Locked-in syndrome

Whatever the name given to this disease by different people at different times, can words convey the tragedy of a situation where a living soul and a healthy mind remain locked inside a virtually completely immobilized body for a long time? It is not for nothing that one of the names of this terrible disease is locked-in syndrome. But can one express it more accurately when a person at some point becomes a hostage of his body, which is bound by illness?

Epidemiology

According to statistics, locked-in syndrome is a very rare pathology. Doctors diagnose a waking coma in one patient out of a million. But these are only the cases where the diagnosis was made promptly and correctly. It is possible that there are actually more such patients, but the low level of development of medicine in some countries simply does not allow them to be identified, because the border between a coma and a pseudo-coma is so thin.

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Causes of locked-in syndrome

Isolated person syndrome, locked-in syndrome, awake coma, Monte Cristo syndrome, Vigil coma, motor function absence syndrome, isolation syndrome - all these are names of the same pathology, the essence of which comes down to the absence of a normal, habitual for a healthy person reaction to what is happening due to the development of certain diseases of the brain, neuromuscular system or blood vessels.

In the literature, one can also find other names for this pathology: deefferentation syndrome, pseudo coma, block syndrome, ventral pontine syndrome, ventral pontine syndrome, "lock-in" syndrome, cerebromodular block, etc. All of them, to a greater or lesser extent, indicate a possible cause of such a person's condition or a connection between the pathology and a certain event.

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Risk factors

Risk factors for the development of isolation syndrome have been and remain many diseases that, under certain conditions, tend to give rise to such a complication.

According to data from various sources, diseases that can be considered possible causes of locked-in syndrome include:

• A cerebral infarction that affects certain areas of the brain that are responsible for the functioning of certain organs and systems of the body (for example, a basal cerebral infarction or a basal midbrain infarction).
• Multiple sclerosis
• Hypertension (long-term)
• brainstem encephalitis with basal lesions
• neuroborreliosis
• Amyotrophic lateral sclerosis
• Myasthenia,
• Brain hemorrhage
• central pontine myelinolysis,
• Guillain-Barre syndrome,
• polio
• paroxysmal myoplegia,
• head injury (albeit a rather rare, but still a possible cause of locked-in syndrome), etc.

Paralysis of motor activity with preserved consciousness and the ability to hear, understand and comprehend speech can also be observed when certain poisons enter the human body.

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Pathogenesis

In our restless age of information technology, which has taken even small children captive, the locked-in syndrome can only be compared to a computer without an Internet connection. It remains the same “intelligent” machine, capable of “thinking,” counting, accumulating information, but it can no longer perform many important functions related to communication. True, everything can be fixed here by simply connecting to local networks, but a person does not have this opportunity, and the isolation syndrome can be considered a life sentence.

The development of locked-in syndrome is associated with disturbances in the functioning of one of the sections of the brain stem - the pons. It is this part of the brain, consisting of white matter, that is responsible for ensuring the connection of the brain and other human organs, as well as for the innervation and sensitivity of organs and systems. White matter is nothing more than nerve fibers responsible for motor activity and providing neural interaction between the hemispheres of the brain, cerebellum and spinal cord.

Damage to the pons most often occurs in cases of infarction or stroke of the brain with disruption of the integrity of its tissues or blood vessels. As a result, only motor and communication functions suffer, while thinking remains at the same level. A person hears, sees and understands everything, but remains unable to react to what is happening through speech, facial expressions and movements. Often, respiratory and digestive functions also suffer (a person cannot breathe, chew and swallow food independently). Eye movements also remain limited.

This condition is in many ways similar to the condition of a person who has fallen into a coma. Hence one of the names of the pathology is a waking coma. A distinctive feature of the isolation syndrome is that the victim is conscious all this time, which means he can hear speech and experience various feelings. Conversations about the unenviable situation of such a patient and discussion of the extremely unattractive prognosis of the disease at the patient's bedside can additionally traumatize the psyche of a person who is already aware of his disadvantaged position.

