Paroxysmal phenomena. Non-epileptic paroxysmal disorders of consciousness

V.S. Myakotnykh
(study guide)

There are several variants of predominantly non-epileptic paroxysmal disorders that require special consideration and are quite common in the clinic of nervous diseases. These conditions are subdivided into several of the most common variants, the clinical description of which is difficult to find in any one study guide, monographs. Basically, they can be divided into:

1. Dystonia or muscle dystonic syndromes
2. Myoclonic syndromes and a number of other hyperkinetic conditions
3. Headaches
4. Vegetative disorders

Often, the clinical manifestation of these pathological conditions is associated with neurological nosology that occurs in young (childhood, adolescence, adolescence) age. But, as practice shows, in adults and even in the elderly, the described syndromes very often either debut or progress, the appearance and severity of which is associated with age-related cerebral disorders, acute and chronic disorders of cerebral circulation. It should be noted that many non-epileptic paroxysmal conditions can also be a consequence of long-term use various medications used for the treatment of circulatory failure, some mental disorders of the elderly and senile age, parkinsonism, etc. Therefore, in this publication we do not seek to present the selected pathological conditions in the form of syndromes that occur in a certain nosology, and even more so in the form of separate nosological units. Let us dwell on the above and the most common variants of non-epileptic seizures.

I. Dystonia.

Dystonia is manifested by persistent or recurrent muscle spasms leading to "dystonic" postures. In this case, of course, we are not talking about the well-known concepts of vegetative-vascular or neurocirculatory dystonia, which are considered completely separately.

Epidemiology. Dystonia is a rare disease: the incidence of its various forms is 300-400 patients per 1 million people (0.03%). Generalized dystonia can be inherited dominantly and recessively. The genetic mechanisms of focal dystonias are unknown, although it has been noted that about 2% of focal dystonias are inherited, and in one third of patients with blepharospasm and spastic torticollis, other families were noted. movement disorders (tics, tremors, etc.).

The pathogenetic mechanisms of dystonia are still not disclosed. Dystonia does not have a clear morphological substrate in the brain and is caused by subcellular and neurodynamic disorders in certain brain systems. The peripheral motor apparatus, the pyramidal pathway, and the proprioceptive servo mechanism (stretch reflex) are intact in dystonia. Disorders in the functional state of interneurons of the brain stem and spinal cord were revealed.

Also, the biochemical defect underlying dystonia is almost unknown. Empirically, it can be assumed that the cholinergic, dopaminergic and GABAergic systems of the brain are involved. But the low efficiency of treatment of dystonia in general suggests the existence of some other, still unknown to us, biochemical disorders underlying the disease. Most likely, the trigger that triggers dystonia is the biochemical systems at the level of the oral part of the brain stem and its connections with subcortical extrapyramidal formations (mainly the shell, optic tubercle and others).

Depending on the distribution of hyperkinesis in muscle groups and the degree of generalization, 5 forms of dystonias, dystonic syndromes are distinguished:

1. focal dystonia,
2. segmental dystonia,
3. hemidystonia,
4. generalized and
5. multifocal dystonia.

Focal dystonia is characterized by the involvement of muscles in any one part of the body ("writing spasm", "blepharospasm", etc.).

Segmental dystonia is manifested by the involvement of two adjacent parts of the body (the circular muscle of the eye and the circular muscle of the mouth; neck and arm; pelvic girdle and legs, etc.).

With hemidystonia, muscle involvement of one half of the body (arms and legs most often) is observed. Such dystonia is often symptomatic and directs the doctor to a diagnostic search for the primary lesion of the nervous system.

Generalized dystonia is characterized by muscle involvement of the entire body.

Multifocal dystonia affects two or more non-adjacent areas of the body (for example, blepharospasm and dystonia of the foot; torticollis and writer's spasm, etc.).

Focal dystonias are much more common than generalized ones and have six main and relatively independent forms:

• blepharospasm,
• oromandibular dystonia,
• spastic dysphonia,
• spastic torticollis,
• writing spasm,
• dystonia of the foot.

Generalized dystonia usually begins with focal dystonic disorders, its onset often occurs in childhood, adolescence. The older focal dystonia begins, the less likely its subsequent generalization is.

Postures and syndromes characteristic of dystonia are presented in Table 1.

Body area	Dystonic pose	Dystonic syndrome
Face	Squinting eyes	Blepharospasm
	Abduction of the eyeballs upward and in other directions	Oculogynous spasm
	Covering or curvature of the mouth, grimace of a smile, curvature of the lips, cheeks, tongue	Oromandibular dystonia
	Jaw clenching	Lockjaw
Neck	Turning the head to the side, tilting it to the shoulder, forward, backward	Torticollis latero-, ante-, retrocollis
Torso	Curvature to the side	Scoliosis, tortipelvis
	Overextension back	Hyperlordosis ("peacock" pose)
	Forward tilt	Bow Pose
	Tension, twisting of the abdominal muscles	"Belly dance"
Proximal extremities	Pronation of the shoulder, forearm, hip with the limb inserted back	Torso spasm
Distal extremities	Flexion at the wrist with extension of the fingers	Athetoid
	Plantar flexion of the foot with dorsiflexion of the big toe	"Ballerina's foot"

But the division of dystonias into focal and generalized reflects only the syndromic principle of classification. The wording of the diagnosis should also include the nosological principle - the name of the disease. The most complete nosological classification of dystonia is presented in the international classification of extrapyramidal disorders (1982), as well as in the generalizing article by McGuire (1988). In these classifications, primary and secondary forms of dystonia are distinguished. In primary forms of dystonia, this is the only neurological manifestation. They can be both hereditary and sporadic. Secondary dystonia occurs in known and diagnosed diseases of the nervous system and is usually accompanied by other neurological disorders. In children, this occurs against the background of infantile cerebral palsy (cerebral palsy), Wilson's disease, storage diseases; in adults, including the elderly - as a result of cerebral infarction, tumors, degenerative processes, the use of drugs, etc.

The defining characteristic of dystonia is the formation of typical dystonic postures, many of which have their own, sometimes figurative names. The most typical dystonic postures and syndromes are shown in table 1 (cited by OR Orlova).

Since any area of \u200b\u200bthe body can be involved in dystonic hyperkinesis, the clinical pattern of dystonic syndrome in each individual patient depends on the distribution and combination of dystonic postures in different areas of the body. This principle (distribution of dystonic syndromes in different regions of the body) is the basis for the modern convenient classification of dystonia (Marsden, 1987), given above.

It would be advisable to list the clinical features common to all focal dystonias.

Dystonic posture. With blepharospasm, there is closure, shutting the eyes, or frequent blinking. Oromandibular dystonia is characterized by dystonic postures in the perioral region, tongue, trismus. Spastic torticollis is manifested by rotation or tilting of the head. When writing spasm, the posture of the hand resembles the "hand of an obstetrician." Pathological postures that occur in the swallowing and voice-forming muscles during spastic dysphagia and dysphonia can be considered with a special ENT examination.

Dystonia of action. In patients, the performance of some of the actions carried out by the muscles that form the dystonic posture is selectively disrupted. With blepharospasm, the action suffers - keeping the eyes open, with spastic torticollis - keeping the head in a straight position, with writing spasm, writing is impaired, with oromandibular dystonia, speech and food intake may be impaired. In the case of spastic dysphagia and dysphonia, swallowing and voice are impaired. With outpatient foot spasm, normal walking is disturbed. At the same time, other actions performed by the same muscle group are completely intact. For example, a patient with writing spasm can perfectly use a “sore” hand for all everyday activities.

The dependence and variability of dystonia is diminished by the position of the body. As a rule, all manifestations of dystonia decrease or disappear when the patient is lying down, and intensify when standing.

The influence of the patient's emotional and functional state on the severity of dystonia: decrease or disappearance of dystonia in sleep, in the morning after waking up, after taking alcohol, in a state of hypnosis, the possibility of short-term volitional control, increased dystonia during stress, overwork. This feature is very clearly manifested at a doctor's appointment, when during a 10-20 minute conversation all manifestations of dystonia may disappear, but as soon as the patient has left the doctor's office, they resume with renewed vigor. This feature can cause the doctor to mistrust the patient, suspicion of simulation.

Corrective gestures are special techniques that the patient uses for the short-term elimination or reduction of dystonic hyperkinesis. As a rule, this is either touching with a hand to any point in the area of \u200b\u200binterest, or imitation of some kind of manipulation in this area. For example, patients with spastic torticollis, to reduce hyperkinesis, touch their cheeks or any other point on the head with their hand, or imitate adjusting glasses, hairstyles, ties, patients with blepharospasm - rub the bridge of their nose, take off and put on glasses, with oromandibular dystonia, chewing gum, sucking helps for a short time sweets, as well as the presence in the mouth of a stick, match, cigarette or any other object. When writing spasm, the difficulty of writing can be temporarily reduced by putting a healthy hand over the "sick" hand.

Paradoxical kinesis is a short-term decrease or elimination of hyperkinesis in the nature of the action (change in the locomotor stereotype). For example, patients with writing spasm easily write with chalk on a blackboard, head rotation in patients with spastic torticollis may decrease or disappear when running or driving a car, in patients with spastic dysphonia, the voice "erupts" when singing or screaming, and in patients with outpatient foot spasm of its pathological posture does not occur when walking on tiptoe or backwards.

Remissions are fairly common in focal dystonias. More often than in other forms, they are observed in patients with spastic torticollis (in 20-30%), when the symptoms can spontaneously disappear completely for months and years, even after several years from the onset of the disease. With exacerbation of spastic torticollis, the phenomenon of rotation inversion is sometimes observed - a change in the direction of a violent turn of the head. Less characteristic remissions for writing spasm and other focal dystonia, however, with writing spasm, the phenomenon of inversion is also observed - the transition of writing spasm to the other hand.

The combination of focal forms of dystonia and the transition of some forms to others. When two or more focal forms are combined, as a rule, manifestations of one form prevail, while others may be subclinical, and the symptoms of the erased form often appear before the symptoms of the clinically pronounced form. Example: A few years before the onset of spastic torticollis, about a third of patients have difficulty writing or frequent blinking, but writing spasm or blepharospasm is diagnosed after the onset of torticollis symptoms. There are cases when, after remission, one focal form is replaced by another, and one patient may have several such episodes. The classic is the combination of blepharospasm and oromandibular dystonia. In this case, blepharospasm usually appears first (the first stage of facial paraspasm) and then oromandibular dystonia (the second stage of facial paraspasm) joins it.

The dynamism of dystonia is most likely associated not with a specific anatomical substrate, which has not yet been found, but with a violation of the interaction between the structures of the basal ganglia, brainstem, thalamus, limbic-reticular complex, motor cortex due to impaired metabolism of neurotransmitters in these structures, which constitutes organic neurodynamic substrate of dystonia (Orlova O.R., 1989, 1997, 2001).

Diagnostic criteria of Marsden and Harrison (1975) for the diagnosis of idiopathic dystonia:

1. the presence of dystonic movements or postures;
2. normal childbirth and early development;
3. absence of diseases or taking medications that could cause dystonia;
4. absence of paresis, oculomotor, atactic, sensory, intellectual disorders and epilepsy;
5. normal results of laboratory tests (copper exchange, fundus, evoked potentials, electroencephalography, computed and magnetic resonance imaging).

Spastic torticollis - the most common focal form of dystonia. The essence of dystonic syndrome with it is a violation of keeping the head in a straight position, which is manifested by rotation or tilt of the head. Spastic torticollis usually begins at the age of 30-40 years, 1.5 times more often observed in women, almost never generalizes, can be combined with writer's spasm, blepharospasm and other focal dystonias. A third of patients are in remission.

Writing spasm. This form of dystonia occurs at the age of 20-30, equally often in men and women; among patients, people of "writing" professions (doctors, teachers, lawyers, journalists) and musicians predominate. Writing spasm and its analogs (professional dystonia) often develop against the background of previous hand injuries or other pathology of the neuromotor apparatus. Writing spasm remissions are rare and generally short-lived.

Blepharospasm and oromandibular dystonia. These forms usually begin after the age of 45. As a rule, symptoms of oromandibular dystonia appear several years after the onset of blepharospasm.

Dystonia deserves special attention, manifested by sudden attacks of involuntary movements and pathological postures, which are never accompanied by impaired consciousness and are often mistakenly regarded as hysterical or epileptic seizures. In some patients, seizures occur spontaneously, in others they are provoked by unprepared movements (kinotogenic or kinesigenic and non-kinetogenic or non-kinesigenic forms). Typical paroxysms: choreoathetosis, tonic or dystonic movements (generalized or by hemitype), sometimes leading to the patient falling down if he does not have time to grab onto any object. The attack lasts from several seconds to several minutes. Paroxysmal dystonia is either idiopathic (including familial) or symptomatic. The latter option is described for three diseases: cerebral palsy, multiple sclerosis and hypoparathyroidism. The drugs of choice for treatment are clonazepam, carbamazepine, and diphenin. The effect of the treatment is high.

There is also a special form of dystonia that is sensitive to the treatment of L-DOPA (Segawa's disease). It responds very well to treatment with dopamine-containing drugs, and this is perhaps its main differential diagnostic criterion.

Treatment of dystonia. It is generally known that there is no specific treatment for dystonia. This is due to the fact that neurochemical disorders in this disease are ambiguous, depend on the initial state of neurochemical systems and transform as the disease progresses. The most universal are GABAergic drugs (clonazepam and baclofen), however, previous treatment with drugs from other groups may reduce the effect of GABAergic therapy.

Treatment for dystonia is predominantly symptomatic. The therapeutic effect is rarely complete, more often only a relative regression of dystonic manifestations is achieved. But this is also achieved at the cost of long-term efforts to select drugs and their optimal doses. In addition, about 10% of dystonia are characterized by spontaneous remissions, in the presence of which it is difficult to talk about evaluating the effectiveness of certain drugs.

Traditionally used dopamine agonists and antagonists, anticholinergics, GABAergic and others medicines... Dopamine agonists (nakom, madopar, lisuride, midantan) and antagonists (haloperidol, pimozide, etopyrazine, azaleptin, tiapride, etc.) are effective in an equally low percentage of cases. Anticholinergics give relief to almost every second patient. The most commonly used are cyclodol, parkopan, artan (trihexyphenidil), but a dose of 2 mg in 1 tablet is rarely effective. Recently, 5 mg parkopan has appeared, but here the effect is often achieved at subtoxic doses. The use of cyclodol is described in daily doses even over 100 mg. But at the same time, side effects are very likely, especially pronounced in patients of older age groups.

Among anticholinergics, tremblex, a central anticholinergic of prolonged action, is more effective. The relief of dystonic manifestations is sometimes achieved in about 50 - 80 minutes after one injection (2 ml) of the drug. Side effects - dry mouth, numbness and a feeling of lining of the tongue and throat, dizziness, drunkenness, hypersomnia. This often causes the patient to refuse treatment with tremblex. There is also a drop in the effectiveness of the drug, sometimes literally from injection to injection. Glaucoma is also a contraindication, especially in the treatment of the elderly.

In the treatment of dystonia, lithium salts (lithium carbonate) and clonidine (gemiton, clonidine) are also used. Only a small proportion of patients respond well to treatment, but they need to be identified.

The vast majority of patients tolerate benzodiazepines well, especially clonazepam (antelepsin). But, unfortunately, we do not yet have an ampouled form of the drug. Clonazepam is effective in all types of diseases with the exception of generalized idiopathic torsion dystonia, where the effect is only subjective and can be explained by the psychotropic effect of the drug. Doses of clonazepam are from 3 to 6 to 8 mg per day, sometimes higher.

Blepharospasm, facial paraspasm (Bruegel's syndrome), and other cranial dystonia also respond well to clonazepam.

Among the drugs that have a relaxing effect in muscle spasticity, I would like to highlight the well-known, but until now undeservedly little used for muscular dystonia mydocalm (tolperisone).

Muscle spasticity can be considered as a pathological state of equilibrium that changes rapidly under the influence of various factors (fever, cold, warmth, time of day, pain), therefore it is difficult to develop a medication that, due to a flexible dosage, would reduce the pathologically increased tone only to the desired level. And here tolperisone has, perhaps, the mildest effect, without crossing the "border of what is permitted."

Among the pharmacodynamic properties of tolperisone, it should be noted: the central muscle relaxant effect and an increase in peripheral blood flow independent of it.

Localization of the muscle relaxant action of the drug is established in the following morphofunctional structures:

• in peripheral nerves;
• in the spinal cord;
• in the reticular formation.

Due to the membrane-stabilizing, local anesthetic effect, which manifests itself in the brain stem, in the spinal cord and in peripheral nerves (both motor and sensory), mydocalm prevents the emergence and conduction of an action potential in "overstimulated" neurons and thereby reduces the pathologically increased muscle tone. Depending on the dose, it inhibits nociceptive and non-nociceptive mono- and polysynaptic reflexes in the spinal cord (flexion, direct and cross-extensor), inhibits mono- and polysynaptic reflexes at the level of the spinal roots, and also inhibits the conduction of excitation along the reticulospinal activating and blocking pathways.

The proof of the direct action of mydocalm on the brain stem is the blocking effect on tonic chewing reflexes that occur during periodontal stimulation. This reflex arc includes intermediate neurons in the brain stem. Direct action at the level of the brain stem is also evidenced by the effect of reducing the latent time of nystagmus induced by rotation.

Tolperisone significantly, depending on the dose, reduces the rigidity caused by overactive gamma-motor neurons after intercollicular transsection in the midbrain.

In the event of ischemic rigidity (in this case, the cause of rigidity is the excitement arising in alpha-motor neurons), tolperisone reduced its severity.

Large doses of tolperisone block the experimental seizures of seizures caused by such provoking agents as strychnine, electroshock, pentylenetetrazole.

The drug has no direct effect on the neuromuscular junction.

It is assumed that tolperisone has a weak atropine-like M-anticholinergic and slightly pronounced -adrenergic blocking effects.

Pharmacological studies carried out on cats, rats, rabbits and dogs have shown that only with an intravenous bolus of a high dose of tolperisone can a temporary sharp decrease in blood pressure occur. A more prolonged slight decrease in blood pressure is observed with the use of large doses of the drug (5-10 mg / kg).

In a study of dogs with bradycardia, tolperisone slightly increased the heart rate due to increased vagal tone.

Tolperisone selectively and significantly increases femoral blood flow in dogs while decreasing mesenteric blood flow. Subsequently, when the experiment was repeated by different methods on a large number of animals, it was found that this effect was due to a direct peripheral vasodilator effect.

After intravenous administration tolperisone increased lymph circulation.

The drug does not have a noticeable effect on the ECG picture.

All of the above turns out to be positive when prescribing mydocalm in elderly and even elderly patients suffering from various disorders of the cardiovascular system.

II. Myoclonic syndromes.

Myoclonus is a short jerky jerk of a muscle, analogous to its contraction in response to a single electrical stimulation of the corresponding nerve. Myoclonus can be limited to a single (or separate) muscle, or it can involve many muscle groups up to complete generalization. Myoclonic jerks (jerks) can be synchronous or asynchronous, for the most part they are arrhythmic and may or may not be accompanied by movement in the joint. Their severity varies from a barely noticeable contraction to a sharp start, leading to a fall. Myoclonus tends to repeat itself in the same muscles. There are spontaneous and reflex myoclonus provoked by sensory stimuli of various modalities. There are myoclonus triggered by voluntary movement (action and intentional myoclonus). Known myoclonus, dependent and not dependent on the cycle "sleep - wakefulness".

The pathophysiological and biochemical mechanisms of myoclonus are not well understood. At the place of generation of myoclonic discharges in the nervous system, 4 types of myoclonus are distinguished:

• cortical;
• stem (subcortical, reticular);
• spinal;
• peripheral.

The first two forms (cortical and stem) have the greatest clinical significance; they are more common than the others. The presented classification is a modification of the old division of myoclonus into pyramidal, extrapyramidal and segmental forms.

The involvement of serotonergic mechanisms in the pathogenesis of myoclonus is assumed. Among patients, there are even subgroups that are amenable to successful treatment by directly opposite means: some patients respond to agonists, the other to serotonin antagonists.

Since a large number of diseases, nosological units can be accompanied by myoclonic hyperkinesis, several classifications of myoclonus according to the etiological principle have been proposed. Marsden's classification (1987) identifies 4 groups of myoclonus:

• physiological myoclonus;
• essential myoclonus;
• epileptic myoclonus;
• symptomatic myoclonus.

Examples of physiological myoclonus are myoclonus of falling asleep and awakening, myoclonus of fright, and some myoclonus in the form of hiccups. They usually do not require special treatment.

