West syndrome - diagnosis and treatment in South Korea

Pathologies of the brain and central nervous system significantly reduce a person’s quality of life and lead to severe disorders. Among the diseases of this group, disorders of the congenital type or those that develop in the first years of life are especially serious. They are accompanied by developmental delays and affect human health in the future. Such pathological processes include West Syndrome.

Vivantes clinics only diagnose this disease in children.

At the Vivantes clinics, diagnosis and treatment of West Syndrome in Germany are carried out by doctors of the highest category. Our clinic’s specialists perform early diagnosis of the disease using modern expert-class equipment in their practice.

Treatment of the disease is carried out according to innovative methods, it includes mainly conservative therapy, but if there are appropriate indications, surgical interventions of varying degrees of complexity are performed.

What is West Syndrome

In neurology, West syndrome is a pathological process of a non-inflammatory nature that affects the structures of the brain. This disease is characterized as infantile epileptic encephalopathy. Its main distinguishing feature is spastic muscle contractions, similar to epileptic seizures.

develops especially often in children in the first years of life. According to statistics, the manifestation of the disease occurs in the first year of a child’s life in approximately 90% of cases. In the remaining 10% of cases, the first signs of the disease appear before 4 years of age. But at the same time, in the vast majority of children, spastic spasms disappear by the age of 3, after which they transform into various forms of epilepsy.

West syndrome belongs to a group of rare neurological disorders. According to statistics, the disease accounts for about 2% of all cases of epilepsy. However, if we talk about infantile forms of epilepsy and epileptic encephalopathies, among them West syndrome accounts for about a quarter of all cases.

The causes of the development of West Syndrome in children are quite numerous, of which the most common in neurology are considered to be:

• consequences of intrauterine infections;
• fetal hypoxia at various stages of intrauterine development;
• premature birth;
• intracranial injuries received during labor;
• respiratory problems in an infant;
• some forms of abnormalities in the structure of the brain.

It is also important to understand that in approximately 13% of cases the true cause of West Syndrome in infants remains unknown.

West syndrome is one of the most famous forms of epileptic encephalopathies, a catastrophic epilepsy of early childhood. This syndrome is characterized by the following triad of symptoms: a unique type of attack - epileptic spasm; hypsarrhythmia pattern on interictal electroencephalogram (EEG); stop or regression of the child’s psychomotor development [1-3].

The incidence of West syndrome is 2-3.5 cases per 10,000 newborns; it occurs more often in boys than in girls (ratio 60:40) [1].

The syndrome is polyetiological and is observed in more than 200 different neurological diseases. All causes of the development of West syndrome are usually divided into prenatal, perinatal and postnatal. Prenatal include malformations of the brain, monogenic cases of West syndrome, chromosomal abnormalities, neurocutaneous syndromes, intrauterine infections and inborn defects of metabolism; perinatal - hypoxic-ischemic encephalopathy of newborns and hypoglycemia; to postnatal - infections of the central nervous system (CNS), tumors and strokes [2].

The prognosis for the course of the syndrome is determined by its etiology, as well as therapy. Moreover, regardless of the etiology, the treatment tactics (choice of drug, dose) of the syndrome are the same. An exception is tuberous sclerosis, in which vigabatrin remains the first choice drug for the treatment of attacks [1].

In this review article, we do not consider the clinical and laboratory diagnosis of West syndrome and dwell in detail only on its treatment, in particular on the controversial issues of hormonal therapy. It should immediately be noted that much in hormonal therapy for West syndrome has not yet been studied, and in the vast majority of cases, recommendations are empirical in nature. Nevertheless, it is well known that the longer a child has hypsarrhythmia on the EEG, the worse the prognosis for his further development. The goal of treatment for this syndrome is the fastest possible cessation of spasms and normalization of the EEG in cryptogenic cases, or at least the disappearance of hypsarrhythmia in symptomatic cases [3], although it should be recognized that a decrease in the number of epileptic spasms does not affect the mental development of the child [3] .

