Paroxysmal heart rhythm disturbances (tachycardia, tachyarrhythmias)

Paroxysmal tachyarrhythmias are characterized by a sudden onset (and possibly also an end) with the development of an attack of increased heart rate of more than 100 beats/min, the possible development of acute circulatory failure and require urgent treatment.

Tachyarrhythmias can occur in any part of the heart.

Depending on the location of the focus, tachyarrhythmias most often occur:

• atrial (supraventricular),
• ventricular,
• sinus,
• nodal,
• atrioventricular.

The mechanism of occurrence of tachyarrhythmias may be different. The re-entry mechanism is distinguished - re-entry of the excitation wave, ectopic, trigger, etc. Separately, tachyarrhythmias with a wide or narrow QRS complex are distinguished, which determines further treatment tactics.

Diagnosis of tachyarrhythmias

Typically, diagnosis of tachyarrhythmias is carried out by a clinic doctor, cardiologist, or emergency physician. It is important to take anamnesis, physical examination, and various instrumental diagnostic methods. It is especially necessary to register an attack of tachyarrhythmia on an ECG (for presentation to an arrhythmologist). The Clinic has all possible methods for diagnosing and treating tachyarrhythmias.

The main ones include:

1. 1. ECG in 12 leads.
2. 2. Daily, three-day and seven-day
   ECG monitoring.
3. 3. Endocardial electrophysiological study of the heart (endo-EPS)

   – carried out in a hospital. Endocardial EPI of the heart is carried out in the cath lab. This method allows you to assess the functional state of the cardiac conduction system and determine the mechanism of arrhythmia, determine indications for radiofrequency catheter ablation (RFA) of additional conduction pathways and arrhythmogenic zones.

Paroxysmal states. Fainting

Fainting , or syncope, is an attack of short-term loss of consciousness and impaired muscle tone of the body (fall) due to a disorder of cardiovascular and respiratory activity. Syncope conditions can be neurogenic in nature (psychogenic, irritative, maladaptive, dyscirculatory), develop against the background of somatic pathology (cardiogenic, vasodepressor, anemic, hypoglycemic, respiratory), under extreme influences (hypoxic, hypovolemic, intoxication, medication, hyperbaric). Syncope, despite its short duration, is a process unfolding over time, in which successive stages can be distinguished: precursors (presyncope), peak (syncope itself) and recovery (postsyncope). The severity of clinical manifestations and the duration of each of these stages are very diverse and depend mainly on the pathogenetic mechanisms of fainting.

Fainting can be provoked by an upright position, stuffiness, various stressful situations (unpleasant news, blood drawing), sudden acute pain. In some cases, fainting occurs for no apparent reason. Fainting can occur from once a year to several times a month.

Clinical manifestations . Immediately after the provoking situation, a presyncope (lipothymic) state develops, lasting from several seconds to several minutes. At this stage, severe general weakness, unsystematic dizziness, nausea, flickering of “spots”, “veils” before the eyes are observed, these symptoms quickly increase, there is a premonition of a possible loss of consciousness, noise or ringing in the ears. Objectively, during the lipothymic period, pallor of the skin, local or general hyperhidrosis, decreased blood pressure, pulse instability, respiratory arrhythmia are noted, coordination of movements is impaired, and muscle tone decreases. The paroxysm can end at this stage or move into the next stage - the actual syncope state, in which all the described symptoms increase, the patients fall, and consciousness is impaired. The depth of loss of consciousness varies from slight stupefaction to deep disturbance lasting several minutes. During this period, there is a further decrease in blood pressure, shallow breathing, the muscles are completely relaxed, the pupils are dilated, their reaction to light is slow, and tendon reflexes are preserved. With a deep loss of consciousness, short-term convulsions, often tonic, and involuntary urination may develop. In the post-syncope period, the restoration of consciousness occurs quickly and completely, patients immediately orient themselves in the environment and what happened, and remember the circumstances preceding the loss of consciousness. The duration of the post-syncope period ranges from several minutes to several hours. During this period of time, general weakness, non-systemic dizziness, dry mouth are noted, pale skin remains, hyperhidrosis, decreased blood pressure, and uncertainty of movements.

The diagnosis is made on the basis of a carefully collected anamnesis, examination of somatic and neurological status; all patients with syncope must undergo echocardiography, VEM, Echo-CG, 24-hour blood pressure monitoring, EEG, ultrasound, radiography of the cervical spine, EEG and EEG monitoring

a single treatment regimen for patients in the interictal period, since the causes and pathogenetic mechanisms of development of various variants of syncope are very diverse. Treatment is prescribed only after a thorough examination of the patient and substantiation of the diagnosis not only of the underlying disease, but also clarification of the leading pathogenetic mechanisms of the development of fainting.

Paroxysmal dyskinesias: differential diagnosis with epilepsies

Paroxysmal dyskinesias are neurological conditions with a varied clinical picture, characterized by sudden attacks of pathological involuntary motor activity (i.e., accompanied by attacks of hyperkinesis) in the muscles of the limbs, trunk, face, and neck [3]. We call attacks of hyperkinesis here “attacks of hyperkinesis” so as not to confuse them with the term “epileptic seizures.”

The relevance of the term “paroxysmal” is due to the fact that hyperkinesis suddenly appears and also suddenly disappears, taking the form of attacks. Between attacks of hyperkinesis, the motor sphere and human behavior as a whole remain completely normal. Hyperkinesis attacks may manifest as involuntary, rapid, irregular jerks (chorea); slow snake-like or worm-like movements, smoothly flowing from one muscle to another (athetosis); increased muscle tone with repeated twisting movements and pathological postures (dystonia); uncontrolled circular movements in one or more limbs (ballism) or any combination of these hyperkinesis.

Paroxysmal dyskinesias are divided into paroxysmal kinesigenic dyskinesia (PKD) and paroxysmal non-kinesigenic dyskinesia (PNKD). Hyperkinesis attacks in PCD develop as a result of provoking factors (triggers) that act unexpectedly and suddenly. In contrast, in PNCD, attacks occur spontaneously at rest or interrupt daily physical activity; their severity is enhanced by alcohol, coffee, stress, violent emotions, etc. [1]. Separately, there are two types of paroxysmal dyskinesias: those provoked by prolonged or excessive physical activity (PKFN) and those provoked by sleep (hypnogenic dyskinesia - PHD).

Paroxysmal dyskinesias are characterized depending on the duration of the attacks (attacks can be short-lived - less than 5 minutes or long-lasting - more than 5 minutes), can be familial in nature, develop due to unclear reasons (sporadic forms) or secondary, against the background of any disease (symptomatic forms) .

