[Epileptic syndromes associated with focal clonic seizures]. / Epilepticheskie sindromy, assotsiirovannye s fokal'nymi klonicheskimi pristupami.

OBJECTIVE: To study clinical, electroencephalographic and neuroimaging features in children with epileptic syndromes associated with focal clonic seizures (FCS). MATERIAL AND METHODS: We examined 1258 patients with various forms of epilepsy with the onset of seizures from the first day of life to 18 years. RESULTS: FCS was identified in 263 patients (20.9%). FCS were included in the structure of 13 different epileptic syndromes: Rolandic epilepsy (28.1%), structural focal epilepsy (27.5%), structural focal epilepsy associated with benign epileptiform discharges of childhood (SFE-BEDC) (20.6%), focal epilepsy of unknown etiology (7.5%), epilepsia partialis continua (4.6%), pseudo-Lennox syndrome (3.4%), ESES syndrome (2.7%), Landau-Kleffner syndrome (1.5%), Dravet syndrome (1.1%), benign occipital epilepsy (1.1%), benign focal epilepsy in infancy (0.8%), MISF syndrome (0.8%), cognitive epileptiform disintegration (0.8%). In 50% of cases, epilepsy associated with FCS debuts before the age of 5 years (from 1 month to 18 years, average age 4.26±3.9). CONCLUSION: The groups of syndromes associated with FCS have different prognosis for remission of seizures. Prognostic predictors of seizure remission are: epileptic syndromes associated with BEDC, the presence of periventricular leukomalacia. A severe prognosis for the course of epilepsy is associated with local structural changes in the neocortex. Despite a favorable prognosis for seizures, continued diffuse interictal epileptiform activity with BEDC on the electroencephalogram is a predictor of the onset of cognitive impairment in children.

[Healthcare of Russia today. Epileptological service]. / Zdravookhranenie Rossii segodnya. Epileptologicheskaya sluzhba.

OBJECTIVE: To examine the state of the epileptological service in Russia by conducting a survey of epileptologists working in different regions of the Russian Federation. MATERIAL AND METHODS: To assess the structure and mechanisms of the antiepileptic service in the Russian Federation, the Russian League Against Epilepsy (RLAE) offered a questionnaire to 63 epileptologists, which has been answered by 37 epileptologists from 28 regions of the country. RESULTS AND CONCLUSION: Among the respondents, 43% are focused on the care of outpatients, 35% work both in outpatient and inpatient appointments, 22% work only in hospitals. 24% of doctors are employees of neurological departments. 84% of epileptologists noted the neurological profile of their medical institution. The most common patient routing options were: «therapist-neurologist-epileptologist¼ (59%), «Neurologist-epileptologist¼ (81%), self-referral to an epileptologist (22%), «Psychiatrist-epileptologist¼ (16%). 59.5% of epileptologists work with adults and with children. 27% of respondents noted that they work with psychiatric patients. Interaction between the departments of neurology and practical health care is carried out in the form of consultations and training events, as noted by 68% of epileptologists. 73% of the epileptologist reported the willingness to actively interact with the RLAE. 54% of the respondents conduct socially oriented schools for patients and their relatives. In the context of the planned reorganization of the health care system, the epileptologists gave the outlook on the work of the epileptological service. CONCLUSION: The main initiatives mentioned by the respondents were the following increasing the availability of medicines and examination methods, increase of continuity in the work of the epileptological service, creation of unified databases, creation of specialized epileptological centers and/or offices of epileptologists.

[The effectiveness of antiepileptic therapy in patients with epileptic syndromes associated with myoclonic seizures]. / Effektivnost' protivoepilepticheskoi terapii u patsientov s epilepticheskimi sindromami, assotsiirovannymi s mioklonicheskimi pristupami.

OBJECTIVE: To evaluate the effectiveness of antiepileptic therapy of epileptic syndromes associated with myoclonic seizures. MATERIAL AND METHODS: One hundred and three patients with epilepsy, in whom myoclonic seizures were identified during the observation, were examined. The observed myoclonic seizures entered the structure of 11 different epileptic syndromes. RESULTS AND CONCLUSION: Drug remission is achieved in 67% of patients, a decrease in the frequency of seizures by 50% or more was noted in 23,3%, the lack of effect in 9,7%. The effectiveness of antiepileptic therapy was significantly different in different groups of epileptic syndromes. The high effectiveness of antiepileptic therapy in the general group of patients in this study is mainly due to the prevalence of patients with juvenile myoclonic epilepsy, in which the highest percentage of remission was observed.

