Paroxysmal activity of brainstem structures during hyperventilation. Symptoms of frontal lobe damage. Frontal pole, orbitofrontal cortex

general characteristics

Rolandic epilepsy- one of the forms of idiopathic locally caused epilepsy of childhood, manifested mainly by nocturnal short hemifacial motor seizures and having a favorable prognosis.

Rolandic epilepsy appears between the ages of 2 and 14 years. Seizures can be partial and secondary generalized. Most partial seizures are motor. Typical attacks, occurring both in a state of wakefulness, but increasingly during sleep (mainly the first half of the night), usually occur with preserved consciousness and begin with a somatosensory aura, characterized by paresthesia of the cheek, muscles of the pharynx and larynx, a feeling of pinpricks, numbness in the cheek, gums, tongue on the side opposite to the focus. Then motor phenomena appear in the form of unilateral hemifacial (involving the facial muscles) clonic or tonic-clonic seizures, which can spread to the homolateral arm (facio-brachial seizures) and leg (unilateral seizures).

At the beginning of an attack or during its development, speech difficulties arise, expressed in the complete inability to speak or pronounce individual sounds. Along with anarthria, hypersalivation is noted, characterized by abundant production and release of saliva from the mouth, which contributes to the appearance of squelching, grunting sounds.

The duration of attacks, as a rule, is no more than 2–3 minutes, the frequency on average is 2–6 times a year [Temin P.A., Nikanorova M.Yu., 1999; Petrukhin A.S., 2000].

With Rolandic epilepsy, in isolated cases the development of status epilepticus is possible.

Patients with Rolandic epilepsy are characterized by normal intelligence and neurological status, although a number of modern researchers still point to the presence of neuropsychological deficiency in such children [Epileptiformnaya..., 2006].

A number of authors note the possibility of attacks during rolandic epilepsy, characterized by abdominal pain, dizziness, visual phenomena (flashes of light, blindness, flashing objects before the eyes), which are usually observed in children under 5 years of age; complex partial seizures or typical absence seizures.

TO include a group of patients aged 2–8 years who experience simple partial hemifacial and hemiclonic nocturnal seizures, combined with myoclonic-astatic, atonic paroxysms and, in some cases, absences, but never, unlike true Lennox-Gastaut syndrome, there are tonic seizures [Temin P.A., Nikanorova M.Yu., 1999]. The frequency of attacks is usually high. Before the onset of seizures, children do not experience any disturbances in neuropsychic development.

The seizure and interictal EEG in patients with rolandic epilepsy is characterized by normal baseline activity and usually diphasic spikes followed by a slow wave. Spikes or sharp waves appear singly or in groups in the mid-temporal and central region or the temporo-central-parietal region [Mukhin K.Yu. et al., 1995; Epileptiformnaya..., 2006]. They can be observed either unilaterally (usually contralateral to hemifacial seizures) or bilaterally (synchronously or asynchronously). "Rolandic" complexes are usually most clearly expressed when the eyes are open (Fig. 3.1).

In some cases, generalized “spike-wave” complexes, typical of absence seizures, are recorded on the EEG. Sharp waves can also be located in the occipital region. Also, during a night attack, the EEG can record low-amplitude fast activity in the central-temporal region, turning into the Rolandic complexes with spread to the entire hemisphere and subsequent generalization.

At atypical rolandic epilepsy (pseudolennox syndrome) the background EEG is not changed or is slightly slowed down and disorganized [Mukhin K.Yu., 2000] (Fig. 3.2a), in some cases, mainly in the frontal regions. Typical rolandic spikes may be combined with slow sharp-slow wave complexes characteristic of Lennox-Gastaut syndrome or irregular diffuse peak-wave activity with a frequency of about 2.5 counts/s and amplitude regional predominance in the frontal regions; focal spikes or slow waves can be detected in the central temporal region [Osobennosti..., 2005] or the central temporo-parietal region (Fig. 3.2b).

In an attack EEG, there may be an asynchronous appearance of discharges of high-amplitude irregular diffuse “peak-wave” or “sharp-slow wave” complexes with a frequency of 1.5–4 counts/s lasting 3–10 s, synchronously with a paroxysm of atypical absence seizures [Temin P.A., Nikanorova M.Yu., 1999].

3.2. Benign epilepsy of childhood with occipital paroxysms

general characteristics

Benign epilepsy of childhood with occipital paroxysms is one of the forms of idiopathic locally caused epilepsy of childhood, characterized by attacks that occur mainly in the form of paroxysms of visual disturbances and often ending in migraine headache. The age of manifestation of the disease varies from 1 to 17 years.

Benign occipital epilepsy with early onset occurs in children under 7 years of age and is characterized by rare, predominantly nocturnal paroxysms. The attack, as a rule, begins with vomiting, tonic deviation of the eyeballs to the side and impaired consciousness. In some cases, there is a transition to hemiconvulsions or a generalized tonic-clonic seizure. The duration of attacks varies from several minutes to several hours. These patients may experience partial seizure status.

Benign occipital epilepsy with late onset manifests itself in children 3–17 years old and is characterized by visual phenomena (transient visual impairment, amaurosis, elementary visual hallucinations (flickering of luminous objects, figures, flashes of light before the eyes), complex (scene-like) hallucinations) and “non-visual” symptoms (hemiclonic convulsions, generalized tonic-clonic convulsions, automatisms, dysphasia, dysesthesia, versive movements). Attacks predominantly occur during the daytime and occur, as a rule, with preserved consciousness. In the post-attack state, diffuse or migraine-like headache may occur, sometimes accompanied by nausea and vomiting.

Patients with this form of epilepsy are characterized by normal intelligence and neuropsychic development.

Electroencephalographic patterns

Interictal EEG is characterized by normal basic activity and the presence of high-amplitude mono- or bilateral spikes, sharp waves, sharp-slow wave complexes, including those with “rolandic” morphology, or slow waves in the occipital or posterior temporal regions. It is characteristic that pathological EEG patterns, as a rule, appear when the eyes are closed and disappear when the eyes are open [Zenkov L.R., 1996].

Occipital epileptiform activity can be combined with generalized bilateral spike-wave and polyspike-wave complexes. Sometimes epileptiform activity in this form of epilepsy can be represented by short generalized discharges of peak-wave complexes with a frequency of 3 counts/s, or is localized in the frontal, central-temporal, central-parietal-temporal leads (Fig. 3.3). Also, interictal EEG may not show any changes [Mukhin K.Yu. et al., 2004; Atlas..., 2006].

Seizure EEG may be characterized by unilateral slow activity interspersed with spikes.

At occipital epilepsy with early onset The EEG during an attack is represented by high-amplitude sharp waves and slow “sharp-slow wave” complexes in one of the posterior leads, followed by a diffuse distribution.

At occipital epilepsy with late onset On the EEG during an attack, rhythmic fast activity occurs in the occipital leads, followed by an increase in its amplitude and a decrease in frequency without post-attack slowing; generalized slow “acute-slow wave” complexes may be observed.

3.3. Chronic progressive continuous epilepsy of childhood (Kozhevnikov-Rasmussen syndrome)

general characteristics

Convulsive attacks in this form of epilepsy begin with focal motor paroxysms, followed by the addition of constant myoclonus.

Unilateral partial motor seizures occur, as a rule, with preserved consciousness and are manifested by clonic twitching of the face, one limb or half of the body. Often attacks are accompanied by a “Jacksonian march”. The onset of the disease can also occur with complex partial and somatosensory paroxysms. Partial seizures are short, no more than 1–2 minutes. In most cases, within a year from the onset of the disease, partial paroxysms are joined by constant stereotypical myoclonus localized in one half of the trunk and limbs, which can transform into generalized convulsions.

As the disease progresses, paresis and paralysis occur in the same limbs. In most patients, cranial nerves are involved in the pathological process, cerebellar disorders are noted, and there may be sensory disturbances.

Changes in mental functions may be absent in Kozhevnikov epilepsy, and Rassmusen syndrome is usually characterized by reduced intelligence.

Electroencephalographic patterns

Interictal and seizure EEG during Kozhevnikov's epilepsy may be characterized by unchanged basic activity or reflect its general slowdown with a reduced alpha rhythm index, against the background of which spikes, sharp waves or spike-wave complexes are detected in the central regions (Fig. 3.4) Frequent discharges of high-amplitude theta are often recorded in the interictal EEG -, delta activity both regionally and generally bilaterally synchronously.

At Rassmusen's syndrome in the interictal EEG there is a slowdown in the main activity, the presence of bilateral or diffuse delta waves of medium or high index. The alpha rhythm may be reduced in amplitude and disorganized in the affected hemisphere. High amplitude delta activity may predominate on the affected side. Multifocal spikes, sharp waves, or peak-wave activity within the affected hemisphere are detected. Epileptiform activity may be most pronounced in the temporo-parietal-central leads. The appearance of independent foci in the “healthy” hemisphere is possible; epileptiform activity can be diffuse asynchronous with spread from the affected hemisphere.

During an attack, the EEG reveals local or unilateral rhythmic oscillations of the beta range, turning into polyspikes with subsequent slowing down and transition to the end of the attack into bilateral synchronous theta and delta waves. Sometimes during an attack there is a local or unilateral flattening of the EEG [Mukhin K.Yu. et al., 2004].

3.4. Epilepsy with seizures provoked by specific factors

general characteristics

Epilepsy with seizures provoked by specific factors is characterized by partial and partial-complex seizures, which are regularly reproduced by some direct influence. A large group consists of reflex attacks.

Haptogenic seizures are caused by thermal or tactile irritation of a certain area of ​​the body surface, usually projected into the zone of epileptogenic focus in the cortex with its destructive focal lesion.

Photogenic seizures are caused by flickering light and manifest as small, myoclonic, and grand mal seizures.

Audiogenic seizures are caused by sudden sounds, certain melodies and are manifested by temporal psychomotor, grand mal, myoclonic or tonic seizures.

Startle attacks are caused by a sudden startling stimulus and manifest as myoclonic or brief tonic seizures.

Electroencephalographic patterns

Interictal EEG may be within normal limits, but is somewhat more likely to show the following changes.

At haptogenic attacks During the interictal period, focal epileptiform patterns are recorded on the EEG in the parietotemporal region of the hemisphere (sometimes in both hemispheres) opposite the somatic zone. During an attack, the appearance or activation and generalization of primary focal epileptiform activity is noted.

At photogenic attacks in the interictal period and during an attack, focal slow waves, epileptiform patterns in the occipital, parietal or temporal regions of one (Fig. 3.5a), sometimes both hemispheres and (or) hypersynchronous generalized, usually bilateral synchronous epileptiform activity are recorded on the EEG (Fig. 3.5b).

At audiogenic seizures in the interictal period and during an attack, the EEG reveals slow waves, epileptiform patterns in the temporal regions or diffusely in one, sometimes both hemispheres and/or hypersynchronous generalized, usually bilaterally synchronous epileptiform activity.

At startle-attacks in the interictal period and during an attack, bilaterally synchronous bursts of theta waves, epileptiform patterns in the temporal, parietal areas or diffusely in one, sometimes both hemispheres and/or hypersynchronous generalized discharge, usually bilaterally synchronous epileptiform activity are recorded on the EEG [Zenkov L.R. ., 1996; 2001; Atlas..., 2006].

3.5. Frontal lobe epilepsy

general characteristics

Frontal epilepsy- a locally caused form of epilepsy, in which the epileptic focus is localized in the frontal lobe.

The most characteristic signs of frontal epilepsy are: stereotyped attacks, their sudden onset (usually without an aura), high frequency of attacks with a tendency to be serial and short duration (30–60 sec.) Unusual motor phenomena are often expressed (pedaling with legs, chaotic movements, complex gestures automatisms), absence or minimal post-ictal confusion, frequent occurrence of attacks during sleep, their rapid secondary generalization, frequent occurrence of episodes of status elepticus in the anamnesis.

Depending on the location in the frontal lobe, R. Chauvel and J. Bancaud (1994) distinguish a number of types of frontal seizures.

Seizures of the anterior frontal lobe

Frontopolar seizures manifested by a sudden disturbance of consciousness, frozen gaze, violent thinking and violent actions, tonic turning of the head and eyes, vegetative symptoms, possibly tonic tension of the body and falling.

Orbitofrontal seizures manifested by olfactory hallucinations, visceral sensory symptoms, impaired consciousness, gestural automatisms, nutritional disorders, autonomic symptoms, and involuntary urination.

Seizures of the medial frontal lobe

Medial midline seizures appear frontal absence seizures(characterized by impaired consciousness, speech cessation, interruption of motor activity, gestural automatisms, and sometimes tonic turning of the head and eyes) and psychomotor paroxysms(characterized by impaired consciousness, tonic rotation of the head and eyes, gestural automatisms, tonic postural phenomena, involuntary urination, secondary generalization is possible).