Essential myoclonus is familial as well as sporadic myoclonus, the so-called nocturnal myoclonus. Manifested in the phase of slow sleep in patients with chronic insomnia. Amenable to therapy with clonozepam, valproate, baclofen when using small doses (one tablet at night). Familial and sporadic myoclonus is a rare disease called essential myoclonus or Friedreich's multiple paramyoclonus. The disease debuts in the first or second decade of life and is not accompanied by other neurological, mental and electroencephalographic disorders. Clinical manifestations include irregular, arrhythmic, and asynchronous twitching and flinching with a generalized distribution of myoclonus. Treatment is ineffective. Clonazepam and valproate are used.

Epileptic myoclonus is myoclonus in the picture of an epileptic seizure, where they sometimes become one of the leading manifestations. There is a separate form of epilepsy - myoclonus-epilepsy, considered as even a hereditary disease that manifests itself in childhood.

Symptomatic myoclonus, most likely for the elderly and senile age, is observed in a number of metabolic disorders, such as renal, hepatic or respiratory failure, alcohol intoxication, withdrawal of certain drugs, as well as in diseases occurring with structural damage to the brain (without epileptic seizures), such as epidemic encephalitis, Creutzfeldt-Jakob disease, subacute sclerosing leukoencephalitis, postanoxic brain damage. The list of symptomatic myoclonias can be significantly expanded to include storage diseases (including Laforte's little body disease, sialidosis), paraneoplastic syndromes, toxic, including alcoholic, encephalopathy, with focal damage to the nervous system (angioma, ischemic or traumatic defect, stereotactic thalamotonia) , as well as myoclonus as a side non-obligatory symptom of other diseases (lipidosis, leukodystrophy, tuberous sclerosis, spinocerebellar degeneration, Wilson-Konovalov disease, myoclonic dystonia, Alzheimer's disease, progressive supranuclear palsy, Whipple's disease). Progressive myoclonus epilepsy can, in principle, also be attributed to symptomatic variants of myoclonus (based on epilepsy). The nosological independence of Ramsey-Hunt cerebellar myoclonic dyssynergy is also disputed. Remains in use only Ramsey-Hunt syndrome, which is equated as a synonym for myoclonus-epilepsy syndrome, Unferricht-Lundborg disease ("Baltic myoclonus", progressive myoclonus epilepsy). It seems to us necessary to dwell on the description of this pathology presented in the work of the Italian authors C.A. Tassinari et al. (1994).

Unferricht-Lundborg disease is a form of progressive myoclonus epilepsy. This disease was better known in Finland traditionally as Baltic myoclonus. In recent years, an identical disease has been described in populations of southern Europe - "Mediterranean myoclonus", or "Ramsey Hunt syndrome". In both populations, the disease has the same clinical and neurophysiological features: onset at 6-18 years of age, the appearance of active myoclonus, rare generalized seizures, mild symptoms of cerebellar failure, the absence of severe dementia, slow progression; EEG reveals normal bioelectrical activity and generalized fast wave activity of the "peak" and "polypeak" type. The conducted molecular genetic study showed the genetic unity of the disease in both populations: the localization of the defective gene on chromosome 22q22.3 was determined. However, in 3 out of 6 Italian families, the disease had features of atypicality - more rapid progression with dementia, the presence of occipital spikes on the EEG, which makes it closer to Lafor's disease. In this regard, it is possible that the “Mediterranean myoclonus” is a heterogeneous syndrome.

The diagnostic criteria for Unferricht-Lunborg disease are highlighted:

1. onset between 6 and 15, less often 18 years;
2. tonic-clonic seizures;
3. myoclonus;
4. EEG paroxysms in the form of spikes or polyspike-wave complexes with a frequency of 3 - 5 per second;
5. progressive course.

Some clinical forms of myoclonus:

Posthypoxic encephalopathy, in which the main manifestations are intentional and action myoclonus (Lanze-Adams syndrome), sometimes in combination with dysarthria, tremor and ataxia.

Myoclonus of the soft palate (bicycle-palatine myoclonus - nystagmus of the soft palate, myorrhythmia) - usually rhythmic, 2-3 per second, contractions of the soft palate, often in combination with hyperkinesis in the tongue almost indistinguishable from tremor, lower jaw, larynx, diaphragm and in the distal parts of the hands (classical myorhythmia, or "skeletal myoclonus", as defined by the old authors); myorhythmia disappears during sleep, it can be either idiopathic or symptomatic (tumors in the pons and medulla oblongata, encephalomyelitis, trauma), sometimes ocular myoclonus of the "rocking" type joins. It is suppressed not only by clonazepam, like most myoclonus, but also by finlepsin (tegretol, stazepine, mazepine, carbamazepine).

Spinal (segmental) myoclonus: rhythmic, from 1 - 2 per minute to 10 per second; independent of external stimuli. The reasons lie in local damage to the spinal cord (myelitis, tumor, trauma, degeneration).

Opsoclonus (dancing eye syndrome) is a fast, jerky, chaotic movement of the eyeballs. Increased hyperkinesis can sometimes occur explosively. Continues during sleep and even worse on awakening. Opsoclonus is often mistaken for nystagmus, which is always characterized by the presence of two successively advancing phases - slow and fast. Opsoclonus indicates an organic lesion of the cerebellar-stem connections in tumors of the brain stem and cerebellum, paraneoplastic syndromes, hemorrhages, severe trauma, metabolic and toxic encephalopathies in the final stage, multiple sclerosis and in some other conditions. Viral encephalitis and meningoencephalitis are often the "culprits" of opsoclonus. In children and people over 40, the likelihood of neuroblastoma is high. Treatment is carried out with adrenocorticotropic hormone, corticosteroids, obzidan, benzodiazepine derivatives.

Myokymia of the superior oblique muscle of the eye ("unilateral rotator nystagmus"); patients themselves feel characteristic molecular oscillations ("objects jumping up and down", "wobbling eyes", etc.) and torsion diplopia. The course is benign. There is a good therapeutic effect from finlepsin.

Hyperexlexia and Leaping Frenchman of Maine. Hyperexlexia - pathologically increased involuntary startle, sometimes leading to the fall of the patient, arising in response to unexpected tactile, light or sound stimuli. Sometimes it is an independent hereditary disease, and sometimes it is secondary, like the syndrome in diseases of Little, Creutzfeldt-Jakob, vascular lesions of the brain. With the syndrome of the "jumping Frenchman from Maine", the frequency of jumping paroxysms reaches 100 - 120 times a day. Many are accompanied by falls and bruises, but without loss of consciousness. Helps clonozepam.

Hiccups are myoclonic contractions of the diaphragm and respiratory muscles. May be physiological (after a rich meal), a symptom in diseases of the gastrointestinal tract, organs chest, with irritation of the phrenic nerve, with damage to the brain stem or upper cervical segments of the spinal cord. Hiccups can be both toxicogenic and psychogenic. Treatment is carried out with antipsychotics, antiemetics (cerucal, for example), clonazepam, finlepsin, psycho and physiotherapy, even transection of the phrenic nerve.

III. Other hyperkinetic syndromes.

The described syndromes include, first of all, episodes of tremors and muscle cramps. In terms of the clarity and "picture" of their clinical manifestations, both tremor and some convulsions to some extent occupy an intermediate place between muscular dystonia and myoclonus, often including elements of both.

Muscle cramps denote involuntary and painful contractions that occur spontaneously or after exercise. A prerequisite for the development of muscle cramps is the absence of a regulatory response from antagonistic muscles. With the tension of the antagonist muscles, reciprocal blocking of seizures occurs, but such blocking is also possible with the use of cutaneous efferent endings.

Histologically, in the painfully contracting muscles, a large number of muscle fibers depleted in glycogen and single myolysis are found; this shows that cramps do not go away without leaving a trace, but affect the structure of the muscles. Finds of this kind are partially comparable with the "syndrome of prolonged activity of muscle fibers" described by H. Isaacs and with other, less common syndromes, including those that develop with repeated irritation of peripheral nerves.

Often, muscle cramps and fascicular twitching are the first symptoms of general somatic disorders: anomalies in electrolyte metabolism and metabolic disorders, including endocrine diseases, chronic inflammatory processes, and malignant tumors. Other reasons may be drug abuse (for example, nicotine and caffeine), various kinds of toxicosis, including medication. Hereditary nocturnal muscle cramps have also been described.

Diseases of the peripheral nerves and central nervous system can lead to muscle cramps. Convulsions can also occur when water-electrolyte metabolism is disturbed. Compression of muscle fibers due to edema plays a significant role in the origin of convulsive pain. The pain disappears immediately when the muscle fascia is cut. A similar mechanism can take place in ischemic convulsions of the calf muscles, a predominantly sedentary lifestyle of most people, in which practically no muscle is involved. In peoples for whom it is common to squat, when the muscles are under relatively heavy load, cramps in the leg and other muscles are rare.

Certain medications are able to induce muscle cramps or increase convulsive readiness. Any attempt to isolate certain groups of drugs, especially those affecting the metabolism in the muscles, affecting electrolytes or the function of sarcolemma and thereby predisposing to the development of muscle cramps, was practically unsuccessful, since the effect of medications is usually very multifaceted.

Muscle cramps with tetanus are characteristic. But it must be remembered that in this case, muscle cramps are often complicated by changes in tendons up to calcification (shoulder, elbow and hip joints are most susceptible to this).
Among the endocrine diseases that can occur with characteristic muscle cramps, hypothyroidism should be mentioned.

Increased excitability and rigidity of all muscles of the neck, upper limbs and the patient's faces were described by H. Mertens and K. Ricker as “spindle myotonia”. The picture of the disease is in many ways similar to the stiff-man syndrome, which occurs sporadically in adults, described by F. Moersch and H. Woltman.

Very interesting is the Schwartz-Jampel syndrome, or myotonic chondrodystrophy, which refers to pseudomyotonia. Electromyography (EMG) with this disorder reveals characteristic explosive, irregularly repeated discharges, similar to high-frequency ones.

With neuromyotonia, persistent muscle contractions can spontaneously develop covering the trunk and face. In this state, only slow active movements are possible. With both passive and active movements, muscle rigidity first increases and then weakens. On EMG, irregular bursts of activity, post-discharges, increased insertional activity (developing in response to the introduction of an electromyographic needle) are noted.

Myotonic syndromes, characterized by prolonged muscle contractions, can occur in response to their mechanical, electrical or other sufficiently strong activation.

Here are some of the most common muscle cramps syndromes.

Crumpy: These are painful spasms of muscles, primarily the muscles of the lower leg, but also of the abdomen, chest, back, and less often of the arms and face. Most often we are talking about the triceps muscle of the lower leg. They occur after physical exertion, occurs in various diseases, including an autosomal dominant variant of non-progressive common crampi with minimal anterior limb insufficiency; observed in amyotrophic lateral sclerosis, peripheral neuropathies, pregnancy, dysmetabolism. Quite often, crumpy occurs in patients with lumbar osteochondrosis and in this case has the following features:

1. typical for the stage of remission and almost never occurs in the acute period;
2. not being epileptic in nature, this local convulsive phenomenon is still common in individuals with residual non-severe cerebral insufficiency;
3. it is characterized by local pathology, most often in the form of popliteal neuroosteofibrosis;
4. it is caused by neurogenic mechanisms and humoral changes - hyperacetylcholinemia, hyperserotoninemia (Popelyansky Ya.Yu.).

Like hypercalcemic, thyrotoxic and others, cramps with osteochondrosis are more common in the elderly and occur at night, in warmth, in a state of rest, i.e. in conditions that promote rapid and intense muscle shortening. A sudden muscle shortening is accompanied by an increase in its diameter, induration (the muscle becomes sharply defined), and severe pain. Possible explanations for such pain lie partly in the biochemical plane (the release of the corresponding substances), and partly in the electrophysiological one (sudden loss of gate control, local discharge, formation of a generator of pathological excitation). Clonazepam is effective.

Tics, facial hemispasm, restless legs syndrome (Ekbema), iatrogenic dyskinesias. Tic generalized hyperkinesis is often combined with obsessive-compulsive disorders, which, in principle, determines the clinical picture of Tourette's syndrome accompanying various organic brain lesions. This syndrome must be differentiated from an independent nosology - Tourette's disease, which is hereditary. There are several points of view on the biochemical basis of Tourette's syndrome. Pfeifer C.C. et al. (1969) wrote about the deficiency of the enzyme hypoxanthine-guanine-phosphoribosyl-transferase, which is involved in the metabolic cycle of uric acid formation and is contained in the maximum concentration in the basal ganglia. P.V. Melnichuk et al. (1980) associate the syndrome under consideration with metabolic disorders of catecholamines. But one way or another, today in the treatment of tic hyperkinesis, the drug of choice is primarily haloperidol in a dose of 0.25 - 2.5 mg, prescribed before bedtime, and sometimes additionally in the daytime. Efficiency reaches 75 - 80% even with Tourette's syndrome or disease (Karlov V.A., 1996). The agent of the second stage is pimozide, 0.5 - 10 mg per day. Elderly patients should be prescribed the drug with caution and under ECG control, since the P - Q interval is prolonged. Clonazepam and reserpine are effective, but these drugs are still not as "successful" as antipsychotics.

Obsessive-compulsive disorders are well treated with antidepressants that inhibit serotonin reuptake. Monoamine oxidase inhibitors, tricyclic antidepressants (amitriptyline, imipramine) can be used. Psychostimulants can also be shown: meridil, sydnocarb, but they increase tic hyperkinesis. In recent years, the antidepressant fluoxetine (serotonin inhibitor) at a dose of 20-40 mg per day, deprenyl at 5-15 mg per day has been successfully used (Karlov V.A., 1996).

Tremor. With its non-Parkinsonian origin (essential, alcoholic, thyrotoxic, post-traumatic tremor), we are talking about trembling hyperkinesis, manifested during movement. If parkinsonian tremor is associated with dopaminergic insufficiency, then non-Parkinsonian variants of tremor are based on the principle of excessive functioning of adrenergic and, possibly, GABAergic neurons. It is possible that there is also a violation of the stability of cell membranes, since anaprilin, which has a maximum effect in tremor, has a pronounced membranostatic effect (Elison P.H., 1978; Karlov V.A., 1996). Anaprilin (propranolol) sometimes gives severe allergic manifestations, even bronchospasm, therefore it is contraindicated for patients suffering from bronchial asthma or other allergies. In this case, the drug can be replaced with metoprol, oxprenolol (trazicor), atenolol. Doses of beta-blockers for anaprilin are 60 - 80 mg per day. For the elderly and senile age, small dosages are advisable, since it is easier than in young people, side effects such as depression, sleep disturbances, even toxic psychosis and hallucinosis occur. In many patients, hexamidine (primidene) and clonazepam are effective. Use leponex, isoniazid.

IV.Headaches.

Headache is one of the most frequent complaints with which patients turn to a doctor of any specialty. According to statistical studies by various authors, the frequency of headaches ranges from 50 to 200 per 1000 population. Headache is the leading syndrome or symptom in more than 45 different diseases (Stock V.N., 1987). The problem of headache is so urgent that various specialized centers have been established to study it. The European Association for the Study of Headache was organized, and since 1991 the Russian Association has been a member. The work of the Association is coordinated by the Russian Headache Center, created on the basis of the Moscow Medical Academy. THEM. Sechenov.

Attempts have been made repeatedly to classify headaches. In our country, the pathogenetic classification of headache presented by V.N. Stock and his famous monograph (1987). The author identifies 6 main types of headaches:

1. vascular;
2. muscle tension;
3. liquorodynamic;
4. neuralgic;
5. mixed;
6. psychhalgia (central).

Each variant has its own characteristic pathophysiological mechanism of headache. The author of this classification defends the concept of isolation of one of these headache variants in each patient, while the mixed variant is considered a rare exception to the rule. As practice shows, this kind of approach is far from always correct (Myakotnykh V.S., 1994), especially in patients with polietiological, polypathogenetic nature of the pathological process, one of the clinical manifestations of which is headache.

In elderly and senile people, in the process of accumulation of various diseases, headache is undoubtedly mixed, combined, including various pathophysiological mechanisms of occurrence.

In 1988, the international classification committee proposed the most complete classification of headache, which, however, is not final and continues to be improved, supplemented, and specified. The classification considers the following forms of headaches:

• migraine:
  1. without aura (simple form);
  2. with an aura (associated).

  In the latter, various forms are distinguished depending on the local symptomatology that occurs when the pathological focus is localized in a particular vascular basin;

• tension headaches (synonyms: psychalgia, psycho-myogenic, neurotic); are subdivided into episodic and chronic, with or without involvement of the muscles of the scalp and (or) neck in the pathological process;
• cluster or cluster headaches;
• chronic paroxysmal hemicrania;
• headaches caused by vascular;
• infectious;
• tumor processes;
• traumatic brain injury, etc.

Very interesting and to a certain extent unusual, uncharacteristic for most other types of pathology, is the fact that some variants of headaches, in particular migraine, can be considered as a syndrome or even a symptom of a disease (there are even the terms "migraine" or " migraine-like "syndrome), and as an independent nosological unit. Perhaps this contributed to the fact that until now there is no consensus about the frequency of occurrence of migraine, since some people put only an independent disease into this concept, and others - a variant of a syndrome or even a symptom.

In addition, absolutely reliable diagnosis of a particular type of headache is a difficult task. If we proceed from the classification of 1988 and subsequent years, it may seem that the simplest thing is to diagnose a headache "tied" to any specific pathology - vascular, infectious, tumor, traumatic, etc. To a certain extent, this is so, but only after the diagnosis of the "background" disease for headache has already been made. Therefore, the very factor of the presence of a headache in a patient from the very beginning should set the doctor up to diagnose the pathology in which the headache acts as a symptom or syndrome. By this, the last part of the classification is "cut off", and the first part remains, where the diagnosis of the nature and clinical-pathogenetic, clinical-pathophysiological variant of headache is carried out.

The most interesting, both in clinical and in pathophysiological aspects, are probably the first three types of headache: migraine (occurs in the population with a frequency of 3 to 30% according to various authors); cluster or beam (frequency of occurrence from 0.05 to 6%); tension headaches (found in 32 - 64%, and among other forms of headache in women - up to 88%, in men - up to 69%). There are a number of similarities that these three forms of headache have in common:

• They are all psychogenic in nature;
• Most represented in the population among other forms of headaches;
• Paroxysmal flow is characteristic.

Determined by the sufficient severity of emotional and personal changes, although different in quality: migraine - the predominance of anxious, demonstrative features, a high level of claims, low stress resistance; tension headache - depressive-hypochondriacal, demonstrative character traits; cluster headache - “lion and mouse” syndrome (outwardly courageous, ambitious, ambitious, and internally timid and indecisive), with the presence of psychomotor agitation during the period of paroxysm.

The presence of clinical autonomic disorders is significant. The maximum vegetative disturbances are presented in "panic migraine", when at the height of the typical form of migraine there are signs of a panic attack (emotional agitation, fear, chill-like hyperkinesis, etc.).

There is a significant number of observations of muscular-tonic syndrome in the neck muscles (palpation or according to the results of electroneuromyography). With migraine, this syndrome is predominantly expressed on the side of hemicrania.

The proximity of subjective severity - the intensity of pain in paroxysm. According to the visual analogue scale (VAS): migraine - 78%, tension headache - 56%, cluster headache - 87%.

An important criterion is the quality of life. It reflects the degree of adaptation of patients with the named forms of headache, determines the degree of their activity, efficiency, fatigue, mood changes, and the effectiveness of the activities performed. Quality of life also includes an assessment of the patient's understanding and support from a loved one. The maximum decrease in the quality of life in patients with tension headache - up to 54%, with migraine - up to 70%, with cluster headache (during an attack) - up to 86%.

Some similarity of disturbances in the interaction of nocic and antinociceptive systems in patients with migraine and tension headache at the level of the brainstem systems. This was revealed as a result of special biochemical and electrophysiological studies.

Thus, with the described forms of headaches, there is a certain psycho-vegetative-motor pattern accompanying painful paroxysm. This served as the basis for the use for the treatment of headaches not only widely known and described in the numerous literature means, but also psychotropic drugs and anticonvulsants. For migraine, for example, phenobarbital, finlepsin, diphenin (Karlov V.A., 1987), cepra (Shershever A.S. et al., 2007) are widely used. Anticonvulsants reduce the pain sensitivity of the vascular wall, enhance antinociception at the level of the stem systems. For cluster headaches, sodium valproate is used, which is a GABA mimetic and acts on interneurons of the hypothalamus, thereby affecting circadian rhythms, the violation of which is one of the main pathogenetic links in cluster cephalgias. Finlepsin can be used in combination with other analgesic, vascular drugs, sedatives.