[Indication of antiepileptic drugs to patients with epilepsy: results of the survey of neurologists]. / Prioritety v naznachenii protivoépilepticheskikh preparatov patsientam s épilepsiei po rezul'tatam oprosa vrachei-nevrologov.

AIM: To study knowledge and opinions of neurologists about priorities in using antiepileptic drugs (AEP). MATERIAL AND METHODS: Eighty-one neurologists from Moscow and surrounding regions were surveyed to identify the factors that influenced the choice of AEP. RESULTS AND CONCLUSION: Valproic acid was the most frequently used drug followed by levetiracetam and carbamazepine, which reflected the overall picture of PEP indication in patients with epilepsy in the Russian population. Levetiracetam occupies a leading position as the starting drug for treatment of epilepsy; most often prescribed to women, patients with generalized seizures and idiopathic epilepsy. It is the drug of choice as adjuvant remedies for Duo therapy.

[Differential diagnosis of paroxysms of tonic muscle tension in children of early age with delay of psychomotor development and abnormal neurologic status]. / Differentsial'naia diagnostika paroksizmov tonicheskogo napriazheniia myshts épilepticheskoi i neépilepticheskoi prirody u detei rannego vozrasta s zaderzhkoi psikhomotornogo razvitiia i ochagovymi nevrologicheskimi simptomami.

AIM: To study neurologic status, results of video-EEG monitoring and magnetic resonance imaging in children under 3 years old with paroxysms of tonic muscle tension. MATERIAL AND METHODS: One hundred and forty-six infants and young children with motor disturbances and different variants of clinically similar epileptic seizures, hyperkinesis and stereotypes were examined. RESULTS AND CONCLUSION: Cerebral palsy (91%), genetic and chromosomal abnormalities (6%), brain malformations (2%) were identified. Neurological status was characterized by pseudobulbar syndrome (100% of cases), hemiparesis (1%), tetraparesis (81%), diffuse muscular hypotonia (18%), intellectual and speech development delay (76%), autistic behavior (16%). During the prolong video-EEG monitoring, paroxysmal tonic muscle tensions were recorded in all patients: epileptic seizures were observed in 113 patients (77.40%), non-epileptic paroxysms in 51 (34.93%). The combination of epileptic and non-epileptic paroxysms was observed in 18 patients (12.33%). In 4 patients (2.75%), it was not possible to determine the genesis of paroxysms even during the prolong video-EEG-monitoring because of myographic artefacts. Five clinical and electroencephalographic combinations of dystonic attacks, epileptic seizures and epileptiform activity were identified. These data allow improving the diagnosis of epilepsy and avoiding unnecessary treatment with antiepileptic drugs. Our study has shown a high diagnostic value of video-EEG monitoring with the inclusion of sleep in patients with paroxysmal conditions in infancy and early childhood.

[Epileptic syndromes in childhood associated with secondary generalized tonic-clonic seizures]. / Épilepticheskie sindromy v detskom vozraste, assotsiirovannye s vtorichno-generalizovannymi sudorozhnymi pristupami.

AIM: To study a group of patients with secondary generalized tonic-clonic seizures (SGTCS) in view of nosology, medical history, clinical, electroencephalographic and neuroimaging features. MATERIAL AND METHODS: The study included 471 patients, 244 (51.8%) men and 227 (48.2%) women. RESULTS: SGTCS were observed in many epileptic syndromes. The most frequent were symptomatic focal epilepsy (33.8%), cryptogenic focal epilepsy (23.8%), rolandic epilepsy (12.6%), FEBL-BEDC syndrome (12.3%). Other forms of epilepsy were less frequent. The onset of epilepsy ranged over a wide age range from the first month of life to 18 years. The average age of onset was 5.7±4.96 years. SGTCS as the only type of paroxysms were observed in 28.3% of cases. Two or more types of seizures were observed in 71.7% of patients, three or more types in 39.3%. Epileptiform activity on EEG during long VEM was detected in 91.3% of patients with SGTCS. In 37.2% of patients, benign epileptiform discharges of childhood were recorded. Treatment with antiepileptic drugs (AEP) led to complete remission in 57.1% of cases of epilepsy associated with SGTCS. A reduction of the frequency of seizures by 50% or more was found in 33.6% of patients treated with AEP. No effect was observed in 9.3% of patients. CONCLUSION: Significant differences in the prognosis and therapeutic approaches to specific epileptic syndromes associated with SGTCS necessitate the use of the entire spectrum of diagnostic measures, which should include careful history taking, clinical examination, video-EEG monitoring with the inclusion of sleep dynamics, MRI / CT brain, genetic testing.