Dorsolateral median seizures manifested by impaired consciousness, violent thinking, complex visual illusions, tonic rotation of the head and eyes, tonic postural phenomena, secondary generalization, and sometimes characterized by vegetative symptoms.

Cingular attacks manifested by an expression of fear on the face, impaired consciousness, vocalization, complex gestural automatisms, emotional symptoms, redness of the face, involuntary urination, and sometimes visual hallucinations.

Posterior frontal lobe seizures

Seizures originating from the precentral motor cortex occur with preserved consciousness and are manifested by partial myoclonus (mainly in the distal parts of the extremities), simple partial motor seizures (in the form of a “Jacksonian march”, developing contralateral to the lesion and spreading in an ascending (leg-arm-face) or descending (face-arm) type leg) march), tonic postural paroxysms in combination with clonic twitching, unilateral clonic seizures.

Seizures originating from the premotor area of ​​the motor cortex occur with preserved consciousness and are manifested by tonic postural paroxysms with predominant involvement of the upper extremities, tonic rotation of the head and eyes.

Seizures originating from the supplementary motor area occur with intact (or partially impaired) consciousness and are often manifested by somatosensory aura, postural tonic postures (fencer's pose) with predominant involvement of the proximal limbs, tonic rotation of the head and eyes, stopping speech or vocalization, pedaling movements of the legs, mydriasis.

Opercular seizures manifested by taste hallucinations and illusions, fear, impaired consciousness, chewing and swallowing automatisms, clonic facial twitching, hypersalivation, hyperpnea, tachycardia, mydriasis.

Most researchers emphasize that a clear determination of the localization of the epileptogenic zone in the frontal lobe is not always possible. Therefore, it is more expedient to differentiate attacks in frontal lobe epilepsy into partial motor, manifesting either a contralateral versive component or unilateral focal clonic motor activity in combination (or without) with a tonic component in the late phases of the attack; partial psychomotor, debuting with sudden stupefaction and freezing of the gaze; seizures from the supplementary motor area, characterized by tonic postural positions of the limbs.

Electroencephalographic patterns

In the interictal period, the EEG may show disorganization and/or deformation of the main rhythms.
Epileptic patterns are often absent. If epileptiform activity is recorded, it is represented by spikes, sharp waves, peak-wave or slow (usually theta range) activity in the frontal, fronto-central, fronto-temporal or fronto-central-temporal leads bilaterally in the form of independent foci or bilaterally synchronously with amplitude asymmetry. Characteristic is the occurrence of local epileptiform activity, accompanied by its bilateral spread and (or) generalization (in some cases in the form of an atypical absence pattern); the appearance of generalized bilateral epileptiform activity is possible, more often with its amplitude predominance in the frontal, frontotemporal regions (Fig. 3.6, Fig. 3.7, Fig. 3.8a, Fig. 3.8b, Fig. 3.8c, Fig. 3.8d, Fig. 3.9, Fig. 3.10, Fig. 3.11, Fig. 3.12, Fig. 3.13, Fig. 3.14a, Fig. 3.14b, Fig. 15, Fig. 3.16, Fig. 3.17, Fig. 3.18a, Fig. 3.18b) .

Local disturbances (exaltation or significant reduction) of rhythms are also possible. When the supplementary motor area is damaged, pathological EEG patterns are often ipsilateral to clinical phenomena or bilateral.

Sometimes EEG changes in frontal epilepsy can precede the clinical appearance of seizures and manifest as bilateral high-amplitude single sharp waves immediately following periods of rhythm flattening; low-amplitude fast activity mixed with spikes; rhythmic spike waves or rhythmic slow waves of frontal localization [Petrukhin A.S., 2000].

During an attack, the EEG may show local epileptiform activity with (or without) the subsequent appearance of generalized and (or) bilaterally synchronous discharges of peak-wave complexes, reflecting secondary generalization (Fig. 3.19). High-amplitude regular theta and delta waves may occur, mainly in the frontal and (or) temporal leads [Zenkov L.R., 1996, 2001]. Also, during an attack, diffuse flattening may occur, most pronounced in the focal area, followed by the appearance of rapid activity, increasing in amplitude and decreasing in frequency.

3.6. Temporal lobe epilepsy

general characteristics

Temporal lobe epilepsy- locally caused, often symptomatic, form of epilepsy in which the epileptic focus is localized in the temporal lobe.

Temporal lobe epilepsy manifests itself as simple, complex partial and secondary generalized seizures or a combination thereof.

The most typical signs of temporal lobe epilepsy are: the predominance of psychomotor seizures, a high frequency of isolated auras, oroalimentary and carpal automatisms, frequent secondary generalization of seizures [Troitskaya L.A., 2006].

Complex partials(psychomotor) seizures can begin with or without a preceding aura and are characterized by a loss of consciousness with amnesia, lack of response to external stimuli, and the presence of automatisms.

Auras include epigastric(tickling, epigastric discomfort), mental(fear), olfactory,vegetative(pallor, redness of the face), intellectual(feeling of what has already been seen, already heard, derealization), auditory(auditory illusions and hallucinations (unpleasant sounds, voices, difficult to describe auditory sensations)) and visual(illusions and hallucinations in the form of micro- and macropsia, flashes of light, the sensation of an object being removed) aura.

Automatisms are divided into oroalimentary(smacking, chewing, licking lips, swallowing); facial expressions(various grimaces, facial expressions of fear, surprise, smile, laughter, frowning, forced blinking), gestural(patting your hands, rubbing your hand on your hand, stroking or scratching your body, sorting through clothes, shaking off, shifting objects, as well as looking around, marking time, rotating around its axis, standing up; it has been revealed that automatisms in the hand are associated with damage ipsilateral temporal lobe, and dystonic hand placement - from the contralateral one); outpatient(attempt to sit down, stand up, walking, seemingly purposeful actions); verbal(speech disorders: slurred muttering, pronouncing individual words, sounds, sobbing, hissing; it was revealed that seizure speech is associated with damage to the dominant hemisphere, and aphasia and dysarthria - to the subdominant).

It has been noted that in children under 5 years of age, as a rule, there is no clearly identifiable aura, oroalimentary automatisms predominate and motor activity is most pronounced at the time of the attack.

The duration of psychomotor temporal paroxysms varies from 30 s to 2 min. After an attack, confusion and disorientation and amnesia are usually observed. Seizures occur both while awake and during sleep.

More often, in patients with psychomotor temporal paroxysms, clinical symptoms occur in a certain sequence: aura, then interruption of motor activity (maybe with a stoppage of gaze), then oroalimentary automatisms, repeated carpal automatisms (less often other automatisms), the patient looks around, then movements of the whole body .

Simple partial seizures often precede the onset of complex partial and secondary generalized seizures.

Simple partial motor seizures manifested by local tonic or clonic-tonic convulsions, contralateral to the lesion; postural dystonic paroxysms (in the contralateral hand, foot); versive and phonatory (sensory aphasia) seizures.

Simple partial sensory seizures manifested by olfactory, gustatory, auditory, complex visual hallucinations and stereotypical non-systemic dizziness.

Simple partial vegetative-visceral seizures manifested by epigastric, cardiac, respiratory, sexual and cephalgic paroxysms.

Simple partial seizures with mental impairment manifested by dreamlike states, phenomena of derealization and depersonalization, affective and ideational (“failure of thoughts,” “whirlwind of ideas”) paroxysms [Temporal..., 1992, 1993; Petrukhin A.S., 2000].

With temporal lobe epilepsy, there are also so-called “temporal syncopes”, starting with an aura (usually dizziness) or without it and characterized by a slow shutdown of consciousness followed by a slow fall. During such attacks, oroalimentary or gestural automatisms may be noted; slight tonic tension in the muscles of the limbs and facial muscles.

Epileptic activity from the temporal lobe often spreads to other areas of the brain. Clinical signs indicating the spread of epileptic activity to other parts are versive movements of the head and eyes, clonic twitching of the face and limbs (with the spread of epileptic activity to the anterior parts of the frontal lobe and premotor zone), secondary generalization with the manifestation of generalized tonic-clonic seizures (with involving both hemispheres of the brain in the process).

Neurological status is determined by the etiology of temporal lobe epilepsy.

Electroencephalographic patterns

Interictal EEG may not show pathological patterns. Spike, sharp waves, peak-wave, polypeak-wave activity or bursts of theta waves can be recorded in the temporal, frontotemporal, central-parietal-temporal and/or parieto-occipital-temporal leads regionally or bilaterally (bilaterally synchronous with one-sided accent or independently); regional temporal slowing of electrical activity; general slowdown in core activity. Generalized peak-wave activity at 2.5–3 Hz may be present; generalized epileptiform activity with emphasis and/or spread from the temporal region. An atypical absence pattern is a common finding. Sometimes pathological changes have a frontal focus (,

3.7. Epilepsy of the parietal lobe

general characteristics

Parietal epilepsy- a locally caused form of epilepsy, characterized mainly by simple partial and secondary generalized paroxysms.

Parietal epilepsy usually debuts with somatosensory paroxysms, which are not accompanied by impaired consciousness, have a short duration (from a few seconds to 1–2 minutes) and, as a rule, are caused by the involvement of the postcentral gyrus in the epileptic process.

Clinical manifestations of somatosensory paroxysms include: elementary paresthesia, pain, disturbances in temperature perception (burning or cold sensation), “sexual attacks,” ideomotor apraxia, disturbances in the body diagram.

Elementary paresthesias represented by numbness, tingling, tickling, a sensation of “crawling goosebumps”, “pin pricks” in the face, upper limbs and other segments of the body. Paresthesia can spread like a Jacksonian march and be combined with clonic twitching.

Painful sensations expressed as a sudden sharp, spasmodic, throbbing pain that is localized in one limb, or in part of a limb, sometimes can spread like a Jacksonian march.

"Sexual Attacks" presented by unpleasant one-sided sensations of numbness, tingling, and sometimes pain in the genital and mammary glands. These seizures are caused by epileptic activity in the paracentral lobule.

Ideomotor apraxia is presented by sensations of impossibility of movement in the limb; in some cases, a Jacksonian march-like spread is noted in combination with focal tonic-clonic convulsions in the same part of the body.

Body schema disorders include sensations of movement in a stationary limb or part of the body; feeling of flight, soaring in the air; a feeling of a body part being removed or shortened; feeling of enlargement or reduction of a part of the body; the feeling of the absence of a limb or the presence of an additional limb [Zenkov L.R., 1996].

Parietal seizures tend to spread epileptic activity to other areas of the brain, and therefore, in addition to somatosensory disturbances at the time of the attack, clonic jerking of the limb (frontal lobe), amaurosis (occipital lobe), tonic tension of the limb and automatisms (temporal lobe) can be observed. .

Electroencephalographic patterns

Interictal EEG often does not show pathological patterns. If pathological activity is noted, it is represented by spikes, sharp waves, sometimes acute-slow wave and spike-wave complexes in the parietal leads, according to the nature of the attack [Zenkov L.R., 2001]. Often, epileptiform activity is distributed outside the parietal region and can be represented in the temporal lobe of the same name (Fig. 3.40).

In the seizure EEG, spikes and “spike-wave” complexes can be recorded in the central-parietal and temporal regions, discharges of epileptiform activity can be bilateral (synchronous or in the form of a “mirror focus”) [Temin P.A., Nikanorova M.Yu., 1999].

3.8. Epilepsy of the occipital lobe

general characteristics

Occipital epilepsy- a locally caused form of epilepsy, characterized mainly by simple partial paroxysms not accompanied by impaired consciousness.

Early clinical symptoms of occipital epilepsy are caused by epileptic activity in the occipital lobe, and late clinical symptoms are caused by the spread of epileptic activity to other areas of the brain.

The initial clinical symptoms of occipital paroxysms include: simple visual hallucinations, paroxysmal amaurosis and visual field disturbances, subjective sensations in the area of ​​the eyeballs, blinking, deviation of the head and eyes to the side contralateral to the epileptic focus.

Simple visual hallucinations are represented by bright flashes of light before the eyes, luminous spots, circles, stars, squares, straight or zigzag lines, which can be single-colored or multi-colored, stationary or moving in the field of view.

Paroxysmal amaurosis manifests itself in the form of blurred vision or temporary loss of vision, felt as “blackness before the eyes” or “white veil before the eyes.”

Paroxysmal visual field disturbances manifest as paroxysmal hemianopsia or quadrant hemianopia within seconds or minutes.

Subjective sensations in the area of ​​the eyeballs are expressed predominantly by a feeling of eye movement in the absence of objective symptoms.

blinking It is noted at the very beginning of the attack, is violent in nature and resembles the fluttering of a butterfly’s wings.

