Cataplexy

Cataplexy (Levenfeld-Genneberg syndrome) is a state of sudden loss of muscle tone with complete preservation of consciousness. At a cataplexy the person is not capable to move in general, or the separate sites of its body appear to be immobilized. But the understanding of what is happening, hearing, sight, smell, that is, the perception of the environment, does not suffer in any way. The emergence of cataplexy science associates with a deficiency in the hypothalamus of a special substance of hypocretin (orexin). Usually cataplexy has a paroxysmal course, the duration of an attack on average is a few seconds or minutes. Most often, an attack of cataplexy is provoked by strong emotions. In 70-80% of cases, cataplexy is a symptom of another disease, narcolepsy. This article will introduce you to the basics of cataplexy.

Modern medicine more often considers cataplexy as one of the manifestations of narcolepsy (Jelino disease). In fact, only 70-80% of all cases of cataplexy are a symptom of narcolepsy, and the remaining 30% occur independently. If cataplexy occurs in the absence of possible causes (problems with the nervous system), then it is primary (true Levenfeld-Gennesberg syndrome). In those cases when the onset of cataplexy is associated with a transferred craniocerebral trauma, intoxication (drugs, alcohol, etc.), a tumor brain, infectious process (encephalitis), multiple sclerosis, surgical interventions on the brain, speak of secondary cataplexy.

Cataplexy can be:

• total (total) - when there is a loss of muscle tone of the whole body. Such cataplexy is accompanied by a fall;
• partial (partial) - tone lose individual muscle groups (muscles of the mouth, tongue, abdomen, neck, hands or feet). In such cases, a person loses the ability to move only the affected parts of the body (for example, can not close his mouth or move his hands);
• cataplexic status - when cataplexy attacks follow one another, almost without interruption.

Probable cause of cataplexy

For 15 years, a group of patients with cataplexy were monitored to find the cause of the disease, and in 2006 the results were published. It was found that the lightning-fast shutdown of muscle tonus during cataplexy is directly related to the hypothalamus (department of the brain), more precisely - with the secretion of a special substance called orexin (hypocretin). Orexin is responsible for the regulation of wakefulness, the processes of excitation in the human body. When cataplexy orexin is produced insufficiently or defective receptors, it perceives. The result is a massive suppression of spinal cord neurons that provide muscle tone. Why there is a shortage of orexin (or "break" its receptors) in the body, is not yet clear, but it is revealed a direct link between deficiency of orexin and a lack of histamine, dopamine, adrenaline in the nervous system.
These mediators also support the wakefulness and vigilance of the body. Further research in this field will certainly be able to establish the true cause of cataplexy, the answer is probably already close.

Symptoms

The main symptom of cataplexy is a sudden loss of muscle tone. It can be manifested:

• sagging the lower jaw (it's impossible for her to close her own person at the moment of an attack, only if she holds her hand by the jaw);
• tipping back the head or dangling the head down (violation of the tone of the neck muscles);
• dangling of hands along the trunk;
• underfoot feet (flexion in the knee joints), which is usually accompanied by a fall;
• "Mute" face, that is, the absence of any facial expression (impaired muscle tone of the face).

If cataplexy is total, then all the features described above are identified simultaneously. Almost always while this person falls and lies immobilized in an uncomfortable position until the end of the attack (if he does not help, of course).

In addition to an unforeseen decrease in muscle tone, a cataplectic attack may also exhibit the following symptoms:

• red face;
• loss of heart rate;
• copious sweating;
• inarticulate speech (if the muscle tone involved in speech production is disturbed);
• double vision in the eyes (due to a decrease in the tone of the eye muscles).

Distinguishing signs of an attack of cataplexy should be considered the absence of a violation of breathing and the preservation of consciousness. A person in a fit of cataplexy perceives the world around him (hears and sees, although it is possible to have a double vision), understands everything, but is not able to perform any movements of the affected muscles.

Quite characteristic is the provocation of an attack of cataplexy with strong emotions or sudden physical efforts. Emotions can be both positive (joy, surprise, laughter, orgasm, awe, etc.), and negative (anger, rage, fright and so on). By sudden physical effort means situations to which the body was not ready. For example, the unpredictable fall of an object on the head (the need to dodge lightning fast), an attempt to catch a suddenly thrown object and so on.

Cataplexy is more common in male subjects. Seizures occur more often between 10 am and 9 pm. The duration of the attack is very variable: from a few seconds to several minutes. In 93% of all cases of catalepsy, the attack lasts no more than 2 minutes, in 6% - up to 5 minutes and in 1% - more than 5 minutes. The disease "loves" young people: the first symptoms usually appear during puberty and have the tendency to fade after 40 years (that is, with age, the disease becomes less aggressive). Attacks do not lead to mental disability, but can cause injuries. People who suffer from cataplexy usually have sleep disorders (not necessarily by the patient himself, sometimes changes are detected only with polysomnography).

Diagnostics

The diagnosis of cataplexy is made by a neurologist. For this purpose, the collection of complaints, anamnesis of life and disease, inspection of the patient is made. Then additional methods of examination are appointed (polysomnography, electroencephalography, computed tomography or magnetic resonance imaging, a number of biochemical blood tests). A comprehensive examination of the patient is necessary to exclude other diseases that have a similar clinical picture with cataplexy (for example, transient ischemic attacks, epileptic seizures, syncope, hyperkalemic paralysis and many other diseases).

Treatment

Currently, cataplexy is a chronic incurable disease. The main approach to the treatment of this disease is symptomatic.

In cases when seizures occur very rarely, last for a short time and do not lead to socially significant negative points, it is possible to simply observe the neurologist without prescribing any therapy.

Of the medications for the elimination of attacks of cataplexy usually use antidepressants (tricyclic - Clomipramine, Imipramine;
selective serotonin reuptake inhibitors - sertraline, paroxetine, fluoxetine and others). Sometimes you need to take sedatives that calm the nervous system and reduce emotional fluctuations in the patient.

In America, the main drug for the treatment of cataplexy is Sodium oxybate or Sodium oxybutyrate (Xyrem). In Russia, it is customary to use this drug in anesthesiology for non-anesthetic anesthesia. Since Sodium oxybate leads to daytime drowsiness, its use is combined with stimulants (Modafinil, Alertek, Provigil). Usually sodium oxibate in combination with stimulants is used for cataplexy with narcolepsy. American scientists have also developed a special nasal spray containing orexin. So far, its effectiveness has been tested only on animals, but the results were quite encouraging. Perhaps, over time, the drug will be tested in humans.

As the main preventive measures for cataplexy, it is recommended to stop using coffee, alcoholic beverages, smoke, lead a healthy lifestyle, observe the regime of work and rest (a significant role is given to a sufficient amount of sleep).
Still it would be desirable to note, that the people, suffering a cataplexy, can not work with moving mechanisms, on altitude and so on, that is, in those conditions where a sudden fall can provoke an accident.

Thus, cataplexy is a disease of the nervous system, caused by a lack of special substance in the brain. Cataplexy is not distinguished by a special variety of symptoms: basically it manifests itself by attacks of sudden loss of muscle tone. By itself, the disease is not dangerous, it does not lead to any complications, it does not progress, but it can cause injuries received at the time of the fall. To date, cataplexy is an incurable disease, but research is being done in this direction. Meanwhile, antidepressants of different groups are the main measure of care for such patients.