PCD was previously called paroxysmal choreoathetosis, but the modern term is more correct, since with PCD not only choreoathetosis develops, but also other types of hyperkinesis. In many patients with PCD, the disease is idiopathic (i.e., familial or sporadic), with symptoms manifesting up to 20 years, and most often before 10 years. In general, the age of onset varies from 6 months to 40 years. According to the literature, boys are affected more often than girls, but the exact prevalence is unknown due to the fact that the disease is extremely rare.

Transient attacks of hyperkinesis in PCD in the early stages affect the muscles of the arms and legs, but as the disease progresses, hyperkinesis also affects the muscles of the face, neck and torso. Attacks can be unilateral or bilateral, but their characteristic feature is their asymmetry, even if they are bilateral. Involuntary contraction of the facial or oromandibular muscles results in grimacing, slurred speech (dysarthria), and even mutism. Attacks are never accompanied by a change in consciousness. Hyperkinesis occurring in the legs or trunk leads to sudden falls and often multiple injuries. In addition to sudden motor attacks, automatisms appear - yawning, shortness of breath, echolalia, echopraxia. Attacks become more frequent under the influence of external factors.

Most patients with PCD experience attacks during the day. Their frequency varies - from 1 per month or several months to 100 daily. At the beginning of each attack, some patients experience precursor sensations (tingling, burning and other paresthesias; dizziness; muscle spasms). After this, as a rule, hyperkinesis develops in the same area of ​​the body.

The frequency of attacks in PCD decreases with age. Most patients with PCD have short attacks, ranging from a few seconds to 5 minutes. Less commonly, hyperkinesis attacks last several hours. PKD with prolonged and infrequent attacks make their debut at a later age. The duration of attacks may vary. Let's give an example.

Girl, P.K., 6 years old. Diagnosis: autosomal recessive rolandic epilepsy. Paroxysmal dyskinesia. Complaints: paroxysms of hyperkinesis in the left limbs and left half of the face. Life history: heredity is burdened: on the mother’s side (grandmother has three twins, prematurity, miscarriages). A child from the first pregnancy, which occurred against the background of the threat of early miscarriage, at 4 months - bleeding (inpatient treatment), against the background of repeated acute respiratory viral infections (ARVI); first urgent rapid birth. She screamed immediately. Discharged on the 7th day. Breastfed for up to 1.5 months. I was gaining weight well. Psychomotor development: holds head - by 3 months, sits - by 7 months, walks - by 11 months. He speaks according to his age.

History of the disease: at the age of 1 year, twitching of the left leg (non-synchronous, sometimes rotational movements) appeared on each awakening; after 2-3 months, twitching began to appear simultaneously in the leg and arm; after a few months, twitching in the face on the same side appeared. At 1.5 years old, she was diagnosed with episyndrome, left-sided Jacksonian convulsions. Gluferal was prescribed, then convulsofin, depakine, benzonal in the form of mono- and polytherapy (up to three drugs at the same time) - without effect. During treatment, the frequency of attacks remained from 1 to 15 per day, they occurred not only upon awakening, but also during wakefulness (when provoked by emotional and physical stress); during sleep in the drowsiness phase, before waking up. Maximum duration up to 30 s. She did not lose consciousness. Anticipates attacks.

The neurological status revealed no focal symptoms.

Video electroencephalography (video-EEG): background EEG with eyes closed in the occipital regions is represented by hypersynchronous alpha activity, amplitude 150–200 μV, frequency 8 Hz. When carrying out photostimulation with a frequency of 18 Hz, generalized bursts of peak-wave complexes are observed, not accompanied by clinical patterns of an epileptic seizure. Flash duration is 1–3 s. Sleep EEG recorded stages 1–3 of sleep. In stages 1–2, two-phase sharp waves are recorded, localized in the central region with amplitude predominance on the right. Generalized outbreaks of an epileptic nature, not accompanied by clinical manifestations, have also been recorded during sleep. Upon awakening and while awake, two episodes of movements in the left hand in the form of choreoathetosis were recorded. The motor phenomenon upon awakening was not accompanied by changes in bioelectrical activity (BEA). The second phenomenon coincided with an outbreak of generalized epileptic activity. Conclusion: video-EEG monitoring revealed two types of pathological activity: 1) local changes in the central region, which may be characteristic of rolandic epilepsy; 2) generalized epileptic activity. The motor phenomena were of the nature of hyperkinesis (choreoathetosis) and are probably not epileptic seizures.

Examination results: biochemical blood test, electrocardiogram (ECG), ultrasound examination of internal organs: without features. Magnetic resonance imaging (MRI) of the brain - without pathology. Ophthalmologist: no change. Psychologist: psychological development corresponds to the age norm. She was examined in the laboratory of hereditary metabolic diseases of the Medical Genetic Center of the Russian Academy of Medical Sciences: GM1 gangliosidosis, neuronal ceroid lipofuscinosis 1 and 2, mitochondrial diseases, Wilson-Konovalov disease were excluded.

Upon discontinuation of anticonvulsant therapy (carbamazepine, barbiturates), there was no change in the frequency of attacks. Introduced by the staff. Attacks of choreoathetosis in the left extremities persisted with a frequency of 5–11 times a day, during the day and at night, and were periodically associated with movements and emotional stress (Table 1). After discharge, the frequency of attacks decreased. After 6 months, therapy was adjusted (increasing the dose of Nakoma, Frisium), and the attacks stopped. There were no attacks for 1 month, then they resumed again, mainly in the morning, in connection with awakening, and less often during wakefulness.

EEG during follow-up therapy: positive dynamics are noted in the form of a decrease in the prevalence of diffuse epileptiform discharges, a decrease in the amplitude of regional central-vertex discharges, as well as their decrease during wakefulness.

PNCD. The former name is “paroxysmal dystonic choreoathetosis”. PNCD often manifests before the age of 20, but the age of onset is extremely variable. Boys get sick more often. Attacks are characterized by the presence of any hyperkinesis (chorea, athetosis, dystonia, ballisma). It must be emphasized that they are never accompanied by impaired consciousness. The attacks can be so severe that they cause a sudden fall or disruption of gait and other types of motor activity, causing the severity of the disease. Attacks in PNCD develop suddenly, without any specific triggers, which distinguishes them from PCD. The severity of attacks can be aggravated by fear, anxiety, excitement, joy, cooling or overheating, drinking alcohol, coffee, tea, chocolate, etc. The frequency of attacks with PNCD is much less frequent than with PCD, 2–3 times a month; however, they sometimes develop more than 100 times a day. Attacks are preceded by precursors in the form of unusual sensations (tingling, spreading warmth, etc.), muscle tension. Attacks during PNCD last from 5 minutes to several days. Based on their duration, they are divided into short (less than 5 minutes) and long (more than 5 minutes). With age, the duration of attacks gradually decreases.

