The article is devoted to the treatment and prediction of outcomes of West syndrome. The analysis of literary and own data was carried out. Was estimated the effectiveness of treatment and predicted the outcomes of the disease by data of anamnesis, seizures and the possibilities of therapy. The article presents a stepwise scheme for the treatment of West syndrome and evaluates the results of the application of various drugs, including valproic acid, vigabatrin*, levetiracetam, phenobarbital, clonazepam and topiramate. Special attention is paid to the role of hormone therapy, demonstrated its high efficiency. The most significant prognostic criteria for the outcome of this disease are identified. These criteria are based on literature and our own data. Predictors of an adverse outcome of West syndrome include: symptomatic forms of disease (structural, genetic, infectious), impaired neuropsychological development before onset of epileptic spasms, age of spasms onset more than 8 months, the presence of other seizures types in addition to epileptic spasms, the need for polytherapy of antiepileptic drugs. The pathology of pregnancy, the period of adaptation, neonatal seizures, starting therapy and the response to it are not always important in predicting remission.

Background. Comparative study of antiepileptic drugs allows to make the optimal choice in therapy.

The aim of the study: a comparative analysis of the administration of two similar by chemical structure antiepileptic drugs (zonisamide and topiramate) in the outpatient practice in epilepsy in children, taking into account the efficacy and tolerability.

Materials and methods. A retrospective analysis of outpatient charts of equal in number (n = 18) groups of patients with different forms of epilepsy treated with zonisamide and topiramate in monotherapy and in combined therapy was carried out.

Results. In general, no statistically significant difference in the efficacy of zonisamide and topiramate was obtained (p = 0.692). Efficacy in structural focal epilepsy was also comparable (absence of seizures 54.6 % and 45.5 %, respectively). With comparable efficacy, zonisamide was administered to patients much later in the antiepileptic drugs (Me = 5) than topiramate (Me = 3). Side effects on zonisamide were registered in 27.8 %, side effects on topiramate – in 38.9 % (p = 0.480). The difference in the effect of these drugs on cognitive functions was noted.

Conclusions. Zonisamide shows its effectiveness in different forms of epilepsy in children, despite the later appointment in practice. The drug is well tolerated and probably has less negative effect on children’s cognitive functions than topiramate.

This lecture with a description of clinical cases presents information on such a mitochondrial disease from the group of hereditary metabolic diseases, like MELAS syndrome (OMIM: 540000). The main manifestations of this progressive disease are stroke-like episodes and a specific form of encephalopathy, including epileptic seizures with the presence of a phenomenon of "ragged red fibers" and early dementia. Clinical cases, given in this lecture, supplement the piggy bank of genetically verified mitochondrial diseases leading to the development of polymorphic neurological disorders and characteristic neuroimaging picture, namely the appearance of polymorphic ischemic changes in the temporal, parietal or occipital regions of the brain, that do not correspond to the zones of the main vascular blood supply, as the basis for the formation of such focus is the phenomenon of mitochondrial angiopathy, and not thrombosis. The description of clinical cases reflects our own observations and the main steps in the diagnosis of this severe hereditary disease, taking into account of the latest neurogenetic representations. In addition to the data given in the clinical course of the disease, the results of the genetic interpretation of the MELAS syndrome are given; modern methods of diagnosis and therapy are considered.

Kleefstra syndrome is a rare autosomal dominant genetic disorder caused by haploinsufficiency of the EHMT1 (Euchromatic Histone MethylTransferase 1). Patients with Kleefstra syndrome have following most common symptoms: moderate or severe intellectual deficiency, absence of speech, significant diffuse muscular hypotonia, micro-brachycephaly, congenital defects of heart, kidneys, genitourinary tract and recognizable dysmorphic features of face. The article presents 2 similar clinical cases of Kleefstra syndrome in combination with epilepsy. Both patients, along with a typical clinical picture of the underlying disease, have serial epileptic spasms with an onset after first year of life, modified hypsarrhythmia with tendency to synchronization on the electroencephalogram, pharmacoresistant epilepsy. This indicates that Kleefstra syndrome can include epilepsy as one of symptoms of the disease.

Fetal alcohol syndrome is the result of prenatal effect of alcohol on the fetus and manifests as disorders of neural and mental development and various congenital developmental disorders leading to comorbid diseases. Among congenital developmental disorders for the pathology, adenotonsillar pathology was described. Narrowing of the nasopharyngeal opening, faciomaxillary dysmorphias and muscle hypotony can lead to sleep apnea syndrome. The article presents a clinical case of fetal alcohol syndrome and sleep apnea in a 7‑year-old girl with multiple developmental disorders born form alcohol-abusing mother. Breathing abnormalities during sleep must be differentiated per character and cause. Cardiorespiratory monitoring is used to diagnose respiratory abnormalities, the results determine patient management tactics. Interdisciplinary approach to observation and treatment of a child with fetal alcohol syndrome is necessary to increase the effectiveness of rehabilitation measures and patient’s quality of life.

Epilepsy is a common disease of the nervous system that requires long-term (often lifelong) use of drugs that allow many people with this diagnosis to lead an active social life with no risk of disability or life-threatening situations. Despite the existing normative acts regulating the provision of free medicines to epilepsy patients, there are still many problems associated with the compliance with the current legislation (Constitution of the Russian Federation) and the availability of free medicines for patients with epilepsy. In this article, we tried to cover the main norms of the current legislation regulating the issues of providing patients with epilepsy with free medicines; this can be helpful for both physicians and patients in overcoming bureaucratic barriers when confronting medical officials in defending the rights of patients.