Electroencephalographic patterns

Interictal EEG may not show pathological patterns or may be represented by epileptiform activity in the occipital or posterior temporal region, sometimes bilaterally. The main activity may not be changed or there may be disorganization and slowness. Epileptiform activity can often also be falsely represented in the temporal lobe of the same name (Fig. 3.41)

During an attack, epileptiform activity may spread with the appearance of “mirror” discharges.

Zalevsky Timur Romanovich, 2 years 6 months (born August 30, 2014) AEDs accepted: does not receive. Video-EEG monitoring was carried out for 4 hours in a state of active and passive wakefulness, during daytime sleep and after awakening, with functional tests. Recording parameters: The study was conducted using the international “10-20” electrode placement pattern. Additional electrodes: ECG. Video-EEG monitoring system – Nihon Kohden, Japan. EEG in a state of wakefulness. The recording of wakefulness was carried out mainly with open eyes, the child is motorically active, and a large number of motor and myographic artifacts are noted. The main activity was assessed when looking closely at an object and at the moment of closing the eyes - rhythmic activity with a frequency of 6-7 Hz, an amplitude of up to 70 μV - the equivalent of the alpha rhythm - was fragmentarily recorded in the occipital regions of the hemispheres. In a state of active wakefulness, an arched sensorimotor rhythm with a frequency of 8 Hz and an amplitude of up to 50 μV is recorded in the fronto-central regions. Beta activity is represented maximally in the frontotemporal parts of the hemispheres, with variable lateralization, frequency 14-24 Hz, amplitude up to 20 μV, and is often difficult to differentiate against the background of myographic artifacts. Bioccipito-temporally, periodically with variable lateralization, irregular polyphasic potentials of the theta-delta range are recorded - occipital delta waves of children. Slow forms of activity are presented widely, diffusely in the form of low-amplitude waves, predominantly theta-, less often delta-range, insignificantly. During wakefulness, regional epileptiform activity in the left and right occipital regions is recorded independently in the form of single peaks and sharp waves, with an amplitude of up to 80 μV. . Functional tests. A test with opening and closing the eyes was not performed. The test with rhythmic photostimulation was carried out at frequencies of 3, 6, 9, 12, 15, 18, 21, 24, 27, 30, 33 Hz; photoparoxysmal forms of activity were not recorded. A clear reaction of rhythm assimilation was not detected. A hyperventilation test was not performed. Record while you sleep. As the patient fell asleep, there was a decrease in the index of basic activity, up to a reduction, and an increase in diffuse slow-wave activity in the theta range. Against this background, bilaterally synchronous flashes of slow waves of the delta range are recorded, with an amplitude of up to 220 μV, with an amplitude predominance bifrontally, periodically with a shift to the central regions - the phenomenon of hypnagogic hypersynchronization (a physiological phenomenon of the drowsiness stage). In the first and second stages of sleep, the appearance of vertex potentials in the central parts of the hemispheres is recorded, with an amplitude of up to 170 μV. Sharpened potentials similar to sharp-slow wave complexes in the frontal-central regions with amplitude predominance in the vertex leads were also recorded. Taking into account the morphological and localization features, these patterns can be considered within the framework of atypical physiological sleep transits - vertex potentials. The 2nd stage itself is represented by “sleep spindles” - fast rhythmic forms of activity in the fronto-central parts of the hemispheres, with a frequency of 12-14 Hz, amplitude up to 80 μV and K-complexes in the form of diffuse slow waves or polyphasic potentials, maximum amplitude in the central parts hemispheres, up to 260 µV. During sleep recording, arc-shaped, pointed waves with a frequency of 6-7 Hz, 14 Hz are periodically recorded in the temporal regions of the hemispheres, often with a tendency to diffuse distribution - physiological non-epileptic sleep transits of “6-14 Hz”. Delta sleep was accompanied during some recording epochs by an increase in the representation of diffuse high-amplitude slow-wave activity, first to 50% and then to 80% of the recording, with a simultaneous gradual reduction of physiological sleep patterns. During sleep, periodic regional theta-delta slowing is detected in the right temporal region, as well as in the left occipitotemporal region independently. Against this background, in the structure of regional slowing with a low index, regional epileptiform activity is recorded in the left and right occipital regions independently, less often in the right posterior temporal region (T6) with spread to the temporal parts of the ipsilateral hemisphere, as well as biocipitally in the form of single and grouped peaks and sharp waves , peak-slow wave complexes, sharp-slow wave, amplitude up to 160 μV. No clinical events were recorded during the study. Conclusion: ​ The basic rhythm corresponds to age. ​ Sleep is modulated by stage. Physiological sleep patterns are visualized.  During sleep, periodic regional theta-delta slowing was detected in the right temporal region, as well as in the left occipitotemporal region independently. ​ During wakefulness, regional epileptiform activity was recorded with an extremely low index in the left and right occipital regions independently in the form of single peaks and sharp waves. ​ During sleep, in the structure of regional slowing with a low index, regional epileptiform activity was recorded in the left and right occipital regions independently, less often in the right posterior temporal region (T6) with distribution to the temporal parts of the ipsilateral hemisphere, as well as bioccipitally in the form of single and grouped peaks and sharp waves, peak-slow wave complexes, sharp-slow wave. ​ No epileptic seizures were recorded. I am concerned about the delay in speech development (certain words from pictures, does not use in everyday life, speech is quiet, nasal), understands spoken speech, follows simple instructions, according to the speech pathologist, there are elements of autism. Hearing and vision are normal. Pregnancy and early development by age. We live in the Yaroslavl region, tell me if there is a need for an in-person consultation based on the EEG conclusion.

Central paralysis and paresis occur when lesions are localized in the precentral gyrus. The somatic representation of motor functions roughly corresponds to that for cutaneous sensitivity in the postcentral gyrus. Due to the large extent of the precentral gyrus, focal pathological processes (vascular, tumor, traumatic, etc.) usually affect it not all, but partially. Localization of the pathological focus on the outer surface causes predominantly paresis of the upper limb, facial muscles and tongue (linguofacial brachial paresis), and on the medial surface of the gyrus - predominantly paresis of the foot (central monoparesis). Paresis of gaze in the opposite direction is associated with damage to the posterior part of the middle frontal gyrus (“the patient looks at the lesion”). Less commonly, with cortical lesions, gaze paresis in the vertical plane is noted.

Extrapyramidal disorders in lesions of the frontal lobes are very diverse. Hypokinesis as an element of parkinsonism is characterized by a decrease in motor initiative, aspontaneity (limited motivation for voluntary actions). Less commonly, when the frontal lobes are affected, hyperkinesis occurs, usually during voluntary movements. Muscle rigidity is also possible (more often with deep-seated lesions).

Other extrapyramidal symptoms are grasping phenomena - involuntary automatic grasping of objects placed on the palm (Yanishevsky-Bekhterev reflex), or (less commonly observed) an obsessive desire to grab an object that appears before the eyes. It is clear that in the first case, the reason for the involuntary motor act is the effect on skin and kinesthetic receptors, in the second - visual stimulation associated with the functions of the occipital lobes.

With lesions of the frontal lobes, reflexes of oral automatism are revived. You can evoke the proboscis and palmomental (Marinescu-Radovici), less often nasolabial (Astvatsaturova) and distant-oral (Karchikyan) reflexes. Sometimes the “bulldog” symptom (Yanishevsky’s symptom) occurs - in response to touching the lips or mucous membrane of the oral cavity with some object, the patient convulsively clenches his jaw.

When the anterior parts of the frontal lobes are affected with the absence of paresis of the limbs and facial muscles, one can notice an asymmetry in the innervation of the facial muscles during the patient’s emotional reactions - the so-called “facial paresis of the facial muscles,” which is explained by a disruption in the connections of the frontal lobe with the visual thalamus.

Another sign of frontal pathology is a symptom of resistance or resistance, which appears when the pathological process is localized in the extrapyramidal regions of the frontal lobes. During passive movements, involuntary tension of antagonist muscles occurs, which creates the impression of conscious resistance of the patient to the actions of the examiner. A particular example of this phenomenon is the symptom of eyelid closure (Kokhanovsky's symptom) - involuntary tension of the orbicularis oculi muscle with closure of the eyelids when the examiner attempts to passively lift the patient's upper eyelid. It is usually observed on the side of the pathological focus in the frontal lobe. The same involuntary contraction of the occipital muscles during passive tilting of the head or extension of the lower limb at the knee joint can create a false impression that the patient has a meningeal symptom complex.

The connection of the frontal lobes with the cerebellar systems (fronto-pontocerebellar tract) explains the fact that when they are damaged, disorders of coordination of movements occur (frontal ataxia), which is manifested mainly by trunk ataxia, the inability to stand and walk (astasia-abasia) with deviation of the body in the opposite direction side of the lesion.

The frontal lobe cortex is an extensive field of the kinesthetic analyzer, so lesions of the frontal lobes, especially premotor areas, can cause frontal apraxia, which is characterized by incomplete actions. Frontal apraxia occurs due to a violation of the program of complex actions (their purposefulness is lost). Damage to the posterior part of the inferior frontal gyrus of the dominant hemisphere leads to motor aphasia, and to the posterior part of the middle frontal gyrus to “isolated” agraphia.

Changes in the sphere of behavior and psyche are very peculiar. They are referred to as the “frontal psyche.” In psychiatry, this syndrome is called apathetic-abulic: patients seem to be indifferent to their surroundings, their desire to carry out voluntary actions (motivation) decreases. At the same time, there is almost no criticism of their actions: patients are prone to shallow jokes (moria), and they are often complacent even in a serious condition (euphoria). These mental disorders can be combined with untidiness (a manifestation of frontal apraxia).

Symptoms of irritation of the frontal lobes are manifested by epileptic seizures. They are varied and depend on the location of the foci of irritation.

Jacksonian focal seizures occur as a result of irritation of certain areas of the precentral gyrus. They are limited to unilateral clonic and tonic-clonic convulsions on the opposite side in the muscles of the face, upper or lower limbs, but can further generalize and develop into a general convulsive seizure with loss of consciousness. When the tegmental part of the inferior frontal gyrus is irritated, attacks of rhythmic chewing movements, smacking, licking, swallowing, etc. occur (opercular epilepsy).

Adversive seizures are a sudden convulsive turn of the head, eyes and entire body in the direction opposite to the pathological focus. The attack may result in a general epileptic seizure. Adverse seizures indicate the localization of epileptic foci in the extrapyramidal parts of the frontal lobe (posterior parts of the middle frontal gyrus - fields 6, 8). It should be noted that turning the head and eyes to the side is a very common symptom of seizures and it indicates the presence of foci in the opposite hemisphere. When the cortex is destroyed in this zone, the head turns towards the location of the lesion.

General convulsive (epileptic) seizures without visible focal symptoms occur when the poles of the frontal lobes are affected; they are manifested by sudden loss of consciousness, muscle spasms on both sides of the body; tongue bite, foam at the mouth, and involuntary urination are often observed. In some cases, it is possible to determine the focal component of the lesion in the post-attack period, in particular temporary paresis of the limbs on the opposite side (Todd's palsy). An electroencephalographic study can reveal interhemispheric asymmetry.

Attacks of frontal automatism are complex paroxysmal mental disorders, behavioral disorders, in which patients unconsciously, unmotivated, automatically perform coordinated actions that can be dangerous to others (arson, murder).

Another type of paroxysmal disorder with lesions of the frontal lobes is petit epileptic seizures with a sudden loss of consciousness for a very short period of time. The patient's speech is interrupted, objects fall out of his hands, and less often, continuation of the started movement (for example, walking) or hyperkinesis (usually myoclonus) is observed. These short-term switches off consciousness are explained by the close connections of the frontal lobes with the midline structures of the brain (subcortical and brain stem).

When the base of the frontal lobe is damaged, homolateral anosmia (hyposmia), amblyopia, amaurosis, and Kennedy syndrome develop (atrophy of the optic nerve nipple on the side of the lesion, on the opposite side - congestion in the fundus).

The described symptoms show that when the frontal lobes are affected, movement and behavior disorders are observed mainly. There are also autonomic-visceral disorders (vasomotor, breathing, urination), especially with foci in the medial parts of the frontal lobes.

