[Epileptic encephalopathy with continuous spikes and waves during sleep (CSWS): a review]. / Épilepticheskaia éntsefalopatiia s prodolzhennoi spaik-vol-no-voi aktivnost'iu vo sne: obzor literatury.

Epileptic encephalopathy (EE) with continuous spikes and waves during sleep (CSWS) (epilepsy with electric epileptic status during slow sleep phase or encephalopathy with electric epileptic status during slow sleep phase) is partially reversible age-dependent epileptic encephalopathy. It is characterized by a triad of symptoms: seizures, neurocognitive regression, and an electroencephalography pattern of electrical status epilepticus during sleep. This rare condition occurs in 0,5% of children and adolescents with epilepsy. Seizures usually start when the child is 2-4 years old. Several stages in CSWS are identified: dormant stage, prodromal stage, acute stage, and residual stage. EE epileptiform activity may be focal, multifocal, unilateral, asymmetric or symmetric bilateral, and diffusive. Treatment goals of CSWS include not only improved seizure control, but also a significant reduction in EEG epileptiform activity. Benzodiazepines and steroids are most effective.

[Focal characteristics in the clinical examination and EEG in children with idiopathic generalized epilepsy].

The aim of the study was to determine the prevalence of focal characteristics in the clinical symptoms of seizures and in EEG in children with different clinical variants of generalized idiopathic epilepsy. We studied 71 patients, 29 boys and 42 girls, aged from 2 to 18 years. Video-EEG-monitoring and MRI were performed in all cases. The results provided further evidence fort the continuum between focal and generalized epilepsy supported by the presence of focal characteristics in the semiology of seizures and in EEG as well.

[Transitory cognitive dysfunction in rolandic epilepsy].

Forty-four patients with rolandic epilepsy (32 boys, 12 girls), aged from 5 to 14 years, were examined in the prospective study during 5 years. Before the antiepileptic treatment, most of patients had transitory cognitive disturbances. There were the impairment of verbal functions, especially verbal intellect, while non-verbal intellect remained intact; dyspraxia, impairment of auditory-speech memory, disturbances of arbitrary regulation and optical-motor coordination. The cognitive impairment was not severe and did not impact on learning of school program. No significant correlations were found between the lateralization of regional EEG changes and the character of cognitive dysfunction though the age-related lateralization of the focal epileptiform activity was shown: the right-side localization of central-temporal EEG spikes predominated in children at the age of 6.29 +/- 0.9 years, the left-side localization - in children at the age of 8.4 +/- 1.4 years. The clinical remission was achieved 4-5 years earlier than the recovery of cognitive functions. Valproates used as monotherapy or in the combination with ethosuximidum and levetiracetam were drugs of choice.

[Epilepsies with electric status epilepticus in sleep: peculiarities of clinical course and rational approaches to treatment].

We studied 52 patients with electric status epilepticus in slow sleep (EESSS) during 3-5 years. Age-dependent peculiarities of clinical course of the disease, risk factors for EESSS and rational approaches to antiepileptic treatment for these cases were singled out. Symptomatic and idiopathic EESSS variants were revealed. Combinations of valproates, levetiracetam and ethosuximidum were the most effective antiepileptic drugs in the treatment of EESSS.

[Idiopathic focal epilepsies of infancy and childhood].

We studied 1036 children with epileptic seizures, aged from 1 to 18 years, during 2004-2008. One hundred and six patients were diagnosed with idiopathic focal epilepsy (IFE). The following forms of IFE were singled out: benign seizures of infancy (familial and non-familial) - Watanabe--Vigevano syndrome - 5,7%, occipital epilepsy of childhood with early manifestation (Panayiotopoulos syndrome) -26,4%, occipital epilepsy of childhood with late manifestation (Gastaut syndrome) - 12,3%, benign epilepsy of childhood with central-temporal spikes (rolandic epilepsy) - 51%, benign focal epilepsy with affective symptoms - 4,7%. The efficacy of the first monotherapy was significantly worse in rolandic epilepsy compared to the other IFE forms. Prescription of valproate or the combination of valproate, ethosuximidum and levetiracetam, in case of resistant course, as a starting therapy was found optimal.

