[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.

[Post-graduated education and medical pedagogics: a new approach based on the competence-oriented educational program for neurologists in the field of children epileptology]. / Poslediplomnoe obrazovanie i meditsinskaia pedagogika: novyi podkhod na osnove kompetentnostno-orientirovannoi obrazovatel'noi programmy dlia vrachei-nevrologov v oblasti detskoi épileptologii.

The highly qualified performance of jobs according to professional standards due to the reformed system of additional professional education is one of the objectives of practical health care. The development of the educational program 'Children epileptology. Clinical, diagnostic and treatment issues' for increasing the qualification of neurologists was based on the competent approach that orients the content and process of education on the formation of competence in work performance. Work performance, in its turn, allowed the determination of professional tasks by neurologists. Module principles used in the development of the Program provided its structuration in accordance to step by step formation of physician competencies aimed to solve professional tasks. In its turn, professional tasks determined a choice of didactic conditions of each educational module (form, methods and means of education) that leads to the achievement of planned results.

[Modern diagnostic of agenesis of the corpus callosum in children]. / Sovremennaia diagnostika agenezii mozolistogo tela u detei.

Observations of the authors regarding main MRI symptoms of agenesis of the corpus callosum (ACC) and literature review on the structure of the corpus callosum in normalcy and pathology are presented. The authors emphasize that some cases of isolated ACC has been found during routine prenatal ultrasound examination. In this regard, prenatal MRI is more effective. In 74% patients with ACC, MRI results are consistent with the results of ultrasound and CT. MRI has advantages in the differentiation of inherited corpus callosum malformation as well as concomitant CNS abnormalities.

[Neurological complications and outcomes of bacterial meningitis in children]. / Nevrologicheskie oslozhneniya i iskhody bakterial'nykh meningitov u detei.

AIM: To analyze neurological complications and outcomes of bacterial meningitis (BM) in children. MATERIAL AND METHODS: Fifty-five patients with BM, aged from 2 months to 12 years, were examined. Bacteriological study, clinical and biochemical blood tests and blood serology and cerebrospinal fluid (CSF) tests as well as serum molecular-genetic study were performed. Neuroimaging methods (neurosonography, computed (X-ray) tomography and brain magnetic resonance imaging) were used. RESULTS AND СONCLUSION: A key role of generalized meningococcal infection in the development of BM in children was confirmed. Brain edema was an early and life-threatening complication of BM. It was found in 9% of the patients with meningococcal infection, 7.3% with pneumococcal meningitis and 3.6% with haemophilus meningitis. Changes in the brain structure were not found in 80% of the patients, in 20%, the residual stage of BM was characterized by cerebral destructive/proliferative or atrophic changes of different severity. The dissociation between clinical and neuroimaging parameters and poor outcomes of BM, related to the complicated premorbid state, concomitant somatic/neurological pathology, BM severity, late laboratory diagnosis and untimely etiotropic antibacterial treatment, were identified in 65.5%.

[Neurological presentations of bacterial meningitis in children: current possibilities of diagnosis and treatment]. / Nevrologicheskie proyavleniya bakterial'nykh meningitov u detei: sovremennye vozmozhnosti diagnostiki i lecheniya.

AIM: To improve clinical/laboratory diagnosis of bacterial meningitis (BM) in children. MATERIAL AND METHODS: Fifty-seven patients, aged from 4 months to 12 years, with BM were examined. Bacteriological study of the mucus from the nasopharynx, sterility tests of blood and cerebrospinal fluid (CNF), clinical and biochemical tests, serological study of paired samples of plasma - indirect hemagglutination test, latex agglutination assay, immunological study of plasma and CNF with the determination of interferon -α, -ß and -γ, interferon procalcitonin and neopterin with ELISA were performed at admission and after CNF remediation. Molecular-genetic study of the blood serum and CNF using PCR, neurosonography, computed tomography and MRI of the brain were done as well. RESULTS AND CONCLUSION: Most of the patients had generalized meningococcus infection (64,9%). Hib-meningitis, pneumococcus meningitis and other forms were less frequent. Characteristics of neurological presentations of BM depending on the etiology, somatic complications and time from the manifestation of symptoms were described. Clinical/biochemical criteria of BM and correlations between procalcitonin, neopterin and interleukin-8 in the CNF and blood of the patients and disease severity as well as between the level of inflammatory reactions in the CNF and the BM form were established. These results are important for the development of informative biomarkers for the prognosis of BM course and outcome.