[Eye movement disorders in children].

The article includes review of literature on anatomy, physiology, symptoms of ocular movement and their disturbance in children. Differential diagnosis between early developmental disturbances of vision in the normal child and during the diseases of central nervous system is very hard. There is data on such pediatric neuro-ophthalmology complex disorders as nystagmus, paroxysmal tonic upgaze, opsoclonus, spasmus nutans, seizures (eyelid myoclonia, absences).

[Clinical presentation and diagnosis of epileptic auras].

To define clinical presentations of visual auras and to reveal their clinical, encephalographic and neuroimaging correlates, we examined 23 patients, aged from 5 to 25 years (mean 14±6 years), with focal forms of epilepsy. Patients had visual auras regardless of the etiology of epilepsy which developed immediately before epileptic seizures or were isolated. Patients had simple or complex visual hallucinations, the former occurring more frequently, visual illusions and ictal amaurosis. Positive visual phenomena were noted more frequently than negative ones. In most of the patients, visual hallucinations were associated with the pathological activity in cortical occipital regions of the brain and, in some cases, in temporal and parietal regions. The different pathologies (developmental defects, post-ischemic, atrophic and other disturbances) identified by MRI were found in a half of patients.

[Atypical absences: prevalence, electroclinical and neuroimaging characteristics].

The study included 1261 patients with different forms of epilepsy. Ages at onset of disease varied from the first days of life to 18 years old. Absence seizures were identified in 231 (18.3%) patients, atypical absences (AA) in 129 (10.2%) of patients, with the frequency of absence seizures 55.8% in the total group. Patients with AA had different forms of epilepsy with the prevalence of cryptogenic/symptomatic forms with the phenomenon of secondary bilateral synchronization in the EEG. If epilepsy manifested itself at the age above 12 years old, AA were not noted. The study demonstrated the relevance of using video-EEG monitoring for diagnosis of epilepsy with AA. The antiepileptic treatment was effective in 53.5% of patients.

Epilepsia partialis continua in tick-borne Russian spring-summer encephalitis.

OBJECTIVES: Epilepsia partialis continua (EPC) is characterized by localized continuous jerks, from time to time with spreading Jacksonian seizures and, more rarely, secondarily generalized tonic-clonic seizures. EPC has numerous possible etiologies. In this paper we describe EPC in the tick-borne Russian spring-summer encephalitis (TBRSSE) and compare it with Rasmussen syndrome. METHODS AND METHODS: We included patients with EPC in TBRSSE (between 2003 and 2010). The diagnosis was verified by immunology (antibodies against TBRSSE virus). The patients were followed 1-7 (mean 3.4) years. RESULTS: We studied 10 patients (eight males, age 10-21 years) with MRI and video-EEG. Nine developed EPC after acute TBRSSE (meningoencephalitic form), and one had a tick bite without clinical symptoms of encephalitis, but with subsequent EPC. All patients came from Ural and Siberia. The onset was at age 4-14 (mean 8.6 years). The interval from onset of TBRSSE or the tick bite to seizure onset was 1 day-4 years. We identified three phases of clinical course EPC in TBRSSE: (i) acute (meningoencephalitic/encephalitic); (ii) development of EPC; and (iii) chronic EPC. The effect of antiepileptic drugs differed according to seizure types. CONCLUSION: EPC caused by TBRSSE is relatively frequent in the Eastern parts of the Russian Federation but not west of the Ural. Unlike Rasmussen encephalitis, EPC with TBRSSE does not progress even in the long term. It appears as disabling but not fatal condition with a time course where three phases can be distinguished.

[Efficacy and safety of the monotherapy with trileptal (oxcarbazepine) in children and adolescents].