Syndromes of local damage to the frontal lobes

I. Precentral gyrus (motor area 4)

1. Facial area (unilateral damage - transient impairment, bilateral - permanent)
   • Dysarthria
   • Dysphagia
2. Hand area
   • Contralateral weakness, awkwardness, spasticity
3. Leg region (paracentral lobule)
   • Contralateral weakness
   • Apraxia of walking
   • Urinary incontinence (long-term with bilateral injuries)

II. Medial divisions (F1, cingulate gyrus)

1. Akinesia (bilateral akinetic mutism)
2. Perseverations
3. Grasp reflex in the hand and foot
4. Alien hand syndrome
5. Transcortical motor aphasia
6. Difficulty initiating movements of the contralateral arm (may require medical assistance)
7. Bilateral ideomotor apraxia

III. Lateral divisions, premotor area

1. Middle frontal gyrus (F2)
   • Deterioration of contralateral saccades
   • Pure agraphia (dominant hemisphere)
   • Contralateral weakness of the shoulder (mainly abduction and elevation of the arm) and hip muscles plus limb apraxia.
2. F2 of the dominant hemisphere. Motor aphasia

IV. Frontal pole, orbitofrontal region (prefrontal)

1. Apathy, indifference
2. Reduce criticism
3. Deterioration of goal-directed behavior
4. Impotence
5. Foolishness (moriah), disinhibition
6. Environment dependence syndrome
7. Apraxia of speech

V. Epileptic phenomena characteristic of the frontal localization of the epileptic focus.

VI. Damage to the corpus callosum (callosal syndromes)

1. Insufficiency of interhemispheric kinesthetic transfer
   • Inability to imitate the position of the contralateral hand
   • Apraxia of the left hand
   • Agraphia of the left hand
   • Constructive apraxia of the right hand
   • Intermanual conflict (alien hand syndrome)
2. Tendency to confabulation and unusual explanations for the behavior of one's left hand
3. Double hemianopsia.

The most common manifestation of frontal dysfunction is a defect in the ability to organize ongoing cognitive and behavioral acts. Motor functions can be impaired either in the direction of hyperkinesia (motor hyperactivity) with increased distractibility to external stimuli, or in the form of hypokinesia. Frontal hypokinesia is manifested by decreased spontaneity, loss of initiative, slowed reactions, apathy, and decreased facial expression. In extreme cases, akinetic mutism develops. It is caused by bilateral damage to the inferomedial frontal and anterior parts of the cingulate gyrus (interruption of connections of the frontal cortex with the diencephalon and the ascending activating reticular formation).

Characterized by problems in maintaining attention, the appearance of perseverations and stereotypies, compulsive-imitative behavior, mental torpidity, weakening of memory and attention. Unilateral inattention, affecting motor and sensory functions, most often observed with parietal damage, can also be observed after damage to the supplementary motor and cingulate areas. Global amnesia has been described in cases of massive damage to the medial frontal lobe.

An accentuation of premorbid personality characteristics is also characteristic, often the appearance of depressive disorders, especially after damage to the anterior sections on the left side. Typically decreased criticism, hyposexuality or, conversely, hypersexuality, exhibitionism, foolishness, puerile behavior, disinhibition, moria. Elevation of mood in the form of euphoria is more common with right-sided injuries than left-sided ones. Here, Mori-like symptoms are accompanied by elevated mood combined with motor agitation, carelessness, a tendency to flat, rude jokes and immoral acts. The patient's sloppiness and untidiness are typical (urinating on the floor in the room, in the bed).

Other manifestations include changes in appetite (especially bulimia) and polydipsia, gait disturbances in the form of apraxia of walking or a “marche a petite pas” type gait (walking in small short steps with shuffling).

Precentral gyrus (motor area 4)

Varying degrees of motor paresis in the hand can be observed with posterior frontal damage, as well as speech impairment with damage to these parts in the left hemisphere. Dysarthria and dysphagia with unilateral damage are often transient in nature, and with bilateral damage they are permanent. Impaired motor functions in the leg are characteristic of damage to the paracentral lobule (contralateral weakness or apraxia of walking). For the same localization, urinary incontinence is typical (long-term in case of bilateral injuries).

Medial divisions (F1, cingulate gyrus)

Damage to the medial parts of the frontal lobe is characterized by the so-called “anterior syndrome of akinetic mutism”, in contrast to the “posterior” (or mesencephalic) similar syndrome. In incomplete syndrome, “frontal akinesia” occurs. Damage to the medial sections is sometimes accompanied by impaired consciousness, oneiric states, and memory impairment. Motor perseverations may appear, as well as a grasping reflex in the hand and its analogue in the leg. “Bowing” seizures have been described, as well as such an unusual phenomenon as alien hand syndrome (a feeling of foreignness in the upper limb and involuntary motor activity in it.) The latter syndrome has also been described in cases of damage to the corpus callosum (less often in other localizations). It is possible to develop transcortical motor aphasia (described only in frontal lesions), bilateral ideomotor apraxia.

Lateral divisions, premotor area

Damage to the posterior parts of the second frontal gyrus causes gaze paralysis in the direction opposite to the lesion (the patient “looks at the lesion”). With less severe lesions, deterioration of contralateral saccades is observed. In the left hemisphere, close to this zone, there is an area (upper premotor), damage to which causes isolated agraphia (“pure agraphia”, not associated with motor aphasia). A patient with agraphia is unable to write even individual letters; mild damage to this area can only manifest itself as an increase in the frequency of spelling errors. In general, agraphia can also develop with local lesions of the left temporal and left parietal lobes, especially near the Sylvian fissure, as well as with involvement of the basal ganglia on the left.

Damage to the posterior part of the third frontal gyrus in Broca's area causes motor aphasia. With incomplete motor aphasia, there is a decrease in speech initiative, paraphasia and agrammatism.

Frontal pole, orbitofrontal cortex

Damage to these departments is characterized by apathy, indifference, spontaneity, as well as mental disinhibition, decreased criticism, foolishness (moriah), disorders of goal-directed behavior, and syndrome of dependence on the immediate environment. Impotence may develop. Oral and manual apraxia is very typical for damage to the left anterior regions. When the orbital surface of the brain is involved (eg, meningioma), unilateral anosmia or unilateral optic atrophy may occur. Foster-Kennedy syndrome is sometimes observed (decreased sense of smell and vision on one side and congestive nipple on the opposite).

Damage to the corpus callosum, especially its anterior sections, separating the frontal lobes, is accompanied by peculiar syndromes of apraxia, agraphia (mainly in the left non-dominant hand), and other rarer syndromes (see the section “Damages to the corpus callosum” below),

The above neurological syndromes can be summarized as follows:

Any (right or left) frontal lobe.

1. Contralateral paresis or incoordination of an arm or leg.
2. Kinetic apraxia in the proximal parts of the contralateral hand (lesion of the premotor area).
3. Grasp reflex (contralateral supplementary motor area).
4. Decreased activity of facial muscles in voluntary and emotional movements.
5. Contralateral oculomotor neglect during voluntary gaze movements.
6. Hemi-inattention.
7. Perseverations and torpidity of the psyche.
8. Cognitive impairment.
9. Emotional disturbances (lack of spontaneity, decreased initiative, affective flattening, lability.
10. Deterioration of olfactory discrimination of odors.

Non-dominant (right) frontal lobe.

1. Instability of the motor sphere (motor program): what is referred to in foreign literature by the term “motor impersistence”, which does not have a generally accepted translation into Russian.
2. Inadequate perception (understanding) of humor.
3. Disturbances in the flow of thinking and speech.

Dominant (left) frontal lobe.

1. Motor aphasia, transcortical motor aphasia.
2. Oral apraxia, limb apraxia with preserved understanding of gestures.
3. Impaired fluency of speech and gestures.

Both frontal lobes (simultaneous damage to both frontal lobes).

1. Akinetic mutism.
2. Problems with bimanual coordination.
3. Aspontaneity.
4. Apraxia of walking.
5. Urinary incontinence.
6. Perseverations.
7. Cognitive impairment.
8. Memory impairment.
9. Emotional disturbances.

Epileptic phenomena characteristic of the frontal localization of the epileptic focus

Frontal lobe irritation syndromes depend on its location. For example, stimulation of Brodmann field 8 causes deviation of the eyes and head to the side.

Epileptic discharges in the prefrontal cortex tend to rapidly generalize into a grand mal seizure. If the epileptic discharge extends to field 8, then before secondary generalization a versive component of the seizure can be observed.

Many patients with complex partial seizures are of frontal rather than temporal origin. The latter are usually shorter (often 3-4 seconds) and more frequent (up to 40 per day); there is partial preservation of consciousness; patients recover from the seizure without a state of confusion; characteristic automatisms are typical: rubbing hands and hitting, snapping fingers, shuffling movements of legs or kicking them; head nodding; shrugs; sexual automatisms (manipulation of the genitals, thrusts of the pelvic area, etc.); vocalization. Vocal phenomena include swearing, screaming, laughing, and simpler unarticulated sounds. Breathing may be irregular or unusually deep. With seizures originating from the medial prefrontal region, there is a tendency to mildly develop status epilepticus.

Unusual ictal manifestations can cause erroneous overdiagnosis of pseudo-seizures (so-called epileptic “pseudo-pseudo-seizures”, “fireworks” seizures, etc.). Since most of these seizures originate from the medial (supplementary area) or orbital cortex, conventional scalp EEG often does not detect any epileptic activity. Frontal seizures occur more easily during sleep than other types of epileptic seizures.

The following specific epileptic phenomena of frontal origin have been described:

Primary motor area.

1. Focal clonic jerks (shudders), more often seen in the opposite arm than in the face or leg.
2. Stopping speech or simple vocalization (with or without salivation).
3. Jackson Motor March.
4. Somatosensory symptoms.
5. Secondary generalization (transition to a generalized tonic-clonic seizure).

Premotor area.

1. Simple tonic movements of the axial and adjacent muscles with versive movements of the head and eyes to one side
2. Secondary generalization is typical.

Accessory motor area.

1. Tonic lifting of the contralateral arm and shoulder with flexion at the elbow joint.
2. Turn your head and eyes towards the raised hand.
3. Stopping speech or simple vocalization.
4. Stopping ongoing motor activity.

Cingulate gyrus.

1. Affective disorders.
2. Automatisms or sexual behavior.
3. Autonomic disorders.
4. Urinary incontinence.

Fronto-orbital region.

1. Automatisms.
2. Olfactory hallucinations or illusions.
3. Autonomic disorders.
4. Secondary generalization.

Prefrontal region.

1. Complex partial seizures: Frequent, brief seizures with vocalizations, bimanual activity, sexual automatisms, and minimal postictal confusion.
2. Frequent secondary generalization.
3. Forced thinking.
4. Adverse movements of the head and eyes or contraversive movements of the body.
5. Axial clonic jerks and patient falls.
6. Vegetative signs.

Damage to the corpus callosum (callosal syndromes)

Damage to the corpus callosum leads to disruption of the processes of interaction between the hemispheres, disintegration (disconnection) of their joint activity. Diseases such as trauma, cerebral infarction or tumor (less commonly, multiple sclerosis, leukodystrophies, radiation damage, ventricular shunting, agynesia of the corpus callosum) that affect the corpus callosum usually involve the interhemispheric connections of the middle parts of the frontal lobes, parietal or occipital lobes. Disruption of interhemispheric connections in itself has almost no effect on everyday activities, but is detected when performing some tests. This reveals the inability of one hand to imitate the positions of the other (contralateral) due to the fact that kinesthetic information is not transferred from one hemisphere to the other. For the same reason, patients are unable to name an object that they feel with their left hand (tactile anomia); they have agraphia in the left hand; they cannot copy movements with their right hand that are made with their left (constructive apraxia in the right hand). Sometimes an “intermanual conflict” (“alien hand” syndrome) develops when uncontrolled movements in the left hand are initiated by voluntary movements of the right hand; the phenomenon of “double hemianopsia” and other disorders have also been described.

Perhaps of greatest clinical significance is the “alien hand” phenomenon, which can result from combined callosal and medial frontal damage. Less commonly, this syndrome occurs with parietal injuries (usually in the picture of paroxysmal manifestations of an epileptic attack). This syndrome is characterized by a feeling of alienation or even hostility in one hand, involuntary motor activity in it, which is unlike any other known form of movement disorder. The affected hand seems to “live its own independent life”; involuntary motor activity is observed in it, similar to voluntary purposeful movements (palpating, grasping and even auto-aggressive actions), which constantly stresses these patients. A typical situation is also when, during involuntary movements, the healthy hand “holds” the sick one. The hand is sometimes personified with a hostile, uncontrollable alien “evil and disobedient” force.

“Alien hand” syndrome has been described in vascular infarctions, corticobasal degeneration, Creutzfeldt-Jakob disease, and some atrophic processes (Alzheimer’s disease).

A rare syndrome of damage to the central part of the anterior parts of the corpus callosum is Marchiafava-Benyami syndrome, which is related to alcoholic lesions of the nervous system. Patients suffering from severe alcoholism have a history of periodic alcohol withdrawal syndrome with tremors, epileptic seizures and delirium tremens. Some of them develop severe dementia. Characterized by dysarthria, pyramidal and extrapyramidal symptoms, apraxia, and aphasia. In the last stage, patients are in a deep coma. The diagnosis is made during life very rarely.

