Research on comparative efficаcy and tolerability of monotherapy with Depakine chronosphere, drugs of Carbamazepine group with extended release and oxcarbazepine in symptomatic and cryptogenic focal epilepsy has been conducted at Svt. Luka’s Institute of Child Neurology and Epilepsy (ICNE) (Moscow). This retrospective study covers a random sample of patients treated in ICNE in the period from December 1, 2013 to September 1, 2014.  The study included 131 patients aged 1 to 18 years with symptomatic and cryptogenic focal epilepsy receiving treatment with one of the study drugs in monotherapy: group 1 – monotherapy with Depakine chronosphere (n = 56); group 2 – monotherapy with drugs of carbamazepine group with extended release (n = 55); group 3 – monotherapy with oxcarbazepine (trileptal) (n = 20). The obtained results allow us to conclude that the effectiveness of Depakin chronosphere, carbamazepine with extended release and oxcarbazepine in monotherapy of symptomatic and cryptogenic focal epilepsy was comparable (statistically significant differences in efficacy were not found). However, carbamazepine was awarded the highest frequency of seizures aggravation. Drugs showed approximately same tolerability (statistically significant differences in tolerability were not found). However, withdrawal of the drug due to side effects was the rarest in Depakine (3.5 %), and withdrawal due to intolerance was higher in carbamazepine and oxcarbazepine (5 and 10 % respectively). Depakinum and oxcarbazepine had the best results in the blocking of pathological activity on the electroencephalogram, whereas carbamazepine was clearly inferior to them. In this regard, complete clinical-electroencephalographic remission (lasting 12 months or more) was achieved under treatment of Depakine chromosphere in 21.5 % of cases, oxcarbazepi on therapy for 12 months was similar in all study drugs. Considering that the objective of epilepsy treatment is to achieve complete control over seizures, or at least substantially reduce and weaken them in the absence of significant side effects that disturb the patient’s quality of life, it can be concluded that the best treatment results were obtained in group of depakine chronosphere therapy.

Women with epilepsy are referred to the special risk group due to the development of side effects of antiepileptic drugs (АED). Women’s neuroendocrinal disorders can be caused by the disease itself-epilepsy, as well as by the undertaken therapy. We have carried out a retrospective research in order to assess the safety and the tolerance of different AED at young girls and women of reproductive age. Was analyzed the data base of patients of Svt. Luka’s Institute of Child Neurology and Epilepsy, comprising all patients, who have been monitored in the period between 2000 and 2014 inclusive at the age between 15–40 years (n = 301). The research included patients, with different diagnosed forms of focal or generalized epilepsy, who were taking AED both during mono and polytherapy. Were analyzed all cases of neuroendocrinal, especially reproductive disorders, including the considerable gain of weight, menstrual disorder, sterility at AED background. Also was analyzed the result of all registered pregnancies at women with epilepsy (at the background of the antiepileptic therapy, as well as without treatment during pregnancy). The retrospective data analysis has revealed 51 сase (17 % in the group under review) of expressed neuroendocrinal, reproductive and cosmetic side effects (including the menstrual disorder: dysmenorrhea, opsomenorrhea, amenorrhea, anovulatory cycles, sterility, unfavorable pregnancy outcomes, as well as cosmetic endocrinal side effects: obesity, hirsutism, hair loss. Most patients have got such combined side effects. Our research results show, that in most cases the pregnancy at women with epilepsy ends by birth of a healthy child, the pregnancy outcome depends on many factors, it also differs according to applied AED. Valproic acid drugs show the highest teratogenic risk. Also at the back ground of the therapy with valproic acid have been registered most cases of neuroendocrinal reproductive diseases at women, who had been included in our research. The second most frequent type of the teratogenic effect and reproductive diseases are AED of carbamazepine group. Most favorable results are shown, when new AED are applied (topiramate, levetiracetam, oxcarbazepine).

The epilepsy treatment is to be based on existing general principles and standards of therapy with differential approach to each patient. Besides peculiarities of treatment of different types of seizures and forms of epilepsy there are also differential approaches to special groups of patients. To one of such groups are referred to women of reproductive age. These patients are referred to special group of risk due to the development of certain side effects of antiepileptic drugs (АED). This article focuses in details on peculiarities of treatment of women of reproductive age with epilepsy with accent made on tolerability and safety of the antiepileptic therapy. It is necessary to take into consideration, that at women neuroendocrinal disorders can be caused both by the disease itself – epilepsy (in such case disorders depend on the starting age, form of epilepsy, focal localization, duration of disorder and other factors, referred to the disease), as well as by the undertaken therapy. The article
hereunder considers only issues, referred to the treatment, i. e. AED side effects and its input in the decrease of life quality of women with epilepsy. As women’s reproductive function starts forming long ago before childbearing age, it is necessary for this category to comprise not only women and adolescents, but girls as well. Notwithstanding the fact that so called benign forms of epilepsy pass before the pubescence period (idiopathic focal epilepsies, several forms of idiopathic generalized epilepsy), in many cases the epilepsy, which has started in childhood, continues in the adult age as well. In the same time there can be possible remote negative consequences of the antiepileptic therapy, which can show at a woman of a reproductive age. The data, given in the article, witnesses the need of the right AED selection at women of reproductive age, suffering from epilepsy. The AED should be selected not only depending on the form of the epilepsy and on the kind of seizures, but also provided its influence on the neuroen docrinal status of a woman, as well as on its influence on the reproductive system. With that it should be kept in mind that the reproductive function is very important not only from the point of view of woman’s health, but also from the point of view of her family and social status. If possible, women and men of reproductive age (including adolescents) should avoid AED with strong induction of the fermentative system of the liver (all barbiturates, hydantoins, carbamazepine), women should also avoid AED, containing valproic acid. In such cases the priority can be given to new AED, not affecting neuroendocrinal functions and the reproductive system.