For migraines and tension headaches, tricyclic antidepressants are used, especially amitriptyline, which is due to the presence of psychovegetative and psychomotor clinical manifestations in paroxysms. The use of alprozolam (cassadan) turned out to be quite effective, especially for headaches of neurotic or partially neurotic genesis. Since this drug has an anoxiolytic, antidepressant, muscle relaxant effect, affects the GABAergic system, it can be used for the following types of headaches: panic migraine, combined migraine plus tension headaches, mainly episodic tension headaches with muscle dysfunction.

Of interest is the question of whether it is possible and how often it is possible to combine several variants of headache in one patient and whether it is possible to change, or even "kaleidoscopic" (constant change of variants with periodic repetitions) in the same patient. In this case, of course, two more questions often arise - what is this connected with and how can the therapeutic problems be solved in this case?

From the indicated positions, we can consider two main options for the clinical "change of scenery":

1. one patient has several variants of one type of headache at the same time, for example, several variants of migraine attacks;
2. one patient has several types of headache.

Perhaps the most fully and distinctly described are the various variants of migraine. Let us once again give the main ones.

1. Simple form (no aura).
2. Associated form (with aura).

In the latter form, a number of clinical variants can be distinguished, depending on the clinical picture of the aura (ophthalmic, ophthalmoplegic, olfactory, illusory, vestibular, etc.).

V. Vegetative disorders.

According to epidemiological studies, up to 80% of the population experience some type of autonomic disturbance. This is due to the key role of the autonomic nervous system in such basic processes as maintaining homeostasis and adapting to changing environmental conditions. Events and situations of both biological and psychosocial nature can lead to a disruption of autonomic regulation, which clinically manifests itself in the form of autonomic dysfunction or autonomic dystonia syndrome. In our opinion, it is completely wrong to believe that vegetative-dystonic manifestations become less pronounced with age than in young people, and the total number of patients suffering from neurocirculatory or vegetative-vascular dystonia drops sharply. It seems to us, on the contrary, that the number of patients with dystonic, vegetative-vascular pathological manifestations in old and senile age is increasing, but this pathology moves from the category of nosology or syndromology to predominantly symptomatic aspects. In the first place as an independent disease or syndrome are various clinical variants of atherosclerosis, arterial hypertension, pathological processes in the gastrointestinal tract, urinary, endocrine systems, osteochondrosis, finally. All these diseases can be clinically represented by vegetative-dystonic disorders, but these disorders are no longer perceived as syndromes, not as independent diseases, but as one, two or more symptoms of more severe pathological processes. This does not mean at all that in old and senile age the problem of vegetative-vascular dystonia is absent, or at least goes to the second, third plans. After all, if we cannot completely stop the development of atherosclerosis, for example, then it would be wrong to completely abandon symptomatic treatment; the patient is not worried about the disease, as such, he is worried about the manifestations of this disease. And therefore, in the elderly, very often therapy can and should be directed precisely at manifestations that level the quality of life of our patients. Within the framework of the syndrome of vegetative dystonia, it is customary to distinguish 3 groups of vegetative disorders (Wayne A.M., 1988):

• psycho-vegetative syndrome;
• progressive autonomic failure syndrome;
• vegetative-vascular-trophic syndrome.

In some cases, autonomic disorders are constitutional in nature, manifesting already from early childhood or from puberty, but in most patients they develop secondarily, within the framework of neuroses, psychophysiological reactions, against the background of hormonal changes, organic somatic, neurological diseases, and endogenous mental disorders.

The group of psycho-vegetative disorders should be especially highlighted, which are most common and manifest clinically in the form of emotional disorders in combination with polysystemic vegetative disorders (cardiovascular system, respiration, gastrointestinal tract, thermoregulation, sweating, etc.). These disorders can occur in the form of permanent, paroxysmal, permanent-paroxysmal disorders. The most obvious and striking representatives of autonomic disorders in this group are vegetative crises (panic attacks) and neurogenic syncope (syncope).

Panic attacks are the most dramatic manifestation of autonomic dystonia syndrome (Wayne A.M. et al., 1994). Many terms have been proposed, denoting apparently identical conditions: diencephalic crises, cerebral autonomic seizures, hyperventilation attacks, anxiety attacks, etc. It seems to us necessary, therefore, when considering panic attacks, at least briefly dwell on the problem of vegetative-vascular dystonia.

For many years, vegetative-vascular dystonia was considered either within the framework of neuroses, or as a pathology of the autonomic nervous system, or as the initial form of other diseases, for example, arterial hypertension, cerebral atherosclerosis. Nevertheless, vegetative-vascular dystonia is an independent form of pathology, which, in essence, etiopathogenetic relations is a functional disease of polyetiological genesis, manifested mainly by vascular and veterative disorders.

Consider the chain of pathophysiological and biochemical reactions that occur in vegetative-vascular dystonia. The most important, perhaps, is the question of the formation of functional hypoxia of the brain. Several mechanisms are important in its occurrence: hyperventilation as a manifestation of the sympathotonic effect, followed by the vasoconstrictor effect of the microvasculature. There is a direct vasoconstrictor effect due to an increase in the level of adrenaline, norepinephrine and cortisol (as a nonspecific effect of stress activation), followed by a decrease in the maximum oxygen consumption, a decrease in metabolism and a slowdown in lactate utilization. Finally, there is a change in the rheological properties of blood (increased viscosity, aggregation properties of erythrocytes and platelets), the affinity of hemoglobin to oxygen, which, in combination with microcirculation disorders, aggravates the level of brain hypoxia. With emotional stress, the body's need for energy supply increases, which is compensated mainly by increasing lipid metabolism.

Lipid peroxidation processes play an important role in the development of adaptation diseases associated with stress and, in particular, diseases of the cardiovascular system. Many authors in their works indicate the activation of lipid peroxidation in gastric ulcer and duodenal ulcer, with neurodermatitis and diabetes. In experiments on animals, in response to severe stress, lipid peroxides accumulated, which led to damage to body tissues, and the introduction of antioxidants in this case inhibited the development of stress-induced disorders of internal organs with a sharp decrease in the release of corticosteroid hormones. Dependences between the activity of lipid peroxidation and clinical features of neurotic disorders were revealed. It is obvious that microcirculation disorders and brain hypoxia are the intermediate link that transforms psychogenic effects into a stable pathological state of the brain. This dictates the need to include in the therapeutic complex drugs used in the treatment of neuroses and, in particular, vegetative-vascular dystonia, which, in addition to affecting the listed biological targets (aggregation properties of blood, microcirculation disorders, oxygen metabolism and lipid peroxidation of biological membranes), ruptured would be a chain of pathological adaptive reactions to anxiety and indirectly reduce the severity of emotional stress.

Since 1980, with the advent of the American Classification of Mental Illness (DSM - III), the term "panic attack" has been established in international practice to denote paroxysmal conditions with polysystemic autonomic, emotional and cognitive disorders. These states are included in the broader class " anxiety". The main criteria for distinguishing panic attacks are:

• recurrence of seizures;
• their occurrence outside of emergency and life-threatening situations;
• attacks are manifested by a combination of at least 4 of the 13 following symptoms:
• dyspnea;
• "Pulsation", tachycardia;
• pain or discomfort in the left side of the chest;
• a feeling of suffocation;
• dizziness, unsteadiness, feeling of impending fainting;
• feeling of derealization, depersonalization;
• nausea or abdominal discomfort;
• chills;
• paresthesia in the arms and legs;
• feeling of "hot flashes", "waves" of heat or cold;
• sweating;
• fear of death;
• fear of going crazy or committing an uncontrollable act.

Panic attacks occur in 1 - 3% of the population, twice as often in women and mainly between the ages of 20 and 45, although they are also far from rare in menopause. The clinical picture of suffering is represented by paroxysms, the core of which is the aforementioned symptoms. However, it was noted that a number of patients at the time of an attack do not have a feeling of fear, anxiety ("panic without panic", "fearless attacks"), in some patients emotional manifestations may consist in a feeling of melancholy or depression, in others it is irritation, aggression or just an inner tension. In the majority of patients in an attack, functional neurotic symptoms are present: lump in the throat, pseudoparesis, speech and voice disorders, convulsive phenomena, etc. Attacks can occur both spontaneously and situationally, in some patients they develop at night, during sleep, often accompanied by unpleasant, disturbing dreams. The latter often precede the deployment of the attack at the moment of waking up, and after the end of the panic attack, they amnesia in whole or in part. With the repetition of paroxysms, a feeling of their anxious expectation is formed, and then the so-called avoidance behavior. The latter, in its extreme version, acts as an agoraphobic syndrome (patients become completely maladapted, cannot stay at home alone, move unaccompanied along the street, urban transport is excluded, etc.). In 30% of cases, the recurrence of panic attacks leads to the onset and development of depressive syndrome. Hysterical and hypochondriacal disorders are not uncommon.

Syncope (neurogenic syncope). The generalized concept of fainting is as follows: "Fainting is a short-term impairment of consciousness and postural tone due to reversible disturbances of cerebral functions with spontaneous recovery."

Fainting occurs in 3% of the population, however, at puberty, the frequency of recurrent syncope can reach 30% (Wayne A.M. et al., 1994). There is no unified classification of syncope yet, but all researchers of this problem distinguish 2 main groups of syncope:

1. neurogenic (reflex),
2. somatogenic (symptomatic).

The first include:

• vasodepressor syncope;
• orthostatic syncope;
• sinocarotid;
• hyperventilating;
• tussive;
• nocturic;
• fainting on swallowing and lingo-pharyngeal neuralgia.

Among the second group of syncope are:

• associated with cardiac pathology, where a violation of cardiac output occurs due to a disturbance in the rhythm of the heart or a mechanical obstruction to blood flow;
• associated with hypoglycemia;
• associated with peripheral autonomic failure;
• associated with the pathology of the carotid and vertebro-basilar arteries;
• associated with organic damage to the brain stem;
• hysterical pseudosyncopes, etc.

The clinical picture of syncope is rather stereotyped. The syncope itself usually lasts from a few seconds to 3 minutes; the patient turns pale, muscle tone decreases, mydriasis is noted with a decrease in the reaction of the pupils to light, a weak, labile pulse, shallow breathing, a decrease in blood pressure. With deep syncope, there may be several tonic or clonic-tonic twitchings, involuntary urination and defecation.

Allocate pre- and post-fainting symptoms.

Lightheadedness (lipotimia), lasting from a few seconds to 2 minutes, is manifested by feelings of lightheadedness, nausea, general discomfort, cold sweat, dizziness, blurred vision, muscle weakness, tinnitus, and a feeling of fading consciousness. At the same time, a number of patients develop fear, anxiety, palpitations, a feeling of lack of air, paresthesia, "lump in the throat", ie. panic attack symptoms. After an attack, the patients quickly come to their senses, although they are alarmed, pale, there is tachycardia, general weakness.

Most patients clearly distinguish factors that provoke fainting: stuffiness, prolonged standing, getting up quickly, emotional and pain factors, transport, vestibular stress, overheating, hunger, alcohol, lack of sleep, premenstrual period, getting up at night, etc.

Some aspects of the pathogenesis of panic attacks and syncope can be very similar and at the same time have distinct differences. There are psychological and biological aspects of pathogenesis. From the point of view of psychophysiology, syncope is a pathological reaction resulting from anxiety or fear in conditions when physical activity (fight or flight) is impossible. A panic attack from the point of view of psychodynamic concepts is a signal to the "ego" about the threat of repressed, unconscious impulses to mental balance. A panic attack helps the ego prevent the unconscious aggressive or sexual impulse from spilling out, which could lead to more serious consequences for the individual.

The biological factors of the pathogenesis of syncope and panic attacks are currently being actively studied. Physiological mechanisms of realization of these two states are to a certain extent opposite. In patients with syncope due to sympathetic insufficiency (especially in the sympathetic postganglionic fibers of the lower extremities), active vasodilation occurs, which leads to a decrease in cardiac output. In case of panic attacks, on the contrary, vascular insufficiency was found, in favor of which is evidenced by:

1. the development of spontaneous panic attacks during the relaxation period;
2. a sharp increase in heart rate in a short period of time;
3. decrease in the content of adrenaline, norepinephrine in serum in the pre-crisis period;
4. characteristic changes in the oscillatory structure of the heart rhythm (detected by cardiointervalography, for example).

When studying the central mechanisms of the pathogenesis of mainly panic attacks, the direct relationship of the noradrenergic nucleus of the brain stem to anxious behavior has been shown. It is no coincidence that drugs that affect the noradrenergic systems - tricyclic antidepressants and monoamine oxidase (MAO) inhibitors - have become so widespread in the treatment of panic attacks. The role of serotonergic systems in the pathogenesis of panic attacks is widely studied. The result is the creation of a large group of drugs, the action of which is directed to these systems - clomipramine, zimeldine, fluvoxamine, fluvoxetine.

Particular interest is shown in biochemical systems associated with the functions of excitation and inhibition - glutamatergic and GABAergic. These systems play a key and opposite role in realization as anxiety; and paroxysmal. In this regard, it seems appropriate to summarize the main clinical and experimental data indicating the proximity of paroxysmal vegetative states and epilepsy:

There are a number of common provoking factors - hyperventilation, inhalation of carbon dioxide;

Paroxysmal flow;

Both spontaneous panic attacks and epileptic seizures occur more often during the period of relaxed wakefulness, often in the stage of slow wave sleep. 2/3 of patients with panic attacks react to sleep deprivation, including electroencephalographically, similarly to patients with epilepsy;

In patients with fainting, paroxysmal activity on the EEG and a decrease in the seizure threshold, as well as asymmetric interest in the deep temporal formations, which is typical for patients with epilepsy, are often recorded;

Relatives of patients suffering from panic attacks or fainting often have typical epileptic seizures;

Vegetative crises can often be risk factors for the subsequent occurrence of epileptic seizures, especially in adults (Myakotnykh V.S., 1992);

The therapeutic activity of antiepileptic drugs (anticonvulsants) is high in patients with fainting and panic attacks.

Treatment of vegetative paroxysms.

Until the mid-1980s, antidepressants dominated the treatment of panic attacks. Tricyclic antidepressants (imipramine, amitriptyline, etc.), MAO inhibitors (phenelzine), four-cyclic antidepressants (mianserin, pyrazidol) were considered basic drugs. But side effects turned out to be significant, there were problems with increasing the dose, the obvious first effect appeared only after 14-21 days, while on days 10-12 an exacerbation of the disease was observed - anxiety increased, seizures became more frequent. The patients also had an increase in blood pressure (BP) and persistent tachycardia, decreased potency, weight gain.

Now the emphasis in drug treatment has shifted to a group of drugs that primarily affect the GABAergic systems. Benzodiazepines are exogenous ligands of benzodiazepine receptors mediated by GABA. There are at least 2 types of central benzodiazepine receptors (BDR): BDR-1, responsible for the anti-anxiety and anticonvulsant action, and BDR-2, responsible for the sedative (hypnotic) effect and muscle relaxant effect. The effects of a new generation of drugs (atypical benzodiazepines) are associated with a specific effect on BDR-1, the most famous of which are clonazepam (antelepsin) and alprozole (xanax, cassadan).

Clonazepam gives a distinct anti-panic effect at a dose of 2 mg per day at 1 - 2 times intake. The effect of the treatment begins already in the first week. The effectiveness of the drug is up to 84% (Wayne A.M. et al., 1994). Side effects are minimal. The independence of the effect on the duration of the disease and the effectiveness in persons with previous episodes of alcoholic excesses, complaining even of hereditary burden of alcoholism, are specific. To a lesser extent, clonazepam affects secondary symptoms panic attacks - depression and agoraphobia, which makes it advisable to include antidepressants in therapy. At a dose of 3-4 mg per day, the drug has proven itself well in the treatment of syncope paroxysms, lipothymia and "hot flashes" in the climacteric period.

Alprozolam is effective in panic attacks from 85 to 92%. The effect is in the first week of treatment. The drug relieves anticipation anxiety and normalizes social and family maladjustment. There is also a rather pronounced antidepressant effect, but with agoraphobia, it is still advisable to add antidepressants to the treatment. The drug can be used for long courses of treatment (up to 6 months) and for maintenance therapy, and does not require an increase in doses. The range of doses used is from 1.5 to 10 mg per day, on average 4 - 6 mg. It is recommended to take it in divided doses. Major side effects: sedation, drowsiness, fatigue, memory loss, libido, weight gain, ataxia. The drug should not be prescribed to patients with substance abuse and alcoholism, because drug dependence may develop. A gradual reduction in dosage at the end of the course of treatment is recommended.

Finlepsin in recent years has been increasingly used in the treatment of paroxysmal conditions of non-epileptic origin.

I would especially like to mention such a well-known drug as Cavinton (Vinpocetine), Cavinton-Forte. Cavinton, as a drug that optimizes metabolism (neurometabolic cerebroprotector) and cerebral hemodynamics, can be considered as a means that affects the pathogenetic mechanisms of the formation of vegetative-vascular dysfunctions. In addition, a number of works indicate the use of Cavinton for the purpose of targeting anxiety, which is a concomitant symptom of various neurotic manifestations. In addition, Cavinton has a pronounced vegetotropic effect, which consists in reducing the reactivity of the sympathetic division of the autonomic nervous system. All this makes it possible to successfully use this drug in the treatment of neuroses and autonomic dysfunctions.

In the treatment of non-epileptic paroxysmal conditions, physiotherapy and balneotherapy, psychotherapy, acupuncture, and bioenergetic effects are widely used. The methods and duration of exposure are selected strictly individually and do not contradict the appointment of basic drug therapy.

Paroxysm is always based on cerebral disorders, therefore, despite a large set of diverse pathologies, which may be characterized by the manifestation of paroxysm, in all cases a general etiological picture is found. In addition, the characteristic features of paroxysm are: short duration, reversibility of disorders, tendency to regular relapses and stereotypes.

In other words, paroxysm is not a separate disease, but the property of some diseases to manifest itself in the form of increased symptoms due to dysfunction of the brain. For this reason, the onset of paroxysms is prone precisely to cerebral pathologies.

In order to identify common etiological factors causing paroxysms in various diseases, a huge number of patients with such diagnoses as epilepsy, migraine, vegetative vascular dystonia, neuroses and neuralgia were studied. A wide range of diagnostic criteria was considered, ranging from a blood picture and ending with the study of the mental state of patients against a general background of risk factors. Thanks to these studies, a comprehensive picture of the factors contributing to the onset of paroxysms was obtained, and the list of diagnoses for which the manifestation of a paroxysmal state is characteristic was supplemented.

As already mentioned, paroxysms arise due to disturbances in the functionality of the brain and complement the general picture of the disease with symptoms characteristic of cerebral disorders, which is one of the main features of paroxysms.

It is necessary to distinguish between primary and secondary paroxysmal genesis. The primary nature of paroxysms is due to congenital factors of manifestation - genetic disposition or disorders in the brain that arose during the period of embryonic development. Secondary paroxysms occur during life due to the influence of various internal or external factors.

In addition, modern science is distinguished by:

• A paroxysmal reaction is a one-time, episodic manifestation of paroxysm, in response to a shock state of the nervous system, for example, with a sharp increase in body temperature, injury, acute blood loss.
• Paroxysmal syndrome accompanies the disease throughout the course.
• Paroxysmal state - regular short-term paroxysmal seizures affecting all areas of the body. The most common paroxysmal state accompanies migraine.

In the early stages of the disease, paroxysms serve as a defense mechanism that stimulates the compensatory component, however, with regular manifestation - in the form of a syndrome and condition, they themselves begin to serve as a complicating factor of the disease.

In addition, there is a simplified classification of paroxysms by the presence of causal relationships between epileptic manifestations and a paroxysmal state. Distinguish:

• Paroxysms of an epileptic nature accompanying the disease of the same name or complementing another organic cerebral disorder with epileptic symptoms.
• Paroxysms are non-epileptic, which are characterized by a simple increase in the clinical signs of any disease and do not have an epileptic basis.

In turn, non-epileptic paroxysmal states are divided according to the predominant manifestation of individual clinical signs:

• Muscular dystonic syndromes, characterized by involuntary and uncontrolled repetitive spasms of certain muscle groups - tics, seizures.
• Myoclonic syndromes are sharp, short, single twitching of an individual muscle, muscle group, or generalized condition. Unlike dystonic syndromes, they differ in a single flinch for a certain period of time.
• Headaches. The main symptom of migraine-like paroxysms.
• Autonomic disorders with an appropriate set of symptoms.

Migraine-like paroxysms

Headaches are one of the most common signs of cerebral pathologies. Several main etiological reasons contributing to the onset of headache have been identified: vascular disorders, muscle tension, liquorodynamic causes, neuralgic etiology, mixed and central.

Each etiological factor is characterized by a separate mechanism for the onset of pain, but the basis is always a violation of the functioning of the nerve cells of the brain. In particular, vascular disorders are characteristic of migraines, when an increased or decreased blood pressure in the network of cerebral capillaries provide regular insufficient trophism of neurons, or there is a pressure of dilated blood vessels on the brain tissue.