ON THE. Ermolenko 1, A.Yu. Ermakov 2, I.A. Buchneva 3

1 -Voronezh State Medical Academy named after. N.N. Burdenko;
2 -Moscow Research Institute of Pediatrics and Pediatric Surgery of Rosmedtekhnologii;
3 - Voronezh Regional Children's Clinical Hospital No. 1

The discovery of a new category of epilepsies arising from local cortical dysfunction, with regional epileptiform discharges on the EEG and a benign prognosis for seizure resolution, is considered the most interesting contribution to epileptology in the last 50 years (Fejerman N. et al., 2007). The electroencephalographic correlate of these states are age-dependent patterns, morphologically representing a three-phase electric dipole with a period of an acute wave of more than 70 ms, followed by a slow wave and constant activation during sleep (Panayiotopoulos C.P., 2005). EEG patterns, known in the literature as “rolandic spikes” (Lundberg S. et al., 2003) or “benign focal epileptiform discharges of childhood” (Panayiotopoulos C.P., 2005), tend to be grouped in series, and in some cases occupy a significant part EEG recordings, recording almost continuously. Despite the use of the word “benign” in the name of the single complex, continued activity of DERD patterns can be the cause of mental, communicative, cognitive, behavioral and social disorders in children. Long-term persistent focal or diffuse epileptiform activity in the form of DERD patterns with a high index of representation on the EEG during sleep causes a functional rupture of neuronal connections, has an adverse effect on brain development during the critical period of synaptogenesis and causes neuropsychological disorders, even in the absence of epileptic seizures (Zenkov L R., 2007; Aarts J., 1984; Gobbi G., 2002). Therefore, these conditions are diagnosed late and have a poor prognosis.

Purpose The present study was to determine the clinical and neurophysiological features of epilepsy in children associated with continued epileptiform activity during sleep, and approaches to the rational treatment of these conditions.

Patients and methods

A preliminary screening examination was carried out on 1,862 children aged 2 to 18 years who were admitted to the specialized psychoneurological department of the State Healthcare Institution “VODKB No. 1” for epileptic seizures and diseases of the nervous system not accompanied by epileptic seizures in the period from 2004 to 2007.

The patients were examined using a clinical method, including examination of the neurological status, neuropsychological testing using A.R. methods. Luria, Toulouse-Pieron and Wechsler tests, as well as video-EEG monitoring (for the purpose of long-term continuous recording of EEG and patient behavior). Video-EEG monitoring was carried out using a computer complex of an electroencephalograph-analyzer “Encephalan 9”, Medicom MTD, Taganrog using 19 channels according to the international “10–20” system and an additional polygraphic ECG channel. The duration of continuous recording varied from 4 to 8 hours. When recording epileptiform activity during sleep, the spike-wave saturation index (SWI/SWI) was calculated (Patry G. et al., 1971; Tassinari C.A. et al., 1982). Neuroradiological examination was carried out on a Siemens magnetic resonance imaging scanner (with a magnetic field voltage of 1.5 Tesla).

results

During the examination, DERD were detected in the background EEG recording and during sleep in 229 (12.3%) patients, including 190 (22.6%) patients with a verified diagnosis of epilepsy (n=840) and 39 (3, 8%) patients with neurological pathology (n=1022), not accompanied by epileptic seizures (Table 1).

Table 1. Frequency of occurrence of EEG changes with the DERD pattern in patients with various nosological forms

In children with cerebral palsy (CP), epilepsy and brain malformations, EEG patterns of DERD were recorded in 10.3%, 22.6% and 52% of cases, respectively, which was 2–10 times higher than the general population values ​​(Panayiotopoulos C.P., 2005 ; Covanis A., 2009).

In patients with cerebral palsy, the hemiparetic form was found in 46% of cases, which significantly exceeds the general population frequency of occurrence of this form of cerebral palsy - up to 13% in the population of patients with cerebral palsy (Ermolenko N.A., 2006).

In 122 patients (53%) there was a combination of epileptic seizures and/or cognitive disorders with prolonged (diffuse or regional) epileptiform activity in the form of SERD patterns during slow-wave sleep (SWS), occupying from 30% to 100% of the recording epoch.

Based on neuroradiological examination data, all children with PEMS (n=122) were divided into 2 groups: the first group (group I; n=62) consisted of patients who had no structural changes in the brain and focal neurological symptoms - idiopathic option (ratio of girls and boys - 1.1:1); the second group (group II; n=60) included patients with focal structural changes in the brain and/or focal neurological symptoms - symptomatic variant (ratio of girls to boys - 1:1.2).

In group II patients, various brain malformations were verified in 22% of cases; in 19% of patients arachnoid cysts were found in the area of ​​the lateral fissures, which are difficult to differentiate from polymicrogyria according to MRI (Alikhanov A.A., 2000), in 53.7% of cases atrophic changes were detected due to strokes, periventricular leukomalacia, and intrauterine infections; in 5.6% of patients, changes on MRI were not verified, but a pronounced neurological deficit was detected in combination with impaired development of cognitive functions. The ILAE Commission on Classification and Terminology (2001) recommends that these cases be treated as probably symptomatic (Engel J., 2001). No predominant localization of focal changes in brain regions was identified, but significantly more often (p<0,05) они обнаруживались в левой гемисфере по сравнению с правой (в 35,3% (n=18) и в 25,5% (n=13) случаев соответственно).

Based on the history, clinical course and results of video-EEG monitoring in patients with continued epileptiform activity during sleep (n=122), the following nosological forms were verified: benign focal epilepsy of childhood with central temporal spikes (18.9% ( n=23) cases); benign occipital epilepsy of childhood with early onset (4.8% (n=6) patients); symptomatic focal epilepsy (14.6% (n=18) of patients); epilepsy with electrical status epilepticus of slow-wave sleep (42.2% (n=52) of patients), including idiopathic (35% (n=18) and symptomatic (65% (n=34)) variants; epileptiform cognitive disintegration (17.1% (n=21) patients); Landau-Kleffner syndrome (1.6% (n=2) patients).

Normal background bioelectrical activity was significantly more often observed in patients of group I compared with group II (47% (n=29) and 20% (n=12), p<0,05 соответственно). В бодрствовании у пациентов в двух группах достоверно чаще регистрировалась региональная продолженная эпилептиформная активность с индексом от 15 до 85% (46% случаев) по сравнению с диффузной (24% больных), мультифокальной (20% пациентов) и унилатеральной (10% детей) активностью. У всех обследованных пациентов отмечалось усиление эпилептиформной активности во сне с появлением диффузной продолженной активности в 40% случаев и достоверным увеличением индекса эпилептиформной активности более 85% - у 41% пациентов, индексом 30–80% - у 59% больных.

Fronto-central-temporal regional accentuation of PEMS (77% (n=43) of patients) was recorded significantly more often (p<0,05), чем теменно-затылочная и затылочная (14% (n=8) пациентов), лобная (9% (n=5 случаев) и центрально-височная (5% (n=3) детей). В 5% (n=6) случаев было зарегистрировано перемещение (шифт) эпилептиформной активности из одной гемисферы в другую при последующих записях ЭЭГ, без достоверной разницы между группами I и II. Смещение региона в пределах одной гемисферы отмечалось в 6% (n=7) случаев. У 11,6% пациентов зарегистрировано несовпадение региональной продолженной эпилептиформной активности на ЭЭГ со стороной локализации очаговых структурных изменений в головном мозге, выявленных при нейровизуализации.

Cognitive disorders of varying severity were verified in 89% of patients included in the study. 11% of children had autistic behavior disorder without a significant difference between groups I and II (13% and 8%, respectively). Patients in group II were significantly more likely, compared to patients in group I, to have more severe cognitive disorders with total impairment of the development of all higher mental functions (60% and 24%, respectively, p<0,05), а также «преморбидная» задержка их формирования с раннего возраста (у 50%), с резким нарастанием когнитивного дефицита после появления эпилептических приступов и/или продолженной эпилептиформной активности на ЭЭГ.

Epileptic seizures were absent throughout the entire observation period in 24.6% (n=30) of patients. Patients in group I showed a predominance of focal motor seizures (100% compared to 61%, p<0,05), связанных со сном (78% против 41%, p<0,05). Однако гемиклонические (22% в сравнении с 11%, p<0,05) и вторично-генерализованные судорожные приступы (30% в сравнении с 9%, p<0,05) чаще отмечались у пациентов группы II (рис. 1) и достоверно чаще возникали в бодрствовании, по сравнению с больными группы I (35% в сравнении с 17%, p<0,05). Ингибиторные моторные эпизоды отмечались у 23% (n=21) детей, достоверно чаще у больных в группе I, чем в группе II (76% (n=16) и 24% (n=5) соответственно, p

It has been established that the long-term prognosis of the disease (3 years after the start of treatment) is determined by the following factors: duration of epileptiform activity, age of onset of the disease, severity of cognitive impairment before treatment and the effectiveness of antiepileptic therapy during the first year. The onset of the disease before 3 years of age, continued epileptiform activity during sleep, persisting for more than 1 year, premorbid delay in the formation of cognitive functions, as well as the absence of clinical-electroencephalographic remission during the first year of therapy significantly worsen the prognosis of the disease. For such parameters as the frequency and nature of epileptic seizures, the nature and persistence of inhibitory symptoms, the index of epileptiform activity on the EEG during sleep and changes on MRI, no statistically significant relationship with the long-term prognosis of the disease was found.

Analysis of the effectiveness of antiepileptic therapy in the first year of treatment revealed a higher effectiveness of duotherapy compared to monotherapy, due to a significantly higher rate of achieving clinical electroencephalographic remission (23% compared to 12%, p<0,05). Наиболее эффективными оказались комбинации вальпроатов с этосуксимидом и леветирацетамом, при этом, клинико-электроэнцефалографическая ремиссия была достигнута в 30–75% случаев (табл. 2). В лечении эпилепсии с эпилептическим электрическим статусом медленного сна был наиболее эффективен леветирацетам: на фоне приема леветирацетама в монотерапии у всех детей (n=3) зарегистрирована клинико-электроэнцефалографическая ремиссия. Однако сопоставление данных по эффективности для сравнения с вальпроатами не представляется возможным из-за малого числа наблюдений.

In patients receiving carbamazepine (n=25) in initial monotherapy (n=16) and duotherapy (n=9), deterioration of the condition was noted in the form of aggravation and atypical evolution, followed by the formation of resistance to AEDs in 64% (n=16) cases.

Complete pharmaco-induced regression of continued epileptiform activity of DERD patterns was observed in 29% (n=35) of cases, 2 times more often in patients of group I - 37% (n=23) compared to patients of group II - 20% (n=12). The average age of disappearance of continued epileptiform activity of DERD patterns during treatment was 8.4–1.2 years, with no significant difference between groups I and II (8.3–1.6 and 8.7–1.7 years, respectively).

Table 2. Duotherapy in patients (n=52) with continued epileptiform activity in the form of EEG patterns on EEG during sleep

Discussion

The electroencephalographic pattern of DERD, discovered for the first time in patients with rolandic epilepsy (Loiseau P. et al., 1961, 1967), was also identified in patients with various neurological pathologies; including in patients with symptomatic focal epilepsy, in whom structural changes in the brain in 41% of cases were localized in epileptogenic zones, and, thus, could be an independent source of epileptogenesis with the DERD pattern. The risk of epilepsy associated with PEMS in children with cerebral palsy, especially with hemiparetic forms, and brain malformations exceeds general population values ​​by 2–10 times. At the same time, in patients with a structural defect of the brain, “double pathology” cannot be excluded (Mukhin K.Yu., 2005), which is based on the universal mechanism of focal cortical dysfunction (Doose H. et al., 1989). A more benign course of idiopathic forms of epilepsy with the DERD pattern has been proven compared to symptomatic ones.

A five-year follow-up of patients with continued epileptiform activity of DERD patterns with an index of at least 30% during sleep showed evolution into epileptic encephalopathy in 66% of cases: in 49% of cases - into epilepsy with electrical status epilepticus in sleep and in 17% - into cognitive epileptiform disintegration. Thus, a spike-wave index of more than 30% on the sleep EEG in children, even without clinical manifestations of epileptic seizures, serves as an indication for the prescription of antiepileptic drugs.

It has been proven that initial therapy and the timing of its administration are crucial for the long-term prognosis regarding the preservation or restoration of cognitive functions in children and adolescents. The most effective are combinations of valproate with ethosuximide or levetiracetam in duotherapy.