PKFN is a rare form of paroxysmal dyskinesia in which attacks are provoked by prolonged or excessive physical activity (formerly known as “intermediate paroxysmal dyskinesia”).

In patients with the idiopathic (familial or sporadic) form, the disease debuts in childhood. Only in secondary (symptomatic) forms does the age of onset increase to 30 years. Girls get sick more often.

PKFN is characterized primarily by sudden transient dystonic attacks, manifested by involuntary repeated muscle contractions with the formation of pathological postures (often painful). In some patients, attacks of dystonia manifest as choreoathetosis. Attacks occur against the background of strong or prolonged physical activity (running, walking long distances), sometimes PKFN is provoked by passive movements in the affected limbs, and intensifies under the influence of external factors. Attacks mainly occur in the legs, but sometimes, especially during prolonged episodes, the muscles of the face, neck, and torso are involved. As a rule, symmetrical parts of the body are affected (bilateral attacks), but there are also asymmetrical ones. No precursor sensations were noted during PKFN. The frequency of attacks is 1–5 per month. Cases have been described where attacks occur 1–2 times a day. The duration of attacks is from 5 to 30 minutes, less often less than 2 minutes. The duration of attacks decreases with age; in general, they last from a few seconds (in adults) to several days (in children). Let's give a second example.

Girl, K.D., 9 years old. Diagnosis: paroxysmal kinesigenic dyskinesia. Symptomatic epilepsy. Cerebral palsy (CP), spastic-hyperkinetic form. Complaints: lag in psychomotor development, periodic attacks of worm-like violent movements, lasting from several hours to days. Life history: child from young healthy parents, first premature birth at 36 weeks. At birth, weight is 3.0 kg, height is 50 cm. Development with delayed psychomotor skills. At 8 months, a diagnosis of cerebral palsy was made. Systematically receives rehabilitation therapy. Heredity is not burdened.

History of the disease: at the age of 7 years, against the background of acute respiratory viral infection, the first attack of hyperkinesis developed in the form of choreoathetosis, which lasted 3 days. Due to the severity of the condition (respiratory arrhythmia, ongoing hyperkinesis), the child was in the intensive care unit. During therapy (tizercin, finlepsin, depakine, cyclodol), hyperkinesis began to weaken by the end of 3 days and then stopped. Motor activity was completely restored. After 2-3 weeks - a repeated attack of choreoathetosis, lasting about 10 minutes, less severe, without loss of consciousness. Then hyperkinesis attacks, lasting several hours, were repeated once a month.

Neurological status: mild divergent strabismus, weak convergence on the left. Tongue deviation to the right. The gait is spastic-paretic with propulsion. Hemiatrophy of the extremities on the right. Muscle tone is increased according to the spastic type, with elements of plasticity. Tendon and periosteal reflexes are increased with expansion of the zones, more pronounced on the right. Bilateral foot clonus. Pathological foot reflexes on both sides. The severity of the condition has not been studied in the Romberg position. Coordinator tests are performed with intention and dysmetry on the left. No sensitivity disorders were identified. Dystonic attacks in response to emotional or physical provocation.

Survey results. ECG: without pathology. Biochemical blood test: no pathology. Ophthalmologist: anterior segment, refractive media, fundus - no changes. Neuropsychologist: deficit in the kinesthetic organization of movements and speech, as well as modality-specific impairments of auditory-verbal memory and weakness of objective visual gnosis. EEG monitoring of daytime sleep: in the left central-parietal region, single monophasic sharp waves are recorded, with a frequency of 1–2 per second, followed by a period of increased sharp waves up to 5 per second and the appearance of individual “peak-wave” complexes in their series. Taking into account the examination data, the attacks were regarded as attacks of symptomatic epilepsy; Depakine (600 mg/day) was prescribed. With Depakine, there was a complete absence of attacks for 6 months. Against the background of acute nasopharyngitis, hyperkinesis appeared, increasing in intensity, frequency and amplitude over 2 days. The administration of diazepam, then aminazine, did not allow us to control the attacks of hyperkinesis, which were repeated every 5–10 minutes and were of a “status” nature, and therefore the child was transferred to the intensive care unit. In the intensive care unit, due to intractable dystonic attacks (ballism, choreoathetosis), a decision was made to administer thiopental, muscle relaxants, and artificial pulmonary ventilation (ALV) for life-saving reasons. The course of ARVI was complicated by the phenomena of upper lobe pneumonia. During intravenous administration of tiapridal at a dose of 300 mg/day, dystonic attacks persisted for 5 days. On the 6th day, chlorpromazine 1.0 ml was administered intravenously. The hyperkinesis continued. Intravenous administration of depakine, diazepam, and sodium hydroxybutyrate stopped hyperkinesis for a short time. On days 8–10, pulse therapy with methylprednisolone was administered at a dose of 400 mg/day without effect. On the 11th day, we applied 1/8 tablet of nacom (in the morning). Treatment for 4 days without effect. On the 15th day, clonazepam was prescribed orally - up to 1.5 mg/day, Topamax - 50 mg/day, Finlepsin - 300 mg/day - without effect. After temporary cessation of thiopental infusions for 2 hours, choreoathetosis persisted. The child was conscious and responded to examination. It was not possible to stop the thiopental infusion. On the 15th day, to tiapridal at a dose of 600 mg/day, obzidan was added at a dose of 0.5 mg/day (in saline solution 20.0 - through an infusion pump), the next day the dose of obzidan was adjusted - 0.7 mg/day in saline solution 20.0 (1 ml/h) with a clear positive effect in the form of a decrease in hyperkinesis, the appearance of “light spaces”. On the 17th day, only constant myoclonus of the limbs and shoulder girdle persisted. The dose of obzidan was increased to 1 mg/day. EEG monitoring in the intensive care unit: diffuse functional-organic disorders of the BEA of the brain. There is no data indicating the presence of regional, generalized, diffuse epileptiform activity. Numerous myoclonus recorded during the study, taking into account clinical and electroencephalographic correlates, was regarded as myoclonus of non-epileptic origin. As a result of MRI of the brain, the following were revealed: basal-temporal atrophy on the left, secondary expansion of the basal subarachnoid spaces in this area to the degree of a cyst; diffuse cortical-subcortical subatrophy, realized by diffuse expansion of the subarachnoid spaces of the convexital sections of the cortex and reactive asymmetric, predominantly right-sided expansion of the lateral ventricles. Examined by a geneticist: GM1- and GM2-gangliosidoses, NARP (neuropathy, ataxia, retinitis pigmentosa), organic aciduria and aminoacidopathy, mitochondrial diseases, Wilson-Konovalov disease, Hallevorden-Spatz disease were excluded. During therapy (thiopridal, obzidan, depakine), thiopental anesthesia was stopped, and hyperkinesis was reduced. In total, the duration of the attack was 17 days. In a stable condition, the patient was transferred from intensive care to the psychoneurology department, where she received depakine - 600 mg/day, anaprilin - 10 mg/day, nootropil - 600 mg 2 times a day, tiapridal - 100 mg/day. Against the background of a single reduction in the dose of tiapridal to 3.3 mg in the morning, an increase in hyperkinesis was noted. By the time of discharge from the hospital, the child’s neurological status did not differ from the state upon admission. Minor dystonic attacks to provocation persisted. The follow-up period was 3 years.