Migraine paroxysms belong to the non-epileptic series and are expressed in the form of regular attacks of pain in the region of one side of the head. Painful sensations are painful and very long lasting, sometimes extending for several days. A feature of migraine-like paroxysms is sufficient resistance to treatment - it can be extremely difficult to stop the pain.

An extraordinary feature of migraine is the fact that a paroxysmal state in this pathology can simultaneously be a clinical sign, as well as enter into a complex of symptoms of other cerebral pathologies. This situation greatly complicates the formulation of the correct diagnosis - it is extremely difficult to discern third-party diseases behind migraine attacks.

Paroxysmal state what is it

Received 10.11.1999

State Medical Academy, Nizhny Novgorod

Classification of paroxysmal conditions

A paroxysmal state is a pathological syndrome that occurs in the course of a disease and can be of leading importance in the clinical picture. According to V.A. Karlov's definition, a paroxysmal state is a seizure (attack) of cerebral origin, manifested against the background of visible health or with a sudden deterioration of a chronic pathological condition, characterized by a short duration, reversibility of emerging disorders, a tendency to repetition, stereotype. A wide variety of clinical manifestations of paroxysmal conditions is due to their polyetiology. Despite the fact that paroxysmal states are manifestations of completely different diseases, in almost all cases common etiopathogenetic factors are found.

In order to identify them, a study of 635 patients with paroxysmal conditions was carried out, as well as a retrospective analysis of 1200 outpatient cards of patients observed in outpatient settings for various neurological diseases, in the clinical picture of which paroxysmal conditions (epilepsy, migraine, vegetalgia, hyperkinesis , neuroses, neuralgia). In all patients, the functional state of the central nervous system was examined by electroencephalography (EEG), the state of cerebral hemodynamics by rheoencephalography (REG). A structural and morphological study of the brain was also carried out by the method of computed tomography (CT) and the study of the state of the autonomic nervous system: the initial autonomic tone, autonomic reactivity and autonomic support of physical activity according to the methods recommended by the Russian Center for Autonomic Pathology. In the study of the psychological status of patients with paroxysmal conditions, the questionnaires of G. Aysenk, C. Spielberger, A. Lichko were used. We studied such indicators as the introversion of patients, the level of personal and reactive anxiety, the type of personality accentuation. A study of the concentration of catecholamines and corticosteroids (17-KS, 17-OCS) in the urine, as well as the state immune status: content of T– and

B-lymphocytes, immunoglobulins, circulating immune complexes (CICs) in the blood. In addition, the state of the energy level in the "channels" of the body was studied by measuring the electrocutaneous resistance at representative points according to Nakatani, in the modification of VG Vogralik, MV Vogralik (1988).

The results obtained made it possible to identify etiopathogenetic and risk factors that were common for patients with various nosological forms of diseases, in the clinical picture of which the paroxysmal state was the leading one.

Among the etiological factors were common: pathology of the pre- and perinatal periods of development, infections, trauma (including birth), intoxication, somatic diseases.

Common risk factors are such as hereditary predisposition, social conditions (living conditions, nutrition, work, rest), occupational hazards, bad habits (smoking, alcoholism, drug addiction). Of the provoking factors that can cause the development of paroxysmal states, an acute stressful or chronic psychotraumatic situation, heavy physical exertion, disturbances in sleep and nutrition, a sharp change in climatic conditions in connection with moving, unfavorable helium and meteorological factors, strong noise, bright light, strong vestibular irritation (sea rolling, flight in an airplane, long driving in a car), hypothermia, exacerbation of chronic diseases. The results obtained agree with the literature data 2-5.

The study of the functional state of the central nervous system, the autonomic nervous system, the state of cerebral hemodynamics, the nature of pathological changes in the substance of the brain, psychopathological changes in the personality of patients, as well as the nature of the dynamics of immunological and biochemical parameters made it possible to identify a number of common signs characteristic of all patients with paroxysmal conditions. These include:

the presence of pathomorphological changes in the substance of the brain;

common characteristics of EEG and REG indicators, characterized by the predominance of disorganized, hypersynchronous delta, theta, sigma wave activity, symptoms of vascular dystonia, often of the hypertensive type, due to increased tone of cerebral vessels, difficulty in the outflow of blood from the cranial cavity; pronounced autonomic changes with a predominance of parasympathicotonic reactions in the initial autonomic tone, with an increase in autonomic reactivity, more often with excessive autonomic support of physical activity; psychopathological changes, manifested by a tendency to depressive, hypochondriacal states, introversion, a high level of reactive and personal anxiety, the type of personality accentuation was often defined as asthenoneurotic, sensitive, unstable; changes in the concentration of catecholamines and corticosteroids in the urine in all patients with paroxysmal conditions, which tended to increase the content of catecholamines before the onset of paroxysms and their decrease after an attack, as well as to a decrease in the concentration of corticosteroids before a paroxysm and to increase it after an attack.

Immunological indicators are characterized by a decrease in the absolute and relative number of T- and B-lymphocytes, inhibition of natural killer cell activity, functional activity of T-lymphocytes, and a decrease in the content of immunoglobulins A and G in the blood.

The noted common signs in patients with paroxysmal conditions allow us to conclude that there are common etiopathogenetic mechanisms for the development of paroxysmal conditions. The polyethiology of paroxysmal conditions and at the same time the existence of common pathogenetic mechanisms necessitate their systematization.

The conducted research allows us to propose the following classification of paroxysmal conditions according to the etiological principle.

I. Paroxysmal conditions of hereditary diseases

a) hereditary systemic degeneration of the nervous system: hepatocerebral dystrophy (Wilson-Konovalov disease); deforming muscular dystonia (torsion dystonia); Tourette's disease;

b) hereditary metabolic diseases: phenylketonuria; histidinemia;

c) hereditary disorders of lipid metabolism: amaurotic idiocy; Gaucher disease; leukodystrophy; mucolipidosis;

d) hereditary disorders of carbohydrate metabolism: galactosemia; glycogenosis;

e) phakomatoses: Recklinghausen's neurofibromatosis; Bourneville tuberous sclerosis; Sturge-Weber encephalotrigeminal angiomatosis;

f) hereditary neuromuscular diseases: paroxysmal myoplegia; paroxysmal myoplegic syndromes; myasthenia gravis; myoclonus; myoclonus - Unferricht - Lundborg epilepsy;

g) genoin epilepsy.

II. Paroxysmal conditions in organic diseases of the nervous system

a) injuries of the central and peripheral nervous system: post-traumatic diencephalic crises; post-traumatic myoclonus; post-traumatic epilepsy; causalgia;

b) neoplasms of the brain and spinal cord: paroxysmal conditions associated with liquorodynamic disorders; vestibulo-vegetative paroxysms; epileptic seizures;

in) vascular diseases nervous system: acute discirculatory encephalopathy; ischemic strokes; hemorrhagic strokes; hyper- and hypotonic cerebral crises; venous cerebral crises; vascular abnormalities; aorto-cerebral crises; vertebrobasilar crises; transient ischemic paroxysms; epileptiform cerebral crises;

d) other organic diseases: paroxysmal myoplegia syndrome of cerebral genesis; periodic hibernation syndrome; Alone's punishment syndrome; pontine myelinosis; juvenile trembling paralysis;

e) neuralgic paroxysms: trigeminal neuralgia; glossopharyngeal neuralgia; neuralgia of the superior laryngeal nerve.

III. Paroxysmal conditions within the psychovegetative syndrome

a) vegetative-vascular paroxysms: cerebral; cardiac; abdominal; vertebral;

b) vegetalgia: Charlen's syndrome; Slader's syndrome; ear node syndrome; anterior sympathetic Glaser syndrome; posterior sympathetic Barre-Lieu syndrome;

c) neuroses: general neuroses; systemic neuroses; paroxysmal conditions in mental disorders: endogenous depression; masked depression; hysterical reactions; affective shock reactions.

IV. Paroxysmal conditions in diseases of internal organs

a) heart disease: congenital defects; heart rhythm disturbances; myocardial infarction; paroxysmal tachycardia; primary tumors of the heart;

b) kidney disease: renal hypertension; uremia; eclampsic (pseudouremic coma); hereditary renal diseases (Schaffer syndrome, familial juvenile nephrophthisis, Albright's osteodystrophy);

c) liver disease: acute hepatitis; hepatic coma; biliary (hepatic) colic; cirrhosis of the liver; calculous cholecystitis;

d) lung disease: lobar pneumonia; chronic pulmonary failure; bronchial asthma; inflammatory diseases of the lungs with the presence of a purulent process; malignant diseases in the lungs;

e) diseases of the blood and blood-forming organs: pernicious anemia (Addison-Birmer disease); hemorrhagic diathesis (Shenlein-Henoch disease, Verlhof's disease, hemophilia); leukemia (tumor or vascular types); lymphogranulomatosis (Hodgkin's disease); erythremia (Vakez disease).

V. Paroxysmal conditions in diseases of the endocrine system

pheochromocytoma; Itsenko-Cushing's disease; Cohn's syndrome; thyrotoxic periodic paralysis; hypoparathyroidism; addison crisis; syndrome of paroxysmal myoplegia of cerebral genesis; climacteric syndrome.

Vi. Paroxysmal conditions in metabolic disorders

hypoxia; hypercapnia; mixed; other metabolic disorders.

Vii. Paroxysmal conditions in infectious diseases

a) encephalitis: acute hemorrhagic encephalitis; epidemic encephalitis (Economo's disease); Japanese encephalitis, mosquito; periaxial Schilder's encephalitis; subacute encephalitis, sclerosing; Creutzfeldt-Jakob disease; neurolupus; neurosyphilis; neurorheumatism (chorea minor);

b) post-vaccination: anti-rabies; small-sized;

VIII. Paroxysmal states with intoxication

alcoholic; Gaie-Wernicke acute alcoholic encephalopathy; poisoning with technical poisons; drug poisoning, including drugs.

Of course, this classification requires further development and refinement.

Distinguish between paroxysmal states with primary and secondary cerebral mechanisms. Primary cerebral mechanisms are associated with hereditary burden for one or another type of pathology or with a gene mutation, as well as with deviations arising during embryogenesis against the background of various pathological influences of the maternal organism. Secondary cerebral mechanisms are formed as a result of pathological exogenous and endogenous effects on the developing organism.

In our opinion, it is necessary to distinguish between the concepts: paroxysmal reaction, paroxysmal syndrome and paroxysmal state. A paroxysmal reaction is a one-time occurrence of paroxysm, the body's response to acute exogenous or endogenous effects. It can be in acute intoxication, a sharp increase in body temperature, trauma, acute blood loss, etc. Paroxysmal syndrome is paroxysms accompanying acute and subacute current disease. These include acute infectious diseases, in the clinic of which convulsive paroxysms, vegetative-vascular crises, the consequences of craniocerebral trauma, diseases of the internal organs, accompanied by paroxysms of various nature (painful, syncope, convulsive, etc.) are observed. Paroxysmal conditions are short-term, suddenly arising, stereotypical paroxysms of a motor, vegetative, sensitive, painful, disomnic, mental or mixed nature, as a rule, constantly accompanying chronic or hereditary diseases, during the development of which a stable focus of pathological hyperactivity has formed in the suprasegmental structures of the head brain. These include epilepsy, migraine, Hunt's cerebellar myoclonic dyssynergy, etc.

At the initial stage of the development of the disease, the paroxysmal reaction performs a protective function, activating compensatory mechanisms. In some cases, paroxysmal conditions are a way to relieve stress in pathologically altered functioning systems. On the other hand, long-term paroxysmal states themselves have pathogenic significance, contributing to the further development of the disease, causing significant disturbances in the activity of various organs and systems.

Literature

Karlov V.A. Paroxysmal conditions in a neurological clinic: definitions, classification, general mechanisms of pathogenesis. In the book: abstracts. report VII All-Russia. Congress of Neurologists. M: JSC "Booklet"; 1995; from. 397.

Abramovich G.B., Taganov I.N. On the significance of various pathogenic factors in the origin of epilepsy in children and adolescents. Journal of Neur and Psychiat 1969; 69: 553-565.

Akimov G.A., Erokhina L.G., Stykan O.A. Syncope neurology. M: Medicine; 1987; 207 s.

Boldyrev A.I. Epilepsy in children and adolescents. M: Medicine; 1990; 318 s.

Karlov V.A. Epilepsy. M: Medicine; 1990; 327 s.

Paroxysmal syndrome

A paroxysmal state is a pathological syndrome that occurs in the course of a disease and is of leading importance in the clinical picture. A paroxysmal state is a seizure (attack) of cerebral origin, manifested against the background of visible health or with a sudden deterioration of a chronic pathological condition, characterized by a short duration, reversibility of emerging disorders, a tendency to repetition, and stereotyping.

A wide variety of clinical manifestations of paroxysmal conditions is due to their polyetiology. Despite the fact that paroxysmal states are manifestations of completely different diseases, in almost all cases common etiopathogenetic factors are found.

Characteristics and types of diseases with paroxysms: symptoms and first aid

A sharp deterioration in health, exacerbation of any chronic disease (including neuralgic) and its inconstancy indicate serious problems that can portend paroxysm or paroxysmal state.

Paroxysmal condition is serious pathological deviation, which occurs due to a certain kind of disease, and is of primary importance in drawing up a general clinical picture.

In other words, a paroxysmal state is an attack of neuralgic origin, which manifests itself with an exacerbation of a chronic disease. This condition is characterized by suddenness, short duration and a tendency to reappear.

Groups of provoking diseases

Paroxysmal disorders are classified into several groups.

Paroxysm or a paroxysmal condition that could be caused by the activation of a hereditary disease:

• hereditary degeneration of the nervous system, which has a systemic form: Wilson-Konovalov disease; muscular dystonia, leading to pathological changes in muscle tissue; Tourette's disease;
• metabolic disorders that can be inherited: phenylketonuria; histidinemia;
• deformation of metabolic lipoid pathways: amaurotic idiocy; Gaucher disease; leukodystrophy; mucolipidosis;
• violation in the functioning of phakomatosis: neurofibromatous changes named after Recklinghausen; Bourneville tuberous sclerosis;
• various muscle disorders and damage to the nervous system - aggravated paroxysmal myoplegia; myoplegic syndrome with paroxysm; epileptic state of Unferricht - Lundborg;
• aggravated epileptic seizures.

Paroxysmal syndrome caused by another neuralgic disease:

Paroxysmal conditions caused by diseases of internal organs:

• diseases of the cardiovascular apparatus (heart paroxysm): heart attack, stroke, heart disease, heart palpitations;
• kidney and liver diseases: hepatitis, colic and uremia;
• respiratory diseases: pneumonia, asthma, inflammatory processes.
• blood disease: hepatitis, diathesis, anemia.

Paroxysm developed against the background of disruption of the endocrine system:

Paroxysmal syndrome in metabolic diseases and intoxication:

• hypoxia;
• alcoholic or food intoxication.

Paroxysm that develops within the framework of a psychological disorder: vegetatively vascular crisis or disturbances in the work of the main functions of the body (we will talk about this classification below).

Vegetative paroxysms

In the medical literature, vegetative paroxysms are divided into two groups: epileptic and non-epileptic, and they, in turn, are divided into the following classifications.

Epileptic vegetative paroxysms:

• diseases developing against the background of non-epileptic disorders;
• diseases that have developed against the background of a disturbance in the work of the central nervous system, including epilepsy and other neuralgic and psychological disorders.

Non-epileptic paroxysms, in turn, are divided into the following groups:

• paroxysms caused by disruption of the rhinencephalic structures;
• paroxysmal disorders against the background of dysfunction of hypothalamic structures;
• disorders in the caudal regions are also a significant cause of the development of paroxysm.

Reasons and provocateurs

Vegetative paroxysms can develop against the background of:

• mental disorders;
• neuralgic diseases;
• disturbances in the work of blood vessels (vascular dystrophy).

What provokes vegetative paroxysms

Some genetic pathologies can provoke the onset of vegetative paroxysms - an unexpected increase in systemic degenerations of the nervous system, the development of metabolic disorders and epileptic conditions:

• wilson-Konovalov disease (hepatocerebral dystrophy);
• tourette's syndrome (a hereditary disease manifested by tics of motor skills);
• phenylketonuria (severe genetic disorder of amino acid metabolism);
• gaucher disease (glucosylceramide lipidosis);
• leukodystrophy (violation of the myelination process);
• glycogenosis (hereditary defects of various enzymes);
• galactosemia (genetic disorder of carbohydrate metabolism).

In the first row of organic pathologies of the central nervous system with paroxysmal autonomic disorders are:

Paroxysmal conditions characterize a number of manifestations of vegetative dystonia syndrome:

• nasal nerve neuralgia (Charlin's syndrome);
• pathology of the wing-palatine node (Sluder's syndrome);
• neuroses;
• migraine;
• depressive disorders;
• hysteria;
• affective states.

Also, vegetative paroxysms are characteristic of pathologies of the visceral organs:

• congenital pathologies of the heart;
• cardiac necrosis;
• hepatitis;
• disruption in the work of vital organs such as the liver and kidneys;
• pneumonia.

In addition, disorders in the functioning of the endocrine system and metabolic disorders can also provoke an attack.

Having looked in detail at the classification of paroxysm, one can notice that the causes of its occurrence are quite diverse (from ordinary poisoning to blood diseases).

Paroxysm is always closely related to that organ, the functioning of which was disrupted in connection with one or another pathology.

The most common symptoms

• general malaise, weakness, vomiting;
• lowering blood pressure;
• violation of the gastrointestinal tract;
• epileptic seizures;
• fever, chills and shivering.
• emotional tension.

Complex of measures

Effective treatment of vegetative paroxysms requires an integrated approach that combines: thiological, pathogenetic and symptomatic treatment complex.

As a rule, similar medications are used to treat paroxysm and paroxysmal conditions, which are prescribed by the attending physician. These include: stimulating, absorbing and disinfecting medicines.

They increase the activity of the autonomic and nervous system human body... In addition, psychotherapy plays an important role in the treatment of a wide variety of autonomic seizures.

Varieties of manifestations

The state of paroxysm is quite difficult to be tolerated by a person and lasts about several hours. A similar condition is characterized by general malaise and instability of the whole organism (the condition may be accompanied by unreasonable fear and aggression).

Paroxysmal reaction

A paroxysmal reaction is a physiological phenomenon that signifies a disorder of a certain kind, which develops on the basis of a neuralgic disease.

A paroxysmal reaction is a disturbance in the work of the cerebral cortex, which affects the activity of the hemispheres and is characterized by an abrupt onset and an equally sudden end.

Disorder of consciousness with paroxysms

Paroxysmal disorder of consciousness is a short and sudden disorder of consciousness that occurs on the basis of neuralgic diseases.

It should also be noted that paroxysmal disorders of consciousness are characterized by epileptic seizures and unreasonable aggression.

First aid and treatment

The first aid provided for a paroxysmal state directly depends on the patient's condition. As a rule, for the fastest removal of paroxysm, a solution of lidocaine is used, which is injected intramuscularly as an injection.

For vegetative disorders, complex treatment should be used (thiological, pathogenetic and symptomatic treatment complex). The same principle of treatment is used for paroxysms and paroxysmal conditions, which are caused by other diseases.

The main goal of therapy is to influence the disease provoking paroxysm.

Prevention of seizures is also extremely important, which consists in avoiding stress and the correct daily routine and lifestyle, which has a beneficial effect on the entire body.

Non-epileptic paroxysmal states

There are several variants of predominantly non-epileptic paroxysmal disorders that require special consideration and are quite common in the clinic of nervous diseases. These conditions are subdivided into several of the most common variants, the clinical description of which is difficult to find in any one textbook, monograph. Basically, they can be divided into:

1. Dystonia or muscle dystonic syndromes
2. Myoclonic syndromes and a number of other hyperkinetic conditions
3. Headaches
4. Vegetative disorders

Often, the clinical manifestation of these pathological conditions is associated with neurological nosology that occurs in young (childhood, adolescence, adolescence) age. But, as practice shows, in adults and even in the elderly, the described syndromes very often either debut or progress, the appearance and severity of which is associated with age-related cerebral disorders, acute and chronic disorders of cerebral circulation. It should be noted that many non-epileptic paroxysmal conditions can also be a consequence of the long-term use of various medications used to treat circulatory failure, some mental disorders of the elderly and senile age, parkinsonism, etc. Therefore, in this publication we do not seek to present the selected pathological conditions in the form of syndromes that occur in a certain nosology, and even more so in the form of separate nosological units. Let us dwell on the above and the most common variants of non-epileptic seizures.

I. Dystonia.

Dystonia is manifested by persistent or recurrent muscle spasms leading to "dystonic" postures. In this case, of course, we are not talking about the well-known concepts of vegetative-vascular or neurocirculatory dystonia, which are considered completely separately.