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The article presents a group of patients with focal epilepsy associated with DEPD in children with perinatal organic brain damage, which, according to its clinical, electro-neuroimaging characteristics, occupies a special “intermediate” position between idiopathic and symptomatic epilepsy. We observed 35 patients aged from 2 to 20 years. Based on the results obtained, diagnostic criteria for the syndrome are proposed. The disease is characterized by: a predominance of male patients; onset of epileptic seizures before the age of 11 years with a maximum in the first 6 years (82.9%) with two peaks: in the first 2 years of life and at the age of 4 to 6 years; often debuts with infantile spasms; predominance of focal hemiclonic seizures, focal occipital seizures and SHSP. A combination of focal and pseudogeneralized seizures is possible (epileptic spasms, negative myoclonus, atypical absence seizures). Characterized by a relatively low frequency of focal and secondary generalized attacks confined to sleep (occurring upon awakening and falling asleep). Neurological deficits are present in most patients, including motor and cognitive impairment; Cerebral palsy is common. It is typical to detect a DEPD pattern on the EEG. In all cases, signs of perinatal brain damage, predominantly of hypoxic-ischemic origin, are stated. Remission of attacks is achieved in all cases; later epileptiform activity on the EEG is blocked. Neurological (motor and cognitive) impairments generally remain unchanged.

According to modern concepts, focal epileptic seizures arise as a result of local discharges in neuronal networks limited to one hemisphere, with greater or lesser spread (Engel J. Jr., 2001, 2006). Focal (localization-related) epilepsies are traditionally divided into symptomatic, cryptogenic (synonym - probably symptomatic) and idiopathic forms. By symptomatic we mean forms of epilepsy with a known etiological factor and verified structural changes in the brain that are the cause of epilepsy. As the name implies, symptomatic epilepsy is a manifestation of another disease of the nervous system: tumors, brain dysgenesis, metabolic encephalopathy, a consequence of hypoxic-ischemic, hemorrhagic brain damage, etc. These forms of epilepsy are characterized by neurological disorders, decreased intelligence, and resistance to antiepileptic therapy (AED). Probably symptomatic (synonym cryptogenic, from Greek criptos - hidden) forms of epilepsy are syndromes with an unspecified, unclear etiology. It is understood that cryptogenic forms are symptomatic, however, at the present stage, when using neuroimaging methods, it is not possible to identify structural disorders in the brain [ 26]. In idiopathic focal forms, there are no diseases that can cause epilepsy. Idiopathic epilepsies are based on a hereditary predisposition to disorders of brain maturation or genetically determined membrane and channelopathies. In idiopathic focal forms of epilepsy (IFE), neurological deficits and intellectual impairment are not detected in patients, and neuroimaging shows no signs of structural brain damage. Perhaps the most important feature of IFE- absolutely favorable prognosis of the disease with spontaneous cessation of attacks when patients reach puberty. Idiopathic focal epilepsies are classified as “benign epilepsies.” Many authors do not accept the term “benign” to characterize a disease such as epilepsy. However, it is generally accepted that benign epilepsy includes forms that satisfy two main criteria: mandatory relief of seizures (medical or spontaneous) and the absence of intellectual and mnestic disorders in patients, even with a long course of the disease.

For idiopathic focal forms of epilepsy, a characteristic feature is the appearance on the EEG of “ benign epileptiform patterns of childhood» - DEPD, specific graph elements consisting of a five-point electric dipole.

The characteristic features of DEPD on EEG are (Mukhin K.Yu., 2007):

• The presence of a five-point electric dipole consisting of an acute and a slow wave.
• The maximum “positivity” of the dipole is in the frontal leads, and the maximum “negativity” is in the central temporal leads, which is most typical for Rolandic epilepsy.
• The morphology of the complexes resembles QRS waves on an ECG.
• Regional, multiregional, lateralized or diffuse nature of activity.
• Instability of epileptiform activity with possible movement (shift) during subsequent EEG recordings.
• Activation during I - II stages of slow-wave sleep.
• Lack of clear correlation with the presence of epilepsy and the clinical picture of epilepsy.

DEPDs are easily recognizable on EEG due to their unique morphological characteristic: a high-amplitude five-point electric dipole. At the same time, we emphasize the importance of the morphological characteristics of this EEG pattern, and not the localization. Previously, we presented the classification of “DEPD-associated conditions”. It has been shown that DEPD are nonspecific epileptiform disorders that occur in childhood, which can be observed in epilepsy, diseases not associated with epilepsy, and in neurologically healthy children.

In recent years, in clinical practice, we have observed a special group of pediatric patients with focal epilepsy, which, according to its clinical and electroneuroimaging characteristics, occupies a special “intermediate” position between idiopathic and symptomatic. We are talking about focal epilepsy associated with DEPD in children with perinatal organic brain damage. This group of patients has clearly defined clinical, electroencephalographic and neuroimaging criteria, response to AED therapy and prognosis.

The purpose of this study: to study the clinical, electroencephalographic, neuroimaging characteristics, features of the course and prognosis of focal epilepsy associated with DEPD in children with perinatal brain damage; establishing diagnostic criteria for the disease and determining optimal methods of therapeutic correction.

PATIENTS AND METHODS

We observed 35 patients, of which 23 were male and 12 were female. The age of the patients at the time of publication ranged from 2 to 20 years (mean, 10.7 years). The vast majority of patients ( 94.3% of cases ) was a child's age: from 2 to 18 years. The observation period ranged from 1 year to 8 months. up to 14 years 3 months (on average, 7 years 1 month).

Criteria for inclusion in the group:

— presence of focal epilepsy in patients;

— anamnestic, clinical and neuroimaging signs of brain damage of perinatal origin;

— registration of regional/multiregional epileptiform activity, morphologically corresponding to “benign epileptiform patterns of childhood” on the EEG.

Criteria for exclusion from the group:

— progression of neurological symptoms;

— verified hereditary diseases;

— structural disorders in neuroimaging acquired in the postnatal period (consequences of traumatic brain injuries, neuroinfections, etc.).

All patients were examined clinically by a neurologist, neuropsychologist; A routine EEG study was carried out, as well as continued video-EEG monitoring with the inclusion of sleep (electroencephalograph-analyzer device EEGA-21/26 “ENCEPHALAN-131-03”, modification 11, Medicom MTD; video-EEG monitoring “Neuroscope 6.1.508”, Biola). All patients underwent an MRI examination (magnetic resonance system Sigma Infinity GE with a magnetic field voltage of 1.5 Tesla). To monitor antiepileptic therapy over time, the content of AEDs in the blood was studied using gas-liquid chromatography; General and biochemical blood tests were performed (Invitro laboratory).

RESULTS

Among the patients we examined there was a significant predominance in the group of male patients (65.7% of cases); the male to female ratio was 1.92:1.

Onset of seizures . The onset of seizures in our group was observed over a wide age range. The earliest occurrence of seizures was observed in the patient on the 3rd day of life, the latest age of onset of epilepsy - 11 years. After 11 years, the attacks did not debut.

Most often, epileptic seizures occurred in patients in the first year of life - in 28.6% of cases. At older ages, the onset of epileptic seizures was noted: at the 2nd and 4th years of life - 11.4% of cases, at the 1st and 5th years - 8.6% of cases, at the ages of 6, 7. At 8 and 9 years old, respectively, the probability of seizures was 5.7%. The onset of attacks was observed least often at the ages of 3, 10 and 11 years - 2.9% each, respectively (1 patient each) (Fig. 1).

Analyzing the age intervals of onset in our group of patients, we can note a significant predominance of the frequency of attacks during the first 6 years of life - 82.9% of cases with two peaks. Most often, attacks began during the first two years of life. In this interval, debut was noted in 37.1% of cases. The second peak is observed in the range from 4 to 6 years - in 20%.

As patients grow older, there is a gradual decrease in the likelihood of a first attack from 48.6% in the first 3 years of life to 11.4% in the age range from 9 to 11 years.

Seizures at the onset of epilepsy . At the onset of epilepsy in our group of patients, focal seizures predominated - 71.4%. Focal motor seizures were noted in 51.4% of cases, secondary generalized convulsive seizures - 14.3%. Other types of focal seizures were observed much less frequently: focal hypomotor in 1 case and negative myoclonus - also in 1 case.

Epileptic spasms at the onset of epilepsy were observed in 17.1% of patients; Serial tonic asymmetrical seizures predominated, often in combination with short focal versive seizures. In 1 case, myoclonic spasms were detected. In all cases, the onset of epileptic spasms was observed in children in the first year of life.

In 14.3% of cases, epilepsy debuted with the appearance of febrile seizures: in 3 cases - typical, and in 2 - atypical. Generalized convulsive seizures were observed in only 8.6% of patients at the onset of the disease; myoclonic - in 1 case.

Epileptic seizures in the advanced stage of the disease. Analyzing the occurrence of epileptic seizures in our group, we can note a significant predominance of focal and secondary generalized seizures in the clinical picture. Among the focal seizures, the most frequently recorded focal clonic seizures, characteristic in kinematics for Rolandic epilepsy: hemifacial, faciobrachial, hemiclonic - 34.3% of cases. In 28.6% of cases, focal seizures were identified, which, based on clinical features and electroencephalographic characteristics, can be classified as focal occipital. In this group, attacks of simple visual hallucinations predominated, with vegetative phenomena (headache, nausea, vomiting), versive and paroxysms of limpness, often followed by a transition to a secondary generalized convulsive attack. Focal versive tonic seizures were observed in 11.4% of patients. Secondary generalized seizures occurred in 40% of cases, including focal onset in most cases. Pseudogeneralized seizures were observed in 31.4% of patients, of which epileptic spasms were more common - 20.0%; in isolated cases, atypical absences and atonic seizures occurred. Focal automotor seizures were detected only in 2 cases.

In 45.7% of cases, only one type of seizure was detected in patients, and also in 45.7% - a combination of two types. In patients who experienced type 1 seizures throughout the entire period of the disease, focal motor seizures predominated (in 17.1% of cases), secondary generalized seizures (14.3% of cases) and focal paroxysms emanating from the motor cortex (8.6% of cases). %). In the group of patients with two types of seizures, attention was drawn to the frequent association of focal motor (25.7% of cases), secondary generalized (20% of patients) and focal seizures emanating from the occipital regions (17.1% of patients) with other types of seizures . A combination of 3 and 4 types of attacks was observed in isolated cases (in 1 and 2 cases, respectively). The most common combination of focal motor seizures and epileptic spasms was detected - in 11.4% of cases, focal motor and secondary generalized seizures - 8.6%, secondary generalized and focal, emanating from the occipital cortex - in 8.6%.

Based on the frequency of occurrence, we divided epileptic seizures into single ones (1 -3 for the entire period of the disease), rare (1-3 times a year), frequent (several attacks per week) and daily. In 57.6% of cases, attacks were rare (27.3%) or single (30.3%). Attacks occurring several times a month were observed in 15.2% of patients. Daily seizures were detected in 27.3% of patients and were represented mainly by pseudogeneralized paroxysms: epileptic spasms, atypical absence seizures, negative myoclonus.

The duration of epileptic seizures varied among patients. In 56.6% of cases, the attacks ended spontaneously within 1 -3 minutes, while short attacks (up to 1 minute) were observed in 33.3% of cases (mostly pseudogeneralized). The high percentage of prolonged attacks is noteworthy. So attacks lasting 5-9 minutes, noted in 13.3% of patients. In 36.7% of cases, the duration of the attacks exceeded 10 minutes, and in some patients the paroxysms were of the nature of status epilepticus.

The study showed a high chronological dependence of epileptic seizures on the sleep rhythm —wakefulness,” which was observed in 88.6% of patients in our group. Most often, attacks were observed during the period of awakening or falling asleep - in 42.9%. Seizures occurred during sleep in 25.7% of cases; in wakefulness and sleep - 17.1%. In only 11.4% of patients, epileptic seizures did not have a clear connection with sleep.

Neurological status. In 100% of cases, focal neurological symptoms were detected. Pyramidal disorders were observed in 82.9% of cases, of which 40% of patients had paresis or paralysis. Among other neurological symptoms, ataxia was the most common - in 20% of cases, muscular dystonia - 11.4%, tremors in the limbs - 8.6%. A decrease in intelligence of varying degrees of severity was detected in 57.1% of cases. Cerebral palsy syndrome was found in 40% of patients. Of these: the hemiparetic form was observed in 57.2% of cases of all forms of cerebral palsy, spastic diplegia - in 21.4% of cases, double hemiplegia - in 21.4% of cases.

EEG study results. The main activity was close to or corresponded to the age norm in 57.2% of cases. However, in most cases, even against the background of a preserved alpha rhythm, a diffuse or biocipital theta slowdown of the background rhythm was determined. Delta deceleration with an emphasis in the posterior regions was detected in 14.3% of cases, mainly in children with epileptic spasms and the onset of seizures in the first year of life. In this case, delta waves were combined with multiregional epileptiform activity in the occipital regions. In more than 50% of cases, the EEG during wakefulness and sleep showed an increased index of exalted beta activity (excessive fast). In general, for patients in our group, the characteristic EEG pattern in the waking state was theta slowdown of the main activity in combination with an acceleration of cortical rhythms.