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Symptoms of locked-in syndrome

The clinical picture of patients with locked-in syndrome can be as diverse as the symptoms of the pathology that caused this condition allow. That is, the picture that doctors observe in most cases consists of the symptoms of the isolation syndrome itself and the manifestations of the pathology that caused paralysis of motor activity. The degree of severity of the disease also leaves its mark on the overall picture, depending on which we observe certain symptoms of varying intensity.

The first signs that doctors pay attention to when diagnosing locked-in syndrome are tetraparesis, characterized by dysfunction of the limbs up to their complete paralysis with preserved muscle tone, and pseudobulbar syndrome, as a result of which bulbar functions (speech, chewing, swallowing, articulation, facial expressions) suffer. These are the main manifestations of the locked-in syndrome.

From the outside, the picture looks something like this: the person's condition generally resembles a coma, he cannot speak, chew and swallow food, or breathe on his own. The patient's movements are usually completely limited, although the sensitivity of the skin remains at the same level. The patient's only connection with the world is the eyes, with the preserved ability to move them in the vertical plane (eye movements from side to side are impossible).

Some patients can move their eyelids, i.e. close and open their eyes. This gives them the ability to communicate, which, with preserved consciousness and mental activity, remains necessary for the patient. It is precisely this ability that often saved patients who were initially given a completely different diagnosis, leaving no hope for the future.

The fact that brain functions are not affected by isolation syndrome also explains the preserved sleep-wake cycles in such patients. In a waking state, a person hears, sees and is aware of everything that is happening around him, but all his reactions remain hidden from the outside eye (locked inside the body).

This condition can occur suddenly against the background of a progressive pathology. In this case, a person at some point simply falls into a coma, and after coming out of it after some time, can no longer return to normal life, remaining bedridden and motionless in the literal sense of the word.

But sometimes the disease develops gradually. The motor activity of the arms and legs worsens, then difficulties with pronouncing sounds and breathing appear, the person cannot focus his gaze on a specific object. In the end, the patient again falls into a coma for a certain period of time. After emerging from the coma, he discovers that he can communicate only by eye movement, and remains unable to take care of himself independently.

Forms

Isolation syndrome can manifest itself differently in different people. The common symptom is preserved electrical activity of the brain and cognitive abilities against the background of a noticeable decrease in the motor activity of the limbs, facial and articulatory muscles.

The above-described symptoms of locked-in syndrome are characteristic of one of the types of pathology - classical. This is the most common type of pathology associated with disorders of the brain stem structures.

If, in addition to the movements of the eyelids and eyeballs, the patient retains movements of some other muscle groups (even if they are very limited), we are talking about incomplete locked-in syndrome, which is a milder form of the pathology and gives a person more opportunities to overcome the disease at any stage.

The total (or complete) form of isolation syndrome is spoken of when the patient lacks any motor reactions that help him communicate with the world. At the same time, the brain continues to work actively, as indicated by the preservation of its healthy bioelectric activity (according to the results of the encephalogram). This is the most severe form of the disease, which can easily be confused with a coma if a special examination is not carried out.

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Complications and consequences

Locked-in syndrome, which is in most cases a complication of various neurological diseases, completely changes the patient's life. His body can function normally only thanks to artificial respiration devices and special care. Patients are fed through a tube, because they themselves cannot chew or even swallow food.

The life of patients with isolation syndrome now depends entirely on the people around them, their love, patience and care. After all, a bedridden patient requires preventive measures to prevent bedsores, in other words, the patient needs to be turned over regularly, despite the fact that he cannot help with this in any way. A person cannot go to the toilet on his own (despite the fact that this need remains, the patient feels when it is time to empty the bladder or bowels, but is not always able to communicate this), wash himself, change his underwear and clothes. All this will need to be done by other people.

Understanding one's helplessness only worsens the situation of such patients, not to mention conversations at the patient's bedside, when his unenviable situation and not the most favorable prognosis are discussed. Not every person is able to step over this and try to change his life for the better with a minimum of opportunities. However, such precedents have existed, and a person doomed to remain a hostage of his body all his life suddenly found a new life and the opportunity (not without the help of others) to use his mind for the benefit of others.

Thanks in large part to such strong people, doctors have received full information about what patients experience in a pseudo-coma state and what they are capable of. This makes it possible to think through various treatment options for such patients, even if no decent results have been achieved in this direction yet.