Epidemiology. Dystonia is a rare disease: the incidence of its various forms of patients per 1 million people (0.03%). Generalized dystonia can be inherited dominantly and recessively. The genetic mechanisms of focal dystonias are unknown, although it has been noticed that about 2% of focal dystonias are inherited, and in one third of patients with blepharospasm and spastic torticollis, other movement disorders (tics, tremors, etc.) were noted in families.

The pathogenetic mechanisms of dystonia are still not disclosed. Dystonia does not have a clear morphological substrate in the brain and is caused by subcellular and neurodynamic disorders in certain brain systems. The peripheral motor apparatus, the pyramidal pathway, and the proprioceptive servo mechanism (stretch reflex) are intact in dystonia. Disorders in the functional state of interneurons of the brain stem and spinal cord were revealed.

Also, the biochemical defect underlying dystonia is almost unknown. Empirically, it can be assumed that the cholinergic, dopaminergic and GABAergic systems of the brain are involved. But the low efficiency of treatment of dystonia in general suggests the existence of some other, still unknown to us, biochemical disorders underlying the disease. Most likely, the trigger that triggers dystonia is the biochemical systems at the level of the oral part of the brain stem and its connections with subcortical extrapyramidal formations (mainly the shell, optic tubercle and others).

Depending on the distribution of hyperkinesis in muscle groups and the degree of generalization, 5 forms of dystonias, dystonic syndromes are distinguished:

1. focal dystonia,
2. segmental dystonia,
3. hemidystonia,
4. generalized and
5. multifocal dystonia.

Focal dystonia is characterized by the involvement of muscles in any one part of the body ("writing spasm", "blepharospasm", etc.).

Segmental dystonia is manifested by the involvement of two adjacent parts of the body (the circular muscle of the eye and the circular muscle of the mouth; neck and arm; pelvic girdle and legs, etc.).

With hemidystonia, muscle involvement of one half of the body (arms and legs most often) is observed. Such dystonia is often symptomatic and directs the doctor to a diagnostic search for the primary lesion of the nervous system.

Generalized dystonia is characterized by muscle involvement of the entire body.

Multifocal dystonia affects two or more non-adjacent areas of the body (for example, blepharospasm and dystonia of the foot; torticollis and writer's spasm, etc.).

Focal dystonias are much more common than generalized ones and have six main and relatively independent forms:

• blepharospasm,
• oromandibular dystonia,
• spastic dysphonia,
• spastic torticollis,
• writing spasm,
• dystonia of the foot.

Generalized dystonia usually begins with focal dystonic disorders, its onset often occurs in childhood, adolescence. The older focal dystonia begins, the less likely its subsequent generalization is.

Postures and syndromes characteristic of dystonia are presented in Table 1.

But the division of dystonias into focal and generalized reflects only the syndromic principle of classification. The formulation of the diagnosis should also include the nosological principle - the name of the disease. The most complete nosological classification of dystonia is presented in the international classification of extrapyramidal disorders (1982), as well as in the generalizing article by McGuire (1988). In these classifications, primary and secondary forms of dystonia are distinguished. In primary forms of dystonia, this is the only neurological manifestation. They can be both hereditary and sporadic. Secondary dystonia occurs in known and diagnosed diseases of the nervous system and is usually accompanied by other neurological disorders. In children, this occurs against the background of cerebral palsy (cerebral palsy), Wilson's disease, storage diseases; in adults, including the elderly - as a result of cerebral infarction, tumors, degenerative processes, the use of drugs, etc.

The defining characteristic of dystonia is the formation of typical dystonic postures, many of which have their own, sometimes figurative names. The most typical dystonic postures and syndromes are shown in table 1 (cited by OR Orlova).

Since any area of \u200b\u200bthe body can be involved in dystonic hyperkinesis, the clinical pattern of dystonic syndrome in each individual patient depends on the distribution and combination of dystonic postures in different areas of the body. This principle (distribution of dystonic syndromes in different regions of the body) is the basis for the modern convenient classification of dystonia (Marsden, 1987), given above.

It would be advisable to list the clinical features common to all focal dystonias.

Dystonic posture. With blepharospasm, there is closure, shutting the eyes, or frequent blinking. Oromandibular dystonia is characterized by dystonic postures in the perioral region, tongue, trismus. Spastic torticollis is manifested by rotation or tilting of the head. When writing spasm, the posture of the hand resembles the "hand of an obstetrician." Pathological postures that occur in the swallowing and voice-forming muscles during spastic dysphagia and dysphonia can be considered with a special ENT examination.

Dystonia of action. In patients, the performance of certain actions carried out by the muscles that form the dystonic posture is selectively disrupted. With blepharospasm, the action suffers - keeping the eyes open, with spastic torticollis - keeping the head in a straight position, with writing spasm, writing is disturbed, with oromandibular dystonia, speech and food intake may be impaired. In the case of spastic dysphagia and dysphonia, swallowing and voice are impaired. With outpatient foot spasm, normal walking is disturbed. At the same time, other actions performed by the same muscle group are completely intact. For example, a patient with a writer's cramp can perfectly use a “sore” hand for all everyday activities.

The dependence and variability of dystonia is diminished by the position of the body. As a rule, all manifestations of dystonia decrease or disappear when the patient is lying down, and intensify when standing.

Influence of the patient's emotional and functional state on the severity of dystonia: decrease or disappearance of dystonia in sleep, in the morning after waking up, after taking alcohol, in a state of hypnosis, the possibility of short-term volitional control, increased dystonia during stress, overwork. This feature is very clearly manifested at a doctor's appointment, when during a minute's conversation all manifestations of dystonia can disappear, but as soon as the patient leaves the doctor's office, they resume with renewed vigor. This feature can cause the doctor to mistrust the patient, suspicion of simulation.

Corrective gestures are special techniques that the patient uses for the short-term elimination or reduction of dystonic hyperkinesis. As a rule, this is either touching with your hand to any point in the area of \u200b\u200binterest, or imitation of some kind of manipulation in this area. For example, patients with spastic torticollis, to reduce hyperkinesis, touch their cheeks or any other point on the head with their hand, or imitate adjusting glasses, hairstyles, ties, patients with blepharospasm - rub the bridge of their nose, take off and put on glasses, with oromandibular dystonia, chewing gum, sucking helps for a short time sweets, as well as the presence in the mouth of a stick, match, cigarette or any other object. When writing spasm, the difficulty of writing can be temporarily reduced by putting a healthy hand over the "sick" hand.

Paradoxical kinesis is a short-term decrease or elimination of hyperkinesis in the nature of the action (change in the locomotor stereotype). For example, patients with writing spasm easily write with chalk on a blackboard, head rotation in patients with spastic torticollis may decrease or disappear when running or driving a car, in patients with spastic dysphonia, the voice "erupts" when singing or screaming, and in patients with outpatient foot spasm of its pathological posture does not occur when walking on tiptoe or backwards.

Remissions are fairly common in focal dystonias. More often than in other forms, they are observed in patients with spastic torticollis (in 20-30%), when the symptoms can spontaneously disappear completely for months or years even after several years from the onset of the disease. With exacerbation of spastic torticollis, the phenomenon of rotation inversion is sometimes observed - a change in the direction of the violent turn of the head. Less characteristic remissions for writing spasm and other focal dystonia, however, with writing spasm, the phenomenon of inversion is also observed - the transition of writing spasm to the other hand.

The combination of focal forms of dystonia and the transition of some forms to others. When two or more focal forms are combined, as a rule, manifestations of one form prevail, while others may be subclinical, and the symptoms of the erased form often appear before the symptoms of the clinically pronounced form. Example: A few years before the onset of spastic torticollis, about a third of patients have difficulty writing or frequent blinking, but writing spasm or blepharospasm is diagnosed after the onset of torticollis symptoms. There are cases when, after remission, one focal form is replaced by another, and one patient may have several such episodes. The classic is the combination of blepharospasm and oromandibular dystonia. In this case, blepharospasm usually appears first (the first stage of facial paraspasm) and then oromandibular dystonia (the second stage of facial paraspasm) joins it.

The dynamism of dystonia is most likely associated not with a specific anatomical substrate, which has not yet been found, but with a violation of the interaction between the structures of the basal ganglia, brainstem, thalamus, limbic-reticular complex, motor cortex due to impaired metabolism of neurotransmitters in these structures, which constitutes organic neurodynamic substrate of dystonia (Orlova O.R., 1989, 1997, 2001).

Diagnostic criteria of Marsden and Harrison (1975) for the diagnosis of idiopathic dystonia:

1. the presence of dystonic movements or postures;
2. normal childbirth and early development;
3. absence of diseases or taking medications that could cause dystonia;
4. absence of paresis, oculomotor, atactic, sensory, intellectual disorders and epilepsy;
5. normal results of laboratory tests (copper exchange, fundus, evoked potentials, electroencephalography, computed and magnetic resonance imaging).

Spastic torticollis is the most common focal form of dystonia. The essence of dystonic syndrome with it is a violation of keeping the head in a straight position, which is manifested by rotation or tilt of the head. Spastic torticollis usually begins with age, 1.5 times more often observed in women, almost never generalizes, can be combined with writer's spasm, blepharospasm and other focal dystonia. A third of patients are in remission.

Writing spasm. This form of dystonia occurs with increasing age, equally often in men and women; among patients, people of "writing" professions (doctors, teachers, lawyers, journalists) and musicians predominate. Writing spasm and its analogs (professional dystonia) often develop against the background of previous hand injuries or other pathology of the neuromotor apparatus. Writing spasm remissions are rare and generally short-lived.

Blepharospasm and oromandibular dystonia. These forms usually begin after the age of 45. As a rule, symptoms of oromandibular dystonia appear several years after the onset of blepharospasm.

Dystonia deserves special attention, manifested by sudden attacks of involuntary movements and pathological postures, which are never accompanied by impaired consciousness and are often mistakenly regarded as hysterical or epileptic seizures. In some patients, seizures occur spontaneously, in others they are provoked by unprepared movements (kinotogenic or kinesigenic and non-kinetogenic or non-kinesigenic forms). Typical paroxysms: choreoathetosis, tonic or dystonic movements (generalized or by hemitype), sometimes leading to the patient falling down if he does not have time to grab onto any object. The attack lasts from several seconds to several minutes. Paroxysmal dystonia is either idiopathic (including familial) or symptomatic. The latter option is described for three diseases: cerebral palsy, multiple sclerosis and hypoparathyroidism. The drugs of choice for treatment are clonazepam, carbamazepine, and diphenin. The effect of the treatment is high.

There is also a special form of dystonia that is sensitive to the treatment of L-DOPA (Segawa's disease). It responds very well to treatment with dopamine-containing drugs, and this is perhaps its main differential diagnostic criterion.

Treatment of dystonia. It is generally known that there is no specific treatment for dystonia. This is due to the fact that neurochemical disorders in this disease are ambiguous, depend on the initial state of neurochemical systems and transform as the disease progresses. The most universal are GABAergic drugs (clonazepam and baclofen), however, previous treatment with drugs from other groups may reduce the effect of GABAergic therapy.

Treatment for dystonia is predominantly symptomatic. The therapeutic effect is rarely complete, more often only a relative regression of dystonic manifestations is achieved. But this is also achieved at the cost of long-term efforts to select drugs and their optimal doses. In addition, about 10% of dystonia are characterized by spontaneous remissions, in the presence of which it is difficult to talk about evaluating the effectiveness of certain drugs.

Dopamine agonists and antagonists, anticholinergics, GABAergic and other drugs are traditionally used. Dopamine agonists (nakom, madopar, lisuride, midantan) and antagonists (haloperidol, pimozide, etopyrazine, azaleptin, tiapride, etc.) are effective in an equally low percentage of cases. Anticholinergics give relief to almost every second patient. The most commonly used are cyclodol, parkopan, artan (trihexyphenidil), but a dose of 2 mg in 1 tablet is rarely effective. Recently, 5 mg parkopan has appeared, but here the effect is often achieved at subtoxic doses. The use of cyclodol is described in daily doses even over 100 mg. But at the same time, side effects are very likely, especially pronounced in patients of older age groups.

Among anticholinergics, tremblex is more effective - a central anticholinergic of prolonged action. The relief of dystonic manifestations is sometimes achieved in about a minute after one injection (2 ml) of the drug. Side effects - dry mouth, numbness and a feeling of lining of the tongue and throat, dizziness, drunkenness, hypersomnia. This often causes the patient to refuse treatment with tremblex. There is also a drop in the effectiveness of the drug, sometimes literally from injection to injection. Glaucoma is also a contraindication, especially in the treatment of the elderly.

In the treatment of dystonia, lithium salts (lithium carbonate) and clonidine (gemiton, clonidine) are also used. Only a small proportion of patients respond well to treatment, but they need to be identified.

The vast majority of patients tolerate benzodiazepines well, especially clonazepam (antelepsin). But, unfortunately, we do not yet have an ampouled form of the drug. Clonazepam is effective in all types of diseases with the exception of generalized idiopathic torsion dystonia, where the effect is only subjective and can be explained by the psychotropic effect of the drug. Doses of clonazepam are from 3 dmg per day, sometimes higher.

Blepharospasm, facial paraspasm (Bruegel's syndrome), and other cranial dystonia also respond well to clonazepam.

Among the drugs that have a relaxing effect in muscle spasticity, I would like to highlight the well-known, but until now undeservedly little used for muscular dystonia mydocalm (tolperisone).

Muscle spasticity can be considered as a pathological state of equilibrium that changes rapidly under the influence of various factors (fever, cold, warmth, time of day, pain), therefore it is difficult to develop a medication that, due to a flexible dosage, would reduce the pathologically increased tone only to the desired level. And here tolperisone has, perhaps, the mildest effect, without crossing the "border of what is permitted."

Among the pharmacodynamic properties of tolperisone, it should be noted: the central muscle relaxant effect and an increase in peripheral blood flow independent of it.

Localization of the muscle relaxant action of the drug is established in the following morphofunctional structures:

• in peripheral nerves;
• in the spinal cord;
• in the reticular formation.

Due to the membrane-stabilizing, local anesthetic effect, which manifests itself in the brain stem, in the spinal cord and in peripheral nerves (both motor and sensory), mydocalm prevents the emergence and conduction of an action potential in "overstimulated" neurons and thereby reduces the pathologically increased muscle tone. Depending on the dose, it inhibits nociceptive and non-nociceptive mono- and polysynaptic reflexes in the spinal cord (flexion, direct and cross-extensor), inhibits mono- and polysynaptic reflexes at the level of the spinal roots, and also inhibits the conduction of excitation along the reticulospinal activating and blocking pathways.

The proof of the direct action of mydocalm on the brain stem is the blocking effect on tonic chewing reflexes that occur during periodontal stimulation. This reflex arc includes intermediate neurons in the brain stem. Direct action at the level of the brain stem is also evidenced by the effect of reducing the latent time of nystagmus induced by rotation.

Tolperisone significantly, depending on the dose, reduces the rigidity caused by overactive gamma-motor neurons after intercollicular transsection in the midbrain.

In the event of ischemic rigidity (in this case, the cause of rigidity is the excitement arising in alpha-motor neurons), tolperisone reduced its severity.

Large doses of tolperisone block the experimental seizures of seizures caused by such provoking agents as strychnine, electroshock, pentylenetetrazole.

The drug has no direct effect on the neuromuscular junction.

It is assumed that tolperisone has a weak atropine-like M-anticholinergic and slightly pronounced -adrenergic blocking effects.

Pharmacological studies carried out on cats, rats, rabbits and dogs have shown that only with an intravenous bolus of a high dose of tolperisone can a temporary sharp decrease in blood pressure occur. A more prolonged slight decrease in blood pressure is observed with the use of large doses of the drug (mg / kg).

In a study of dogs with bradycardia, tolperisone slightly increased the heart rate due to increased vagal tone.

Tolperisone selectively and significantly increases femoral blood flow in dogs while decreasing mesenteric blood flow. Subsequently, when the experiment was repeated by different methods on a large number of animals, it was found that this effect was due to a direct peripheral vasodilator effect.

After intravenous administration of tolperisone, lymph circulation is enhanced.

The drug does not have a noticeable effect on the ECG picture.

All of the above turns out to be positive when prescribing mydocalm in elderly and even elderly patients suffering from various disorders of the cardiovascular system.

II. Myoclonic syndromes.

Myoclonus is a short jerky jerk of a muscle, similar to its contraction in response to a single electrical stimulation of the corresponding nerve. Myoclonus can be limited to a single (or separate) muscle, or it can involve many muscle groups up to complete generalization. Myoclonic jerks (jerks) can be synchronous or asynchronous, for the most part they are arrhythmic and may or may not be accompanied by movement in the joint. Their severity varies from a barely noticeable contraction to a sharp start, leading to a fall. Myoclonus tends to recur in the same muscles. There are spontaneous and reflex myoclonus provoked by sensory stimuli of various modalities. There are myoclonus triggered by voluntary movement (action and intentional myoclonus). Known myoclonus, dependent and not dependent on the cycle "sleep - wakefulness".

The pathophysiological and biochemical mechanisms of myoclonus are not well understood. At the place of generation of myoclonic discharges in the nervous system, 4 types of myoclonus are distinguished:

• cortical;
• stem (subcortical, reticular);
• spinal;
• peripheral.

The first two forms (cortical and stem) have the greatest clinical significance; they are more common than the others. The presented classification is a modification of the old division of myoclonus into pyramidal, extrapyramidal and segmental forms.

The involvement of serotonergic mechanisms in the pathogenesis of myoclonus is assumed. Among patients, there are even subgroups that are amenable to successful treatment by directly opposite means: some patients respond to agonists, the other to serotonin antagonists.

Since a large number of diseases, nosological units can be accompanied by myoclonic hyperkinesis, several classifications of myoclonus according to the etiological principle have been proposed. Marsden's classification (1987) identifies 4 groups of myoclonus:

• physiological myoclonus;
• essential myoclonus;
• epileptic myoclonus;
• symptomatic myoclonus.

Examples of physiological myoclonus are myoclonus of falling asleep and awakening, myoclonus of fright, and some myoclonus in the form of hiccups. They usually do not require special treatment.

Essential myoclonus is familial as well as sporadic myoclonus, the so-called nocturnal myoclonus. Manifested in the phase of slow sleep in patients with chronic insomnia. Amenable to therapy with clonozepam, valproate, baclofen when using small doses (one tablet at night). Familial and sporadic myoclonus is a rare disease called essential myoclonus or Friedreich's multiple paramyoclonus. The disease debuts in the first or second decade of life and is not accompanied by other neurological, mental and electroencephalographic disorders. Clinical manifestations include irregular, arrhythmic and asynchronous twitching and flinching with a generalized distribution of myoclonus. Treatment is ineffective. Clonazepam and valproate are used.

Epileptic myoclonus is myoclonus in the picture of an epileptic seizure, where they sometimes become one of the leading manifestations. There is a separate form of epilepsy - myoclonus-epilepsy, considered as even a hereditary disease that manifests itself in childhood.

Symptomatic myoclonus, most likely for the elderly and senile age, is observed in a number of metabolic disorders, such as renal, hepatic or respiratory failure, alcohol intoxication, withdrawal of certain drugs, as well as in diseases occurring with structural damage to the brain (without epileptic seizures), such as epidemic encephalitis, Creutzfeldt-Jakob disease, subacute sclerosing leukoencephalitis, postanoxic brain damage. The list of symptomatic myoclonias can be significantly expanded to include storage diseases (including Laforte's corpuscular disease, sialidosis), paraneoplastic syndromes, toxic, including alcoholic, encephalopathy, with focal damage to the nervous system (angioma, ischemic or traumatic defect, stereotactic thalamotonia) , as well as myoclonus as a side non-obligatory symptom of other diseases (lipidosis, leukodystrophy, tuberous sclerosis, spinocerebellar degeneration, Wilson-Konovalov disease, myoclonic dystonia, Alzheimer's disease, progressive supranuclear palsy, Whipple's disease). Progressive myoclonus epilepsy can, in principle, also be classified as symptomatic variants of myoclonus (based on epilepsy). The nosological independence of Ramsey-Hunt cerebellar myoclonic dyssynergy is also disputed. Remains in use only Ramsey-Hunt syndrome, which is equated as a synonym for myoclonus-epilepsy syndrome, Unferricht-Lundborg disease ("Baltic myoclonus", progressive myoclonus epilepsy). It seems to us necessary to dwell on the description of this pathology presented in the work of the Italian authors C.A. Tassinari et al. (1994).

