Cloistered syndrome: types, causes and treatment

Imagine living trapped in a totally soundproof jail, from which all the information from outside but neither your voice nor your actions can be seen through the walls. You can not do anything or talk to anyone, or have an interaction with the medium. The world knows that you exist, but apart from that it cannot practically know how you feel, or how you feel, or what you think.

Now imagine that this prison is nothing other than your own body. It is what happens to people who 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.

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The locked-in syndrome

A locked-in syndrome is a neurological syndrome in which the subject is unable 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. As usual,

the only exception to this fact is eye movement and possibly the upper eyelid, which are kept preserved.

People who suffer from this syndrome maintain practically all their cognitive functions, with only motor involvement. Although his muscles do have the ability to move, motor commands are not transmitted to them. The same goes for the voice.

Subject is tetraplegic, completely paralyzed, and you may lose the ability to breathe on your own. Due to the symptoms (the subject is conscious but cannot move anything except the eyes, and this not in all cases), it is very common for a extreme panic, anxiety, depression and emotional lability.

Generally, this syndrome occurs in two phases: in the first, articulatory capacity is lost, movement and it may be that consciousness and basic physiological abilities, but in the chronic phase consciousness, eye movement and ability are recovered respiratory.

The locked-in syndrome can be easily confused with coma, or even with the brain death, due to the absence of a perceptible motor reaction (if there is a total confinement, there may not be the possibility of moving the eyes). In some cases, it has even taken years to identify the patient as aware of what is happening around him.

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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 affectation and the capacities that have been maintained. Generally three types of presentations can be found.

1. Classic cloister

It is the type of locked-in syndrome in which the subject cannot perform any voluntary motor action beyond eye movement, remaining aware of the environment. They can blink and move the eye, albeit only vertically

2. Incomplete cloister

In this case, the level of paralysis is similar but in addition to eye movement, they can mobilize some fingers or even parts of the head.

3. Total confinement

The worst of the three subtypes. In the syndrome of total confinement, the subject is not able to make any type of movement, not even of the eyes. The gaze remains frozen and immobile. Despite this, the subject remains aware of what is happening around him.

Etiology of this syndrome

The locked-in syndrome occurs due to the existence of a brain injury, specifically in the Brain stem. Most often the damage occurs in the boss. The rupture of the nerve fibers in this area is what generates the generalized motor paralysis and the horizontal control of the gaze.

Generally this fiber break is caused by a stroke or stroke with effects in this area, although it can also appear due to head injuries or diseases or tumors. In some cases it has been caused by overdose.

Depending on its causes, the locked-in syndrome can be chronic or transitory, the latter being of the assumptions the one that admits the partial or even complete recovery of the functions in a progressive.

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Treatment

The locked-in syndrome does not have a treatment or therapy that allows its cure. In some cases, if the cause of the locked-in syndrome is temporary or can be recovered improvements are possible and the patient can perform certain movements.

In most cases the applied treatments are mainly aimed at maintaining the person alive and make sure they can breathe and feed (the latter by tube) adequately. Also to avoid the emergence of complications derived from the absence of movement (for example avoiding ulcerations and sores due to staying in the same position for a long time, monitoring nutrition, injecting drugs that allow blood to flow properly throughout the body and do not form thrombi). Physiotherapy is also used to preserve the flexibility of the joints and muscle groups.

Another major goal of treatment is the development and learning of methods that allow the patient to communicate with loved ones, such as by using pictograms or by moving the eyes. 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 communicative codes through elements that record brain activity, such as the electroencephalogram.

It also has to be aware of feelings of loneliness, misunderstanding, and panic that these subjects usually suffer, with which counseling and possible psychological treatment would be useful. Psychoeducation, both for them and for their families, can also be of great use, in a way that helps to generate guidelines that allow managing the situation.

The general prognosis for this condition is not positive.. Most cases tend to die in the first few months, although they can sometimes live for many years. In some cases, part of muscle function may be restored. And although it is exceptional, on some occasions, as in the case of Kate Allatt, a complete recovery has been achieved.

Bibliographic references:

• Maiese, K. (s.f.). Cloistered syndrome. MSD Manual. Professional version.

• Lara-Reyna, J.; Burgos-Morales, N.; Achim J.; Martínez, D. and Cárdenas, B. (2015). Cloistered syndrome. Presentation of a case. Chilean Journal of Neurosurgery, 41.

• Smith, E. & Delargy, M. (2005). Locked-in syndrome. BMJ; 330-406