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A new classification of limb‑girdle muscular dystrophy, which was adopted in 2018, is given. The reasons why there was a need to revise the classification are indicated. A new definition of this pathology is given. The diseases that were excluded from the new classification of limb‑girdle muscular dystrophy due to inconsistency with the new definition of this form of pathology are given. A comparison of the new and old classification of this group of muscular dystrophies is given. The genes responsible for the development of various forms of this pathology are indicated, as well as proteins of striated musculature, the synthesis of which is disrupted. It has been shown that a mutation in one gene can lead to different types of inheritance of limb‑girdle muscular dystrophy, which is important to take into account when conducting prenatal diagnostics. The new classification will allow introducing new forms of this pathology as new mutations in disease genes are detected.

Background: Growing glioblastoma is associated with an impairment of the blood brain barrier and increased hemodynamic parameters (CBV, CBF), which is related to advanced tumor angiogenesis. Arterial spin labeling (ASL) perfusion, an additional study to the routine intravenous contrast-enhanced magnetic resonance imaging (MRI), may be a technique for assessment of hemodynamics and identification of the residual tumor tissue.Aim: To study the potential of native ASL to assess hemodynamic parameters and detect residual tumor tissue in the patients who had undergone surgical resection of glioblastoma.Materials and methods: At 2 to 4 weeks after surgery for glioblastoma grade IV, brain MRI with native ASL perfusion and subsequent intravenous contrast enhancement was performed in 63 patients. Cerebral blood flow (CBF) values were measured in three areas of interest: in the presumptive tumor tissue (PTT) with maximal perfusion, in the postoperative scar tissue (PST) and in the deep white matter (DWM) of the contralateral hemisphere.Results: According to their CBF values, all patients were categorized into two groups. Group 1 included 43 patients (68.3%) with mean CBF in PTT of 135.4±41.3 ml/100 Gr/min (min 73.9, max 255.9). These values were 5 to 6-fold higher than the CBF values in the PST and 6 to 8-fold higher than those in DWM. Group 2 included 20 patients (31.7%) without any areas of abnormal CBF increase in PST, with its mean value of 22.1±5.6 ml/100 Gr/min (min 13.9, max 37.1), which was close to the CBF level in DWM (19.8±4.6 ml/100 Gr/min, p=0.06). There were no between-group differences for the CBF values in PST (p=0.6), and DWM (p=0.7).Conclusion: ASL MR perfusion technique has good potential for the identification of residual tumor tissue after surgical resection of glioblastoma and can be an alternative to contrast enhancement during long-term follow up.

Guillain–Barré syndrome – an acute demyelinating autoimmune disease characterized by lesions of the peripheral nervous system and consequently peripheral paralysis, paresthesias and/or pain. Guillain–Barré syndrome is a predominant cause of acute flaccid paralysis, which may occur at any age. We present two clinical cases of flaccid paralysis with patients at the age of 7 and 5 years requiring verification of the diagnosis.

Objective. To evaluate the efficiency of ASL-perfusion as a method of estimating of hemodynamics and detection of residual tumor tissue after surgical treatment of glioblastoma.Material and methods. 56 patients after brain tumor’s surgical resection of glioblastoma (GRADE IV). CBF values were determined in 3 different areas - in the presumed tumor tissue with maximum perfusion, in the postoperative scar tissue and in the deep white matter of the opposite hemisphere. All patients were divided into 2 groups according to CBF value.Results. 1st group: 38 (67.9%) patients - the average CBF in suspected tumor was 137.6±35.2 (79.6-227.6) ml/100 g/min. It was 6-8 times higher than CBF in the deep white matter of the opposite hemisphere, and 5-6 times higher than in the postoperative scars.2nd group: 18 (32.1%) patients with no pathological elevation of CBF in postoperative scar tissue. CBF there was 22.3±5.9 (13.9-37.1) ml/100 g/min. CBF in white matter in the contralateral hemisphere was similar.There was no significant differences in CBF of scar tissue (p=0,52) and in white matter of contralateral hemisphere (p=0,96) in both groups.Conclusion. The possibilities of ASL-perfusion are enough to estimating of hemodynamics and detection of residual tumor tissue after surgical removed glioblastoma.

Neuromyelitis optica spectrum disorders (NMOSDs) is an inflammatory demyelinating disease of the central nervous system with a selective injury of the optic nerves and spinal cord. The formation of neuromyelitis optica ((NMO)-IgG autoantibodies against the cell membrane water channel protein aquaporin 4 (AQP4) underlies the pathogenesis of the disease. The article describes the clinical presentation of the disease in pediatric patients followed up by the authors. It presents the criteria for diagnosing NMOSDs and their therapy regimens.

Neuromyelitis optica spectrum disorder is an inflammatory demyelinating disease of the central nervous system with selective damage to the optic nerves and spinal cord. The clinical case of a child 11 years old and its course throughout the year, with an onset in the form of an episode of visual impairment with signs of a previously developed partial atrophy of the optic nerves of both eyes and a manifestation of retrobulbar neuritis on the left eye is considered in the article. Repeatedly conducted magnetic resonance examination revealed a pattern of focal lesion of the left and then of the right optic nerve with a violation of the integrity of the blood brain barrier and an extended focus in the structure of the spinal cord of the cervical spine. The blood test for antibody to aquaporin-4 was positive. Optical coherence tomography revealed decrease in the thickness of the peripapillary fibers of the retina in all quadrants in both eyes, which indicates a partial atrophy of the optic nerves of both eyes, as a result of repeatedly transferred retrobulbar neuritis in both eyes.

We describe clinical case of Rasmussen's encephalitis in a 24-year-old woman that occurred during her hospital stay. Its clinical manifestations included a clonic movement disorder and partial epilepsy. Brain magnetic resonance imaging study showed shrinkage of her left hemisphere with signs of inflammation and scarring. An electroencephalography showed the electrophysiological characteristics of epilepsy and slowing down of the left hemisphere activity. Brain biopsy was not provided.

In the last years there has been a tendency towards a shift of the onset of multiple sclerosis to childhood and adolescence. According to various estimates, its incidence at the age below 18 is in the range from 2 to 10%. The most characteristic for such patients is a relapsing remitting course with a multi symptom onset. We present a "classic" clinical case of a female patient with an aggressive course of multiple sclerosis with the onset in the adolescence, discuss its assessment and management.

We present a clinical observation of a pathological fracture of the spine in a patient with ankylosing spondylitis (Bechterew's disease). The results of a comprehensive radiological assessment (X-ray, multidetector computed tomography, magnetic resonance imaging), which enabled the differential diagnosis with a tumor and inflammatory processes and to determine a management strategy.

The article is devoted to the role of increase of junior schoolchildren’s cognitive activity as an important condition for the development of cognitive abilities of learners. Psycho-pedagogical support should be focused on the development of cognitive functions of junior schoolchildren, and psychologists and parents should be involved in this process, as well as the teachers. Traditionally, psycho-pedagogical support at primary school focuses on the child's adaptation to the new educational environment. We consider that a child needs the same support in the process of mastering the curriculum. There is no true support without an emotional involvement and empathy, and as a result cognitive activity of a child might suffer greatly. It was proven by empirical results obtained in an ordinary school, where no innovative teaching methods are used. At the end of primary school pupils have a significantly reduced motivation, teachers express no serious interest in problems of junior high school students. Not entirely satisfactory educational environment also could not provide a positive influence on the development of cognitive sphere.