Drugs of choice in the treatment of West syndrome

Until recently, the first choice drugs were vigabatrin and hormones equally. The 2007 European Consensus on the Treatment of Epilepsy in Children [4] suggested that vigabatrin should be used first for infantile spasms of any etiology, followed by adrenocorticotropic hormone (ACTH). At the moment, preference is given to hormonal therapy as a starting therapy due to its greater effectiveness [5]. R. Riikonen [6] summarized data from 8 studies on the comparative effectiveness of vigabatrin and ACTH. The cessation of spasms during therapy with vigabatrin was 44%, and with ACTH therapy - in 59%; the rate of further relapses was similar, but cognitive function in the group with unknown etiology of infantile spasms (cryptogenic infantile spasms) was better with hormonal therapy. Presented by A. Lux et al. [7] the results of studies of class 3 evidence (controlled with a sufficient number of patients) showed that with ACTH therapy the effect is achieved 3 times faster than with vigabatrin, and the remission rate is higher.

Why is hormonal therapy effective for West syndrome?

There is no clear answer to this question. Experimental studies have provided evidence that in this case there may be several mechanisms of action, among them: stimulation of the synthesis of glucocorticoids that interact with steroid receptors in the central nervous system, which leads to modification of the functions of calcium channels; stimulation of the synthesis of neurosteroids in glia and neurons, which leads to modulation of GABA receptors; suppression of the activity of proconvulsant molecules (corticotropin-releasing hormone). Corticotropin-releasing hormone is a stress hormone that has a proconvulsant effect in the immature brain and is elevated in the brains of children with West syndrome; direct activation of melanocortin receptors, these receptors suppress the stress hormone, as well as a factor that provides readout of most inflammatory molecules.

By definition T. Granata et al. [8], hormonal therapy for West syndrome «

acts at the intersection of inflammation, stress and epilepsy.”

Choice of drug

For West syndrome, the choice of hormonal therapy presents certain difficulties. In world practice, preference is given to ACTH. In the USA, natural ACTH is used; in Europe it is not available and its synthetic analogue tetracosactide (trade name “Synacthen Depot”) is used. There have been no comparative studies on their effectiveness. Nevertheless, the doses of these drugs are considered equivalent, but the price of natural ACTH is 200 times more expensive than its synthetic counterpart. Both of these drugs are not registered in the Russian Federation, which creates significant difficulties in the treatment of West syndrome. Corticosteroids (prednisolone, methylprednisolone, dexamethasone, hydrocortisone) are available in Russia. In international practice, prednisolone is considered the drug of choice in the treatment of infantile spasms, mainly in developing countries [6]. Most experts believe that there is insufficient evidence that prednisolone, methylprednisolone, and dexamethasone are as effective as ACTH [1]. Nevertheless, in recent years, corticosteroids have been mainly used in practice, and their rather high effectiveness and acceptable tolerability are noted.

Hormone therapy protocols

Considering the difficulties described above, we are forced to admit the absence of a single universal protocol for the treatment of West syndrome. Different clinics around the world use different drugs and their administration regimens, but there is a general treatment strategy.

Its general principle is to start therapy as early as possible. It is believed that the earlier effective treatment is started, the better the prognosis for the child’s further psychomotor development [3, 6]. It is optimal to begin treatment immediately after the onset of infantile spasms and confirmation of the presence of hypsarrhythmia on the EEG. But this is not always possible, since even in developed countries the delay in diagnosing West syndrome is quite significant. Thus, S. Auvin et al. [9] reported that the correct diagnosis was not made in approximately 80% of children (301 of 362 children). In domestic practice, the delay in starting adequate (in this case hormonal) therapy is usually several months. Recently, an international study was initiated [10], in which low doses of ACTH were prescribed to children at high risk of developing infantile spasms in the presence of prehypsarrhythmia. Its authors sought to normalize the EEG. Unfortunately, the results of this study have not yet been fully published.

Another general principle of hormonal therapy is minimal doses and minimal duration of treatment. This principle stems from problems with tolerability of hormonal therapy, in particular associated with the potential for the development of adrenal insufficiency and decreased immunity.