PGD ​​is a rare variant of the disease characterized by transient attacks of involuntary movements during NREM (non-REM) sleep. Occasionally, PGD attacks are preceded by specific precursor sensations. Attacks are often associated with periods of awakening (arousal); occur during sleep, while the patient’s eyes are open, and hyperkinesis occurs in the limbs and torso. Sometimes attacks are accompanied by vocalizations, respiratory rhythm disturbances, and tachycardia. Then normal sleep continues, the attacks themselves are completely amnesiac. The severity of attacks is aggravated by external factors [4].

Idiopathic variants make their debut in childhood, with familial forms earlier than sporadic ones. The age of onset varies from 2 to 23 years for familial cases and from 3 to 47 years for sporadic cases. The frequency of attacks is usually 4–5 times per year, but sometimes it increases to 4–5 times per night. Attacks are usually short - from 20–50 s to 2 minutes.

Some patients, along with nocturnal attacks, experience attacks during wakefulness, kinesigenic or non-kinesigenic. In addition, with familial variants, different family members may experience different forms of paroxysmal dyskinesia. Let's give another example.

Child, I.O., 6 years old. Diagnosis: cerebral palsy, left-sided hemiparesis. Rolandic epilepsy. Paroxysmal dyskinesia. Upon admission, complaints about episodes of “stretching” during sleep when changing body position (tonic tension in the muscles of the neck and back, followed by extension of the torso and throwing back of the head). Paroxysms are accompanied by cessation of breathing, vegetative symptoms in the form of cold skin, and opercular automatisms (smacking). In the morning hours after night paroxysms, he is lethargic, awkward, and stumbles. The frequency of paroxysms is once a month.

From the anamnesis: the parents are healthy, there is no heredity. A child from the 4th pregnancy, characterized by a pathological course (against the background of chronic sinusitis, otitis, threat of miscarriage with placental abruption in the second trimester, hypothyroidism, Rh conflict), with hospital treatment (hormonal drugs); second premature birth

34 weeks by emergency caesarean section. Birth weight 2.7 kg, height 52 cm. The child was born in severe asphyxia, was on a ventilator for 7 days, and suffered from pneumonia. Due to hemolytic disease of newborns, a single replacement blood transfusion was performed. According to neurosonography, during the neonatal period, hemorrhage was observed in the lateral ventricles of the brain, resulting in periventricular leukomalacia. She has been seen by a neurologist since birth. From the age of 2 years, nocturnal attacks are observed in the form of abdominal pain, the urge to defecate, accompanied by facial distortion and opercular movements for half an hour, followed by post-attack sleep. From the age of 3 years, the seizures changed (simple focal motor seizures in the form of clonic convulsions in the left upper limb lasting 15 minutes followed by sleep). Finlepsin retard was prescribed - 300 mg/day, after which night “sipping” appeared when changing body position during sleep. With the introduction and increase in the dose of Depakine Chrono to 1000 mg/day, “sipping” became less frequent. Due to the development of thrombocytopenia, the dose of depakine chrono was reduced to 750 mg/day and the dose of finlepsin was increased to 400 mg/day. At the age of 4, against the background of herpetic stomatitis, generalized tonic-clonic seizures with loss of consciousness developed, the frequency of attacks was 5 times per night.

Neurological status: Convergent strabismus. Muscle tone is increased according to the spastic type, tendon reflexes are high - S > D, pathological foot symptoms. Muscle wasting in the left extremities, more in the leg. Flat valgus placement of the left foot, retraction in the left ankle joint. Retraction in the left elbow joint with rotation of the forearm. Disinhibited, euphoric, decreased sense of distance. Speech is phrasal.

An examination was carried out: biochemical blood test, ECG, ultrasound of internal organs without pathology. According to MRI of the brain: local atrophic, multifocal small-focal cystic-gliotic changes in the right frontoparietal region. Psychologist: intellectual development does not correspond to age level. Oculist: no pathology detected. EEG: moderate functional-organic changes. Regional epileptiform activity in the right central region, similar in morphology to the rolandic one. Video-EEG sleep monitoring: pronounced changes in BEA, expressed in a delay in the formation of basic rhythms, persistent epileptiform activity in the frontal regions of the right hemisphere of the brain, intensifying during sleep. Ictal EEG is characteristic of paroxysms of an epileptic nature; probable localization of the focus of epiactivity in the supplementary motor zone of the frontal lobe of the right hemisphere. The presence of nocturnal paroxysmal dystonia cannot be completely excluded.

Receives therapy: depakine chrono - 500 mg/day, finlepsin retard - 200 mg/day, mydocalm 0.05 - 1 tablet 3 times a day, phenibut 0.25 - 1/2 tablet at night, nacom - 1/4 tablet in the morning . During treatment, no epileptic seizures were observed; rare dystonic attacks during sleep persisted.

The genetics of paroxysmal dyskinesias are presented in Table 2. It should be noted that in our country genetic diagnosis of paroxysmal dyskinesias is not yet carried out. The true incidence of paroxysmal dyskinesias in the general population is unknown because these conditions are commonly misdiagnosed as other diseases. The patients mentioned above were observed for a long time with a diagnosis of epilepsy (one form or another) and received antiepileptic therapy for a long time, which had a different effect on hyperkinetic attacks. The etiology of secondary paroxysmal dyskinesias includes multiple sclerosis, perinatal brain lesions and cerebral palsy, psychogenia, traumatic brain injury, encephalitis, tumors, pathology of the thyroid gland and Fahr's disease, cerebral dysgenesis, strokes, diabetes mellitus, etc. Case histories are presented in this case. two children with cerebral palsy (paroxysmal dyskinesia is symptomatic) [6].