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Diagnostics of locked-in syndrome

At first glance, a patient with locked-in syndrome can be confused only with someone who is in a state of true coma. However, for neurologists, this is not a problem. Usually, it is enough to study the symptoms to make a final diagnosis.

However, in the case of the total form of the syndrome, everything is not so simple. After all, a person in such a situation practically does not show any signs of brain activity. It is possible to prove that the patient is fully conscious and has preserved sleep and wake cycles only through instrumental diagnostics. The picture becomes clearer in particular after performing an encephalography. With isolation syndrome, the encephalogram remains the same as in a healthy person, which is simply impossible with a true coma.

Analysis and other methods of instrumental examination of brain function, such as computer and magnetic resonance imaging of the head (CT and MRI of the brain), diffuse optical tomography, magnetoencephalography, etc., united under the single name "neuroimaging of the brain" are carried out exclusively for the purpose of identifying the pathology that led to the development of locked-in syndrome.

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Differential diagnosis

The goal of differential diagnosis in this case is to distinguish between coma and pseudocoma, as well as to determine the causes of this condition.

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Treatment of locked-in syndrome

Despite the fact that doctors have learned to distinguish locked-in syndrome from a comatose state, therapy for the pathological condition is still at a low level. Few patients manage to fight the disease. The majority of patients die within the first month of the onset of the disease.

The hope, if not for a full recovery, then at least for a partial restoration of the functions of the muscular system, allowing the patient to communicate in society, is mainly for those whose illness is caused by a cause that can be corrected. In the case of incurable diseases, the prognosis is much worse. And yet, there are cases confirming the fact that with a mild and moderate form of isolation syndrome, patients are capable of more than just lying like a vegetable in the care of others.

Unfortunately, effective medications capable of bringing a person out of this state and returning them to normal life have not yet been found. Therefore, treatment of such patients is mainly reduced to combating the cause of the development of locked-in syndrome (i.e., the pathology that caused the pseudo-coma) and preventing possible complications caused by the patient's prolonged immobility (pulmonary congestion with the development of pneumonia, UTI due to insufficient hygiene, etc.).

Glucocorticosteroids, antihistamines, immune-stimulating drugs and other medications are used to treat and prevent various complications. Folk remedies and homeopathy are not very effective in this case.

Physiotherapy treatment for patients with locked-in syndrome may include plasmapheresis (using immunoglobulins), therapeutic exercise to maintain normal joint function and other procedures, electrical myostimulation (functional neuromuscular stimulation to restore the function of certain muscle groups), magnetic stimulation of the motor cortex and other methods of physical influence.

The prognosis becomes more favorable if the body responds to neuromuscular stimulation (for example, the patient begins to move his eyes in a horizontal direction, motor reactions appear in muscle groups other than the eye).

In the case of incomplete and classic forms of locked-in syndrome, good results in restoring cognitive functions are achieved by speech therapy sessions (learning to convey thoughts through eye movement and blinking), watching TV, reading books to the patient. And people caring for such patients should communicate with them more, developing their own system of codes. After all, patients with locked-in syndrome are distinguished by good mental abilities and understanding of other people's speech, which means they can be quickly taught to answer questions and express their requests using eye movements.

Computerized systems have also now been developed that allow patients with disabilities to communicate with others using a computer and to develop their creativity by writing books.

Surgical treatment is performed either in relation to the pathology that caused the isolation syndrome, or to restore physiologically conditioned functions of the body, such as breathing and nutrition. Sometimes, to help the patient breathe, a tracheotomy is performed, and to introduce food into the body - a gastrotomy (a special tube is inserted into the lumen of the stomach, through which mashed semi-liquid and liquid food is introduced, fully satisfying the body's needs for nutrients).

The main focus in case of limited mobility of the patient is on care by relatives, friends or medical personnel. A patient with locked-in syndrome requires a lot of love, patience and attention for 24 hours, because he cannot call for help in case of deterioration of the condition, is not able to take care of himself independently. But this is a living person, capable of thinking and feeling, and therefore living, even in such harsh (and one can even say cruel) conditions.