Locked man syndrome

No matter how different people call this disease in different times, can words convey the tragedy of a situation when a living soul and a healthy mind for a long time remain locked inside a virtually completely immobilized body? No wonder one of the names of a terrible disease sounds like this - the syndrome of a locked man. And is it possible to put it more precisely when a person at some point becomes a hostage to his body bound by the disease?

[1], [2]

Epidemiology

According to statistics, the syndrome of a locked person is a very rare pathology. The wakeful to whom the doctors diagnose in one patient out of a million. But these are only those cases when the diagnosis was delivered in a timely and correct manner. It is possible that such patients are actually more, but the low level of medical development in some countries simply does not allow them to be identified, because the border between coma and pseudocoma is so thin.

[3], [4], [5], [6], [7]

Causes of the syndrome of a locked person

The isolated person syndrome, the locked man syndrome, the wakeful coma, the Monte Cristo syndrome, the Vigil coma, the lack of motor function syndrome, the isolation syndrome are all names of the same pathology, the essence of which is the lack 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, you can find other names for this pathology: de-efferentation syndrome, pseudo-coma, blocking syndrome, ventral pontine syndrome, ventral bridge syndrome, "closure" syndrome, cerebromodular block, etc. All of them to a greater or lesser extent indicate the possible cause of such a person's condition or the relationship of pathology with some event.

[8]

Risk factors

The risk factors for the development of the isolation syndrome have been and still are many diseases that are prone to such complications under certain conditions.

To such diseases, which can be considered as possible causes of the development of the syndrome of the locked person, according to data from different sources, one can attribute:

• A cerebral infarction that affects some of its areas responsible for the work of certain organs and systems of the body (for example, a cerebral base infarction or a midbrain basis infarction).
• Multiple sclerosis
• Hypertensive disease (with prolonged course)
• trunk encephalitis with basal lesion
• neuroborreliosis
• Amyotrophic lateral sclerosis
• Myasthenia gravis,
• Hemorrhage in the brain
• central pontinous myelinolysis, 
• Guillain-Barre syndrome, 
• polio
• paroxysmal myoplegia,
• trauma to the head (albeit quite rare, but still the cause of the syndrome of the locked person), etc.

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

[9], [10], [11], [12], [13]

Pathogenesis

In our troubled age of information technology, even small children captured, the syndrome of a locked man can only be compared with a computer that is not connected to the Internet. It remains the same "intelligent" machine capable of "thinking", counting, accumulating information, but it can not carry out many important functions related to communication. True, everything can be fixed here by the usual connection to local networks, but the person does not have such an opportunity, and the isolation syndrome can be considered a sentence for life.

The development of the syndrome of a locked person is associated with impairment in the work of one of the brain stem sections - the variolium bridge. 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 ensuring neuronal interaction of the hemispheres of the brain, cerebellum and spinal cord.

Damage to the variolium bridge is most often observed with a heart attack or stroke with a violation of the integrity of its tissues or blood vessels. As a result, only motor and communicative functions suffer, and thinking remains at the same level. The person hears everything, sees and understands, but remains unable to react to what is happening through speech, facial expressions and movements. Often, respiratory and digestive functions also suffer (a person can not breathe, chew and swallow food independently). The eye movements are also limited.

This state is in many respects similar to the state of a person who has fallen into a coma. Hence one of the names of pathology is the wakeful coma. A distinctive feature of the isolation syndrome is that the victim is conscious all this time, which means that he can hear speech and experience different feelings. Talking about the unenviable situation of such a patient and discussing an extremely unattractive prognosis of the disease at the patient's bed can further traumatize the psyche of a person who is already aware of his flawed position.

[14], [15], [16], [17], [18], [19]

Symptoms of the syndrome of a locked person

The clinical picture in patients with the syndrome of a locked person can be as diverse as the symptomatology of the pathology that caused such a condition allows. That is, the picture that doctors watch, in most cases, consists of the symptoms of the isolation syndrome and manifestations of the pathology that caused paralysis of motor activity. Its imprint on the overall picture of the disease imposes a degree of its severity, depending on which we observe certain symptoms of different intensity.

The first signs, which doctors pay attention to in diagnosing the syndrome of a locked person, are tetraparesis, characterized by limb dysfunction until complete paralysis with the muscle tone preserved, and pseudobulbar syndrome, which results in bulbar functions (speech, chewing, swallowing, articulation, facial expressions ). These are the main manifestations of the isolation syndrome.

From the side the picture looks like this: the state of the person as a whole reminds someone, he can not talk, chew and swallow food, breathe. The patient's movements are usually completely limited, although the sensitivity of the skin remains at the same level. The only connection of the patient with the world is the eyes with the preserved ability to move them in a vertical plane (eye movements from side to side are impossible).

Some patients can move centuries, i.e. Close and open your eyes. This gives them the opportunity to communicate, which, with preserved consciousness and mental activity, remains a necessary one for the patient as well. It is this ability that often saved patients who initially had a completely different diagnosis, leaving no hope for the future.

The fact that brain functions in the isolation syndrome does not suffer, explains the saved cycles of sleep and wakefulness in such patients. In the waking state, a person hears, sees and understands 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 on the background of progressive pathology. In this case, a person at some instant just falls into a coma, and after leaving it after a while, can no longer return to normal life, remaining confined to bed and immovable in the truest sense of the word.

But sometimes the disease develops gradually. Impaired motor activity of the hands, legs, then there are difficulties with pronunciation of sounds and breathing, a person can not focus on a particular subject. In the end, the patient again falls into a coma for a certain period of time. Coming out of a coma, he discovers that he can communicate only with the help of eye movement, and at the same time remains unable to serve himself.