Researchers recognize dysfunction of the basal ganglia as the leading link in pathogenesis. In some patients, positron emission tomography (PET) and single photon emission computed tomography (SPECT) revealed changes in catecholamine metabolism. In addition, levodopa (a precursor of dopamine neurotransmitters) or antipsychotics (dopamine antagonists) were effective in a number of patients. In animal studies of PGD, increasing the activity of the main inhibitory neurotransmitter, γ-aminobutyric acid (GABA), reduces the severity and frequency of attacks. It is known that anticonvulsants—phenobarbital, valproate, and benzodiazepines—have a GABAergic effect. In clinical observations in patients with PGD, the effectiveness of anticonvulsants was noted, and in cases of PNCD, the course of the disease was mitigated with the use of only valproate and phenobarbital. This proves that the forms of paroxysmal dyskinesia are based on different mechanisms. However, more detailed studies of their pathogenesis have not been carried out to date.

The morphological substrate of paroxysmal dyskinesias is unknown. Since region 2 of chromosome (2q33–q35), damaged during paroxysmal dyskinesias, contains genes for membrane channels, there is a point of view that they are “channelopathies,” i.e., they arise as a result of abnormal operation of electrical channels of the cytoplasmic membrane [2]. Disruption of the channels leads to changes in ion concentrations in the cell and extracellular space. Mutations of the same genes have been found in some other channelopathies, for example familial hyper- or hypokalemic paralysis, paroxysmal ataxia.

Diagnosis of paroxysmal dyskinesia is based on clinical symptoms (duration and frequency of attacks, “provoking” factors; the presence of similar manifestations in relatives). Laboratory tests, EEG (preferably monitoring during an attack of hyperkinesis), MRI should be carried out - all this will exclude diseases with a similar clinical picture, as well as identify current diseases in which paroxysmal dyskinesia is secondary. Idiopathic paroxysmal dyskinesias are not characterized by changes on MRI or computed tomography. The question remains whether paroxysmal dyskinesias are epilepsies. A number of researchers classify paroxysmal dyskinesias as epilepsies, based on clinical manifestations (paroxysmality, precursors, effect of antiepileptic drugs). Other authors indicate that during the attack there are no changes in the EEG, and there are no changes in consciousness and behavior after the attack. Cases of registration of nonspecific pathological patterns on the EEG in patients with paroxysmal dyskinesia and the “coexistence” of paroxysmal dyskinesia and epilepsy in one patient have been described [2, 4]. It is especially difficult to distinguish nocturnal attacks of PCH from nocturnal frontal seizures in epilepsy, especially since antiepileptic drugs are effective in both cases. The only method that allows a correct diagnosis is nighttime video-EEG monitoring, which does not reveal epileptiform activity during night attacks of hypnogenic paroxysmal dystonia [1]. The differential diagnostic algorithm is presented in the figure. From our point of view, paroxysmal dyskinesias are not epilepsies for several reasons:

• they generally have a benign course and are not complicated by an increase in behavioral changes in the personality;
• hyperkinesis attacks are not accompanied by epileptiform activity on the EEG;
• even in the case of a status course (second example), attacks of hyperkinesis do not cause disturbances of consciousness.

Treating PD is very difficult. Unified recommendations for therapy have not been developed. In our opinion, the difficulty of selecting therapy is associated with pronounced fluctuations in endogenous catecholamines in the attack and inter-attack periods. Perhaps that is why, to stop attacks of hyperkinesis (in an acute state), it is better to prescribe antipsychotics (the drug of choice is tiapridal), and to prevent attacks, it is better to prescribe a combination of Nakoma and an antiepileptic drug (Depakine/Frisium).

At the same time, this regimen cannot be considered as a “hard” recommendation for each patient, since in some cases other drugs are also effective (finlepsin, propranolol, high doses of piracetam, etc.). Of course, the likelihood of side effects as a result of treatment should also be taken into account, since attacks regress with age.

Literature

1. Mukhin K. Yu., Maksimova E. V., Glukhova L. Yu., Petrukhin A. S., Mironov M. B., Gaman O. V. A family case of paroxysmal kinesogenic choreoathetosis // Journal of Neurology and Psychiatry. 2000. No. 8. P. 40–43.
2. Berkovic SF Paroxysmal movement disorders and epilepsy. Links across the channel//Neurology. 2000; 55(2): 169–170.
3. Fahn S. The paroxysmal dyskinesias//Movement Disorders. Oxford, England: Butterworth-Heinemann; 1994; 3: 310–345.
4. Hirata K., Katayama S., Saito T. et al. Paroxysmal kinesigenic choreoathetosis with abnormal electroencephalogram during attacks//Epilepsia. 1991; 32(4): 492–494.
5. Sadamatsu M., Masui A., Sakai T., Kunugi H., Nanko S., Kato N. Familial paroxysmal kinesigenic choreoathetosis: an electrophysiologic and genotypic analysis//Epilepsia. 1999; 40(7):942–949.
6. Swoboda KJ, Soong BW, McKenna C. et al. Paroxysmal kinesigenic dyskinesia and infantile convulsions. Clinical and linkage studies//Neurology. 2000; 55:224–230.

M. Yu. Bobylova E. S. Ilyina , Candidate of Medical Sciences S. V. Pilia M. B. Mironov , Candidate of Medical Sciences I. A. Vasilyeva A. A. Kholin , Candidate of Medical Sciences S. V. Mikhailova , Candidate of Medical Sciences Sciences A. S. Petrukhin , Doctor of Medical Sciences, Professor of the Russian State Medical University, Russian Children's Clinical Hospital of the Russian Federation, Moscow

Paroxysmal sleep disorders in children

P. Schwarzmayr, K. Mayer-Ewert

Diagnosis and treatment of epilepsy in children

- M.: Mozhaisk-Terra 1997.-656 p.

INTRODUCTION

Paroxysmal sleep disorders have been known since antiquity, and already in the works of Hypnocrates and Aristotle, some of them were described, both epileptic and non-epilentic conditions observed in sleep. To date, a large number of nosologically independent syndromes accompanied by paroxysmal sleep disturbances are known.

Differential diagnosis of sleep disorder syndromes of epileptic and non-epileptic origin has many problems in clinical practice. At the same time, an adequate assessment of the syndrome is extremely necessary to develop an optimal treatment strategy. In the focus of the most significant aspects of the problem of differential diagnosis of epileptic and non-epileptic paroxysmal sleep disorders, the main attention, from our point of view, should be concentrated on the following fundamentally important issues:

1. Epileptic and non-epileptic paroxysmal disorders are associated with disorders of the micro- and macroarchitecture of sleep (occur mainly in the slow-wave sleep phase - non-REM sleep, are associated with the phenomenon of “arousal” awakening, and are characterized by specific patterns in the form of K-complexes and vertex waves) .