Unferricht-Lundborg disease is a form of progressive myoclonus epilepsy. This disease was better known in Finland traditionally as Baltic myoclonus. In recent years, an identical disease has been described in populations of southern Europe - "Mediterranean myoclonus", or "Ramsey Hunt syndrome". In both populations, the disease has the same clinical and neurophysiological features: onset, appearance of active myoclonus, rare generalized seizures, mild symptoms of cerebellar failure, absence of severe dementia, slow progression; EEG reveals normal bioelectrical activity and generalized fast wave activity of the "peak" and "polypeak" type. The conducted molecular genetic study showed the genetic unity of the disease in both populations: the localization of the defective gene on chromosome 22q22.3 was determined. However, in 3 out of 6 Italian families, the disease had features of atypicality - more rapid progression with dementia, the presence of occipital spikes on the EEG, which makes it closer to Lafor's disease. In this regard, it is possible that the “Mediterranean myoclonus” is a heterogeneous syndrome.

The diagnostic criteria for Unferricht-Lunborg disease are highlighted:

1. onset between 6 and 15, less often 18 years;
2. tonic-clonic seizures;
3. myoclonus;
4. EEG paroxysms in the form of spikes or polyspike-wave complexes with a frequency per second;
5. progressive course.

Some clinical forms of myoclonus:

Posthypoxic encephalopathy, in which the main manifestations are intentional and action myoclonus (Lanze-Adams syndrome), sometimes in combination with dysarthria, tremor and ataxia.

Myoclonus of the soft palate (bicycle-palatine myoclonus - nystagmus of the soft palate, myorrhythmia) - usually rhythmic, per second, contractions of the soft palate, often in combination with hyperkinesis almost indistinguishable from tremor in the tongue, lower jaw, larynx, diaphragm and in the distal parts of the arms (classical myorhythmia, or "skeletal myoclonus", as defined by the old authors); myorhythmia disappears during sleep, it can be either idiopathic or symptomatic (tumors in the pons and medulla oblongata, encephalomyelitis, trauma), sometimes ocular myoclonus of the "rocking" type joins. It is suppressed not only by clonazepam, like most myoclonus, but also by finlepsin (tegretol, stazepine, mazepine, carbamazepine).

Spinal (segmental) myoclonus: rhythmic, from a minute to 10 per second; independent of external stimuli. The reasons lie in local damage to the spinal cord (myelitis, tumor, trauma, degeneration).

Opsoclonus (dancing eye syndrome) is a fast, jerky, chaotic movement of the eyeballs. Increased hyperkinesis can sometimes occur explosively. Continues during sleep and even worse on awakening. Opsoclonus is often mistaken for nystagmus, which is always characterized by the presence of two successively advancing phases - slow and fast. Opsoclonus indicates an organic lesion of the cerebellar-stem connections in tumors of the brain stem and cerebellum, paraneoplastic syndromes, hemorrhages, severe trauma, metabolic and toxic encephalopathies in the final stage, multiple sclerosis and in some other conditions. Viral encephalitis and meningoencephalitis are often the "culprits" of opsoclonus. In children and people over 40, the likelihood of neuroblastoma is high. Treatment is carried out with adrenocorticotropic hormone, corticosteroids, obzidan, benzodiazepine derivatives.

Myokymia of the superior oblique muscle of the eye ("unilateral rotator nystagmus"); patients themselves feel characteristic molecular oscillations ("objects jumping up and down", "wobbling eyes", etc.) and torsion diplopia. The course is benign. There is a good therapeutic effect from finlepsin.

Hyperexlexia and Leaping Frenchman of Maine. Hyperexlexia - pathologically increased involuntary startle, sometimes leading to the fall of the patient, arising in response to unexpected tactile, light or sound stimuli. Sometimes it is an independent hereditary disease, and sometimes it is secondary, like the syndrome in diseases of Little, Creutzfeldt-Jakob, vascular lesions of the brain. With the syndrome of the "jumping Frenchman of Maine" the frequency of jumping paroxysms reaches once a day. Many are accompanied by falls and bruises, but without loss of consciousness. Helps clonozepam.

Hiccups are myoclonic contractions of the diaphragm and respiratory muscles. It can be physiological (after a plentiful meal), a symptom in diseases of the gastrointestinal tract, chest organs, irritation of the phrenic nerve, with damage to the brain stem or upper cervical segments of the spinal cord. Hiccups can be both toxicogenic and psychogenic. Treatment is carried out with antipsychotics, antiemetics (cerucal, for example), clonazepam, finlepsin, psycho and physiotherapy, even transection of the phrenic nerve.

III. Other hyperkinetic syndromes.

The described syndromes include, first of all, episodes of tremors and muscle cramps. In terms of the clarity and "picture" of their clinical manifestations, both tremor and some convulsions to some extent occupy an intermediate place between muscular dystonia and myoclonus, often including elements of both.

Muscle cramps denote involuntary and painful contractions that occur spontaneously or after exercise. A prerequisite for the development of muscle cramps is the absence of a regulatory response from antagonistic muscles. With the tension of the antagonist muscles, reciprocal blocking of seizures occurs, but such blocking is also possible with the use of cutaneous efferent endings.

Histologically, in the painfully contracting muscles, a large number of muscle fibers depleted in glycogen and single myolysis are found; this shows that cramps do not go away without leaving a trace, but affect the structure of the muscles. Finds of this kind are partially comparable with the "syndrome of prolonged activity of muscle fibers" described by H. Isaacs and with other, less common syndromes, including those that develop with repeated irritation of peripheral nerves.

Often, muscle cramps and fascicular twitching are the first symptoms of general somatic disorders: anomalies in electrolyte metabolism and metabolic disorders, including endocrine diseases, chronic inflammatory processes, and malignant tumors. Other reasons may be drug abuse (for example, nicotine and caffeine), various kinds of toxicosis, including medication. Hereditary nocturnal muscle cramps have also been described.

Diseases of the peripheral nerves and central nervous system can lead to muscle cramps. Convulsions can also occur when water-electrolyte metabolism is disturbed. Compression of muscle fibers due to edema plays a significant role in the origin of convulsive pain. The pain disappears immediately when the muscle fascia is cut. A similar mechanism can take place in ischemic convulsions of the calf muscles, a predominantly sedentary lifestyle of most people, in which practically no muscle is involved. In peoples for whom it is common to squat, when the muscles are under relatively heavy load, cramps in the leg and other muscles are rare.

Certain medications are able to induce muscle cramps or increase convulsive readiness. Any attempt to isolate certain groups of drugs, especially those affecting the metabolism in the muscles, affecting electrolytes or the function of sarcolemma and thereby predisposing to the development of muscle cramps, was practically unsuccessful, since the effect of medications is usually very multifaceted.

Muscle cramps with tetanus are characteristic. But it must be remembered that in this case, muscle cramps are often complicated by changes in tendons up to calcification (shoulder, elbow and hip joints are most susceptible to this).

Among the endocrine diseases that can occur with characteristic muscle cramps, hypothyroidism should be mentioned.

The increased excitability and rigidity of all muscles of the neck, upper limbs and face in the patient were described by H. Mertens and K. Ricker as “spindle myotonia”. The picture of the disease is in many ways similar to the stiff-man syndrome, which occurs sporadically in adults, described by F. Moersch and H. Woltman.

Very interesting is the Schwartz-Jampel syndrome, or myotonic chondrodystrophy, which refers to pseudomyotonia. Electromyography (EMG) with this disorder reveals characteristic explosive, irregularly repeated discharges, similar to high-frequency ones.

With neuromyotonia, persistent muscle contractions can spontaneously develop covering the trunk and face. In this state, only slow active movements are possible. With both passive and active movements, muscle rigidity first increases and then weakens. On EMG, irregular bursts of activity, post-discharges, increased insertional activity (developing in response to the introduction of an electromyographic needle) are noted.

Myotonic syndromes, characterized by prolonged muscle contractions, can occur in response to their mechanical, electrical or other sufficiently strong activation.

Here are some of the most common muscle cramps syndromes.

Crumpy: These are painful muscle spasms, primarily the muscles of the lower leg, as well as the abdomen, chest, back, and less often the arms and face. Most often we are talking about the triceps muscle of the lower leg. They occur after physical exertion, occurs in various diseases, including an autosomal dominant variant of non-progressive common crampi with minimal anterior limb insufficiency; observed in amyotrophic lateral sclerosis, peripheral neuropathies, pregnancy, dysmetabolism. Quite often, crumpy occurs in patients with lumbar osteochondrosis and in this case has the following features:

1. typical for the stage of remission and almost never occurs in the acute period;
2. not being epileptic in nature, this local convulsive phenomenon is still common in individuals with residual non-severe cerebral insufficiency;
3. it is characterized by local pathology, most often in the form of popliteal neuroosteofibrosis;
4. it is caused by neurogenic mechanisms and humoral changes - hyperacetylcholinemia, hyperserotoninemia (Popelyansky Ya.Yu.).

Like hypercalcemic, thyrotoxic and others, cramps with osteochondrosis are more common in the elderly and occur at night, in warmth, in a state of rest, i.e. in conditions that promote rapid and intense muscle contraction. A sudden shortening of a muscle is accompanied by an increase in its diameter, induration (the muscle becomes sharply defined), and severe pain. Possible explanations for such pain lie partly in the biochemical plane (the release of the corresponding substances), partly in the electrophysiological (sudden loss of gate control, local discharge, the formation of a generator of pathological excitation). Clonazepam is effective.

Tics, facial hemispasm, restless legs syndrome (Ekbema), iatrogenic dyskinesias. Tic generalized hyperkinesis is often combined with obsessive-compulsive disorders, which, in principle, determines the clinical picture of Tourette's syndrome accompanying various organic brain lesions. This syndrome must be differentiated from an independent nosology - Tourette's disease, which is hereditary. There are several points of view on the biochemical basis of Tourette's syndrome. Pfeifer C.C. et al. (1969) wrote about the deficiency of the enzyme hypoxanthine-guanine-phosphoribosyl-transferase, which is involved in the metabolic cycle of uric acid formation and is contained in the maximum concentration in the basal ganglia. P.V. Melnichuk et al. (1980) associate the syndrome under consideration with metabolic disorders of catecholamines. But one way or another, today in the treatment of tic hyperkinesis, the drug of choice is primarily haloperidol in a dose of 0.25 - 2.5 mg, prescribed before bedtime, and sometimes additionally in the daytime. Efficiency reaches even with Tourette's syndrome or disease% (Karlov V.A., 1996). The agent of the second stage is pimozide at 0 mg per day. Elderly patients should be prescribed the drug with caution and under ECG control, since the P - Q interval is prolonged. Clonazepam and reserpine are effective, but these drugs are still not as "successful" as antipsychotics.

Obsessive-compulsive disorders are well treated with antidepressants that inhibit serotonin reuptake. Monoamine oxidase inhibitors, tricyclic antidepressants (amitriptyline, imipramine) can be used. Psychostimulants can also be shown: meridil, sydnocarb, but they increase tic hyperkinesis. In recent years, the antidepressant fluoxetine (serotonin inhibitor) has been successfully used at a dose of mg per day, deprenyl pomg per day (Karlov V.A., 1996).

Tremor. With its non-Parkinsonian origin (essential, alcoholic, thyrotoxic, post-traumatic tremor), we are talking about tremulous hyperkinesis manifested during movement. If parkinsonian tremor is associated with dopaminergic insufficiency, then non-parkinsonian variants of tremor are based on the principle of excessive functioning of adrenergic and, possibly, GABAergic neurons. It is possible that there is also a violation of the stability of cell membranes, since anaprilin, which has a maximum effect in tremor, has a pronounced membranostatic effect (Elison P.H., 1978; Karlov V.A., 1996). Anaprilin (propranolol) sometimes gives pronounced allergic manifestations, even bronchospasm, therefore it is contraindicated for patients suffering from bronchial asthma or other allergies. In this case, the drug can be replaced with metoprol, oxprenolol (trazicor), atenolol. Doses of beta-blockers for anaprilin are mg per day. For the elderly and senile age, small dosages are advisable, since it is easier than in young people, side effects such as depression, sleep disturbances, even toxic psychosis and hallucinosis occur. In many patients, hexamidine (primidene) and clonazepam are effective. Use leponex, isoniazid.

IV.Headaches.

Headache is one of the most frequent complaints with which patients turn to a doctor of any specialty. According to statistical studies by various authors, the frequency of headaches ranges from 50 to 200 per 1000 population. Headache is the leading syndrome or symptom in more than 45 different diseases (Stock V.N., 1987). The problem of headache is so urgent that various specialized centers have been created to study it. The European Association for the Study of Headache has been organized, since 1991 the Russian Association has been a member of it. The work of the Association is coordinated by the Russian Center for Headache, created on the basis of the Moscow Medical Academy. THEM. Sechenov.

Attempts have been made repeatedly to classify headaches. In our country, the pathogenetic classification of headache presented by V.N. Stock and his famous monograph (1987). The author identifies 6 main types of headaches:

1. vascular;
2. muscle tension;
3. liquorodynamic;
4. neuralgic;
5. mixed;
6. psychhalgia (central).

Each variant has its own characteristic pathophysiological mechanism of headache. The author of this classification defends the concept of isolation of one of these headache variants in each patient, while the mixed variant is considered a rare exception to the rule. As practice shows, this kind of approach is far from always correct (Myakotnykh V.S., 1994), especially in patients with polietiological, polypathogenetic nature of the pathological process, one of the clinical manifestations of which is headache.

In elderly and senile people, in the process of accumulation of various diseases, headache is undoubtedly mixed, combined, including various pathophysiological mechanisms of occurrence.

In 1988, the international classification committee proposed the most complete classification of headache, which, however, is not final and continues to be improved, supplemented, and specified. The classification considers the following forms of headaches:

• migraine:
  1. without aura (simple form);
  2. with an aura (associated).

  In the latter, various forms are distinguished depending on the local symptomatology that occurs when the pathological focus is localized in a particular vascular basin;

• tension headaches (synonyms: psychalgia, psycho-myogenic, neurotic); are subdivided into episodic and chronic, with or without involvement of the muscles of the scalp and (or) neck in the pathological process;
• cluster or cluster headaches;
• chronic paroxysmal hemicrania;
• headaches caused by vascular;
• infectious;
• tumor processes;
• traumatic brain injury, etc.

Very interesting and to a certain extent unusual, uncharacteristic for most other types of pathology, is the fact that some variants of headaches, in particular migraine, can be considered as a syndrome or even a symptom of a disease (there are even the terms "migraine" or " migraine-like "syndrome), and as an independent nosological unit. Perhaps this contributed to the fact that until now there is no consensus about the frequency of occurrence of migraine, since some people put only an independent disease into this concept, and others - a variant of a syndrome or even a symptom.

In addition, absolutely reliable diagnosis of a particular type of headache is a difficult task. If we proceed from the classification of 1988 and subsequent years, it may seem that the simplest thing is to diagnose a headache "tied" to any specific pathology - vascular, infectious, tumor, traumatic, etc. To a certain extent, this is so, but only after the diagnosis of the "background" disease for headache has already been made. Therefore, the very factor of the presence of a headache in a patient from the very beginning should set the doctor up to diagnose the pathology in which the headache acts as a symptom or syndrome. By this, the last part of the classification is "cut off", and the first part remains, where the diagnosis of the nature and clinical-pathogenetic, clinical-pathophysiological variant of headache is carried out.

The most interesting in both clinical and pathophysiological aspects are probably the first three types of headache: migraine (occurs in the population with a frequency of 3 to 30% according to various authors); cluster or beam (frequency of occurrence from 0.05 to 6%); tension headaches (occur in%, and among other forms of headache in women - up to 88%, in men - up to 69%). There are a number of similarities that these three forms of headache have in common:

• They are all psychogenic in nature;
• Most represented in the population among other forms of headaches;
• Paroxysmal flow is characteristic.

Determined by the sufficient severity of emotional and personal changes, although different in quality: migraine - the predominance of anxious, demonstrative features, a high level of claims, low stress resistance; tension headache - depressive-hypochondriacal, demonstrative character traits; cluster headache - “lion and mouse” syndrome (outwardly courageous, ambitious, ambitious, and internally timid and indecisive), with the presence of psychomotor agitation during the period of paroxysm.

The presence of clinical autonomic disorders is significant. The maximum vegetative disturbances are presented in "panic migraine", when at the height of the typical form of migraine there are signs of a panic attack (emotional agitation, fear, chill-like hyperkinesis, etc.).

There is a significant number of observations of muscular-tonic syndrome in the neck muscles (palpation or according to the results of electroneuromyography). With migraine, this syndrome is predominantly expressed on the side of hemicrania.

The proximity of subjective severity - the intensity of pain in paroxysm. According to the visual analogue scale (VAS): migraine - 78%, tension headache - 56%, cluster headache - 87%.

An important criterion is the quality of life. It reflects the degree of adaptation of patients with the named forms of headache, determines the degree of their activity, efficiency, fatigue, mood changes, and the effectiveness of the activities performed. Quality of life also includes an assessment of the patient's understanding and support from a loved one. The maximum decrease in the quality of life in patients with tension headache - up to 54%, with migraine - up to 70%, with cluster headache (during an attack) - up to 86%.

Some similarity of disturbances in the interaction of nocic and antinociceptive systems in patients with migraine and tension headache at the level of the brainstem systems. This was revealed as a result of special biochemical and electrophysiological studies.

Thus, with the described forms of headaches, there is a certain psycho-vegetative-motor pattern accompanying painful paroxysm. This served as the basis for the use for the treatment of headaches not only widely known and described in the numerous literature means, but also psychotropic drugs and anticonvulsants. For migraine, for example, phenobarbital, finlepsin, diphenin (Karlov V.A., 1987), cepra (Shershever A.S. et al., 2007) are widely used. Anticonvulsants reduce the pain sensitivity of the vascular wall, enhance antinociception at the level of the stem systems. For cluster headaches, sodium valproate is used, which is a GABA mimetic and acts on interneurons of the hypothalamus, thereby affecting circadian rhythms, the violation of which is one of the main pathogenetic links in cluster cephalgias. Finlepsin can be used in combination with other analgesic, vascular drugs, sedatives.

For migraines and tension headaches, tricyclic antidepressants are used, especially amitriptyline, which is due to the presence of psychovegetative and psychomotor clinical manifestations in paroxysms. The use of alprozolam (cassadan) turned out to be quite effective, especially for headaches of neurotic or partially neurotic genesis. Since this drug has an anoxiolytic, antidepressant, muscle relaxant effect, affects the GABAergic system, it can be used for the following types of headaches: panic migraine, combined migraine plus tension headaches, mainly episodic tension headaches with muscle dysfunction.

Of interest is the question of whether it is possible and how often it is possible to combine several variants of headache in one patient and whether it is possible to change, or even "kaleidoscopic" (constant change of variants with periodic repetitions) in the same patient. In this case, of course, two more questions often arise - what is this connected with and how can the therapeutic problems be solved in this case?

From the indicated positions, we can consider two main options for the clinical "change of scenery":

1. one patient has several variants of one type of headache at the same time, for example, several variants of migraine attacks;
2. one patient has several types of headache.

Perhaps the most fully and distinctly described are the various variants of migraine. Let us once again give the main ones.

1. Simple form (no aura).
2. Associated form (with aura).

In the latter form, a number of clinical variants can be distinguished, depending on the clinical picture of the aura (ophthalmic, ophthalmoplegic, olfactory, illusory, vestibular, etc.).

V. Vegetative disorders.

According to epidemiological studies, up to 80% of the population experience some type of autonomic disturbance. This is due to the key role of the autonomic nervous system in such basic processes as maintaining homeostasis and adapting to changing environmental conditions. Events and situations of both biological and psychosocial nature can lead to a disruption of autonomic regulation, which is clinically manifested in the form of autonomic dysfunction or autonomic dystonia syndrome. In our opinion, it is completely wrong to believe that vegetative-dystonic manifestations become less pronounced with age than in young people, and the total number of patients suffering from neurocirculatory or vegetative-vascular dystonia drops sharply. It seems to us, on the contrary, that the number of patients with dystonic, vegetative-vascular pathological manifestations in old and senile age is increasing, but this pathology is moving from the category of nosology or syndromology to predominantly symptomatic aspects. In the first place as an independent disease or syndrome are various clinical variants of atherosclerosis, arterial hypertension, pathological processes in the gastrointestinal tract, urinary, endocrine systems, osteochondrosis, finally. All these diseases can be clinically represented by vegetative-dystonic disorders, but these disorders are no longer perceived as syndromes, not as independent diseases, but as one, two or more symptoms of more severe pathological processes. This does not mean at all that in old and senile age the problem of vegetative-vascular dystonia is absent or at least goes to the second, third plans. After all, if we cannot completely stop the development of atherosclerosis, for example, then it would be wrong to completely abandon symptomatic treatment; the patient is not worried about the disease, as such, he is worried about the manifestations of this disease. And therefore, in the elderly very often therapy can and should be directed precisely at manifestations that level the quality of life of our patients. Within the framework of the syndrome of vegetative dystonia, it is customary to distinguish 3 groups of vegetative disorders (Wayne A.M., 1988):

• psycho-vegetative syndrome;
• progressive autonomic failure syndrome;
• vegetative-vascular-trophic syndrome.