Next, the doses and frequency of administration of drugs are discussed, including natural ACTH and its synthetic analogue - tetracosactide, which are not registered in the Russian Federation. Note that the latter drug was included in the Russian Clinical Guidelines for the diagnosis and treatment of epilepsy [11].

Doses and frequency of administration

ACTH and tetracosactide

. The doses of these two drugs are equivalent. There are discrepancies regarding the doses of ACTH and tetracosactide. In the USA, 0.6-0.8 mg (equal to 0.6-0.8 ml) ACTH is used daily, in Japan - 0.1 mg (0.1 ml) ACTH daily, in Finland - 0.25 mg (0 .25 ml) per day or 0.5 mg (0.5 ml) tetracosactide (Synacthen depot) every second day. In the UK, 0.5 mg (0.5 ml) of tetracosactide is administered every second day. Why daily doses in the United States are several times higher than those taken in Japan is unclear [6]. There are studies [6] that compared the effectiveness of high and low doses of ACTH. High doses of ACTH (1.50 mg) stopped spasms in 79% of all cases, low doses (0.25 mg) - in 76% of all cases, which suggests that low doses of ACTH are no less effective than high doses. It is clear that the use of lower doses improves tolerability of the drug. Currently, the tetracosactide administration regimen described in the Clinical Guidelines for the diagnosis and treatment of epilepsy is not used [11], namely: an initial dose of 0.1 mg (ml) with a gradual increase in it to a dose at which seizures disappear (but not more than 1 mg), and switching to regimens more commonly used in Europe of 0.4-0.5 mg (ml) daily or every other day [6].

Prednisolone and methylprednisolone.

Recommended doses vary from 2 to 10 mg per 1 kg of body weight per day [6, 11]. In principle, there are two treatment options: high-dose pulse therapy and longer-term oral administration of the drug. Based on the work of J. Mytinger et al. [12], a combination of these two methods was used, in which the combination of pulse therapy with high doses of prednisolone (20 mg per 1 kg of body weight for 3 days) and oral administration of low doses for 2 months led to remission in 50% of cases. This approach to the use of prednisolone is advisable, since high doses of pulse therapy allow you to quickly (within several days) get rid of hypsarrhythmia and spasms), and the subsequent use of low doses of prednisolone ensures long-lasting effect and good tolerability. Currently, pulse therapy with methylprednisolone is also used in a daily dose of 30 mg per 1 kg of body weight intravenously or intramuscularly for 3 days with a transition to oral administration of the drug. The dose for oral administration should be no more than 2 mg per 1 kg of body weight with its further reduction. It is believed that the optimal dose for long-term oral administration is no more than 10 mg per day, and this is what we should strive for. There is evidence that higher doses (15 mg per day or more) for 2 weeks can cause further development of adrenal insufficiency [6]. Further studies are needed to establish the optimal dose of prednisolone.

Dexamethasone.

The recommended dose is 0.3-0.5 mg per 1 kg of body weight per day daily intramuscularly 10 injections, then 5 injections every other day and 5 injections every 2 days in the same doses [11, 13]. E. Haberlandt et al. [14] suggest administering 20 mg of dexamethasone intravenously for 3 days, and then 5 courses of 3 days each with an interval of 4 weeks. K.Yu. Mukhin and A.S. Petrukhin [15] provide the following regimen for prescribing dexamethasone orally: 2 mg per 1 kg of body weight for 1 week, then 1 mg per 1 kg of body weight per day for 2 weeks, then switching to an alternative therapy - 1 dose 1 time in 2- 3 days.

Hydrocortisone.

We were unable to find recommended regimens for the use of hydrocortisone in the literature. The following empirical regimen can be used: an initial dose of 10 mg per 1 kg of body weight per day for 1 week, and then the daily dose is reduced by 2 mg per 1 kg of body weight once a week (8, 6, 4 and 2 mg per 1 kg of body weight per day, respectively).