2. Individual nocturnal paroxysms of epileptic and non-epileptic origin are similar both in clinical manifestations and in polysomnographic characteristics.

3. During nocturnal paroxysms of both epileptic and non-epileptic origin, the patient’s consciousness can be preserved or impaired.

4. Non-epileptic nocturnal paroxysms in some cases can bring more suffering to the patient than epileptic seizures.

It should be noted that nocturnal paroxysms of epileptic origin and many parasomnias have a close relationship with the phenomenon of awakening. Epileptic patterns that occur during the phase of rapid eye movement (REM sleep) and slow-wave sleep (non-REM sleep) are considered by a number of researchers as unique analogues of parasomnia (Clarenbach, Bukau, 1995). So-called “false” or pseudo-awakenings are characterized by the presence of K-complexes and vertex waves on the sleep EEG, which are regarded as epileptogenic (Niedermeyer, 1972). The role of sleep spindles is not well defined (Niedermeyer, 1995).

In recent years, in order to diagnose and differentially diagnose paroxysmal sleep disorders of epileptic and non-epileptic origin, as well as to analyze the semiology of attacks, the method of polysomnography with simultaneous video monitoring during sleep has been used (Niedermeyer, 1983; Broughton, 1987; Niedermeyer, Lopez da Silva, 1987). This method allows you to record both interictal and seizure EEG changes and the clinical condition of the patient. In clinical practice, the telemetry method (mobile long-term EEG), a combination of short-term recording of sleep EEG and mobile long-term EEG monitoring, is also used (Newzella, Platte, 1993).

In some cases, when it is difficult to differentiate between paroxysmal sleep disorder of epileptic and non-epileptic origin, it is recommended to prescribe the patient a “trial” anticonvulsant therapy, for example, carbamazepine. The patient's response (relief of attacks or absence of changes) allows the doctor to draw a conclusion about the nature of the existing paroxysmal disorder.

EPILEPTIC PAROXYSMS IN SLEEP

Gowers (1881) was the first to focus physicians' attention on the circadian distribution of epileptic paroxysms and identified attacks that occur during sleep, shortly after falling asleep, and upon awakening. Langdon-Down (1929) also emphasized the advisability of differentiating individual epileptic paroxysms, taking into account their relationship with the sleep-wake cycle. Janz (1962) proposed a classification of generalized epilepsies according to their relationship with sleep and awakening. Further differentiation of nocturnal paroxysms became possible thanks to the clarification of the semiology of attacks, the use of the polysomnography method, as well as a system of special application of electrodes (Luders et al, 1987). At the same time, a number of issues in the differential diagnosis of epileptic and non-epileptic nocturnal paroxysms remain insufficiently clarified.

There is a close relationship between epilepsy and sleep. 75% of all paroxysms occur during sleep (Clarenbach, Bulaun, 1995). Nocturnal epileptic paroxysms themselves often lead to various disturbances in the sleep profile. Changes in sleep characteristics are also observed under the influence of anticonvulsant therapy (Clarenbach et al, 1981; Wolf, 1987; Borbely, 1992). It has been shown that sleep can improve in some cases under the influence of anticonvulsant therapy (Johnson, 1982).

Epileptic brain activity contributes to both changes in sleep stages and the overall sleep profile (Baldy-Mouliner et al, 1984; Montplaisir et al, 1985; Dadmehr et al, 1987). In this case, clinical manifestations of paroxysms may be absent. The following sleep disturbances may occur due to nocturnal epileptic activity:

• increased and accelerated changes in sleep stages;
• fragmented R EM sleep;
• increased frequency of awakenings;
• decreased sleep efficiency (the relationship between sleep duration and night awakenings);
• shortening the total duration of sleep;
• prolongation of phases I and II of sleep;
• prolongation of sleep latency;
• disappearance of K-complexes and sleep spindles.

Epileptic seizures most often occur during the slow-wave sleep phase (non-REM sleep), particularly in stages I and II of sleep (Zeitihofer et al, 1991). Almost all primary generalized epileptic paroxysms, in comparison with complex partial seizures, are observed during slow-wave sleep (Montplaisir et al, 1985). The incidence of sleep disorders increases with the frequency of attacks. These disorders do not depend on the age of the patient (Horrner et al, 1984), but are often associated with the nature of the attacks. Patients with simple and complex partial paroxysms often have complaints of sleep disturbances.

Epilepsy and sleep have mutual influence on each other. In this regard, there are many problems in the diagnosis and differential diagnosis of nocturnal epileptic seizures:

• epileptic seizures that occur during sleep are often not regarded as epileptic or are not recognized at all;

• the semiology of epileptic paroxysms at night and during the day can differ significantly;

• individual nocturnal epileptic paroxysms are often mistakenly regarded as psychogenic;

• in some cases, during nocturnal epileptic seizures, the EEG does not show typical patterns.

Such diagnostic difficulties predetermine the need for special studies of nocturnal epileptic paroxysms based on sleep monitoring.

GENERALIZED EPILEPSY

Janz (1962) proposed a division of generalized paroxysms according to their relationship with the sleep-wake cycle into attacks that occur upon awakening, during sleep and diffuse ones, observed both during the day and at night. It should be noted that the circadian distribution of seizures may change as the disease progresses. Thus, attacks that occur upon awakening or during sleep may become diffuse; seizures observed on awakening evolve into seizures occurring during sleep. At the same time, “sleep epilepsy” almost never turns into “waking epilepsy” (Janz, 1962).

There are a number of epileptic syndromes in which seizures are associated with awakening: grand mal awakening (idiopathic generalized epilepsy with tonic-clonic seizures), absence seizures, juvenile myoclonic epilepsy, West syndrome (infantile spasms). In most cases (90%) “awakening epilepsies” are idiopathic genetically determined forms of epilepsy. Seizures usually occur 1-2 hours after waking up, after a daytime nap, or in the evening, between 6 and 8 p.m. (“end-of-working day epilepsy”) (Janz, 1962). It is characteristic that many patients who have attacks upon awakening wake up slowly in the morning, are often sleepy and become able to work after a certain interval of time. These patients prefer to go to bed late, fall asleep slowly, have difficulty reaching the superficial stages of sleep (stage III non-REM sleep), and only in the morning do they experience deep sleep.

Diffuse epilepsies, in which attacks do not have a clear circadian distribution, are symptomatic focal (temporal and frontal) epilepsies in 53% of cases.

Of particular interest are some forms of generalized epilepsies, in particular epilepsy with grand mal seizures during sleep and Lennox Gastaut syndrome, which were called “sleep epilepsies” by Janz (1962).