[Treatment of asthenoneurotic disorders in children]. / Korrektsiya astenonevroticheskikh rasstroistv u detei.

AIM: To evaluate the efficacy of cogitum in the treatment of asthenoneurotic disorders in children after bacterial meningitis (BM) or brain injury (BI). MATERIAL AND METHODS: Twenty-four patients were examined. Group 1 included 14 patients with BM, 8 boys and 6 girls, aged 7 - 12 years, mean age 9,91 ± 1,71 years; group 2 consisted of 10 patients with BI, 6 boys and 4 girls, aged 7-12 years, mean age 10,4 ± 2,36 years. All patients received cogitum in dose of 250- 500 mg daily during 8 weeks. Neurological and neuropsychological (Bourdon's test, Luria's tests) examinations, EEG, MRI were performed before and after treatment. RESULTS AND CONCLUSION: The study of cognitive functions showed a decrease in the accuracy and speed during performance of Bourdon's and Luria's tests. After the beginning of treatment with cogitum, 80% of the patients in both groups demonstrated a significant improvement in the accuracy of Bourdon's test (р<0,01) and verbal retention (р<0,001). Repeated EEG (3-6 months after treatment) showed the distinct theta- , alpha-, beta- rhythms and clear zonal differences. Given a spectrum of clinical effects of cogitum, the authors recommend it for active use in pediatric practice.

[Cortical dysgenesis with epileptic syndromes and symptomatic epilepsy in children].

Cortical dysgenesis (CD) is a frequent inherited brain malformation. CD is a key cause of epileptic syndrome in children. In this review, the author presents a current classification of CD, etiological factors of their development, including gene mutations and adverse effects of various toxins and perinatal factors. A spectrum of clinical symptoms of CD with epileptic seizures is discussed in details. A role of current MRI-regimes in the diagnosis, the management of patients and prognosis of the course of CD are highlighted.

[Optimization of treatment focal forms of epilepsy in young children].

A clinical trial included 30 children, aged from 24 to 46 months, stratified into two groups on the basis of clinical-instrumental data: 22 (73%) patients with symptomatic focal epilepsy and 8 (27%) patients with cryptogenic (possibly symptomatic) focal epilepsy. Topamax (topiramate) was used as mono- (8 children) and combined (22 children) therapy. Mean treatment dose was 5,9 mg/kg per day. Efficacy of treatment was evaluated by the changes in frequency of seizures. After 6 months, the positive result (completed stopping of seizures or their reduction by 50% and more) was seen in 19 (63,75%) patients. The effect was absent or was minimal in 9 (29,5%). In 2 (6,75%) cases the frequency of seizures increased. Adverse events were recorded in 11 (36%) patients, side-effects that led to the treatment discontinuation (vomiting, increasing of seizure frequency, enuresis) were found in 5 (16%) patients.

[Macro- and microscopic systematization of cerebral cortex malformations in children].

For the first time in pediatric pathologicoanatomic practice the complete systematization of cerebral cortex malformations is represented. Organ, macroscopic forms: microencephaly, macroencephaly, micropolygyria, pachygyria, schizencephaly, porencephaly, lissencephaly. Histic microdysgenesis of cortex: type I includes isolated abnormalities such as radial (IA) and tangential (I B) subtypes of cortical dislamination; type II includes sublocal cortical dislamination with immature dysmorphic neurons (II A) and balloon cells (II B); type III are the combination focal cortical dysplasia with tuberous sclerosis of the hippocampus (III A), tumors (III B) and malformations of vessels, traumatic and hypoxic disorders (III C). Band heterotopias. Subependimal nodular heterotopias. Tuberous sclerosis. Cellular typification of cortical dysplasia: immature neurons and balloon cells.