Duration of hormone therapy

The optimal duration is still unknown. According to various researchers, it ranges from 4 to 12 weeks (1-3 months) [6]. But it is almost never possible to limit yourself to one or two months of therapy. The main problem is the high relapse rate after stopping hormone therapy, so the longer low-dose hormone therapy is continued, the lower the risk of relapse. There are virtually no relapses for 6-12 months in those patients who are slowly withdrawn from hormonal therapy, and the total course is about 5 months. In the above-mentioned study by E. Haberlandt et al. [14] also noteworthy is the long total duration of therapy (in general, about 6 months). True, we are talking about pulse therapy. How justified such a long course is (and it contradicts one of the principles - the minimum duration of treatment) is a debatable issue and requires further study.

Expected effectiveness of hormone therapy

The cessation of attacks and the disappearance of hypsarrhythmia is observed with the use of ACTH in 60-80% of patients with infantile spasms [3, 6, 16]. The dispersion of effectiveness indicators is apparently associated both with different treatment protocols (choice of a specific drug, dose, duration of treatment) and with different criteria for assessing effectiveness (in particular, different features of the diagnosis of hypsarrhythmia). For corticosteroids, there is less data on the frequency of seizure cessation, and they vary widely - from 50% in the work of J. Mytinger [12] (prednisolone was used) to 100% in the work of E. Haberlandt (dexamethasone was used) [14]. In a study by J. Wanigasinghe et al. [17] the effectiveness of prednisolone was higher than ACTH: cessation of attacks and disappearance of hypsaritymia was achieved in 28 (58.3%) of 48 patients treated with prednisolone and in 18 (36.7%) of 49 patients treated with ACTH. A positive effect was achieved faster with prednisolone (3.85±2.4 days) than with ACTH (8.65±3.7 days) [17]. In contrast, in the work of K. Knupp et al. [18] the effectiveness of ACTH was higher than with the use of oral corticosteroids - cessation of spasms was observed in 55 and 39%, respectively. The effectiveness of hormonal therapy does not appear to depend on the timing of its initiation. The results of the study by M. Farnosova et al. [19] in 150 children with West syndrome showed that early initiation of tetracosactide therapy (within the first month of life) was no more effective than later initiation of therapy in stopping infantile spasms.

In case of relapses, and they are very common in West syndrome, it is recommended to repeat hormonal therapy. Everything regarding the effectiveness of repeated hormonal therapy for relapses has not been sufficiently studied. M. Ikegami et al. [20] describe the high effectiveness of ACTH during relapse: attacks stopped in 52% of patients. If the patient had only infantile spasms (without other types of seizures), then the effect was even better - seizures stopped in 75.6%.

Tolerance to hormone therapy

When using both ACTH and corticosteroids, side effects from various systems and organs can be observed. Therefore, it is advisable to hospitalize the child for some period of time (1-2 weeks) or carry out initial therapy with high doses in a day hospital under the supervision of a doctor.

Serious side effects of hormone therapy include co-infections, hypertension, and adrenal insufficiency after discontinuation of therapy. It is not recommended to carry out hormonal therapy in the presence of an active infectious process. In this situation, it is better to consider the possibility of prescribing vigabatrin. The possibility of developing arterial hypertension requires monitoring blood pressure and prescribing diuretics if it develops. In patients with arterial hypertension while taking hormones, one should be wary of the development of hypertrophic cardiomyopathy (echocardiography is necessary). Adrenal insufficiency usually develops after hormone withdrawal when concomitant infections occur and can be fatal for the child. Therefore, long-term use of high doses of corticosteroids should be avoided. Some authors [6, 21] recommend determining the level of cortisol in the blood after hormonal treatment (the norm is 500 nmol/l). If the cortisol level is lower, a consultation with an endocrinologist is indicated to prescribe hydrocortisone replacement therapy. It is believed that ACTH, unlike prednisolone, is less likely to cause the further development of adrenal insufficiency. Frequent side effects include weight gain, irritability, sleep disturbances and, conversely, drowsiness [6, 17]. These side effects are dose dependent.

The following is the incidence of side effects in one of the latest studies on the effectiveness and tolerability of hormones in West syndrome. In the table

it is listed in descending order of frequency.