Clinical characteristics. Most grand mal attacks that occur during sleep are very mild and sometimes not recognized even during polysomnographic testing. In Lennox-Gastaut syndrome, nocturnal paroxysms are often characterized only by the opening of the eyes and tonic abduction of them upward, in some cases by tonic abduction of the head. The duration of such a paroxysm is no more than 10 seconds. Night attacks with Lennox Gastaut syndrome usually occur in the first half of the night, but then may become more frequent. Attacks of “sleep epilepsy” develop predominantly soon after falling asleep, between 22 and 24 hours, or 1–2 hours before awakening during non-REM sleep (Janz, 1974). Compared to patients suffering from epilepsy with awakening seizures, patients with “sleep epilepsy” wake up in the morning alert, fresh, and well rested. Their maximum performance is observed in the first half of the day. At the same time, they prefer to go to bed in the early evening and usually fall asleep early, quickly reaching the deep stage of sleep.

Data from laboratory and functional studies. A polysomnographic study reveals a reduction in sleep spindles and a shortening of the duration of REM sleep. During the non-REM sleep phase, fragmentary spike-wave complexes or generalized spike-wave complexes with a frequency of 3 Hz are recorded. Grand mal attacks most often occur during stage 1 non-REM sleep (Billiard, 1982). Typical EEG patterns are symmetrical synchronized sharp waves or temporal spikes. In Lennox-Gastaut syndrome, activation of epileptic activity is observed during sleep. Typical EEG changes are: focal or multifocal “sharp-slow wave” complexes with a frequency of 1-2.5 Hz with a strong tendency to generalize (Stefan, 1991).

Differential diagnosis. The most difficult is the differential diagnosis between nocturnal generalized attacks and nocturnal complex partial paroxysms. The “key” diagnostic signs for complex partial paroxysms—aura and disturbance of consciousness—cannot be adequately assessed at night. The presence of attacks, along with night and daytime, indirectly indicates the likelihood of complex partial paroxysms. The presence of focal changes in the sleep EEG testifies in favor of partial epileptic paroxysms. However, nocturnal partial paroxysms are often accompanied by rapid secondary generalization, making it difficult to identify focal EEG patterns. Particular difficulties arise in the differential diagnosis of generalized and frontal lobe epilepsies. Frontal paroxysms often occur with preserved consciousness. Typical for frontal epilepsy are: a high frequency of attacks, turning the head and eyes to the side, the presence of peculiar postures (the “fencer’s” pose) and automatisms (pedaling movements of the legs). To make a differential diagnosis between generalized and frontal lobe epilepsies, EEG video monitoring is necessary.

The differential diagnosis between primary generalized epilepsy and Rolandic epilepsy is based on the detection of typical “Rolandic” spikes localized in the centrotemporal region on the EEG. Differential diagnosis with electrical status epilepticus during slow-wave sleep (ESES) is not difficult, since ESES is characterized by specific typical EEG patterns - generalized spike-wave complexes, recorded in all leads throughout the curve (Patry et al, 1971) .

Treatment. In the treatment of epilepsy with grand mal attacks during sleep, the drug of first choice is sodium valproate. Phenobarbital is sometimes used. In the treatment of Lennox-Gastaut syndrome, sodium valproate, ethosuximide, and mesuximide are recommended. It is often necessary to resort to combination therapy with carbamazepine, phenytoin, primidone or phenobarbital. Additionally, ACTH and benzodiazepines are sometimes used (Matthes, Schneble, 1992).

FOCAL EPILEPSY

Temporal lobe epilepsy

Temporal lobe epilepsy is the most common form of focal epilepsy. Temporal lobe epilepsy can manifest itself as simple partial, complex partial and secondary generalized paroxysms. The following types of temporal lobe epilepsy are distinguished (Wieser, Hajek, 1995):

• hippocampal-amygdalar (mediobasal limbic);
• neocortical lateral temporal;
• opercular-insular.

Among the various types of temporal lobe epilepsy, the most common is mesial temporal lobe epilepsy (mediobasal limbic) (Wieser et al, 1993). It is important to note that hippocampal-amygdala temporal lobe epilepsies are activated by REM-CHOM (Wieser et al, 1993).

Clinical characteristics. Temporal paroxysms can be observed shortly after falling asleep (38% of cases), shortly after waking up (28%), or during the day (Janz, 1974). A typical sign of temporal lobe epilepsy is an aura. Most often, with temporal lobe epilepsy in children, an aura is noted in the form of a feeling of fear, unusual sensations (tickling) in the epigastric region, sensations of “already seen,” dream states, and less commonly, complex illusions and hallucinations (Wieser, 1987). Following the aura, a disturbance of consciousness often occurs. Oroalimentary automatisms (chewing, smacking, lip licking) are often observed, sometimes in combination with unilateral wrist automatisms and dystonic placement of the hand on the contralateral side (Kotagal et al, 1989). Autonomic symptoms such as increased heart rate and breathing are also characteristic. Secondary generalization is also possible.

Data from laboratory and functional studies. Typical EEG patterns in the interictal period are often absent. A polysomnographic study is necessary, which can detect epileptic activity (sharp waves or “sharp-slow wave” complexes) in 95% of cases (Gibbs, Gibbs, 1947; Sutherling, 1987). These EEG patterns appear most clearly in stages I-II of sleep and in the first sleep cycle (Passouant, 1967; Montplaisir, et al, 1980).

Differential diagnosis. The differential diagnosis of temporal lobe epilepsy and parasomnia (with somnambulism, nightmares, nocturnal enuresis, stereotypical head shaking, nocturnal paroxysmal dystonia) seems difficult. Compared with temporal lobe epilepsy, stereotypic head shaking and nightmares have an earlier age of onset. and paroxysmal nocturnal dystonia is later (Meier-Ewert, Schuiz, 1990). Nightmares often cause changes in heart rate and breathing rate.

Significant assistance in differential diagnosis is provided by laboratory research methods (NMR, positron emission tomography, photon positron emission tomography), which make it possible to detect structural damage to the temporal lobe (mesial temporal sclerosis) (Cascino et al, 1991). In some cases, for the differential diagnosis of temporal lobe epilepsy and parasomnias, a trial prescription of anticonvulsants is also recommended (Roder, Wolf, 1981). The lack of effect when prescribing anticonvulsants is more evidence in favor of narasomnia. One of the main differential diagnostic methods is EEG recording during sleep, which makes it possible to identify patterns typical for temporal lobe epilepsy that are absent in parasomnias. It should be noted, however, that with superficial application of electrodes it is not always possible to detect temporal lesions. In this regard, in some cases, the use of sphenoidal, subdural and intracerebral electrodes is recommended.