[Efficacy of carbamazepine, valproate and topiramate in the treatment of medial temporal epilepsy in children].

For a retrospective observational investigation based on real clinical practice of relative efficacy of valpoic acid (VPA), carbamazepine (CBZ) and topiramate (TPM) we have selected 205 patient with age of seizure onset before 16 years with a undoubted diagnosis of medial temporal lobe epilepsy, who had received treatment according to ILAE recommendations, and observation time since the last treatment change was from 2 to 5 years. The groups of patient receiving CBZ, VPA small i, Cyrillic TPM did not differ significantly in presenting unfavorable prognostic factors and dose regimes that allowed to conduct direct comparison of efficacy of the investigated drugs. Efficacy of VPA in children with medial temporal lobe epilepsy was higher compared with CBZ (79% vs 61%; p< or =0,05) and TPM (79% vs 53%; p< or =0.001). CBZ caused seizure aggravation more frequently than VPA (10% vs 1%; p< or =0,001). In case of presence of clinico-electroencephalografic signs of significant organic brain damage and in patient with seizure onset before age of 1 year CBZ was not effective while TPM showed efficacy of 20%, (p< or =0,05) and VPA was the most effective drug in this case (50%; p< or =0,001). In case of focal cortical dysphasia or the states after periventricular leucomalacia the efficacy of CBZ was lower than VPA (0% for CBZ vs 89% for VPA - p< or =0,01 and 40% for CBZ vs 77% for VPA - p< or =0,05, respectively) and TPM (0% for CBZ vs 100% for TPM - p< or =0,01 and 40% for CBZ vs 100% for TPM - p< or =0,01, respectively). In MRI-negative cases VPA was most effective (90% vs 53% for CBZ; p< or =0,001 and 67% for TPM; p< or =0,05). Efficacy CBZ reduces proportionally the number of previously used antiepileptic drugs (AEDs) (52% as a first AED vs 17% as a second AED; p< or =0,01), this tendency is noted also for TPM but in less extend (80% vs 46%, respectively p< or =0,05), but not for VPA (77% vs 75%; p>0,05, respectively). Adverse effects were more frequent during treatment with CBZ, than VPA (19% vs 5%; p< or =0,001) and TPM (19% vs 9%; p< or =0,05).

[Comparative efficacy of carbamazepine, valproic acid and topiramate in symptomatic and cryptogenic frontal lobe epilepsy in children].

For a retrospective observational investigation based on real clinical practice of relative efficacy of valpoic acid (VPA), carbamazepine (CBZ) and topiramate (TPM) we have selected 277 patients with seizure onset before 17 years with a undoubted diagnosis of symptomatic or cryptogenic frontal lobe epilepsy (FLE), who had received treatment according to ILAE recommendations, and observation time since the last treatment change was from 2 to 10 years. Patients suspicious for idiopathic epilepsies were excluded. The groups of patient receiving CBZ, VPA and TPM did not differ significantly in presenting unfavorable prognostic factors that allowed conducting direct comparison of efficacy of the investigated drugs. Efficacy of VPA in children with FLE was higher compared with CBZ (56% vs 22%, p<0,01) and TPM (56% vs 10%, p<0,001). CBZ and TPM caused seizure aggravation rather frequently, but no aggravation was noted while VPA treatment (14% and 17% respectively vs 0%, p<0,001). In case of presence of clinico-electroencephalografic and MRI signs of significant organic brain damage and seizure onset after 1 year of age VPA was most effective and TPM showed minimal effect. TPM was ineffective in case of focal cortical dysphasia and cerebral atrophy; in other lesions its efficacy was comparable with CBZ. In MRI-negative cases VPA was most effective (71% vs 24% for CBZ, p<0,001 and 20% for TPM, p<0,001). Efficacy of VPA, CBZ and TPM does not change with the number of previously used antiepileptic drugs (AEDs). VPA was also most effective as a first AED (63% vs 26%, for CBZ, p<0,001 and 13%, p<0,001 for TPM), as well as a second AED (50% vs 30% for CBZ and 7% for TPM, p<0,05). Adverse effects were more frequent during treatment with CBZ and TPM, than VPA (20% vs 6%, p<0,001 and 31% vs 6%, p<0,05, respectively).