From the data in the table it follows that ACTH is slightly better tolerated than prednisolone. Another side effect not mentioned in the table is a transient increase in the size of the ventricles and subarachnoid spaces, detected by magnetic resonance imaging (MRI). The frequency of this side effect has been little studied, but it is mentioned [10] that when using high doses of ACTH, this phenomenon was observed in approximately 60% of patients. This is why an MRI is usually recommended before starting hormone therapy. The rapid development of transient brain atrophy in the presence of hormones is also associated with an increased risk of intracranial hemorrhage, which is also associated with high doses of hormones [10]. The exact incidence of intracranial hemorrhage as a complication of hormonal therapy for West syndrome is not known.

The frequency of side effects during hormone therapy for West syndrome according to J. Wanigasinghe et al. [17]

In general, despite the high frequency of side effects, limiting the timing of taking high doses of hormones and the duration of treatment helps to minimize their negative impact.

Possible reasons for the lack of effect of hormonal therapy

Despite the fairly high effectiveness of hormonal therapy, there are still patients who either do not respond to it at all or do not respond sufficiently. If seizure cessation is not achieved within 2 weeks of hormonal use, continuation of therapy is not advisable, although there have been isolated cases of ineffective prednisolone in which switching to tetracosactide was effective, as well as the effectiveness of prednisolone in the absence of effect from ACTH. Failure to respond to any hormonal therapy option may occur in genetically determined cases of West syndrome. This applies to both monogenic mutations associated with West syndrome (for example, mutations of the CDKL 5

), as well as numerous congenital metabolic defects, including West syndrome. Although the effectiveness of hormones has also been described for inborn defects of metabolism (but does not lead to improvement in the neuropsychological development of the child), it is clear that infantile spasms caused by biotinidase deficiency or pyridoxine dependence will be sensitive to specific therapy, and not to hormones. The lack of effect of hormonal therapy, along with other indications, serves as a serious reason to clarify the genetic nature of West syndrome. In a situation where the metabolic nature of West syndrome is suspected, it always makes sense to try sequentially high doses of pyridoxine, pyridoxal phosphate, biotin, and folic acid [22].

The place of antiepileptic drugs in the treatment of West syndrome

In the available domestic literature, the role of various antiepileptic drugs (especially valproate, topiramate and benzodiazepine) in the treatment of West syndrome is widely discussed. In the international literature, they take a back seat to vigabatrin and hormones, and are used only in the absence of effect from the first two. In domestic practice, the situation when hormonal therapy is the starting one (without a previous prescription of an antiepileptic drug - AED) is extremely rare. This may be due to the fact that hospitalization for hormone therapy does not take place immediately (it is often planned). Most patients admitted to our institute's departments with West syndrome are already receiving some kind of AED. Most often it is valproate. When hormonal therapy is effective, the question arises: what to do with an AED already taken that is ineffective or partially effective? There is no answer to this question in the guidelines and recommendations, and it is decided individually after discussing the situation with the child’s parents. Parents, as a rule, are afraid of relapse, and often previously received AEDs are not discontinued, although this tactic is controversial. When hormones are effective, it always makes sense to simplify antiepileptic therapy, gradually reducing the number of AEDs to a minimum (many children receive 2-3 ineffective drugs for months).

The literature presented in this review allows us to draw the following conclusions: 1. Hormonal therapy for West syndrome is more effective than the use of vigabatrin (with the exception of patients with tuberous sclerosis). 2. The effect of hormonal therapy is achieved faster than with vigabatrin, and rapid initiation of treatment improves the prognosis of the child’s mental development. 3. In international practice, preference in therapy is given to ACTH and tetracosactide (not registered in the Russian Federation); in our country, the use of corticosteroids is also acceptable. 4. In order to improve tolerability, the minimum effective doses and minimum duration of treatment should be adhered to. The optimal dose of hormones and the optimal duration of treatment are still unknown. 5. Further research is needed to develop optimal treatment protocols for West syndrome.

There is no conflict of interest.