In some cases, autonomic disorders are constitutional in nature, manifesting already from early childhood or from puberty, but in most patients they develop secondarily, within the framework of neuroses, psychophysiological reactions, against the background of hormonal changes, organic somatic, neurological diseases, and endogenous mental disorders.

The group of psycho-vegetative disorders should be especially highlighted, which are most common and manifest clinically in the form of emotional disorders in combination with polysystemic vegetative disorders (cardiovascular system, respiration, gastrointestinal tract, thermoregulation, sweating, etc.). These disorders can occur in the form of permanent, paroxysmal, permanent-paroxysmal disorders. The most obvious and striking representatives of autonomic disorders in this group are vegetative crises (panic attacks) and neurogenic syncope (syncope).

Panic attacks are the most dramatic manifestation of autonomic dystonia syndrome (Wayne A.M. et al., 1994). Many terms have been proposed that denote apparently identical conditions: diencephalic crises, cerebral autonomic seizures, hyperventilation attacks, anxiety attacks, etc. It seems to us necessary, therefore, when considering panic attacks, at least briefly dwell on the problem of vegetative-vascular dystonia

For many years, vegetative-vascular dystonia was considered either within the framework of neuroses, or as a pathology of the autonomic nervous system, or as the initial form of other diseases, for example, arterial hypertension, cerebral atherosclerosis. Nevertheless, vegetative-vascular dystonia is an independent form of pathology, which, in essence, etiopathogenetic relations is a functional disease of polyetiological genesis, manifested mainly by vascular and veterative disorders.

Consider the chain of pathophysiological and biochemical reactions that occur in vegetative-vascular dystonia. The most important, perhaps, is the question of the formation of functional hypoxia of the brain. Several mechanisms are important in its occurrence: hyperventilation as a manifestation of the sympathotonic effect, followed by the vasoconstrictor effect of the microvasculature. There is a direct vasoconstrictor effect due to an increase in the level of adrenaline, norepinephrine and cortisol (as a nonspecific effect of stress activation), followed by a decrease in the maximum oxygen consumption, a decrease in metabolism and a slowdown in lactate utilization. Finally, there is a change in the rheological properties of blood (increased viscosity, aggregation properties of erythrocytes and platelets), the affinity of hemoglobin to oxygen, which, in combination with microcirculation disorders, aggravates the level of brain hypoxia. With emotional stress, the body's need for energy supply increases, which is compensated mainly by increasing lipid metabolism.

Lipid peroxidation processes play an important role in the development of adaptation diseases associated with stress and, in particular, diseases of the cardiovascular system. Many authors in their works indicate the activation of lipid peroxidation in gastric ulcer and duodenal ulcer, with neurodermatitis and diabetes. In experiments on animals, in response to severe stress, lipid peroxides accumulated, which led to damage to body tissues, and the introduction of antioxidants in this case inhibited the development of stress-induced disorders of internal organs with a sharp decrease in the release of corticosteroid hormones. Dependences between the activity of lipid peroxidation and clinical features of neurotic disorders were revealed. It is obvious that microcirculation disorders and brain hypoxia are the intermediate link that transforms psychogenic effects into a stable pathological state of the brain. This dictates the need to include in the therapeutic complex drugs used in the treatment of neuroses and, in particular, vegetative-vascular dystonia, which, in addition to affecting the listed biological targets (aggregation properties of blood, microcirculation disorders, oxygen metabolism and lipid peroxidation of biological membranes), ruptured would be a chain of pathological adaptive reactions to anxiety and indirectly reduce the severity of emotional stress.

Since 1980, with the advent of the American Classification of Mental Illness (DSM - III), the term "panic attack" has been established in international practice to denote paroxysmal conditions with polysystemic autonomic, emotional and cognitive disorders. These conditions are included in the broader class of "alarm conditions". The main criteria for distinguishing panic attacks are:

• recurrence of seizures;
• their occurrence outside of emergency and life-threatening situations;
• attacks are manifested by a combination of at least 4 of the 13 following symptoms:
  • dyspnea;
  • "Pulsation", tachycardia;
  • pain or discomfort in the left side of the chest;
  • a feeling of suffocation;
  • dizziness, unsteadiness, feeling of impending fainting;
  • feeling of derealization, depersonalization;
  • nausea or abdominal discomfort;
  • chills;
  • paresthesia in the arms and legs;
  • feeling of "hot flashes", "waves" of heat or cold;
  • sweating;
  • fear of death;
  • fear of going crazy or committing an uncontrollable act.

Panic attacks occur in 1 - 3% of the population, twice as often in women and mainly between the ages of 20 and 45, although in menopause they are also far from uncommon. The clinical picture of suffering is represented by paroxysms, the core of which is the aforementioned symptoms. However, it was noted that a number of patients at the time of an attack do not have a feeling of fear, anxiety ("panic without panic", "fearless attacks"), in some patients emotional manifestations may consist in a feeling of melancholy or depression, in others it is irritation, aggression or just an inner tension. In the majority of patients in an attack, functional neurotic symptoms are present: lump in the throat, pseudoparesis, speech and voice disorders, convulsive phenomena, etc. Attacks can occur both spontaneously and situationally, in some patients they develop at night, during sleep, often accompanied by unpleasant, disturbing dreams. The latter often precede the deployment of the attack at the moment of waking up, and after the end of the panic attack, they amnesia in whole or in part. With the repetition of paroxysms, a feeling of their anxious expectation is formed, and then the so-called avoidance behavior. The latter, in its extreme version, acts as an agoraphobic syndrome (patients become completely maladapted, cannot stay at home alone, move unaccompanied along the street, urban transport is excluded, etc.). In 30% of cases, the recurrence of panic attacks leads to the onset and development of depressive syndrome. Hysterical and hypochondriacal disorders are not uncommon.

Syncope (neurogenic syncope). The generalized concept of fainting is as follows: "Fainting is a short-term impairment of consciousness and postural tone due to reversible disturbances of cerebral functions with spontaneous recovery."

Fainting occurs in 3% of the population, however, at puberty, the frequency of recurrent syncope can reach 30% (Wayne A.M. et al., 1994). There is no unified classification of syncope yet, but all researchers of this problem distinguish 2 main groups of syncope:

1. neurogenic (reflex),
2. somatogenic (symptomatic).

The first include:

• vasodepressor syncope;
• orthostatic syncope;
• sinocarotid;
• hyperventilating;
• tussive;
• nocturic;
• fainting on swallowing and lingo-pharyngeal neuralgia.

Among the second group of syncope are:

• associated with cardiac pathology, where a violation of cardiac output occurs due to a disturbance in the rhythm of the heart or a mechanical obstruction to blood flow;
• associated with hypoglycemia;
• associated with peripheral autonomic failure;
• associated with the pathology of the carotid and vertebro-basilar arteries;
• associated with organic damage to the brain stem;
• hysterical pseudosyncopes, etc.

The clinical picture of syncope is rather stereotyped. The syncope itself usually lasts from a few seconds to 3 minutes; the patient turns pale, muscle tone decreases, mydriasis is noted with a decrease in the reaction of the pupils to light, a weak, labile pulse, shallow breathing, a decrease in blood pressure. With deep syncope, there may be several tonic or clonic-tonic twitchings, involuntary urination and defecation.

Allocate pre- and post-fainting symptoms.

Lightheadedness (lipotimia), lasting from a few seconds to 2 minutes, is manifested by feelings of lightheadedness, nausea, general discomfort, cold sweat, dizziness, blurred vision, muscle weakness, tinnitus, and a feeling of fading consciousness. At the same time, a number of patients develop fear, anxiety, palpitations, a feeling of lack of air, paresthesia, "lump in the throat", ie. panic attack symptoms. After an attack, the patients quickly come to their senses, although they are alarmed, pale, there is tachycardia, general weakness.

Most patients clearly distinguish factors that provoke fainting: stuffiness, prolonged standing, getting up quickly, emotional and pain factors, transport, vestibular stress, overheating, hunger, alcohol, lack of sleep, premenstrual period, getting up at night, etc.

Some aspects of the pathogenesis of panic attacks and syncope can be very similar and at the same time have distinct differences. There are psychological and biological aspects of pathogenesis. From the point of view of psychophysiology, syncope is a pathological reaction resulting from anxiety or fear in conditions when physical activity (fight or flight) is impossible. A panic attack from the point of view of psychodynamic concepts is a signal to the "ego" about the threat of repressed, unconscious impulses to mental balance. A panic attack helps the ego prevent the unconscious aggressive or sexual impulse from spilling out, which could lead to more serious consequences for the individual.

The biological factors of the pathogenesis of syncope and panic attacks are currently being actively studied. Physiological mechanisms of realization of these two states are to a certain extent opposite. In patients with syncope due to sympathetic insufficiency (especially in the sympathetic postganglionic fibers of the lower extremities), active vasodilation occurs, which leads to a decrease in cardiac output. In case of panic attacks, on the contrary, vascular insufficiency was found, in favor of which is evidenced by:

1. the development of spontaneous panic attacks during the relaxation period;
2. a sharp increase in heart rate in a short period of time;
3. decrease in the content of adrenaline, norepinephrine in serum in the pre-crisis period;
4. characteristic changes in the oscillatory structure of the heart rhythm (detected by cardiointervalography, for example).

When studying the central mechanisms of the pathogenesis of mainly panic attacks, the direct relationship of the noradrenergic nucleus of the brain stem to anxious behavior has been shown. It is no coincidence that drugs that affect the noradrenergic systems - tricyclic antidepressants and monoamine oxidase (MAO) inhibitors - have become so widespread in the treatment of panic attacks. The role of serotonergic systems in the pathogenesis of panic attacks is widely studied. The result is the creation of a large group of drugs, the action of which is directed to these systems - clomipramine, zimeldine, fluvoxamine, fluvoxetine.

Particular interest is shown in biochemical systems associated with the functions of excitation and inhibition - glutamatergic and GABAergic. These systems play a key and opposite role in realization as anxiety; and paroxysmal. In this regard, it seems appropriate to summarize the main clinical and experimental data indicating the proximity of paroxysmal vegetative states and epilepsy:

There are a number of common provoking factors - hyperventilation, inhalation of carbon dioxide;

Both spontaneous panic attacks and epileptic seizures occur more often during the period of relaxed wakefulness, often in the stage of slow wave sleep. 2/3 of patients with panic attacks react to sleep deprivation, including electroencephalographically, similarly to patients with epilepsy;

In patients with fainting, paroxysmal activity on the EEG and a decrease in the seizure threshold, as well as asymmetric interest in the deep temporal formations, which is typical for patients with epilepsy, are often recorded;

Relatives of patients suffering from panic attacks or fainting often have typical epileptic seizures;

Vegetative crises can often be risk factors for the subsequent occurrence of epileptic seizures, especially in adults (Myakotnykh V.S., 1992);

The therapeutic activity of antiepileptic drugs (anticonvulsants) is high in patients with fainting and panic attacks.

Treatment of vegetative paroxysms.

Until the mid-1980s, antidepressants dominated the treatment of panic attacks. Tricyclic antidepressants (imipramine, amitriptyline, etc.), MAO inhibitors (phenelzine), four-cyclic antidepressants (mianserin, pyrazidol) were considered basic drugs. But the side effects turned out to be significant, there were problems with increasing the dose, the obvious first effect appeared only after a day, while an exacerbation of the disease was observed - anxiety intensified, seizures became more frequent. The patients also had an increase in blood pressure (BP) and persistent tachycardia, decreased potency, weight gain.

Now the emphasis in drug treatment has shifted to a group of drugs that primarily affect the GABAergic systems. Benzodiazepines are exogenous ligands of benzodiazepine receptors mediated by GABA. There are at least 2 types of central benzodiazepine receptors (BDR): BDR-1, responsible for the anti-anxiety and anticonvulsant action, and BDR-2, responsible for the sedative (hypnotic) effect and muscle relaxant effect. The effects of a new generation of drugs (atypical benzodiazepines) are associated with a specific effect on BDR-1, the most famous of which are clonazepam (antelepsin) and alprozole (xanax, cassadan).

Clonazepam gives a distinct anti-panic effect at a dose of 2 mg per day when taken multiple times. The effect of the treatment begins already in the first week. The effectiveness of the drug is up to 84% (Wayne A.M. et al., 1994). Side effects are minimal. The independence of the effect on the duration of the disease and the effectiveness in persons with previous episodes of alcoholic excesses, complaining even of hereditary burden of alcoholism, are specific. To a lesser extent, clonazepam affects the secondary symptoms of panic attacks - depression and agoraphobia, which makes it appropriate to include antidepressants in therapy. At a dose of mg per day, the drug has proven itself well in the treatment of syncope paroxysms, lipotimias and "hot flashes" in the climacteric period.

Alprozolam is effective in panic attacks from 85 to 92%. The effect is in the first week of treatment. The drug relieves anticipation anxiety and normalizes social and family maladjustment. There is also a rather pronounced antidepressant effect, but with agoraphobia, it is still advisable to add antidepressants to the treatment. The drug can be used for long courses of treatment (up to 6 months) and for maintenance therapy, and does not require an increase in doses. The range of doses used is from 1.5 to 10 mg per day, on average mg. It is recommended to take it in divided doses. Major side effects: sedation, drowsiness, fatigue, memory loss, libido, weight gain, ataxia. The drug should not be prescribed to patients with substance abuse and alcoholism, because drug dependence may develop. A gradual reduction in dosage at the end of the course of treatment is recommended.

Finlepsin in recent years has been increasingly used in the treatment of paroxysmal conditions of non-epileptic origin.

I would especially like to mention such a well-known drug as Cavinton (Vinpocetine), Cavinton-Forte. Cavinton, as a drug that optimizes metabolism (neurometabolic cerebroprotector) and cerebral hemodynamics, can be considered as a means that affects the pathogenetic mechanisms of the formation of vegetative-vascular dysfunctions. In addition, a number of works indicate the use of Cavinton for the purpose of targeting anxiety, which is a concomitant symptom of various neurotic manifestations. In addition, Cavinton has a pronounced vegetotropic effect, which consists in reducing the reactivity of the sympathetic division of the autonomic nervous system. All this makes it possible to successfully use this drug in the treatment of neuroses and autonomic dysfunctions.

In the treatment of non-epileptic paroxysmal conditions, physiotherapy and balneotherapy, psychotherapy, acupuncture, and bioenergetic effects are widely used. The methods and duration of exposure are selected strictly individually and do not contradict the appointment of basic drug therapy.

A paroxysmal state is a pathological syndrome that occurs in the course of a disease and can be of leading importance in the clinical picture. According to V.A. Karlov's definition, a paroxysmal state is a seizure (attack) of cerebral origin, manifested against the background of visible health or with a sudden deterioration of a chronic pathological condition, characterized by a short duration, reversibility of emerging disorders, a tendency to repetition, stereotype. A wide variety of clinical manifestations of paroxysmal conditions is due to their polyetiology. Despite the fact that paroxysmal states are manifestations of completely different diseases, in almost all cases common etiopathogenetic factors are found.

In order to identify them, a study of 635 patients with paroxysmal conditions was carried out, as well as a retrospective analysis of 1200 outpatient records of patients observed in outpatient settings for various neurological diseases, in the clinical picture of which paroxysmal conditions (epilepsy, migraine, vegetalgia, hyperkinesis , neuroses, neuralgia). In all patients, the functional state of the central nervous system was studied by the method of electroencephalography (EEG), the state of cerebral hemodynamics by the method of rheoencephalography (REG). A structural and morphological study of the brain was also carried out by the method of computed tomography (CT) and the study of the state of the autonomic nervous system: the initial autonomic tone, autonomic reactivity and autonomic support of physical activity according to the methods recommended by the Russian Center for Autonomic Pathology. In the study of the psychological status of patients with paroxysmal conditions, the questionnaires of G. Aysenk, Ch. Spielberger, A. Lichko were used. We studied such indicators as the introversion of patients, the level of personal and reactive anxiety, the type of personality accentuation. A study was made of the concentration of catecholamines and corticosteroids (17-KS, 17-OCS) in the urine, as well as the state of the immune status: the content of T– and

B-lymphocytes, immunoglobulins, circulating immune complexes (CICs) in the blood. In addition, the state of the energy level in the "channels" of the body was studied by measuring the electrocutaneous resistance at representative points according to Nakatani, in the modification of VG Vogralik, MV Vogralik (1988).

The results obtained made it possible to identify etiopathogenetic and risk factors that were common for patients with various nosological forms of diseases, in the clinical picture of which the paroxysmal state was the leading one.

Among the etiological factors were common: pathology of the pre- and perinatal periods of development, infections, trauma (including birth), intoxication, somatic diseases.

Common risk factors are such as hereditary predisposition, social conditions (living conditions, nutrition, work, rest), occupational hazards, bad habits (smoking, alcoholism, drug addiction). Of the provoking factors that can cause the development of paroxysmal states, an acute stressful or chronic psychotraumatic situation, heavy physical exertion, disturbances in sleep and nutrition, a sharp change in climatic conditions in connection with moving, unfavorable helium and meteorological factors, strong noise, bright light, strong vestibular irritation (sea rolling, flight in an airplane, long driving in a car), hypothermia, exacerbation of chronic diseases. The results obtained agree with the literature data 2-5.

The study of the functional state of the central nervous system, the autonomic nervous system, the state of cerebral hemodynamics, the nature of pathological changes in the substance of the brain, psychopathological changes in the personality of patients, as well as the nature of the dynamics of immunological and biochemical parameters made it possible to identify a number of common signs characteristic of all patients with paroxysmal conditions. These include:

the presence of pathomorphological changes in the substance of the brain;

common characteristics of EEG and REG indicators, characterized by the predominance of disorganized, hypersynchronous delta, theta, sigma wave activity, symptoms of vascular dystonia, often of the hypertensive type, due to increased tone of cerebral vessels, difficulty in the outflow of blood from the cranial cavity; pronounced autonomic changes with a predominance of parasympathicotonic reactions in the initial autonomic tone, with an increase in autonomic reactivity, more often with excessive autonomic support of physical activity; psychopathological changes, manifested by a tendency to depressive, hypochondriacal states, introversion, a high level of reactive and personal anxiety, the type of personality accentuation was often defined as asthenoneurotic, sensitive, unstable; changes in the concentration of catecholamines and corticosteroids in the urine in all patients with paroxysmal conditions, which tended to increase the content of catecholamines before the onset of paroxysms and their decrease after an attack, as well as to a decrease in the concentration of corticosteroids before a paroxysm and to increase it after an attack.

Immunological indicators are characterized by a decrease in the absolute and relative number of T- and B-lymphocytes, inhibition of natural killer cell activity, functional activity of T-lymphocytes, and a decrease in the content of immunoglobulins A and G in the blood.

The noted common signs in patients with paroxysmal conditions allow us to conclude that there are common etiopathogenetic mechanisms for the development of paroxysmal conditions. The polyethiology of paroxysmal conditions and at the same time the existence of common pathogenetic mechanisms necessitate their systematization.

The conducted research allows us to propose the following classification of paroxysmal conditions according to the etiological principle.

I. Paroxysmal conditions of hereditary diseases

a) hereditary systemic degeneration of the nervous system: hepatocerebral dystrophy (Wilson-Konovalov disease); deforming muscular dystonia (torsion dystonia); Tourette's disease;

b) hereditary metabolic diseases: phenylketonuria; histidinemia;

c) hereditary disorders of lipid metabolism: amaurotic idiocy; Gaucher disease; leukodystrophy; mucolipidosis;

d) hereditary disorders of carbohydrate metabolism: galactosemia; glycogenosis;

e) phakomatoses: Recklinghausen's neurofibromatosis; Bourneville tuberous sclerosis; Sturge-Weber encephalotrigeminal angiomatosis;

f) hereditary neuromuscular diseases: paroxysmal myoplegia; paroxysmal myoplegic syndromes; myasthenia gravis; myoclonus; myoclonus - Unferricht - Lundborg epilepsy;

g) genoin epilepsy.

II. Paroxysmal conditions in organic diseases of the nervous system

a) injuries of the central and peripheral nervous system: post-traumatic diencephalic crises; post-traumatic myoclonus; post-traumatic epilepsy; causalgia;

b) neoplasms of the brain and spinal cord: paroxysmal conditions associated with liquorodynamic disorders; vestibulo-vegetative paroxysms; epileptic seizures;

c) vascular diseases of the nervous system: acute discirculatory encephalopathy; ischemic strokes; hemorrhagic strokes; hyper- and hypotonic cerebral crises; venous cerebral crises; vascular abnormalities; aorto-cerebral crises; vertebrobasilar crises; transient ischemic paroxysms; epileptiform cerebral crises;

d) other organic diseases: paroxysmal myoplegia syndrome of cerebral genesis; periodic hibernation syndrome; Alone's punishment syndrome; pontine myelinosis; juvenile trembling paralysis;

e) neuralgic paroxysms: trigeminal neuralgia; glossopharyngeal neuralgia; neuralgia of the superior laryngeal nerve.