Forms

The isolation syndrome in different people can manifest itself in different ways. A common symptom in this case remains the stored electrical activity of the brain and cognitive abilities against a background of a marked decrease in the motor activity of the limbs, facial and articulatory muscles.

The above symptoms of the syndrome of a locked person are characteristic for one of the types of pathology - the classical one. This is the most common type of pathology associated with impaired brain stem structures.

If, in addition to the movements of the eyelids and eyeballs, the patient retains the movements of some other muscle groups (even if they are severely restricted), it is an incomplete syndrome of a locked person, which is an easier form of pathology and gives the person more opportunities to defeat the disease at any stage.

The total (or complete) form of the isolation syndrome is said in the event that the patient lacks any motor reactions that help him communicate with the world. In this case, 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 not conducted a special examination.

[20], [21]

Complications and consequences

The syndrome of the locked person, which in most cases is a complication of various neurological diseases, completely changes the life of the patient. His body can function normally only through artificial respiration apparatus and special care. Feeding patients through a probe, because they themselves can not only chew, but also swallow food.

The life of patients with the syndrome of isolation now completely depends on the people around him, their love, patience and care. Still, the patient lying down requires preventive measures to prevent the occurrence of bedsores, in other words the patient needs to be regularly turned over, although he can not help it in any way. A person can not go to the toilet alone (although this need is preserved, the patient feels when it's time to empty the bladder or intestine, but not always able to report it), wash, change clothes and clothes. All this will need to be done by other people.

The understanding of their helplessness only aggravates the situation of such patients, not to mention conversations at the patient's bedside, when his unenviable position and not the most favorable forecasts are being discussed. Not every person is able to step over it and try to change their lives for the better with a minimum of opportunities. However, there were such precedents, and a person who was doomed to remain a hostage to 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.

In many respects thanks to such strong people, doctors have received full information about what patients experience in a pseudocoma condition, and what they are capable of. This makes it possible to think over various options for the treatment of such patients, even if there have not yet been any worthy results in this direction.

[22], [23], [24], [25], [26], [27], [28]

Diagnostics of the syndrome of a locked person

A patient with a syndrome of a locked person at first glance can be confused only with someone who is in a state of true coma. However, for specialists in neurology and this is not a problem. Usually it is enough just to study the symptomatology to make the final diagnosis.

However, in the case of the total form of the syndrome, everything is not so simple. After all, a person in this situation practically does not give any signs of brain activity. Prove that the patient is fully conscious and his cycles of sleep and wakefulness are preserved only through instrumental diagnostics. The picture becomes clear in particular after the encephalography. With the isolation syndrome, the encephalogram remains the same as in a healthy person, which is simply impossible with a true coma.

Analyzes and other methods of instrumental study of the brain, such as the computer and magnetic resonance tomogram of the head (CT and MRI of the brain), diffuse optical tomography, magnetoencephalography, etc., united by the same name "neuroimaging of the brain" are performed solely for the purpose of revealing the pathology that led to development of the syndrome of the locked person.

[29], [30], [31], [32]

Differential diagnosis

The purpose of differential diagnosis in this case is the delineation of coma and pseudocomas, as well as the determination of the causes of this condition.

[33], [34], [35], [36], [37], [38], [39], [40], [41], [42], [43]

Treatment of the syndrome of a locked person

Despite the fact that physicians have learned to distinguish the syndrome of a locked person from a coma, the therapy of a pathological condition is still at a low level. Few of the patients manage to oppose the disease. Most of the patients die within the first month of the onset of the disease.

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

Effective drugs that can lead a person out of this state and return to normal life, alas, have not yet been found. Therefore, the treatment of such patients reduces mainly to the fight against the cause of the development of the syndrome of the locked person (ie, with the pathology that caused the pseudocome condition) and the prevention of possible complications caused by prolonged immovable position of the patient (congestion in the lungs with the development of pneumonia, UTI due to lack of hygiene and etc.).

For the treatment and prevention of various complications, glucocorticosteroids, antihistamines, drugs that stimulate immunity and other drugs are used. Alternative treatment and homeopathy in this case are of little effect.

Physiotherapeutic treatment of patients with the syndrome of a locked person can include plasmaphoresis (with the use of immunoglobulins), exercise exercises to maintain the normal functioning of the joints and other procedures, electromyostimulation (functional neuromuscular stimulation to restore the work of certain muscle groups), magnetic stimulation of the motor cortex, methods of physical impact.

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 other muscle groups, except for the eyeballs).

In the case of incomplete and classical forms of the syndrome of the locked person, good results in restoring cognitive functions are given by exercises with a speech therapist (training the transmission of thoughts through the movement of eyeballs and blinking), watching TV programs, reading books to the sick. And people caring for such patients, it is more to communicate with them, having developed their system of codes. After all, patients with the syndrome of isolation are distinguished by good mental abilities and understanding of someone else's speech, which means they can be quickly taught by eye movement to answer questions and make their requests.

Currently, computerized systems have also been developed that allow patients with disabilities to communicate with others by computer, and develop their creative abilities by writing books.

Surgical treatment is performed either with respect to the pathology that caused the isolation syndrome, or for the restoration of physiologically conditioned functions of the body, such as breathing and nutrition. Sometimes, to help the patient to breathe, a tracheotomy is performed, and gastro- tomia is introduced into the body (a special probe is introduced into the gastric lumen, through which the frayed semi-liquid and liquid food is introduced, completely providing the body's nutritional needs).

The main emphasis in the case of limited movements in the patient is to care for him by relatives, friends or persons from the medical staff. A patient with a syndrome of a locked person requires great love, patience and attention to himself within 24 hours, because he can not call for help in case of deterioration, unable to independently service himself. But this is a living person, able to think and feel, and therefore live, even in such tough (and even cruel) conditions.