According to our small personal experience, when using methylprednisolone according to the scheme of pulse therapy plus oral administration, it was possible to achieve the disappearance of attacks and the elimination of hypsarrhythmia in 15 (75%) patients out of 20. We also observed the disappearance of hypsarrhythmia very early (immediately after the 3rd day of pulse therapy ), but it was not durable unless accompanied by oral maintenance therapy.

Symptoms and diagnosis of West Syndrome

As mentioned earlier, in most cases the pathological process debuts in the first year of a child’s life. At the same time, the clinical picture becomes more diverse as West Syndrome develops , and the progression of the pathology begins mainly with a delay in psychomotor development. Among the most characteristic clinical signs, experts include:

• loss of the infant’s grasping reflex;
• disorders of fixation, gaze focusing;
• the child cannot follow a specific object with his eyes;
• generalized epileptic seizures of varying duration.

It is important to understand that intervals between spastic muscle contractions can be less than 1 minute. Moreover, the frequency of seizures varies significantly, from several dozen per day to hundreds. In this case, the head bends and twitches, the arms are extended, the body bends into an arc (the attack ends with complete relaxation of the body).

During the diagnostic process, our doctors pay special attention to the symptoms of West Syndrome , record every parental complaint, and find out the frequency, frequency, and nature of attacks. A detailed examination of the medical history, examination of the child is also carried out, and the specifics of psychomotor developmental delay are assessed. After this, a series of laboratory and hardware tests are carried out:

• general clinical blood and urine tests;
• biochemical blood test;
• electroencephalography;
• polysomnography;
• computed tomography or magnetic resonance imaging of the brain.

A mandatory stage of diagnosis is consultation with specialized specialists, including a pediatrician, pediatric neurologist, and epileptologist.

First signs

The main symptom of epilepsy is seizures. With cryptogenic epilepsy, generalized or partial seizures may occur. Before the onset of partial seizures, more typical of cryptogenic epilepsy, a so-called aura occurs. It signals that a seizure is about to occur. The appearance of the aura allows the doctor to determine the location of the brain lesion.

A motor type aura is accompanied by unexpected movements of the patient, which indicates the location of the affected part in the frontal lobe of the brain. With a rapid deterioration in visual and auditory functions, it can be assumed that the localization of the disorders is in the occipital or temporal lobe.

Expert opinion

Author: Polina Yuryevna Vakhromeeva

Neurologist

In recent years, doctors have noted an increase in the incidence of epilepsy. The exact causes of this disease have not yet been established. It is known that about 4-10 people per 1000 people suffer from seizures. Drug-resistant cryptogenic epilepsy is considered one of the most severe forms of the disease. It is difficult to select treatment for this species due to drug resistance.

A qualified team of doctors at the Yusupov Hospital takes on complex cases. Diagnosis of epilepsy in our clinic is carried out using modern medical equipment. European CT, MRI and EEG equipment are used for examination. They allow you to determine the location of the pathological focus with high accuracy. An analysis of blood and the degree of resistance of the body to drugs is carried out in a modern laboratory.

Therapy for cryptogenic epilepsy is carefully developed by experienced neurologists and epileptologists. In this case, an individual approach is used. Drugs are selected taking into account the degree of drug resistance and other associated conditions. The prescribed medications meet the latest European standards for the treatment of epilepsy.

Conservative

As part of conservative therapy, our specialists use innovative techniques, use the latest generation drugs, the duration of administration and dosage of which are calculated individually. The main groups of drugs aimed at treating West epilepsy syndrome include:

• muscle relaxants;
• glucocorticosteroids;
• valproate group agents;
• adrenocorticotropic hormone;
• vitamins of group B6;
• immunomodulators.

Diagnostics

When diagnosing the disease, the main criterion is to exclude organic brain damage. It is for this purpose that the following procedures are used:

• collection of family and individual anamnesis with clarification of the presence of brain injury, birth or congenital pathology;
• electroencephalogram, based on recording the biorhythms of the brain during various activities, for example, sleep, intellectual or physical activity;
• conducting magnetic resonance imaging, which helps detect impaired brain activity or localized brain damage;
• performing angiography, which allows you to study how well the blood supply to the brain flows;
• neuropsychological diagnostics, designed to study the quality of mental activity;
• a blood serum test aimed at detecting syphilis and other pathogenic microorganisms in the body.

