Антенатальный период - короткий, но один из самых важных в жизни индивидума. Именно он является первым этапом существования человека, определяющим благополучие в последующем, которое зависит от экологической и социальной ситуации, состояния здоровья матери к моменту зачатия и на протяжении всей гестации. При их нарушениях развивающаяся ишемия плаценты сопровождается различными патогенетическими процессами, стимулирующими состояние хронического воспаления, активацию иммунных клеток, апоптоз трофобластов, присутствующих в децидуальной оболочке. Чрезмерная активация этих механизмов приводит к аномальной плацентации с нарушением инвазии, ремоделирования сосудов и микроциркуляции плаценты, что приводит к одному из самых сильных стрессов, лежащих в основе гипоксически-ишемического поражения головного мозга у плода и новорожденного. The antenatal period is short, but one of the most important in the life of an individual. It is he who is the first stage of human existence, determining well-being in the future, which depends on the ecological and social situation, the state of health of the mother at the time of conception and throughout gestation. With their violations, the developing ischemia of the placenta is accompanied by various pathogenetic processes that stimulate the state of chronic inflammation, activation of immune cells, apoptosis of trophoblasts present in the decidua. Excessive activation of these mechanisms leads to abnormal placentation with impaired invasion, vascular remodeling and microcirculation of the placenta, which leads to one of the most severe stresses underlying hypoxic-ischemic brain damage in the fetus and newborn.

A clinical case of congenital hyperinsulinism, diffuse form, pharmacoresistant course (heterozygous mutation of p. 1361 1363 dup CGG in the GCK gene) in an early child is presented as an example of an orphan severe disease with an extremely unfavorable course and a probability of deterioration of the long-term prognosis. The goal was to highlight the clinical manifestations, course options, and complexity of treatment of this pathology to a wide range of doctors of different specialties in the field of Pediatrics in terms of improving the quality and timeliness of diagnosis, reducing the number of complications with the formation of irreparable consequences. Attention is drawn to the most severe course of hypoglycemic conditions in the early neonatal period, the dependence of the formation of a pronounced neurological deficit on the degree and duration of hypoglycemia, which emphasizes the importance of their timely correction to preserve the quality of life of this contingent of children.

Objective: the study is to determine the effectiveness of EEG-biofeedback training on β-rhythm for correcting voluntary attention in children 6 – 8 years of age with the consequences of perinatal CNS lesions of hypoxic-ischemic genesis. Materials and methods: a total of 120 children aged 6 – 8 years who included 30 children with cerebrastenic syndrome (I group) were examined; 58 children with hyperactivity and attention deficit syndrome (II group); 32 children with cerebral palsy, spastic diplegia (III group). EEG-biofeedback training on β-rhythm correction was carried out in an amount of 15 sessions lasting 20 – 30 minutes on the Kinesis machine (Neurot, Russia). Before and after the biofeedback therapy, a psychodiagnostic evaluation of the functions of voluntary attention on the Toulouse-Pieron scale was carried out (in the adaptation of LA Yasyukova, 2001). EEG recording was performed on an EEG 21/26 encephalograph Encephalan-131-03, modification 10 (Manufacturer: NPKF Medikom MTD, Russia). For statistical analysis of the obtained indicators before and after treatment, the significance criterion was used to compare the two proportions (fractions) under the assumption that the variables are distributed according to the normal law (package STATISTICS 6.0). Results: the effectiveness of EEG-biofeedback training on β-rhythm correction was revealed, as evidenced by a significant decrease in the number of children with isolated types of attention disorders in all groups of patients as well as with mixed disorders in the II group. Conclusion: аctivation of cortical processes, increasing the level of attention, memory and cognitive abilities of the psyche, when EEG-biofeedback training is included in the therapy complex, depend on the nature and severity of central nervous system lesions in children with various nosological forms of neurological pathology, which requires rethinking of therapeutic complex methods of correction and selection of personalized biofeedback training protocols, taking into account data on various types of metabolic and neurophysiological abnormalities in the head in children with different nosological forms of neurological disorders.

Glutaric aciduria type I (deficiency of glutaryl-COA dehydrogenase, glutaric acidemia type I) is a rare autosomal recessive disease caused by mutations in the gene encoding the enzyme glutaryl – COA - dehydrogenase (GCDH). Cerebral organic aciduria, caused by a deficiency of glutaryl-COA - dehydrogenase, is generally considered a neurological disorder.The phenotypic spectrum of untreated GA-1 varies from a more common and pronounced form (a disease with infancy) to a low-symptom and less common form. In people with the same genotype, the clinical manifestations and depth of CNS damage can vary widely depending on the age of manifestation of acute encephalopathic crises. It is assumed that with early detection and treatment of “asymptomatic” newborns (in the context of screening for this disease), most people who would have developed manifestations of GA-1 with childhood or late onset will remain asymptomatic.