Objective. To evaluate morphological changes of placenta in 21 dead preterm babies with extremely low body weight. Materials and Methods. We evaluated the grade of immaturity of the placenta, inflammatory changes of placenta and extraplacental membranes in premature babies who died because of intraventricular hemorrhage (IVH) 2-3 grades and without sonographic and pathomorphological signs of IVH. Results. We identified the signs of ascending amniotic infections in 13 (100%) children with IVH and only in 4 (50%) babies without IVH. We also found that placenta damages in babies with IVH were associated with bacterial and mixed viral-bacterial infections, and the viral agent was represented mainly by the herpetic group. Only viral pathogens (mainly of the herpetic group) were identified as etiological factors of ascending infection in children without IVH. The role of compensatory changes of the placenta observed in children without signs of IVH in prolongation of pregnancy was noted. Conclusions. The presence of viral-bacterial lesions of placenta has a more pronounced impact on premature delivery, which increases the risk of complications in premature newborns in the form of damage to the central nervous system – intraventricular hemorrhages.

Toxic damages in fetuses and newborns rank highly in perinatal pathology structure, though their true importance is not understood thoroughly. Among toxic encephalopathies of newborns the accent should be given to the conditions caused by administration of medicines by a pregnant woman. The use of medicines, especially neurotropic, during pregnancy is often forced. The widespread prevalence of epilepsy in women of reproductive age leads, in certain cases, to anticonvulsants administration during pregnancy. The dominance of valproic acid in epilepsy treatment in recent decades requires studying its teratogenic effect in newborns fetal valproate syndrome. The fetal valproate syndrome has casuistic nature and polymorphic phenomenology. The clinical case study is presented of a newborn with fetal valproate syndrome (born to a mother who took valproic acid in dose 14 mg/kg per day or 750 mg per day while pregnant, due to Janz syndrome), confirmed by valproic acid presence in the blood of a newborn (47.36 mol/l), with the main clinical manifestation of incomplete cleft palate (hard and soft) without other typical disturbances. Most anticonvulsants have a teratogenic effect, and may cause malformations and toxic encephalopathies in fetus and newborns. The similarity of clinical manifestations of intrauterine exposure to various anticonvulsants is associated with the presence of common metabolic links (epoxy and dihydrodiol derivatives of phenytoin, phenobarbital and carbamazepine). In spite of similarity of some teratogenic effect mechanisms, the anticonvulsants nature introduces specific clinical manifestations of these disorders.

Epileptic encephalopathy (EE) is a group of genetic monogenic diseases with leading feature of intractable epilepsy with onset at an early age and the development of neurocognitive deficit. Thanks to the development of molecular genetic diagnostic methods, more than 90 hereditary forms of EE have been identified, more of which have been discovered over the past decade. EE can be associated with impaired molecular function of neuron transporters (voltage-dependent and ligand-dependent transporters), metabolic disorders, and chromosomal diseases. Among monogenic EE, a group of diseases is distinguished, in which brain damage and the development of epilepsy are caused by hereditary disorders of mitochondrial functions. Given the wide variety of forms of mitochondrial dysfunctions, the absence of specific manifestations, different age of manifestation, the diagnosis of this group of diseases is not a routine process and requires DNA test (whole-exome/genome sequencing, gene panels). With the creation of new drugs that correct mitochondrial disorders, in-time diagnosis of mitochondrial dysfunctions, identification of a genetic disorder contributes to the in-time manage of pathogenetic treatment, the choice of an antiepileptic drug, which can reduce the risk of mortality and the degree of patient disability. We describe the case of early neonatal epilepsy in the structure of hereditary deficiency of coenzyme Q10. However, unfortunately, the late started specifical energotropic therapy and the severe course of the disease led to an early death. Hereditary defects in coenzyme Q are rare genetic disorders. In this regard, for the specialists leading the patient, the discovery of this particular defect was most likely an “unexpected” finding. Considering the complexity and duration of the whole exome study, the severity of the phenotype and the delay in energotropic therapy, the course of the disease in the child turned out to be extremely unfavorable. The presentation of the clinical case, in our opinion, will be important for practitioners who rarely encounter this type of pathology.

The differential diagnosis of paroxysmal conditions, as well as disorders of muscle tone (hypertension) in the neonatal period and in young children is quite complicated. Various states of the nervous system in newborns are transient and permanent, optimal and suboptimal, normal and pathological. Among them, we can mention non-epileptic paroxysmal states of early childhood. In some cases, non-epileptic paroxysmal states of early childhood is accompanied by motor disorders, manifested by an excessive increase in limb tone in newborns. This pathological condition of muscle tone in the English-language literature is referred to by the term stiffness baby (the syndrome of a rigid or fettered baby). Neonatal pathological muscle hypertonicity, unlike physiological hypertonicity of muscles of a newborn, is a rather rare condition. The article presents literature data and a description of the clinical observation of a patient with hyperekplexia. Hyperekplexia is a rare paroxysmal movement disorder in young children. The main clinical variants of the disease, methods of diagnosis and correction, the main mutations associated with this condition are considered. The article describes the own clinical observation of an early-age patient with hyperekplexia, its clinical picture, features of paroxysmal states and therapy, neuroimaging data, electroencephalographic phenomena recorded in the patient and genetic testing that confirmed the diagnosis of non-epileptic paroxysmal disorders. The child has a mutation in the ATAD1 gene associated with type 4 Hyperekplexia (618011).