The victim must undergo laboratory tests, including genetic diagnostics, biochemical and general blood tests, as well as a urine test. Epilepsy is diagnosed by doctors only after conducting a full range of examinations and excluding the possibility of other pathologies affecting the body.

Surgical

Surgical interventions in the treatment of West Syndrome occur in rare cases when attacks are caused, for example, by a brain tumor or tuberous sclerosis. In such cases, surgeons of the highest category are involved, who perform operations to remove lesions and tumor processes. The maximum accuracy and efficiency of neurosurgical methods is due to both the qualifications of doctors and the use of the latest equipment, for example, the Da-Vinci robotic system.

What kind of disease is this

Cryptogenic epilepsy has a whole set of epileptic symptoms, the causes of which cannot be determined. The type of illness is hidden. Manifestations of cryptogenic epilepsy are not differentiated from symptoms of other types of disease. With this form, spontaneous electrical impulses occur in the cerebral cortex, causing overexcitation of nerve transmission. As a result, a convulsive focus is formed, provoking an attack.

Seizures can be focal or gradually spreading throughout the victim’s body. The first type of pathology represents limited areas of increased activity in the cerebral cortex.

The symptomatic nature of the disease is detected only with a comprehensive examination of the body. Local abnormalities are detected using magnetic resonance imaging.

Depending on the pathological focus, epilepsy can occur in such lobes as:

• frontal;
• temporal;
• occipital;
• parietal

The frontal form is often accompanied by sudden attacks that last in short series. In rare situations, secondary generalization occurs due to damage to brain cells. If the focus of epileptic activity is localized in the temporal zone, the patient, against the background of regularly manifested emotions, exhibits a phenomenon such as déjà vu. It is this subtype of the disease that is most often encountered in practical medicine. Cryptogenic frontotemporal epilepsy is diagnosed in the same way; the symptoms in this situation are combined.

Pathology is always accompanied by increased activity in the crown area, which is responsible for sensory functions. Victims experience unusual sensory sensations, such as:

• tingling on only one side of the body;
• distorted perception of one's own limbs.

Accompanied by partial seizures, it is the rarest form.

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Prof. Dr. Bettina Schmitz

Neurology – Epilepsy

Head of the Department of Neurology

Specialization

• Epilepsy
• Differential diagnosis of non-epileptic paroxysms
• Long-term EEG - video monitoring
• Medical and non-medical care for paroxysms and complications associated with epilepsy
• Member of the British Medical Association
• Instructor and member of the European Academy for the Study of Epilepsy (EUREPA)
• Member of the Commission on Psychobiology and the International League Against Epilepsy (ILAE)
• Psychoorganic syndrome, World Federation of Societies of Biological Psychiatry (WFSBP)
• Video consultation

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Symptoms

The clinical picture of cryptogenic epilepsy is manifested by the following symptoms:

• a more severe course of attacks, in contrast to the classic type of disease;
• reduced effectiveness of drug treatment;
• significantly accelerated pace of personality change;
• different results of diagnostic studies with each subsequent execution.

Each type of cryptogenic epilepsy has its own characteristic features and symptomatic manifestations that make it possible to differentiate one form from another.

Partial seizures characteristic of cryptogenic focal epilepsy can be simple (without loss of consciousness), complex (with loss of consciousness) and complicated by secondary generalized seizures.

The leading symptom complex of cryptogenic focal epilepsy is repeated partial paroxysm.

The nature of simple partial seizures can be motor, sensory, autonomic, somatosensory, accompanied by auditory, visual, olfactory or gustatory hallucinations and mental disorders.