A prospective non-randomized non-controlled multicenter trial has been conducted. The trial included 254 children, aged from 11 months to 18 years (mean age 9.3 +/- 4.5 years), with predominantly focal forms of epilepsy treated with trileptal (oxcarbazepine). The observation period was 31 weeks. Efficacy and safety of therapy was assessed in 3 visits: screening and assignment to therapy (visit 1), the end of titration and achievement of maintenance dose (visit 2), assessment of maintenance therapy (visit 3). The percentage of patients with a positive response to the trileptal therapy (the decrease of seizure frequency by 50% and more) was 91.1%. The complete reduction of seizures was achieved in 59.4% of patients. Most of patients (95.3%) continued to receive trileptal until the end of the trial. The significant decrease (p < 0.001) of seizure frequency from visit 1 to visit 3, the reduction of epileptiform activity (p < 0.05) on the awake EEG in visit 3 were found. The mean effective dose of trileptal was 902.4 +/- 442.7 mg/day, i.e. was less than 30 mg/kg/day, daily doses did not exceed 600 mg. Adverse effects were observed in 11.2% of patients but in 40% of cases they seemed not be related to the drug. The adverse effects were from mild to moderate extent. In conclusion, trileptal as the monotherapy is effective and well-tolerated in the treatment of focal epilepsies in the age groups studied.

[A use of high dosages of piracetam in the treatment of Kozhevnikov epilepsy syndrome].

An efficacy of high dosages of piracetam in the treatment of children with Kozhevnikov epilepsy syndrome (epilepsia partialis continua) has been studied. The study included 6 patients, aged from 9 to 16 years, with diagnosis of Kozhevnikov epilepsy (KE) who received piracetam (nootropil, UCB) in mean dose of 1g/kg/day (up to 35 g/day) intravenous in drops. The treatment course was 30 days. During therapy, there was decrease of myoclonus frequency by 75% in 3 patients and its full stopping in 3 patients. Focal clonic seizures were stopped in 2 cases, their frequency decreased in 4 cases. Secondary generalized seizures did not relapse in 2 cases and there was a decrease of seizures by 50% and over in 4 remained cases. The use of nootropil led to the improvement of neurological status in 5 out of 6 patients with KE. The intensity of hemiparesis was reduced in 3 patients treated with nootropil. The improvement of cognitive functioning was observed in 5 out of 6 patients. The duration of clinical improvement was 1-2 months after infusions cessation. The study revealed the efficacy of high dosages of nootropil in combination with basic antiepileptic drugs in the treatment of Kozhevnikov epilepsy.

[Topamax in monotherapy of epilepsy]. / Topamaks pri monoterapii épilepsii.

Thirty-three patients, aged 3-29 years, with the following epileptic types: symptomatic forehead (15), symptomatic temporal (6), symptomatic occipital (2), juvenile myoclonic, in combination with eyelid myoclonus syndrome with absences, (5), epilepsy with isolated generalized seizures (3) and rolandic epilepsy (2), were treated with topamax. A medication dose was 50-200 mg per day in children younger 12 years and 100-550 mg per day in those older 12 years and in adults. The results obtained suggest the high efficacy and well tolerability of topamax in monotherapy of epilepsy. Therapeutic effect was achieved in 28 out of 33 patients (84.8%), i.e. seizures stopping--in 18 patients (54.5%). Monotherapy was mostly effective in symptomatic forehead epilepsy: seizures stopped in 53.3% patients and a frequency of seizures reduced in 33.3%. Side-effects were detected only in 18% cases, they were mostly transient and resulted in treatment withdrawal in 6% patients only.

[Clinical efficacy and tolerability of topamax in combined therapy of epilepsy]. / Klinicheskaia éffektivnost' i perenosimost' topamaksa pri kombinirovannoi terapii épilepsii.

Topamax in combination with other antiepileptic medications was used in a treatment of 45 patients with different epilepsy types. The efficacy of topamax was shown in all types of epilepsy and in most types of epileptic seizures. Positive effect was revealed in 69% of the cases, in 22.5% complete remission was achieved. The best results were obtained in idiopathic generalized epilepsy (in respect to stopping generalized tonico-clonic seizures) and in the cases of symptomatic (cryptogenic) partial epilepsy (in the treatment of simple partial motor and secondary generalized seizures). Seizures aggravation was only 6.5% in complex partial and tonic axial paroxysms. In 19% of the patients, topamax efficacy decreased, with seizures frequency gradually returning to a baseline 1-3 months after the positive effect achievement. Side effects (SE) were detected in 24% of the cases. The most frequent ones were dose-dependent SE in relation to CNS in the absence of idiosyncratic reactions. Therapy withdrawal because of SE was necessary only in 11% of the cases. Topamax is considered a promising antiepileptic medication.