This article investigates the value of early diagnosis and prognostic evaluation of Doppler ultrasound for preterm neonates very low birth weight in early neonatal life. The results showed that cerebral hemodynamic disturbance of intracranial carotid arterials would be associated with later brain impairments (intraventricular hemorrhage, lesions of periventricular white matter) detected by MRI.

The perinatal lesions of the nervous system in newborns include a number of diseases of the brain, spinal cord and peripheral nerves, united in a group by the time of exposure to damaging factors. The MRI is recognized as the most infor mative and specific method for the diagnosis of perinatal lesions of the central nervous system. By using the MRI, it is possible not only to identify structural changes in the brain, but also to qualitatively assess the myelination of cerebral structures in preterm infants. It was shown that in severe hypoxic-ischemic damage in preterm infants, dysmyelination is more often determined in the posterior pedicle of the inner capsule. DTI is a promising method to quantify the degree of myelination of the brain, visualize pathways, assess their structure and integrity. The aim of the study was to identify significant differences in diffusion values according to DTI in preterm infants with severe hypoxic-ischemic brain injures in the form of periventricular leucomalatia and peri-intraventricular hemorrhage. Methods: Group 1 (10 children) consisted of patients who did not have structural changes on MRI. Group 2 (8 children) consisted of children with periventricular leukomalacia. Group 3 (4 children) consisted of children with peri-intraventricular hemorrhages. The patients was underwent MRI, diffusion-tensor MRI. A comparison was made of the diffusion values in the posterior limb of inner capsules and the thalami between the groups. Results: there were revealed changes in diffusion values, indicating a delay of myelination at the level of the posterior legs of the inner capsules in children with severe hypoxic-ischemic brain injures.

The aim of the study was to compare the volumes according to MR morphometry and diffusion values according to diffusion-tensor MRI (DT-MRI) of the corpus callosum in children with spastic diplegia. Methods: the main group consisted of 12 children aged from 1 year 8 months to 4 years 3 months, the comparison group — 5 children with normal motor development, who did not have motor impairments, at the age from 1 year and 3 months up to 3 years 8 months. All patients underwent MR-morphometry, DTI. A comparison was made between the volumes and diffusion values of the corpus callosum between groups. Results: Significant differences were found (p<0,05) in the volume of the genu, splenium and the middle third of the trunk of the corpus callosum. Significant differences (p<0,05) of diffusion values in the middle and posterior third of the trunk of the corpus callosum were also revealed. The data obtained demonstrate the diffuse nature of pathological changes in the corpus callosum in children with spastic diplegia.

The results of autopsy material of preterm newborns very low birth weight (VLBW) and extremely low birth weight (ELBW) brains are presented. In life the brains lesions were detected in the examined newborns with cranial ultrasound and MRI (intraventricular herminal hemorrhages/IVH and hypoxic — ischemic encephalopathy /HIE). In this researching were been using method of microscopy. Postmortem examination confirmed brain cell migration continues after birth in all examined newborns. This study have detailed the directions, positions, thickness cells migration columns. There were found that cells migration thickness and length depend on gestation age of preterm newborn with IVH and HIE. Besides we found the age morphologic differences in cell structures cerebral cortex in preterm newborns.

Patterns of neuroplasticity and cerebral maturation in preterm neonate can be assessed by MRI and cranial ultrasound. The score system of brain maturation includes the account of germinal matrix (GM) regression by MRI. The GM regression can be considered as pattern of neuroplasticity. There have been investigated the changes of neuroplasticity pattern or GM regression in preterm neonates with extremely low birth weight (ELBW) without intragerminal/intraventricular hemorrhages ( n = 21). It is believe that the main causes of impair of GM are the intragerminal hemorrhages and hypoxia. The methods of study were cranial ultrasound (CU) and MRI. The measurement of GM was carried out by CU in anterior horn of the lateral ventricles of neonates in the study group (25-29 weeks). It was detected the GM regression in preterm neonates with increasing age, and complete GM regression to 30 week. MRI has been performed in 15 neonates from the study group on 27-38 weeks age with using the common pulse sequences – T1 WI, T2 WI and Flair. GM was detected by MRI up to 34 weeks inclusive by using the additional pulse sequence – DWI. By using common pulse sequences the GM was visualized up to 32 weeks age. Furthermore there has been pathological examination of GM in anterior horn of lateral ventricle in dead neonates from the study group ( n = 3). We revealed the thickness reduction of GM in the lateral ventricles with increasing age of the dead neonates. Also we identified the delay of the GM reduction in two dead neonates 36-38 weeks age (post conceptual age) what may indicate the disorder of neuroplasticity in those preterm neonates. The performed study showed the capability of CU and MRI in examination of neuroplasticity in preterm neonates.

Long-term neurological sequels in preterm babies in great cases are determinated by injury of immature cerebral white matter. There are several known forms of the white matter injure including noncystes forms. The noncystes forms of the white matter injury are general cerebral pathology in the preterm babies with low gestation age (born before 28 weeks). The main causes of the white matter damage related with extent of the cerebral myelination, the modification of cerebral immature cells, glial cells ability to prolong proliferations, migration and activity for exposed damaging factors as well.