A mandatory criterion for inclusion in the group was the identification of benign epileptiform patterns of childhood (BECP) on EEG. DEPD were presented in the form of regional/multiregional epileptiform activity in 100% of cases, as well as in the form of lateralized, and much less often, bilateral and diffuse discharges.

In 75% of cases, regional epileptiform activity was noted in the central-temporo-frontal regions (p is. 2), in 30% of cases, DEPD were recorded in the occipital leads (Fig. 3). It should be noted that in our group a focus was often detected in the vertex areas. In 57.1% of cases, regional/multiregional epileptiform activity was limited to one hemisphere; in 42.9%, independent foci of epileptiform activity were noted in two hemispheres (Fig. 4). In 57.1% of patients, a bilateral distribution of epileptiform activity was noted, which included: cases of continued discharges in symmetrical areas in the two hemispheres with the formation of a picture of bilateral asynchronous complexes ( rice. 3), bilateral spread of discharges from one focus to homologous parts of the contralateral hemisphere, bilateral acute-slow wave complexes, diffuse discharges of acute-slow wave complexes.

The study showed a high chronological association of DEPD with sleep. In 100% of cases, DEPD was recorded during sleep, in 77.1%, epileptiform activity was detected both during sleep and wakefulness. It is important to note that in no case was the appearance of epileptiform activity of the DEPD isolated in a state of wakefulness noted.

Analysis of the results of video-EEG monitoring made it possible to identify the characteristic features of epileptiform activity in the examined group. Benign epileptiform patterns of childhood were characterized by a tendency to form groups in the form of doublets, triplets and longer groups (pseudo-rhythmic discharges). The DEPD index increased in the state of passive wakefulness and was maximum during the transition to the state of drowsiness and in sleep. In a state of active wakefulness, the DEPD index was significantly blocked. In sleep, the representation of DEPD is maximum in the stages of slow-wave sleep, during In REM sleep, a significant reduction in this EEG pattern was observed. It was in our patients’ sleep that we recorded continuous peak-wave epileptiform activity in slow-wave sleep (PEMS) and electrical status epilepticus in slow-wave sleep - PEMS with an index of more than 85% of the sleep recording.

The study showed that there was no significant relationship between the DEPD index and the frequency of focal motor seizures. DEPD were not an EEG pattern of focal seizures. However, in the case of lateralized or diffuse discharges, the likelihood of epileptic negative myoclonus or atypical absence seizures was high.

The dynamics of epileptiform activity in patients during treatment is of interest. Having appeared on the sleep EEG once, DEPD continued to be recorded continuously in all subsequent EEG recordings for many months or years. In all cases, relief of epileptic seizures was first noted, and only then — disappearance of DEPD. During AED therapy, a decrease in the index and amplitude of epileptiform complexes was gradually observed over time. In cases of PEMS, epileptiform activity and especially the electrical status gradually “faded” and “released” more and more epochs of EEG recording for a normal rhythm. PEMS became less regular and rhythmic, and increasingly large gaps appeared, free from epileptiform activity. At the same time, regional patterns somewhat intensified, both in sleep and wakefulness, replacing diffuse activity. At first, epileptiform activity completely disappeared when recording while awake, and then during sleep. By the onset of puberty, epileptiform activity was not recorded in any of the cases.

Neuroimaging data When conducting neuroimaging, various structural disorders in the brain were identified in 100% of cases. The most frequently detected signs of hypoxic-ischemic perinatal encephalopathy (62.8% of cases): diffuse atrophic/subatrophic changes of varying severity - 31.4%, periventricular leukomalacia - 31.4% (Fig. 5). Arachnoid cysts (Fig. 6) were detected in 13 (37.1%) patients, of which in 7 cases temporal lobe cysts were found (53.9% among patients with cysts), in 4 patients - parietal lobe cysts (30.8%) , in 2 patients - the frontal region (15.4%), in 2 - the occipital region (15.4%). Changes in the cerebellum (hypoplasia of the cerebellar vermis, cerebellar atrophy) were detected in 11.4% of cases. Cortical tubers were observed in 1 patient; in 2 cases signs of polymicrogyria were detected.

Clinical-electro-neuroimaging correlations. Separately, we analyzed the correlations of clinical, electroencephalographic and neuroimaging data in the examined patients. The degree of correlation was based on a comparison of survey data indicating a common focus. The relationship between 4 main parameters was assessed: neurological status (side of the lesion), seizure semiology (localization of the lesion), EEG data and neuroimaging results:

• 1st degree of correlation: coincidence of all clinical, electroencephalographic and neuroimaging parameters (4 parameters indicated above).
• 2nd degree of correlation: coincidence of three out of four parameters.
• 3rd degree of correlation: coincidence of 2 out of 4 parameters.
• Lack of clear correlation.

The frequency of occurrence of diffuse symptoms in the structure of the above parameters was separately assessed. We included the following: bilateral neurological symptoms, pseudogeneralized seizures, diffuse discharges on the EEG and diffuse changes in the brain during an MRI study.

A clear correlation (coincidence of all 4 parameters) was observed only in 14.3% of patients; 2nd degree of correlation — 25.7% of cases; 3rd degree - 22.9%. A significant lack of correlation was found in 37.1% of patients. Various diffuse symptoms were noted in 94.3% of cases. However, there was not a single patient who experienced exclusively diffuse symptoms.

Therapy and prognosis The study showed a good prognosis for the control of epileptic seizures and high effectiveness of antiepileptic therapy. During treatment, seizure relief was achieved in all but one patient - 97.1%! In 28.6%, complete electro-clinical remission was achieved, which is 32.3% of all patients with clinical remission for more than a year. In 1 case, a patient with hemiclonic and secondary generalized seizures and signs of hypoxic-ischemic perinatal encephalopathy on MRI achieved seizure remission that lasted for 3 years. Further, a recurrence of attacks was noted. Currently, after correction of AEDs, the attacks have been stopped, but at the time of publication, the duration of remission was 1 month. Remission for more than 1 year was observed in 31 patients, which was 88.6% of cases. It should be noted that, despite such a high percentage of remissions, in most cases, at the initial stages of therapy, the disease was resistant to seizures and epileptiform activity on the EEG. Only in 8 cases (22.9%) attacks were stopped with monotherapy. In other cases, remission was achieved with duo- and polytherapy, including the use of corticosteroids. The most effective drugs in the treatment of patients in the examined group were: valproate (Convulex) and topiramate (Topamax), both in monotherapy and in combination. When using carbamazepine in monotherapy, high efficiency was noted in a number of cases, but aggravation phenomena were often observed in the form of an increase in focal seizures and the appearance of pseudogeneralized paroxysms, as well as in the form of an increase in the index of diffuse epileptiform activity on the EEG. When focal attacks were resistant, a good response was obtained when prescribing combinations: Convulex + Topamax, Convulex + Tegretol or Trileptal. Succinimides (suxilep, petnidan, zarantin), which were used only in combination, mainly with valproate, were highly effective. Succinimides were effective against both pseudogeneralized seizures and epileptiform activity on the EEG. Sulthiam (oppolot) has also been used successfully in combination with valproate. In resistant cases, mainly in patients with infantile spasms, as well as in the presence of “electrical status epilepticus of slow-wave sleep” on the EEG, we prescribed corticosteroid hormones (synacthen depot, hydrocortisone, dexamethasone) with the highest effect: stopping attacks, blocking or significantly reducing the index epileptiform activity in all cases. The use of hormones was limited by the high frequency of side effects of therapy.

Analysis of the results showed that at the initial stages of treatment, in most cases it is not possible to block or even reduce the DEPD index on the EEG. Cases of diffuse spread of DEPD with the formation of a picture of continued epileptiform activity during the slow-wave sleep phase were particularly resistant. In these cases, the addition of succinimides or oppolot to the basic AEDs showed the greatest effectiveness. The administration of these drugs significantly blocked regional and diffuse epileptiform activity on the EEG. The use of corticosteroids has also shown high effectiveness against DEPD.

It should be noted the positive effect of AEDs observed in the examined patients in relation to cognitive functions and motor development. This effect, first of all, can be associated with the “freeing” of the brain from seizures and epileptiform activity, as well as with more intensive rehabilitation assistance, which became possible after seizure control was established. However, complete or significant restoration of motor and cognitive functions was not observed in any case, even after complete relief of seizures and blocking of epileptiform activity.

DISCUSSION

The study of the described group of patients was carried out at the Center for Pediatric Neurology and Epilepsy (K.Yu. Mukhin, M.B. Mironov, K.S. Borovikov), together with German colleagues (H. Holthausen et al.) from 2002 to 2009 . Currently, there are more than 130 patients under our supervision who meet the criteria described in the article. In our opinion, this group represents a completely special epileptic syndrome with a favorable course of epilepsy, but with severe neurological disorders. We called it " focal epilepsy of childhood with structural changes in the brain and benign epileptiform patterns on the EEG", abbreviated — FEDSIM-DEPD. A not entirely successful synonym used earlier is “double pathology”; by this term, different authors mean various pathological conditions, in particular, a combination of mesial temporal sclerosis with dysplastic changes in the hippocampus.

I would like to draw your attention to the fact that we did not find such studies in the domestic and foreign literature available to us. Some publications describe only isolated observations of patients with focal motor seizures reminiscent of those in IFE, a favorable prognosis for the course of epilepsy, and the presence of structural changes in the brain. The authors call these cases “idiopathic copies of symptomatic focal epilepsies.” In fact, these isolated cases are identical to the group of patients with FEDSIM-DEPD that we described. However, the fundamental difference is in the name, which radically changes the idea of ​​this syndrome.

FEDSIM-DEPD is not, in the strict sense, symptomatic epilepsy. Firstly, in many cases, the ictogenic zone does not coincide with the localization of structural changes in the brain, not only within the brain lobe, but even within the hemisphere. In 28.6% of the patients we examined, diffuse cortical atrophy was observed, and there were no local structural changes in the brain. Secondly, epileptiform activity in patients of this group is represented mainly by multiregional and diffuse DEPD, and not by clearly regional EEG patterns, as in symptomatic focal epilepsies. Moreover, if the phenomenon of secondary bilateral synchronization occurs, then the zone of discharge generation does not always coincide with the zone of the pathological substrate. Thirdly (this - the main thing!), in the overwhelming majority of cases, epileptic seizures disappear during puberty, despite the persistence of the morphological substrate in the brain.

The lack of a clear correlation of the ictogenic zone and the localization of epileptiform activity with the localization of structural changes in the brain, the eventual disappearance of epileptic seizures in almost all patients, casts doubt on the symptomatic nature of epilepsy, that is, its development directly as a result of exposure to the morphological substrate. On the other hand, there is a high incidence of epilepsy in families of probands; onset of epilepsy exclusively in childhood; attacks identical in nature to IFE with their timing at the time of awakening and falling asleep; presence of DEPD on EEG; relief of seizures in puberty (under the influence of therapy or spontaneously) - clearly indicate the idiopathic nature of epilepsy. However, in idiopathic focal epilepsy there are no structural changes in the brain, no focal neurological symptoms and intellectual deficits, no slowing of the underlying background EEG activity and no continued regional slowing. Also, IFE is not characterized by prolonged attacks, often with a status course and the formation of Todd's palsy. In our opinion, these symptoms are not caused by epilepsy, but are the result of perinatal pathology. Thus, we are talking about a unique syndrome in which epilepsy is essentially idiopathic, and the accompanying symptoms (neurological and intellectual deficits) are caused by structural damage to the brain. It follows from this that FEDSIM-DEPD is not an “idiopathic copy of symptomatic epilepsy,” but, most likely, idiopathic focal epilepsy, developing in patients with morphological changes in the brain of perinatal origin. This form is idiopathic, but by no means benign. The concept of “benign epilepsy” includes not only the possibility of stopping (or self-limiting) seizures, but also the absence of neurological and cognitive impairment in patients, which does not happen with FEDSIM-DEPD, by definition. FEDSIM-DEPD is idiopathic (by the nature of the attacks and the characteristics of the course) epilepsy in children with local or diffuse changes in the brain of perinatal origin. This a group of patients, taking into account clinical, electro-neuroimaging features, in our opinion, is a separate, clearly defined epileptic syndrome in children, which occupies a special intermediate place in a number of focal forms of epilepsy of various etiologies.

The pathogenesis of the development of such a unique epileptic syndrome will likely be the subject of further study. We would like to discuss some possible mechanisms for the occurrence of FEDSIM-DEPD. From our point of view, the development of FEDSIM-DEPD is based on two mechanisms: a congenital disorder of brain maturation and pathology of the perinatal period, mainly hypoxic-ischemic damage to the central nervous system. The term “ hereditary impairment of brain maturation” - a congenital disorder of brain maturation - was first used by the famous German pediatric neurologist and epileptologist Hermann Doose. The Doose hypothesis, which we wholeheartedly support, lies in the existence in a number of patients of a genetically determined disorder of brain maturation in the prenatal period. In our opinion, there are 3 main diagnostic criteria for the condition designated as “congenital disorder of brain maturation.”