Treatment. The first-line drugs of choice for the treatment of temporal lobe epilepsy are carbamazepine and phenyin (Matthes, Schneble, 1992). For temporal epileisias that are resistant to anticonvulsant therapy, neurosurgical correction is recommended (Engel, 1987).

Frontal epilepsy

Clinical characteristics. Frontal paroxysms are characterized by a number of features that distinguish them from paroxysms of other localization: sudden (often without an aura) onset, high frequency of attacks with a tendency to be serial, short duration (30-60 sec), often preserved consciousness, vocalization, elaborate motor automatisms and postures , pronounced stereotypicality of attacks, absence or minimal post-attack confusion. Frontal seizures often occur during sleep (Baldy-Mouliner, 1986). Consciousness at the time of paroxysm is often preserved, and some patients are able to describe or even depict their attacks (Shondienst, 1995). Many patients are quite indifferent to attacks.

A special place among frontal paroxysms is occupied by attacks emanating from the supplementary motor area. The supplementary motor area is located between the paracentral lobules and the cingulate gyrus (PenField, Welch, 1951). It presents a somatotopic projection of various parts of the body. Seizures emanating from the supplementary motor area, like other frontal paroxysms, occur predominantly at night. Tonic motor phenomena dominate in the proximal parts of the upper and lower extremities, pretentious postures and automatisms in the form of flapping hands, wheel-like rotation of the whole body (Lim et al, 1991). A typical “fencing pose” is often noted. Intelligence is usually not affected (Chauvel et al, 1992).

Data from laboratory and functional studies. In EEG studies, pathological epileptic patterns are often absent (Mayer, Schondienst, 1993). A significant number of artifacts are often recorded on the seizure EEG, which complicates the interpretation of the data (Morris et al, 1988). EEG using special electrodes (subdural, intracerebral) is more informative.

Differential diagnosis is carried out with various syndromes of both epileptic and non-epileptic origin (generalized epilepsy, temporal lobe epilepsy, pseudoepileptic seizures, nightmares).

Pseudoepileptic seizures, in comparison with frontal paroxysms, rarely occur at night, have a longer duration and are not stereotypical (Kanner et al, 1990).

Nightmares occur between the ages of 4 and 12 years, usually occurring in the first third of the night and never during the daytime. Attacks of nightmares are characterized by the absence of an aura, motor automatisms, and partial impairment of consciousness. At the time of the attack, vegetative symptoms are expressed - tachycardia, tachypnea. The duration of nightmare attacks is 5-6 minutes. An EEG study during nightmares does not reveal epileptic patterns.

Differential diagnosis between frontal and temporal paroxysms is based on the following criteria: the presence of an aura, the nature of automatisms, impaired consciousness, duration of attacks, the presence of post-attack confusion. With temporal attacks, in comparison with frontal ones, auras, oroalimentary automatisms are often observed, consciousness is often impaired, post-attack confusion, and a longer (1-4 minutes) duration of paroxysm.

When differential diagnosis with primary generalized epilepsies, the semiology of seizures should be taken into account. The EEG is not always sufficiently informative, since frontal paroxysms tend to undergo rapid secondary generalization (Holthausen, 1995).

Treatment. The first-line drugs of choice for the treatment of frontal lobe epilepsy, as well as temporal lobe epilepsy, are carbamazepine and phenytoin.

ESES (electrical status epilepticus during slow sleep) Electrical status epilepticus during slow sleep

Patry et al in 1971 described peculiar EEG patterns in the form of “electrical status epilepticus” (ESES). Later, Tassinari et al (1977, 1982), Billiard et al (1982) presented a clinical description of the syndrome, calling it “epilepsy with prolonged spike-wave complexes during slow sleep” (CSWS-continuos spike-wave during slow sleep). Under this name, the disease was included in the International Classification of Epilepsies and Epileptic Syndromes. In recent years, it has been established that ESES is an electrographic diagnosis and in some cases may not be accompanied by clinical manifestations.

Clinical characteristics. The disease manifests itself at the age of 8 months to 11.5 years (Tassinari et al, 1985), more often between 4 and 14 years (Morikawa et al, 1985, 1989). After 15 years, the syndrome usually does not occur (Tassinari et al, 1985; Morikawa et al, 1989). Often attacks occur at night. By nature, attacks can be either generalized or partial. Depending on the semiology of attacks, Tassinari et al (1994) identified 3 groups of patients:

1. Patients with only motor seizures (myoclonic absences, generalized clonic seizures, orofacial paroxysms). 2. Patients with initially unilateral partial motor seizures or generalized tonic-clonic paroxysms in combination with atypical absences, which develop as the disease progresses. 3. Patients with nocturnal generalized tonic-clonic seizures in combination with atypical absences and atonic paroxysms. The frequency of attacks is variable - from rare to daily.

Data from laboratory and functional studies. On interictal EEG in a state of wakefulness, generalized spike-wave complexes are recorded, sometimes in the form of flashes, or focal spikes or sharp waves with a predominant localization in the frontotemporal or centrotemporal regions (Tassinari et al, 1994). During slow-wave sleep, the EEG shows prolonged (85-100% of the entire curve), bilateral or diffuse “spike-wave” complexes. Physiological sleep patterns (sleep spindles, K-complexes or vertex waves) are practically absent (Tassinari et al. 1994).

Differential diagnosis should be made with Landau-Kleffner, Lennox-Gastaut syndromes, and Rolandic epilepsy. The most difficult is differential diagnosis with Landau-Kleffner syndrome. With Landau-Kleffner syndrome, speech disorders and behavioral disorders come to the fore. The nature of the attacks is usually atonic, while with ESES syndrome, generalized tonic-clonic, atypical absences are most often observed. The question of the possible transformation of ESES syndrome into Landau-Kleffner syndrome is being discussed (Shoumaker et al, 1974; Kellerman, 1978; Hirsch et al, 1990).

In some cases, with ESES syndrome, both daytime and nighttime seizures are observed, combined with speech disorders, which predetermines the need for differential diagnosis with Rolandic epilepsy. The most significant differential diagnostic criteria are the absence of neuropsychological disorders in rolandic epilepsy and the presence of specific spike patterns on the EEG with a predominant localization in the centrotemporal regions. In ESES syndrome, focal EEG changes are recorded predominantly in the frontal areas (Tassinari et al, 1985).

In ESES syndrome, in comparison with Lennox-Gastaut syndrome, there are no tonic seizures and slow spike-wave complexes with a frequency of 2-2.5 Hz (Patry et al, 1971).

Treatment. The order of choice of drugs depends on the nature of the attacks. In the presence of atypical absence seizures, sodium valproate and ethosuximide are recommended; for partial paroxysms, carbamazepine or phenytoin is recommended.

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