Cataplexy: a modern view of the problem

Cataplexy is a neurological disorder characterized by paroxysmal disturbances of motor activity.

Expressed as a short-term( partial or complete) muscle atony.

Specificity of the disease

Muscular weakness, from subtle tension to complete blockade of movements, slurred speech, visual impairment is a typical picture of an attack. The average duration varies from a few seconds to 3 minutes, the frequency - both once and daily.

A person is not able to interrupt his current on his own, but is in full consciousness.

Clinical neurology specialists distinguish several forms of cataplexy activity, differing in intensity and severity:

1. Total( total) .It is accompanied by a complete weakening of the muscular skeleton, resulting in the risk of falling and injury.
2. Partial( partial) .Affected individual muscle groups, there are involuntary movements - head overturning, knee bending, lowering of the jaw, and limp drooping of the hands.
3. Awakenings of the .It manifests itself in unsuccessful attempts to perform an action after a dream, mostly night.

Both adults and children with teenagers are exposed to attacks. The disease is successfully treatable, does not cause defects in mental development, eventually regresses.

Pathogenesis of the development of the

disorder Cataplexy syndrome is caused by disorders in the functioning of the central nervous system of a person. The most important integrative device of the activating function of the brain is the aggregate of neurons of reticular pharmacy.

It provides muscular control, balance of states of wakefulness and sleep, processing of information signals.

There are 2 hypotheses about the appearance of cataplexic disorders:

1. The destruction or insufficient number of cells of the posterior and lateral parts of the hypothalamus , which produce hypocretin, which is necessary for the physiological maintenance of wakefulness, alertness and excitability. Such a deficit starts the process of inhibition in the transmission of nerve impulses, as a consequence - there is an instantaneous weakening of the muscle tone.
2. Damage to the brain receptors of , sensitive to this neuropeptide.

Scientists do not agree on the true nature of the onset of this disorder, but all of them are similar in opinion - this pathology belongs to the group of diencephalic, pituitary diseases of dyshormonal and autoimmune characters.

Where is the reason behind it?

The main factor provoking the development of the disorder is the presence of a narcolepsy syndrome in the patient - a pathological disturbance of the sleep regime, expressed in daytime drowsiness and sudden falling asleep.

The European Association has sounded data according to which this indicator accounts for about 80% of all episodes.

Based on the results of studies by Dr. Guilleminaulta( professor at Stanford University) and co-authors, of 10 children diagnosed with narcolepsy, 10 have cataplexy symptoms.

The emotional shock is the strongest catalyst, both positive( more often) and negative - feelings of uncontrollable laughter, crying, admiration, surprise, horror or joy. And the higher the degree of excitement, the heavier the consequences of an attack.

Patients with concomitant diseases of the brain, endocrine-vegetative disorders, amyema, lymphocytosis, obesity, hypotension, and craniocerebral trauma are at risk.

Severe physical exertion and affective states are also on the list of irritating factors.

Symptoms of the disease

The main indicator of the presence of cataplexic disorder is a sudden, as a rule, non-prolonged immobilization of the whole body or parts of it.

Along with this, there are:

• numbness of facial muscles;
• redness / pallor of the skin;
• slowing of the heart rate( bradycardia);
• increased sweating;
• general numbness;
• impossibility of holding the body in an upright position;
• defocus of vision;
• decreased sensitivity of mucous membranes;
• speech impairment.

Diagnosis and its role in the prevention of risks

Cataplexy patients daily endanger themselves and others. The attack can overtake at critical times of the responsible production, driving, eating.

This explains the importance of the timely installation of a correct diagnosis, which is the competence of a certified neurologist.

Based on the collected information on the transferred diseases and injuries, available complaints, medications taken, the scheme of the further examination is determined, which includes the procedures:

• of electroencephalography ;
• magnetic resonance and computed tomography ;
• sleep monitoring by hardware - polysomnography ( parallel recording of respiratory function, muscle contraction, palpitation);
• MTLS - testing ( assessment of the level of daytime drowsiness, quantity and speed of falling asleep);
• questionnaire on the Epworth scale .

Complex diagnosis is necessary to confirm( exclude) the relationship with concomitant neurological pathologies - epileptic attacks, fainting, ischemic and drop-attacks, hyperkalemic paralysis and other disorders.

Principles of treatment

In the absence of risk of traumatic effects, drug therapy is not required. The patient is encouraged to adjust the image and lifestyle in accordance with the recommendations of the coolant:

• to avoid emotional, physical fatigue;
• not to abuse alcohol;
• adhere to the diet;
• not to take sedatives and hypnotics;
• eliminate irritating factors.

Otherwise, the patient is prescribed a set of therapeutic measures:

• therapy with tricyclic antidepressants, psychostimulants, selective inhibitors, restorative drugs;
• attending psychotherapy courses;
• autogenic training( self-hypnosis, self-learning, muscle relaxation).

In order to normalize and stabilize night sleep, physiotherapy techniques( hydrotherapy, encephalophonia, phototherapy) are used.

Research of the effectiveness of the development of American scientists - special nasal sprays based on hypocretin is being conducted today.

Recommendations

The patient needs to learn to control his consciousness, to anticipate situations that can cause emotional outbursts.

Cataplexy attacks pose a threat to the life and health of the patient, significantly affect the quality of his life, do not allow full exercise of professional duties.

If you have any symptoms, you should consult with specialists - a neurologist, a somnologist, which will allow you to start treatment on time and avoid the progression of the disease.