Cataplexy

Cataplexy is the sudden disappearance of muscle tone caused by emotional stress or a state of affect, from a subtle weakening of the musculature of the face and neck to a total short-term atony and disabling the ability to hold the body in a certain position. In clinical neurology, cataplexy is considered to be one of the symptoms of such a disease of the nervous system as to rkolepsia (Gelino's disease).

[1], [2], [3], [4], [5], [6], [7]

Causes of the cataplexy

Before considering the most important causes of cataplexy, it should be borne in mind that, according to the European Federation of Neurological Societies (EFNS), narcolepsy and cataplexy, more precisely, cataplexy syndrome, is observed in an average of 70-80% of patients with diagnosed narcolepsy - increased drowsiness in daytime time. And this syndrome is not related to the pathology of the musculoskeletal system, but is directly related to the brain and the central nervous system. This is the primary cataplexy (or the Levenfeld-Genneberg syndrome).

Scientific studies of the last 15 years have shown that the periodic inhibition of the transmission of nerve impulses and the instantaneous decrease in muscle tone in this pathology is due to problems with the hypothalamus. The currently accepted version of the etiology of narcolepsy and cataplexy is the lack or destruction of hypothalamus cells that produce hypocretin (orexin), a neurotransmitter that regulates the processes of excitation and wakefulness. What exactly leads to the loss of brain cells producing this neuropeptide has not yet been established, but scientists agree that this is a genetically transmitted (modified form of the DQB1 0602 gene) pituitary pathology of an autoimmune nature.

According to the second version, cataplexy causes not a hypocretin deficiency, but a defect of receptors perceiving it. In general, the neurophysiological mechanism of the development of cataplexy remains unclear, although a certain correlation between the lack of this mediator of the nervous system and the decrease in the level of such important neurotransmitter hormones as histamine, dopamine and adrenaline is found. On this basis, this condition could be classified as diencephalic syndromes of a disgormonal nature.

Secondary cataplexy may occur as a result of damage to the hypothalamic region of the brain by tumor formations or metastases of breast, thyroid or lung cancer, due to congenital anomalies of the cerebral vascular system, multiple sclerosis, craniocerebral trauma, or infections.

[8], [9], [10], [11], [12]

Symptoms of the cataplexy

Typical symptoms of cataplexy are expressed in the form of atonic muscle attacks lasting from a few seconds to several minutes, during which a person - due to the disappearance of normal physiological muscle tension - the mandible is hanging down, the head is lowered or tilted, the legs bend at the knee joints "), Hands hang along the body. In most cases, when the cataplectic seizure is caught in a standing position, a person can not restrain himself and falls.

At the same time, the person does not lose consciousness, breathing does not stop, but the heartbeat may slow down; face turns red, throws into sweat; speech inarticulate (due to relaxation of mimic and chewing muscles of the face). Vision also deteriorates: double vision (diplopia) and problems with focusing. But hearing and understanding are not violated.

As specialists note, atony can be partial, affecting only the muscles of the face and neck. Symptoms most often occur in adolescence or 20-30 years, in children narcolepsy and cataplexy is diagnosed in less than 5% of cases, most of the patients are male.

In addition, in the history of patients with cataplexy syndrome there is a short-term loss of muscle tone right after a night's sleep - the so-called cataplexy of awakening, and there are disturbances in the normal structure of sleep in the form of anxiety, hallucinations when falling asleep and negative emotional coloring of dreams (often all nightmares are dreamed).

[13], [14]

Diagnostics of the cataplexy

The diagnosis of cataplexy is performed by a neurologist and consists of:

• examination of the patient, fixing his complaints and collecting anamnesis;
• finding out all the patient's illnesses and the medications taken by him;
• studying the features of night sleep with polysomnography;
• setting the level of biological need for sleep by conducting MSLT testing - a multiple latency test for sleep (no later than two hours after waking from a night's sleep);
• detection of pathological drowsiness (hypersomnia) by questioning according to the Euphord Sleepiness Scale;
• electroencephalography (EEG);
• CT or MRI of the brain.

When diagnosing, it is necessary to adhere strictly to the diagnostic criteria accepted in neurology, because cataplexy requires differentiation with respect to syncope, epileptic seizures, fall attacks, transient ischemic attacks, vertebral artery syndrome, periodic hyperkaliemic paralysis, acute intermittent porphyria, Thomsen's disease, syndromes Lambert-Eaton and Guillain-Barre, as well as iatrogenic intoxications of the body.

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

Treatment of the cataplexy

To date, the treatment of cataplexy - taking into account the clinical approach to the etiology of the disease - is carried out with drugs that control the manifestation of symptoms. But if cataplexy does not create significant problems for the patient, then no drug treatment is required.

Despite the lack of medical evidence of the effectiveness of antidepressants in the treatment of this pathology, the European recommendations of EFNS assign antidepressants the main position in the tactics of therapy of cataplexy. According to the appointment of neurologists, you should take Clomipramine - 10-20 mg per day. Also, selective inhibitors of serotonin and norepinephrine and drugs that inhibit serotonin reuptake in nerve endings (SSRIs) can be used. However, all of them, especially antidepressants, have a lot of negative side effects.

Specialists of the American Academy of Sleep Medicine recommend with cataplexy a drug based on the sodium salt of gamma-hydroxybutyric acid - sodium oxybate or sodium oxybutyrate (syrup or solution for ingestion). The main use of this drug is anesthesiology (for non-anional anesthesia), ophthalmology (to improve vision in glaucoma), and in neurology - as a sedative to improve the quality of night sleep. Standard dosage: one tablespoon of syrup or 5% solution before bedtime. The drug undergoes complete biochemical transformation with metabolites in the form of water and carbon dioxide, however a prolonged intake can cause an increased level of potassium in the body.

Because Sodium oxybate can lead to daytime drowsiness, simultaneously stimulant medications are prescribed - in particular, Modafinil (other trade names - Modalert Alertec, Provigil) based on the active substance 2- (diphenylmethyl) -sulfinyl acetamide. The drug is taken once a day (in the morning hours) with diagnosed narcolepsy with frequent attacks of cataplexy. This remedy is contraindicated for patients under 18 years of age, with psychosis, depression, suicidal thoughts or mania; should monitor blood pressure and heart rate.

Prevention

Prevention of cataplexy is, rather, a general health-improving character, since the disease is incurable. Doctors advise to give up coffee, alcohol and alcohol-containing drinks, quit smoking, as well as engage in physical education, eat well and not overwork.

[20], [21], [22], [23], [24], [25]

Forecast

Forecast cataplexy: with inferior sleep at night, memory and concentration of attention can be significantly impaired; There can be difficulties (and dangerous situations) when driving or managing complex mechanisms. In addition, with an unexpected fall during atonic muscular attacks, a person can get a serious injury, in the first place, craniocerebral.

As a rule, the symptoms of narcolepsy and cataplexy are manifested for life. However, sometimes - with the passage of time or as a result of therapy - cataplexy disappears.