[Epilepsy with prolonged epileptiform activity during sleep in children and adolescents].

Benign epileptic discharges of childhood (BEDC) are typical age-related EEG patterns associated with idiopathic benign focal epilepsy (BFE). The study of BFE revealed the symptomatic phenocopies in patients with structural brain lesions in infantile cerebral paralysis and malformations. The authors discuss the question of "benignity" of BEDC that may lead to various disturbances of cognitive functions and behavior, i.e. to signs of epileptic encephalopathy. Based on the examination of 1862 children, including 840 patients with epileptic seizures and 1022 neurologic patients, clinical and neurophysiological features of epileptic syndromes associated with prolonged epileptiform EEG activity in children were found. The most rational antiepileptic therapy was determined.

[Comparative efficacy of carbamazepine, valproic acid and topiramate in symptomatic and cryptogenic occipital lobe epilepsy in children].

For a retrospective observational investigation based on real clinical practice of relative efficacy of valpoic acid (VPA), carbamazepine (CBZ) and topiramate (TPM) we have selected 106 patients with age of seizure onset before 17 years with a undoubted diagnosis of symptomatic or cryptogenic occipital lobe epilepsy (OLE), who had received treatment according to ILAE recommendations, and observation time since the last treatment change was from 2 to 10 years. Patients suspicious for idiopathic epilepsies were excluded. The groups of patient receiving CBZ, VPA and TPM did not differ significantly in presenting unfavorable prognostic factors and dose regimes that allowed to conduct direct comparison of efficacy of the investigated drugs. Efficacy of VPA in children with OLE was higher compared with CBZ (69% vs 36%, p < 0.01) and TPM (69% vs 8%, p < 0.001). CBZ and TPM caused seizure aggravation more frequently than VPA (12% and 13% respectively vs 1%, p < 0.001). In case of presence of clinico-electroencephalografic and MRI signs of significant organic brain damage and in patients with seizure onset under 11 years TPM was not effective. In case of focal cortical dysphasia the efficacy of CBZ was lower than VPA (20% vs 63%, p < 0.05). In MRI-negative cases VPA was most effective (79% vs 44% for CBZ, p < 0.001 and 29% for TPM, p < 0.01). Efficacy of CBZ and TPM reduces proportionally the number of previously used antiepileptic drugs (AEDs), this tendency is noted also for VPA but as a second AED it was more effective than CBZ and TPM (56% vs 15%, p < 0.01 and 14%, p < 0.05, respectively); as a first AED VPA was also most effective (82% vs 37%, p < 0.001 for CBZ and 82% vs 33%, p < 0.01 for TPM). Adverse effects were more frequent during treatment with CBZ and TPM, than VPA (21% vs 6%, p < 0.001 and 17% vs 6%, p < 0.05).

[The efficacy of topiramate (topamax) in the treatment of resistant epilepsy in children].

Authors presented the data on the efficacy and tolerability of the new anticonvulsant drug topiramate (topamax) in the treatment of 110 children aged from 6 months to 16 years with pharmacoresistant types of epilepsy including 42 patients with tuberous sclerosis. The medication dosage varied from 1 to 20 mg/kg daily. As a monotherapy the drug was used in 28 children, in the other cases it was combined with different anticonvulsant medications. Topiramate was effective in 78% patients; complete remission has been achieved in 35% cases. Side-effects were observed in 17% patients but they were transient and did not result in the drug withdrawal. The results of the study allowed to recommend the drug as an add-on and monotherapy in the treatment of resistant epilepsy.

[Chromosome instability in multiple sclerosis]. / Nestabil'nost' khromosom pri rasseiannom skleroze.

The results of the study have confirmed the existing data on an elevation in the spontaneous levels of sister chromatid exchange (SCE) in multiple sclerosis patients as compared to control subjects (in the patients the mean number of SCEs per cell was 10.5 +/- 0.3 whereas in the control group it was 8.2 +/- 0.25).