Complex partial seizures in cryptogenic focal epilepsy in rare cases begin with a simple seizure, after which the patient’s consciousness is impaired, and sometimes automatisms occur. After an attack, patients experience confusion. Secondary generalization of partial seizures is allowed. In these cases, at the beginning, an epileptic attack can be simple or complex, and as it develops, there is a diffuse spread of excitation to other parts of the cerebral cortex, after which the paroxysm becomes generalized.

With symptomatic epilepsy, in addition to seizures, other symptoms develop, corresponding to the main lesion of the cerebral cortex. In patients with this diagnosis, intelligence decreases, cognitive impairment occurs, and children experience delayed mental development.

In idiopathic epilepsy, which is characterized by benignity, neurological deficits and mental and intellectual disorders are not observed.

In addition, the disease may be accompanied by the occurrence of twilight states, in which the following occurs:

• auditory and visual hallucinations;
• erratic motor and speech activity;
• clouding of reason.

Attacks of focal cryptogenic epilepsy in adults are more severe than in children. Finding effective therapy is also more difficult for them. Conventional antiepileptic drugs for this pathology have less effect and have virtually no effect in minimal dosages. During the period between attacks, patients continue to have nonspecific symptoms not associated with convulsive attacks: depression, resentment, unreasonable temper, memory impairment.

Prof. Dr. Jörg Müller

Neurology – Parkinson's, MS

Head of the Department of Neurology

Specialization

• Recognized international expert in Parkinson's disease, dystonia and tremor
• Neuromuscular diseases
• Multiple sclerosis
• Treatment using butolotoxin (dystonia, spasticity)
• Deep brain stimulation treatment
• Author of more than 70 scientific publications, is a member of international expert councils

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Prof. Dr. Bruno-Marcel Mackert

Neurology

Head of the Department of Neurology

Specialization

• Internationally recognized specialist in stroke, peripheral nervous system diseases and clinical electrophysiology
• Author of over 40 original publications
• Board of Directors of the Berlin Stroke Society (BSA)
• Member of the Board of Directors of the Berlin Center for Stroke Research (CSB)

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Treatment

Timely diagnosis of the disease will help you select an effective treatment program and reduce the risk of side effects. Therapy can be carried out in an inpatient or outpatient setting. Correction with medications is prescribed only after the final diagnosis is made and the second attack of pathology is completed. Therapeutic treatment for a disease has several goals:

• relief of pain accompanying attacks;
• preventing the occurrence of new epileptic episodes;
• reduction in the frequency of seizures;
• reducing the duration of the attack;
• reducing the likelihood of new side effects;
• restoring the functioning of the nervous system without the need to use medications.

Drug therapy for cryptogenic epilepsy involves taking medications that reduce the frequency and duration of epileptic seizures. Additionally, nootropic substances that affect nerve impulses can be prescribed. In addition, patients with a similar diagnosis are prescribed psychotropic medications.

Some patients with cryptogenic epilepsy are prescribed surgical correction, a therapeutic diet, and physiotherapeutic techniques. There is no single method to completely get rid of epileptic seizures. However, competent treatment at the Yusupov Hospital using innovative techniques helps alleviate the condition of patients in 90% of cases.

Considering the fact that the patient’s condition depends on the influence of external factors, he is recommended to lead a correct lifestyle, try to eliminate stressful situations and overwork.

The diet of a patient with epilepsy should include foods with increased amounts of protein. It is necessary to give up hot and spicy foods, coffee and tea. A dairy-vegetable diet is recommended. Due to the fact that alcohol can aggravate the course of the disease, drinking alcohol even in minimal doses should be strictly avoided.

Prof. Dr. Jörg Wissel, MD, FRCP

Neurological rehabilitation

Head of the Department of Neurological Rehabilitation and Physiotherapy, Department of Neurology and Rehabilitation Center

Specialization

• Neurological rehabilitation after stroke, traumatic brain injury and spinal cord injury
• Rehabilitation therapy for Parkinson's disease and dystonia
• Botulinum therapy
• Treatment of spasticity
• Neurological rehabilitation using intrathecal baclofen therapy and deep brain stimulation
• Author of more than 90 scientific publications, member of several expert commissions

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