1. The presence of “pathology of neuropsychic development” in patients: global impairment of cognitive functions, mental retardation, dysphasia, dyslexia, dyscalculia, attention deficit hyperactivity disorder, autistic-like behavior, etc.

2. The combination of these disorders with interictal epileptiform activity, corresponding in morphology to benign epileptiform patterns of childhood.

3. Improvement in the course of the disease and complete disappearance of epileptiform activity when patients reach puberty.

A variety of endogenous and exogenous factors acting in the prenatal period can cause congenital disorders of brain maturation processes. In this case, it is possible that “genetic predisposition” plays a leading role. H. Doose (1989), H. Doose et al. (2000) showed that benign epileptiform patterns of childhood on the EEG (isolated, in combination with epilepsy or other “developmental pathology”) are genetically determined, inherited in an autosomal dominant manner with low penetrance and variable expressivity. Each gene locus or allelic genes influences the synthesis of a specific polypeptide or enzyme. The developmental pathology is based on a violation of the prenatal differentiation of neurons, the formation of the dendritic tree and the reorganization of synaptic contacts, due to which neurons must be connected into “cellular ensembles” or neuronal networks. Under the influence of various damaging factors, erroneous neuronal connections may occur. - aberrant synaptic reorganization. According to some researchers, impaired plasticity (aberrant sprutting) is most characteristic of childhood and may be one of the causes of epilepsy, as well as the development of cognitive disorders. Impaired neuronal plasticity during brain development leads to the formation of “broken,” “perverted” cellular ensembles of cortical neurons, which is clinically expressed as persistent congenital impairments of cognitive functions. Phylogenetically, the youngest parts of the brain - the frontal lobes - are especially vulnerable to disorders of neuronal organization.

A congenital disorder of brain maturation, manifested by various “developmental pathologies” ( table 1). These pathological conditions arise mainly from birth. However, the appearance of epileptiform activity, and in some cases seizures, occurs, as a rule, during a certain “critical” period of child development - most often between the ages of 3 and 6 years. It is important to note that as the child grows and the brain matures, there is a gradual improvement in mental development, relief of attacks and complete blocking of DEPD with the onset of puberty. Sex hormones play a critical role in brain development. A.S. Petrukhin (2000) believes that disturbances in exposure to hormones in the prenatal period can induce mechanisms leading to perverted differentiation of the brain. On the other hand, the onset of the functioning of sex hormones during puberty leads to a “smoothing out” of the symptoms of cognitive epileptiform disintegration and, in many cases, to complete normalization of the electroencephalogram. We believe that the mechanism of congenital disorders of brain maturation processes is the main one in the development of the symptom complex “idiopathic focal epilepsy”. At the same time, it is more correct to consider benign epileptiform patterns of childhood not as markers of epilepsy, but as a sign of brain immaturity.

The second mechanism for the development of FEDSIM-DEPD is the presence of morphological changes in the brain caused by the pathology of the prenatal period. H. Holthausen (2004, personal communication) proposed the term “ dual pathology" We are talking about patients with two pathological conditions: morphological changes in the brain and the presence of DEPD on the EEG and/or epileptic seizures. Structural changes, according to MRI, are always congenital in nature, caused by pathology of the prenatal period. On the other hand, epileptic seizures in patients with “double pathology” and epileptiform activity of the DEPD type do not have a clear localization relationship with morphological substrates in the brain. Among the patients we examined, grade 1 correlation (coincidence of the localization of the lesion according to the neurological examination, the nature of the attacks, EEG and MRI results) was observed only in 14.3% of cases. And a complete lack of correlation was found in 34.3% of patients, that is, in more than 1/3 of patients!

Epilepsy that occurs in these patients has all the features of idiopathic focal (more often - rolandic, less often - occipital), and DEPD activity is usually observed multiregionally. The most typical occurrence is pharyngo-oral, hemifacial, facio-brachial, versive and secondary generalized seizures. Attacks occur almost exclusively upon awakening and falling asleep, their frequency is low, and they necessarily (!) disappear by puberty - as a result of therapy or spontaneously. During the treatment of our patients, seizure relief was achieved in all, with the exception of one patient - 97.1%!

Thus, despite the presence of morphological changes in the brain, both local and diffuse, the clinical picture (the nature of the attacks, EEG data) and the course of epilepsy are identical to those in idiopathic focal epilepsy. However, the problem is that, despite the absolutely favorable course of epilepsy (meaning obligate relief of seizures), the prognosis for motor and cognitive functions in this category of patients can be very difficult. In this regard, FEDSIM-DEPD cannot in any way be called a “benign” form of epilepsy. While maintaining the first criterion of benign epilepsy (obligate relief of seizures), the second criterion (normal motor and mental development of children) - usually absent. This is the fundamental difference between FEDSIM-DEPD and IFE.

The most common congenital morphological substrates in patients with FEDSIM-DEPD are: arachnoid cysts, periventricular leukomalacia, diffuse cortical atrophy of hypoxic-ischemic origin, polymicrogyria, congenital occlusive shunted hydrocephalus. When visualizing on MRI periventricular leukomalacia (premature children with hypoxic-ischemic perinatal encephalopathy) and shunted occlusive hydrocephalus, the development of cerebral palsy (atonic-astatic form or double diplegia) with epilepsy and/or multiregional DEPD on the EEG is typical. In the presence of polymicrogyria, a clinical picture of a hemiparetic form of cerebral palsy with epilepsy and/or DEPD is formed. In patients with arachnoid and porencephalic cysts, it is possible to detect congenital hemiparesis, speech, behavioral (including autism) and intellectual-mnestic disorders in combination with DEPD on the EEG. Once again, it should be noted that the course of epilepsy in patients in this group is always favorable. At the same time, movement disorders and intellectual-mnestic disorders can be very serious, leading to severe disability.

Some publications indicate the role of early organic damage to the thalamus as a result of hypoxic-ischemic disorders in the perinatal period. Structural abnormalities in the thalamus can lead to hypersynchronization of neurons, their “firing,” helping to maintain “increased convulsive readiness” until the onset of puberty. Guzzetta et al. (2005) presented a description of 32 patients with thalamic lesions in the perinatal period; Moreover, 29 of them showed electro-clinical signs of epilepsy with electrical status epilepticus in the slow-wave sleep phase. It has been suggested that the ventrolateral and reticular nuclei of the thalamus, as well as an imbalance of the GABA-transmitter systems, are responsible for the development of constant ongoing epileptiform activity (by morphology - DEPD) in the slow-wave sleep phase. According to H. Holthausen ( Holthausen, 2004, personal communication), DEPD are an electroencephalographic reflection of perinatal leukopathy. It is damage to the white matter (conducting pathways) of the brain that leads to the development of “idiopathic” focal epilepsy, combined with DEPD. Therefore, FEDSIM-DEPD often occurs in premature infants with cerebral palsy and periventricular leukomalacia on MRI. However, this does not explain the appearance of DEPD in neurologically healthy children and in IFE, in cases where there are no motor disorders, that is, there is no damage to the white matter.

Cognitive impairment in FEDSIM-DEPD is due to three main reasons. Firstly, morphological changes in the brain that occur in the prenatal period. These changes are irreversible, we cannot influence them with medication, however, they do not progress. Secondly, frequent epileptic seizures and, especially, constant continued epileptiform activity can lead to severe disturbances in praxis, gnosis, speech, and behavior. Forming in the developing brain of a child, epileptiform activity leads to constant electrical “bombardment” of the cortical centers of praxis, gnosis, speech and movements; leads to their “overexcitation”, and then functional “blocking” of these centers. A functional rupture of neuronal connections occurs due to long-term epileptiform activity. At the same time, what is important for us is the index of epileptiform activity, its prevalence (the diffuse nature and bifrontal distribution are the most unfavorable), as well as the age at which this activity manifests itself.

There is a third mechanism for the formation of cognitive impairment in patients with FEDSIM-DEPD. From our point of view, an important factor in the development of cognitive deficit in this category of patients is “ congenital disorder of brain maturation processes" The etiology of this process is unknown. Apparently, it is determined by a combination of two reasons: genetic predisposition and the presence of various stress factors affecting the intrauterine development of the child. Specific marker of brain immaturity - appearance on the EEG of “benign epileptiform patterns of childhood” - DEPD. In this regard, the use of steroid hormones that promote “brain maturation”, and not AEDs, has the most effective effect in improving cognitive functions in patients with FEDSIM-DEPD. Doose H., Baier W.K. (1989) suggested that the EEG pattern of DEPD is controlled by an autosomal dominant gene with age-dependent penetrance and variable expressivity. Unfortunately, antiepileptic therapy, while affecting epileptiform activity, does not always have a clear positive effect on reducing neuropsychological disorders. As they grow and mature (primarily - puberty) there is a gradual improvement in cognitive functions, learning abilities and socialization of patients. However, impairment of cognitive functions, of varying severity, can persist throughout life, despite the relief of seizures and blocking of epileptiform activity.

Based on the results obtained and literature data, we developed diagnostic criteria for FEDSIM-DEPD syndrome.

1. Predominance of male patients by gender.

2. Onset of epileptic seizures before the age of 11 years with a maximum in the first 6 years (82.9%) with two peaks: in the first 2 years of life and at the age of 4 to 6 years. Often debuts with infantile spasms.

3. The predominance of focal motor seizures (hemifacial, brachiofacial, hemiclonic), focal seizures originating from the occipital cortex (visual hallucinations, versive seizures, limp seizures) and secondary generalized convulsive seizures.

4. A combination of focal and pseudogeneralized seizures is possible (epileptic spasms, negative myoclonus, atypical absence seizures).

5. Relatively low frequency of focal and secondary generalized attacks.

6. Chronological association of focal attacks with sleep (occurrence upon awakening and falling asleep).

7. Neurological deficits in most patients, including motor and cognitive impairment; often the presence of cerebral palsy.

8. Background EEG activity: characterized by theta slowdown of the main activity against the background of an increased index of diffuse beta activity.

9. The presence on the EEG, mainly in the central temporal and/or occipital leads, of a specific EEG pattern - benign epileptiform patterns of childhood, which more often arise multiregionally and diffusely with an increase in the slow-wave sleep phase.

10. Neuroimaging in all cases reveals signs of perinatal brain damage, predominantly of hypoxic-ischemic origin. These morphological changes can be either local or diffuse, with predominant damage to the white matter (leukopathy).

11. Remission of epileptic seizures is achieved in all cases; later epileptiform activity on the EEG is blocked. Neurological (motor and cognitive) impairments generally remain unchanged.

Thus, 5 main criteria remain in all cases of FEDSIM-DEPD syndrome: onset of epileptic seizures in childhood; the presence of focal seizures (variants of hemiclonic or focal, emanating from the occipital cortex) and/or secondary generalized seizures confined to sleep; the presence of benign epileptiform patterns of childhood (BEPD) on the EEG; the presence of structural changes in the brain of perinatal origin during neuroimaging; complete relief of epileptic seizures before patients reach adulthood.

Rice. 2. Patient Z.R.

Video-EEG monitoring: During sleep, multiregional epileptiform activity is recorded: in the right central-temporal region spreading to the right parietal-occipital region, in the frontal-central-parietal vertex regions, in the left frontal region in the form of single low-amplitude spikes. Epileptiform changes have the morphology of benign epileptiform patterns of childhood (BECP).

Rice. 3. Patient M.A., 8 years. Diagnosis: FEDSIM-DEPD. Delayed psycho-speech development.

Video-EEG monitoring: Epileptiform activity is recorded, presented in the form of bilateral DEPD discharges with an amplitude of up to 200-300 μV of varying degrees of synchronization in the occipito-posterior temporal regions with a pronounced spread to the vertex regions with an alternative onset both in the right posterior regions (more often) and in the left departments

Fig.4. Patient A.N., 10 years. Diagnosis: FEDSIM-DEPD. Right-sided hemiconvulsive seizures.

Video-EEG monitoring : Regional epileptiform activity (READ) is recorded, presented independently in the left temporo-central-frontal region with periodic spread to the left posterior regions and in the right central-frontal region with a tendency to spread to all electrodes of the right hemisphere.

Rice. 5. Patient Z.R., 2 years. Diagnosis: FEDSIM-DEPD. Left-sided hemiclonic seizures with Todd's palsy.

MRI of the brain: Phenomena of residual post-hypoxic leukopathy of the periventricular white matter of both parietal lobes: clearly limited areas of increased T2 signal, hyperintense in FLAIR, localized in the white matter of the fronto-parietal and parieto-occipital lobes. Secondary ventriculomegaly of the lateral ventricles.

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