic, completely paralyzed, and may lose the ability to breathe on his or her own. Due to the symptoms (the subject is conscious but cannot move anything except the eyes, and this is not in all cases), it is very common for extreme panic, anxiety, depression and emotional lability to occur.
Generally, this syndrome occurs in two phases: in the first, articulatory ability and movement are lost, and consciousness and basic physiological abilities may be lost, but in the chronic phase, consciousness, eye movement, and respiratory capacity are recovered.
Locked-in syndrome can be easily confused with coma, or even with brain death, due to the absence of a perceptible motor reaction (if total enclosure occurs, there may not be the possibility of moving the eyes). In some cases it has even taken years to identify the patient as being aware of what is happening around them.
Types according to the level of paralysis
It’s possible to do a classification of types of locked-in syndrome, depending on the degree of impact and the capabilities that have been maintained. Generally three types of presentations can be found.
1. Classic enclosure
This is the type of locked-in syndrome in which the subject cannot perform any voluntary motor actions beyond eye movement, remaining aware of the environment. They can blink and move their eyes, although only vertically
2. Incomplete enclosure
In this case, the level of paralysis is similar but In addition to eye movement, they can move some fingers or even parts of the head.
3. Total enclosure
The worst of the three subtypes. In total locked-in syndrome the subject is not able to make any type of movement, not even of the eyes. The gaze remains paralyzed and motionless. Despite this, the subject remains aware of what is happening around it.
Etiology of this syndrome
Locked-in syndrome occurs due to the existence of a brain injury, specifically in the brain stem. Most often, the damage occurs in the bump. The rupture of the nerve fibers in this area is what generates generalized motor paralysis and horizontal control of gaze.
Generally this fiber rupture It is caused by a cerebrovascular accident or stroke with effects in said area, although it can also appear due to head trauma or diseases or tumors. In some cases it has occurred due to overdose.
Depending on its causes, locked-in syndrome can be chronic or transient, with the latter being the one that allows partial or even complete recovery of functions in a progressive manner.
Treatment
Locked-in syndrome does not have a treatment or therapy that allows it to be cured. In some cases, if the cause of the locked-in syndrome is temporary or can be recovered improvements may occur and the patient can perform certain movements.
In most cases, the treatments applied are mainly aimed at keeping the person alive and ensuring that they can breathe and feed (the latter by tube) adequately. Also to avoid the emergence of complications arising from the absence of movement (for example, avoiding ulcerations and sores due to remaining in the same position for a long time, monitoring nutrition, injecting drugs that allow blood to flow properly throughout the body and prevent blood clots from forming). Physiotherapy is also used to maintain the flexibility of joints and muscle groups.
Another of the major objectives of treatment is development and learning of methods that allow the patient to communicate with their loved ones, such as through the use of pictograms or through eye movement. In some cases it is even possible to use ICT as an element of communication thanks to the translation of said eye movements. In cases where the eyes are not mobile either, it is possible to establish simple communication codes through elements that record brain activity, such as the electroencephalogram.
It must also be take into account feelings of loneliness, misunderstanding and panic that these subjects usually suffer from, so counseling and possible treatment at a psychological level would be useful. Psychoeducation for both them and their family members can also be very useful, helping to generate guidelines that allow them to manage the situation.
The general prognosis for this condition is not positive Most cases tend to die in the first months, although sometimes they can live for many years. In some cases, part of the muscle function may be recovered. And although it is exceptional, on some occasions, such as in the case of Kate Allatt, a complete recovery has been achieved.
Smith, E. & Delargy, M. (2005). Locked-in syndrome. BMJ; 330-406