III. Paroxysmal conditions within the psychovegetative syndrome

a) vegetative-vascular paroxysms: cerebral; cardiac; abdominal; vertebral;

b) vegetalgia: Charlen's syndrome; Slader's syndrome; ear node syndrome; anterior sympathetic Glaser syndrome; posterior sympathetic Barre-Lieu syndrome;

c) neuroses: general neuroses; systemic neuroses; paroxysmal conditions in mental disorders: endogenous depression; masked depression; hysterical reactions; affective shock reactions.

IV. Paroxysmal conditions in diseases of internal organs

a) heart disease: congenital defects; heart rhythm disturbances; myocardial infarction; paroxysmal tachycardia; primary tumors of the heart;

b) kidney disease: renal hypertension; uremia; eclampsic (pseudouremic coma); hereditary renal diseases (Schaffer syndrome, familial juvenile nephrophthisis, Albright's osteodystrophy);

c) liver disease: acute hepatitis; hepatic coma; biliary (hepatic) colic; cirrhosis of the liver; calculous cholecystitis;

d) lung disease: lobar pneumonia; chronic pulmonary failure; bronchial asthma; inflammatory diseases of the lungs with the presence of a purulent process; malignant diseases in the lungs;

e) diseases of the blood and blood-forming organs: pernicious anemia (Addison-Birmer disease); hemorrhagic diathesis (Shenlein-Henoch disease, Verlhof's disease, hemophilia); leukemia (tumor or vascular types); lymphogranulomatosis (Hodgkin's disease); erythremia (Vakez disease).

V. Paroxysmal conditions in diseases of the endocrine system

pheochromocytoma; Itsenko-Cushing's disease; Cohn's syndrome; thyrotoxic periodic paralysis; hypoparathyroidism; addison crisis; syndrome of paroxysmal myoplegia of cerebral genesis; climacteric syndrome.

Vi. Paroxysmal conditions in metabolic disorders

hypoxia; hypercapnia; mixed; other metabolic disorders.

Vii. Paroxysmal conditions in infectious diseases

a) encephalitis: acute hemorrhagic encephalitis; epidemic encephalitis (Economo's disease); Japanese encephalitis, mosquito; periaxial Schilder's encephalitis; subacute encephalitis, sclerosing; Creutzfeldt-Jakob disease; neurolupus; neurosyphilis; neurorheumatism (chorea minor);

b) post-vaccination: anti-rabies; small-sized;

VIII. Paroxysmal states with intoxication

alcoholic; Gaie-Wernicke acute alcoholic encephalopathy; poisoning with technical poisons; drug poisoning, including drugs.

Of course, this classification requires further development and refinement.

Distinguish between paroxysmal states with primary and secondary cerebral mechanisms. Primary cerebral mechanisms are associated with hereditary burden for one or another type of pathology or with a gene mutation, as well as with deviations arising during embryogenesis against the background of various pathological influences of the maternal organism. Secondary cerebral mechanisms are formed as a result of pathological exogenous and endogenous effects on the developing organism.

In our opinion, it is necessary to distinguish between the concepts: paroxysmal reaction, paroxysmal syndrome and paroxysmal state. A paroxysmal reaction is a one-time occurrence of paroxysm, the body's response to acute exogenous or endogenous effects. It can be in acute intoxication, a sharp increase in body temperature, trauma, acute blood loss, etc. Paroxysmal syndrome is paroxysms accompanying acute and subacute current disease. These include acute infectious diseases, in the clinic of which convulsive paroxysms, vegetative-vascular crises, the consequences of craniocerebral trauma, diseases of the internal organs, accompanied by paroxysms of various nature (painful, syncope, convulsive, etc.) are observed. Paroxysmal conditions are short-term, suddenly arising, stereotypical paroxysms of a motor, vegetative, sensitive, painful, disomnic, mental or mixed nature, as a rule, constantly accompanying chronic or hereditary diseases, during the development of which a stable focus of pathological hyperactivity has formed in the suprasegmental structures of the head brain. These include epilepsy, migraine, Hunt's cerebellar myoclonic dyssynergy, etc.

At the initial stage of the development of the disease, the paroxysmal reaction performs a protective function, activating compensatory mechanisms. In some cases, paroxysmal conditions are a way to relieve stress in pathologically altered functioning systems. On the other hand, long-term paroxysmal states themselves have pathogenic significance, contributing to the further development of the disease, causing significant disturbances in the activity of various organs and systems.

Literature

Karlov V.A. Paroxysmal conditions in a neurological clinic: definitions, classification, general mechanisms of pathogenesis. In the book: abstracts. report VII All-Russia. Congress of Neurologists. M: JSC "Booklet"; 1995; from. 397.

Abramovich G.B., Taganov I.N. On the significance of various pathogenic factors in the origin of epilepsy in children and adolescents. Journal of Neur and Psychiat 1969; 69: 553-565.

Akimov G.A., Erokhina L.G., Stykan O.A. Syncope neurology. M: Medicine; 1987; 207 s.

Boldyrev A.I. Epilepsy in children and adolescents. M: Medicine; 1990; 318 s.

Karlov V.A. Epilepsy. M: Medicine; 1990; 327 s.

Paroxysms are short-term, sudden-onset and abruptly terminating disorders prone to recurrence. A wide variety of mental (hallucinations, delusions, confusion, anxiety, fear or drowsiness), neurological (convulsions) and somatic (palpitations, headaches, sweating) disorders can occur paroxysmally. In clinical practice, the most common reason the occurrence of paroxysms - epilepsy.

Epileptic and epileptiform seizures - the manifestation of organic brain damage, as a result of which the entire brain or its individual parts are involved in pathological rhythmic activity, recorded in the form of specific complexes on the EEG. Pathological activity can be expressed by loss of consciousness, seizures, episodes of hallucinations, delusions, or ridiculous behavior.

Characteristic signs of epileptic and epileptiform paroxysms:

Spontaneity (lack of provoking factors);

Sudden onset;

Relatively short duration (seconds, minutes, sometimes tens of minutes);

Sudden cessation, sometimes through a sleep phase;

Stereotype and repeatability.

The specific symptomatology of a seizure depends on which parts of the brain are involved in the pathological activity. It is customary to divide seizures into generalized and partial (focal). Generalized seizures, in which all parts of the brain are simultaneously susceptible to pathological activity, are manifested by complete loss of consciousness, sometimes by general convulsions. Patients have no memory of the seizure.

Partial (focal) seizures do not lead to complete loss of consciousness, patients retain separate memories of paroxysm, pathological activity occurs only in one of the brain regions. So, occipital epilepsy is manifested by periods of blindness or flashes, flickering in the eyes, temporal epilepsy - by episodes of hallucinations (auditory, olfactory, visual), damage to the precentral gyrus - by unilateral convulsions in one of the limbs (Jackson seizures).

The partial nature of the seizure is also indicated by the presence of precursors (unpleasant sensations in the body that occur several minutes or hours before the seizure) and aura (a short initial phase of the seizure, which is stored in the patient's memory). Doctors pay special attention to partial seizures because they can be the first manifestation of focal brain lesions, such as tumors.

Seizures are usually classified according to their main clinical presentation.

Epileptic paroxysms include:

Large seizures (grand mal, clonicotonic seizures);

Minor seizures (petit mal, simple and complex absences, myoclonic seizures);

Twilight obscurations (ambulatory automatisms, somnambulism, trances, hallucinatory-delusional variant);

Dysphoria;

Special states of consciousness (psychosensory seizures, attacks of "deja vu" and "jame vu", paroxysms of delusional and hallucinatory structure);

Jacksonian seizures with convulsions in one of the limbs.

Large seizure (grand mal)

manifests itself as a sudden shutdown of consciousness with a fall, a characteristic change in tonic and clonic seizures and subsequent complete amnesia. The duration of the seizure is from 30 seconds to 2 minutes. The patient's condition changes in a certain sequence. The tonic phase is replaced by a clonic one and the seizure ends with the restoration of consciousness, however, somnolence is observed for several hours after that. At this time, the patient can answer simple questions from the doctor, but, left to himself, falls asleep deeply.

In about half of cases, the onset of seizures is preceded by an aura (various sensory, motor, visceral or mental phenomena, extremely short-lived and the same in the same patient). Some patients experience an unpleasant feeling of weakness, malaise, dizziness, irritability a few hours before the onset of a seizure. These phenomena are called seizure harbingers.

Small seizure (petit mal) - a short-term shutdown of consciousness followed by complete amnesia. A typical example of a minor seizure is absence during which the patient does not change posture. Turning off consciousness is expressed in the fact that he stops the action begun (for example, he becomes silent in a conversation); the gaze becomes "floating", meaningless; face turns pale. After 1-2 seconds, the patient comes to his senses and continues the interrupted action, not remembering anything about the seizure. Convulsions and falls are not observed. Minor seizures are never accompanied by an aura or precursors.

Non-convulsive paroxysms, equivalent to seizures, are of great difficulty for diagnosis. Equivalents of seizures can be twilight states, dysphoria, psychosensory disorders.

Twilight states are suddenly arising and suddenly ceasing disorders of consciousness with the possibility of performing rather complex actions and deeds and subsequent complete amnesia. In many cases, epileptiform paroxysms are not accompanied by loss of consciousness and complete amnesia. An example of such paroxysms is dysphoria - sudden attacks of altered mood with a predominance of malignant melancholy affect. Consciousness is not darkened, but affectively narrowed. Patients are agitated, aggressive, react viciously to remarks, show dissatisfaction in everything, speak out sharply insultingly, can hit the interlocutor. After the seizure is over, patients calm down. They remember what happened and apologize for their behavior. Almost any symptom of productive disorders can be a manifestation of paroxysms.

Similar information.

Paroxysms are short-term, sudden-onset and abruptly terminating disorders prone to recurrence. A wide variety of mental (hallucinations, delusions, confusion, anxiety, fear or drowsiness), neurological (convulsions) and somatic (palpitations, headaches, sweating) disorders can occur paroxysmally. In clinical practice, the most common cause of seizures is epilepsy, but seizures are common in several other conditions, such as migraine (see section 12.3) and narcolepsy (see section 12.2).

Epileptiform paroxysms

Epileptiform paroxysms include short-term seizures with a very different clinical picture, directly related to organic brain damage. Epileptiform activity can be detected on the EEG in the form of single and multiple peaks, single and rhythmically repetitive (6 and 10 per second) acute waves, short-term bursts of high-amplitude slow waves and especially peak-wave complexes, although these phenomena are also recorded in people without clinical signs of epilepsy.

There are many classifications of paroxysms, depending on the localization of the lesion (temporal, occipital foci, etc.), the age of onset (childhood epilepsy - pycnolepsy), causes (symptomatic epilepsy), the presence of seizures (convulsive and non-convulsive paroxysms). One of the most common classifications is the division of seizures according to the leading clinical manifestations.

Great seizure (grand mal) manifests itself as a sudden shutdown of consciousness with a fall, a characteristic change in tonic and clonic seizures and subsequent complete amnesia. The duration of a seizure is typically between 30 seconds and 2 minutes. The patient's condition changes in a certain sequence. The tonic phase is manifested by sudden loss of consciousness and tonic convulsions. Signs of switching off consciousness are the loss of reflexes, reactions to extraneous stimuli, lack of pain sensitivity (coma). As a result, patients, falling, cannot protect themselves from serious injuries. Tonic cramps are manifested by a sharp contraction of all muscle groups and a fall. If at the time of the onset of the seizure there was air in the lungs, a sharp cry is observed. With the onset of the seizure, breathing stops. The face first turns pale, and then cyanosis grows. The duration of the tonic phase is 20-40 s. Klonichi phase also proceeds against the background of switched off consciousness and is accompanied by simultaneous rhythmic contraction and relaxation of all muscle groups. During this period, urination and defecation are observed, the first respiratory movements appear, however, full breathing is not restored and cyanosis persists. Air expelled from the lungs forms a foam, sometimes stained with blood due to a bite of the tongue or cheek. The duration of the tonic phase is up to 1.5 minutes. The seizure ends with the restoration of consciousness, but for several hours after that, there is doubtfulness. At this time, the patient can answer simple questions from the doctor, but, left to himself, falls deeply asleep.

In some patients, the clinical picture of a seizure may differ from the typical one. Often one of the phases of seizures is absent (tonic and clonic seizures), but the reverse phase sequence is never observed. In about half of the cases, the onset of seizures is preceded by an aura (various sensory, motor, visceral or mental phenomena, extremely short-lived and the same in the same patient). Clinical features of the aura may indicate the localization of a pathological focus in the brain (somatomotor aura - posterior central gyrus, olfactory - hooklike gyrus, visual - occipital lobes). Some patients experience an unpleasant feeling of weakness, malaise, dizziness, irritability a few hours before the onset of a seizure. These phenomena are called harbingers of a seizure.

Small seizure (petit mal) - a short-term shutdown of consciousness with subsequent complete amnesia. A typical example of a minor seizure is absence during which the patient does not change posture. Turning off consciousness is expressed in the fact that he stops the action started (for example, he becomes silent in a conversation); the gaze becomes "floating", meaningless; the face turns pale. After 1-2 seconds the patient regains consciousness and continues the interrupted action, not remembering anything about the seizure. Convulsions and falls are not observed. Other options for minor seizures are - difficult absences, accompanied by abortive convulsive movements forward (propulsions) or backward (retropulsions), bending like an oriental greeting (salam-seizures). In this case, patients can lose balance and fall, but immediately get up and regain consciousness. Minor seizures are never accompanied by an aura or precursors.

Non-convulsive paroxysms, equivalent to seizures, are of great difficulty for diagnosis. Equivalents of seizures can be twilight states, dysphoria, psychosensory disorders.

Twilight states - sudden and abruptly terminating disorders of consciousness with the possibility of performing rather complex actions and deeds and subsequent complete amnesia. Twilight states are detailed in the previous chapter (see section 10.2.4).

In many cases, epileptiform paroxysms are not accompanied by loss of consciousness and complete amnesia. An example of such paroxysms are dysphoria - Sudden attacks of altered mood with a predominance of an angry melancholy affect. Consciousness is not darkened, but affectively narrowed. Patients are agitated, aggressive, react viciously to comments, show dissatisfaction in everything, speak out sharply insultingly, can hit the interlocutor. After the seizure is over, patients calm down. They remember what happened and apologize for their behavior. A paroxysmal appearance of pathological drives is possible: for example, the manifestation of epileptiform activity is periods of excessive drinking - dipsomania ... Unlike patients with alcoholism, such patients outside of an attack do not experience a pronounced craving for alcohol, drink alcohol in moderation.

Almost any symptom of productive disorders can be a manifestation of paroxysms. Occasionally, there are paroxysmal hallucinatory episodes, unpleasant visceral sensations (senestopathies), and seizures with primary delusions. Quite often during attacks, psychosensory disorders and episodes of derealization described in Chapter 4 are observed.

Psychosensory seizures are manifested by the feeling that the surrounding objects have changed their size, color, shape or position in space. Sometimes there is a feeling that parts of your own body have changed. ("Body schema disorders"). Derealization and depersonalization in paroxysms can be manifested by attacks of deja vu and jamais vu. It is characteristic that in all these cases, patients retain rather detailed memories of painful experiences. Real events at the time of the seizure are somewhat worse remembered: patients can remember only fragments from the statements of others, which indicates an altered state of consciousness. M.O. Gurevich (1936) proposed to distinguish such disorders of consciousness from typical syndromes of switching off and clouding of consciousness and designated them as “Special states of consciousness”.

A 34-year-old patient from infancy is observed by a psychiatrist due to mental retardation and frequent paroxysmal seizures. The cause of organic brain damage is otogenic meningitis transferred in the first year of life. Over the past years, seizures have occurred 12-15 times a day and are characterized by stereotyped manifestations. A few seconds before the start, the patient may anticipate the approach of an attack: suddenly he takes his right ear with his hand, holds his stomach with his other hand, and after a few seconds raises it to his eyes. Does not answer questions, does not follow the doctor's instructions. After 50-60 seconds, the attack passes. The patient reports that at this time he smelled tar and heard a rough male voice in his right ear, voicing threats. Sometimes, simultaneously with these phenomena, a visual image appears - a person of white color, whose facial features cannot be seen. The patient describes in some detail the painful experiences during the seizure, also states that he felt the doctor's touch at the moment of the seizure, but did not hear the speech addressed to him.

In the described example, we see that, in contrast to small seizures and twilight clouding of consciousness, the patient retains memories of the attack, but the perception of reality, as should be expected in special states of consciousness, is fragmentary, indistinct. Phenomenologically, this paroxysm is very close to the aura preceding a large seizure. Such phenomena indicate the local nature of the attack, the maintenance of normal activity in other parts of the brain. In the described example, the symptomatology corresponds to the temporal localization of the focus (the history data confirm this point of view).

The presence or absence of focal (focal) manifestations is the most important principle of the International Classification of Epileptiform Paroxysms (Table 11.1). According to the International classification, seizures are divided into generalized (idiopathic) and partial (focal). Great value for differential diagnosis of these variants of paroxysms has an electroencephalographic examination. Generalized seizures correspond to the simultaneous appearance of pathological epileptic activity in all parts of the brain, while with focal seizures, changes in electrical activity occur in one focus and only later can affect other parts of the brain. There are also clinical signs characteristic of partial and generalized seizures.

Generalized seizures always accompanied by a gross disorder of consciousness and complete amnesia. Since the seizure immediately disrupts the work of all parts of the brain at the same time, the patient cannot feel the approach of the seizure, the aura is never observed. Absenteeism and other types of minor seizures are typical examples of generalized seizures. Large seizures are generalized only if they are not accompanied by an aura.

Table 11.1. International classification of epileptic paroxysms

Classes of seizures	Heading in ICD-10	Clinical characteristics	Clinical options
Generalized (idiopathic)	G40.3	They begin for no apparent reason, immediately with a loss of consciousness; EEG shows bilateral synchronous epileptic activity at the time of an attack and the absence of pathology in the interictal period; good effect of standard anticonvulsants	Tonic-clonic (grand mal) Atonic Clonic Tonic Typical absences (petit mal) Atypical absences and myoclonic seizures
Partial (focal)	G40.0,	Are accompanied by an aura, harbingers or not a complete shutdown of consciousness; asymmetry and focal epileptic activity on the EEG; often a history of organic disease of the central nervous system	Temporal lobe epilepsy Psychosensory and Jacksonian seizures With outpatient automatisms Secondary generalized (grand mal)

Partial (focal) seizures may not be accompanied by complete amnesia. Their psychopathological symptomatology is varied and exactly corresponds to the localization of the focus. Typical examples of partial seizures are special states of consciousness, dysphoria, Jacksonian seizures (motor seizures localized in one limb, occurring against the background of clear consciousness). Quite often, local epileptic activity later spreads to the entire brain. Loss of consciousness and the onset of clonic-tonic seizures correspond to this. Such variants of partial seizures are designated as secondary generalized... Examples of these are grand mal seizures, which are preceded by forerunners and aura.

The division of seizures into generalized and partial is essential for diagnosis. So, generalized seizures (both grand mal and petit mal) are mainly a manifestation of the actual epileptic disease (genuinic epilepsy). Partial seizures, on the other hand, are very nonspecific and can occur in a wide variety of organic brain diseases (trauma, infections, vascular and degenerative diseases, eclampsia, etc.). Thus, the appearance of partial seizures (secondary generalized, Jacksonian, twilight states, psychosensory disorders) at the age of more than 30 years is often the first manifestation of intracranial tumors and other volumetric processes in the brain. Epileptiform paroxysms are a common complication of alcoholism. In this case, they occur at the height of the withdrawal syndrome and stop if the patient abstains from alcohol for a long time. It should be borne in mind that some drugs (camphor, bromcamphor, corazole, bemegrid, ketamine, proserin and other cholinesterase inhibitors) can also provoke epileptic seizures.

A dangerous paroxysmal arising condition is status epilepticus - a series of epileptic seizures (usually grand mal), between which patients do not come to a clear consciousness (i.e., a coma persists). Repeated seizures lead to hyperthermia, impaired blood supply to the brain and cerebrospinal fluid dynamics. Increasing cerebral edema causes respiratory and cardiac disturbances, which are the cause of death (see section 25.5). Status epilepticus cannot be called a typical manifestation of epilepsy - it is most often observed in intracranial tumors, head trauma, and eclampsia. It also occurs when anticonvulsants are suddenly stopped.