With a few exceptions patients with tuberous sclerosis (TS) suffering from drug-resistant epilepsies have potentially epileptogenic lesions
within both hemispheres. Until one decade ago in general such a constellation was an xclusion criteria for considerations with respect to epilepsy surgery. However experience has shown that it is not so rare to find patients in whom over the ears seizures are generated from just one single focus and that these patients can be good candidates for epilepsy surgery. Almost revolutionary was the further evelopment: multi-step procedures in patients with bilateral epileptogenic lesions – with promising results in terms of postoperative seizure outcome. Also, with increasing experience, it becomes more and more possible to differentiate already non-invasively which lesions could be epileptogenic and which are rather not the source of the seizures. The most important achievement of epilepsy surgery in TS however is that in selected cases early surgical intervention is able to prevent severe mental retardations, which are often the main burden for families who have members with this peculiar disease.

Despite the significant progress made in epileptology, resistant epilepsies constitute approximately 30 % of all forms of epilepsy. An overview of recent literature is provided on the efficacy and safety of a new antiepileptic drug zonisamide (Zonegran) in the treatment of epilepsy with emphasis on monotherapy. The mode of action, pharmacokinetic characteristics, the effectiveness and tolerance of zonisamide in resistant focal epilepsy, as well as other types of seizures and forms of epilepsy are described. Indications for use of the drug are discussed. Also a wider potential for application of zonisamide are described: in the treatment of generalized seizures, in monotherapy as well as in children and adolescents. At present, Zonegran is registered for application in children from 6 years and older, with focal seizures, in additional therapy. The authors present their own observation of high efficacy of zonisamide in adjunctive therapy in a child of 9 years with cryptogenic focal epilepsy. Onset of epileptic seizures at the age of 5 months. Since the onset of the disease the child has received a lot of antiepileptic drugs in various combinations, with no significant effect. Zonisamide (Zonegran) was introduced in therapy 4 months ago in combination with the valproate acid drug (which the child received over the years), and since the release to the full dose epileptic seizures are absent up to present. It should be noted that there was not a single remission of four-month duration since the onset of the disease. Moreover, normalization of sleep has been noted. Tolerability of the therapy is good. No side effects were noted.

Angleman syndrome (АS) – is a chromosomal syndrome, which is manifested through atypical autism with feeble minding, epilepsy, outrage of the speech development, movement disorders, ataxia, as well as special (happy) behavior of patients, combined with outbursts of laugh. The disease is caused by the mutation of 15q11.2–13 maternal locus or by the gene of UBE3A ubiquitinated complex. Such genes regulate the functional activity of hippocampus neurons, of olfactory bulbs, of the parastriate cortex, of the tentorium. We demonstrate the atypical AS case, which clinical presentation developed after acute respiratory viral infection with febrile temperature. The disease started with episodes of acute ataxia, interrupting daily activities of the child. Step by step the speech development was regressing – several words have fallen out,
leaving the space for babbling sounds. Also appeared stereotypic movements of upper extremities (bending of arms in elbow joints, its retraction and joggling of hands), unmotivated laugh. Due to the nonrelevant starting presentation in the acute period following conditions were differentially diagnosed: 1) opsoclonus-myoclonus syndrome; 2) cerebral circulation diseases; 3) epilepsy with absences and atonic attacks; 4) paroxysmal dyskenisias and ataxias; 5) start of the neurodegenerative disease; 6) early childhood autism. Results of laboratory research allowed to exclude opsoclonus-myoclonus, the magnetic and resonance tomography and vessels research allowed to exclude the cerebrovascular pathology. Changes, revealed in the course of the videoelectroencephalographic monitoring, as well as anamnesis data (clinical symptoms after fever) allowed to narrow the diagnostic search; AS suspected. Provided the combination of ataxia with movement disorders, it was decided to carry out not molecular & genetic, but also micromatrix analysis, in order to exclude the channelopathy, as well as other genetic reasons. The method of polymerase chain reaction did not reveal changes, typical for AS. Anyway, the micromatrix analysis has revealed that the molecular karyotype has got spots with lost heterozygosis of locuses, containing genes, referred to the imprinting phenomena (UBE3A). In such a way, AS was confirmed.

The childhood epilepsy with occipital paroxysms and early onset or Panayiotopoulos syndrome is a benign focal epileptic syndrome,
a benign disease, developing at infants. The disease has been described in 1989 by S. Panayiotopoulos on the basis of results of own observations, who has called this syndrome “benign night childhood occipital lobe epilepsy”. In 1996 N. Fejerman et al. offered the new name of the syndrome – benign childhood occipital lobe epilepsy, with earlier onset (Panayiotopoulos type), versus the childhood occipital lobe epilepsy, starting later in childhood (Gastaut type). The Panayiotopoulos syndrome occurs only at children, develops at healthy children without neurological disorders, the pick of disease falls on 4–5 years. The morbidity of boys and girls is almost the same. In most cases of the syndrome seizures take place during the sleep, 2 / 3 of patients report seizures only during sleep. As a rule, seizures last for more than 5 min, in 40 % of cases it last for more than 30 min, what corresponds (according to the consciousness level) to focal or generalized epileptic status criteria. To typical clinical syndromes at Panayiotopoulos syndrome are referred the ictal vomiting, as well as different vegetative symptoms. So, theictal vomiting, which, generally, is not typical for epilepsy, takes place in about 80 % of cases at Panaytopululos syndrome. Authors describe their own observation of the Panayiotopoulos syndrome at 5 years old girl.