Fahr disease is a rare hereditary or sporadic neurological condition characterized by bilateral calcium deposition in the basal ganglia, dentate nuclei of cerebellum, and subcortical white matter. We can also distinguish Farh syndrome when its etiology is associated with the disorder of calcium metabolism, mitochondriopathies, cerebrum neoplasms, infections, inflammatory diseases of the nervous system, and injuries. The most common manifestations in patients with calcification of the basal ganglia of cerebrum are neurological and/or psychiatric disorders of varying severity. The clinical manifestation of the disease can occur at different ages, but mainly in young and middle-aged adults. However, some patients remain asymptomatic throughout their lives. The main clinical manifestations of the disease are extrapyramidal and movement disorders, emotional and cognitive impairments. At the same time, the correspondence of the form and severity of neurological conditions and the nature of calcification of the basal ganglia is rare. Currently, the treatment strategy for Fahr disease is based on symptomatic therapy and correction of etiological factors in Fahr syndrome. There is information about the reversibility of the calcification process and the complete restoration of mental functions in the early diagnosis and treatment of Fahr syndrome.

The peculiarity of this study was not only the study of the severity of subjectively felt psychopathological manifestations and the level of depression in patients with myasthenia, data on which are found in the literature, but the features of psychological adaptive-compensatory mechanisms were first considered. The psychological characteristics of the perception of time, the level of internality and the meaning of life orientations, substantive psychopathological manifestations are studied. The article discusses the features of psychological adaptive-compensatory mechanisms in patients with myasthenia through the prism of the severity of subjectively felt psychopathological manifestations and characteristics of time, the level of internality, and life-meaning orientations. The data of modern studies of similar parameters in patients with myasthenia are given.

The purpose of the study is the analysis of adaptive-compensatory psychological mechanisms in patients with myasthenia gravis, in comparison with individuals who do not have physical disorders.

Materials and methods. The study involved 61 people: patients with myasthenia gravis diagnosed on the basis of clinical manifestations and the results of instrumental and laboratory tests (study of the level of antibodies to AchR MuSk) (31 people) and people without physical abnormalities (30 people). All participants were examined by experimental psychological methods: symptomatic questionnaire “SCL-90”, “Level of subjective control”, test of lifemeaning orientations, A. Beck’s depression scale, questionnaire “Semantic time differential”.

Results. Patients with myasthenia gravis complain of unpleasant physical sensations, obsessive thoughts, sometimes note repeated actions and rituals. They are characterized by depressive symptoms, the presence of fears, alertness. Symptoms of the disease are assessed by them as causing great discomfort. Compared to the control group, they take less of their successes and failures, consider that their contribution to the development of family and production relations is small, these factors depend on external circumstances. In the subjective sense of the psychological characteristics of time, the present and future are defined as inactive, their course seems uneven, poorly structured, poorly predicted.

Conclusion. The data obtained establish target-centered zones of psychological correction in order to stabilize the adaptive-compensatory mechanisms of patients with myasthenia gravis — this is a work aimed at correcting the self-concept and goal setting, helping the individual to better adapt to the chronic psycho-traumatic effect of the disease.

The state of cognitive deficit in subjects with combination of attention deficit hyperactivity disorder and tic hyperkinesis is among the least studied issues. Identification of the cognitive development features in children with these disorders comorbidity will help to develop and build an adequate model of rehabilitation.

Purpose of the study. To study cognitive deficits in children with tic hyperkinesis (TH) in cases of comorbidity with the combined type of attention deficit disorder (ADHD).

Materials and methods. 404 children aged 6–10 years with a combined type of ADHD were divided into three subgroups according to the state of monoamine activity: 1st subgroup with a combination of hypofunction of dopaminergic and hyperfunction of noradrenergic systems — 120 people, 2nd subgroup with a combination of hyperfunction of noradrenergic system with a relative dopamine balance — 136 people, 3rd subgroup with indicators of monoamines within the reference values — 148 children. The control group comprised of 90 children (54 boys and 36 girls). To diagnose the level of intellectual development, Wechsler Intelligence Scale for Children was used. In each subgroup, with the known direction of monoamine activity, the Wexler test parameters were compared among ADHD groups, both with or without TG.

Results. Subtests “Picture Arrangement”, “Picture Completion” and “Block Design” turned out to be sensitive to concomitant combined type of ADHD and tics in children, reflecting, respectively, enumeration, the ability to logical thinking, visual attention and hand-eye coordination. In a case of dopamine system hypofunction in children with ADHD, the addition of tics leads to the smoothing of the biochemical imbalance by increasing the activity of dopamine and thus, results in a more effective realization of logical thinking ability. The level of visual attention in children with ADHD with the addition of TH also increases, probably due to a growth of the intensity of serotonin system activity. Finally, hand-eye coordination improves with the addition of tics in cases of a shift in the balance of dopamine and noradrenergic systems.

Conclusion. The data obtained are consistent with the “frontal lobe compensation hypothesis” in the part, which describes the presence of a compensatory process when tics emerge in children with combined type of ADHD. Besides, our results expand it, suggesting that not only the prefrontal cortex but also the parietal-occipital cortex participates in the adaptive process associated with spatial synthesis and analysis of information, as well as hand-eye coordination.

The present article addresses the definition of cerebral amyloid angiopathy (CAA) and its symptoms based on the analysis of the medical case; the issues of diagnosis and treatment of this pathology are discussed. The Boston criteria, which became the basis for diagnosis, study of clinical manifestations and progression of CAA and approaches to its therapy, are presented. Methods and modes of neuroimaging, including magnetic resonance imaging (MRI), which verify micro cerebral haemorrhage, are described. At the same time, the role and significance of cardiac arrhythmias in the genesis of ischemic stroke are discussed, and scales for assessing the risk of its occurrence are presented. The observation of the neurological, somatic, neuroimaging, neuropsychological status of a 62-year-old patient confirms quite rare combination of probable CAA, paroxysmal atrial fibrillation and repeated hemorrhagic functional apoplexy (FA). The relevance of the case described, is a complex clinical dilemma based on mutually exclusive recommendations for the pharmacological correction of such conditions. It is emphasized that in many multicenter clinical studies on the effectiveness of antithrombotic medication (antiaggregants, anticoagulants) in the treatment and prevention of ischaemic functional apoplexy , an important exclusion criterion is a hemorrhagic stroke in past history (including the multiple changes in haemostasis indicators). Taking into account the obtained clinical and laboratory data in the dynamics, the tactics of treating the described patient were determined. The results of studies related to the treatment of comorbid pathology that should become the subject of the development of a personalized algorithm for managing patients in each specific case, are discussed.

A rare version of paraproteinemic hemablastosis, with the onset characterized by Peripheral Nerve Disease, is POEMS- syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, Skin Changes). In the foreground of the clinical picture of this oncohematological disease is progressive sensorimotor polyneuropathy, which is the reason for the primary reference to a neurologist long before the provision of specialized hematological care. The article provides an overview of recent scientific publications devoted to this disease. Its pathogenetic mechanisms are discussed, as well as the basis of endothelial growth factor. The authors present their own clinical observation of a 62-year-old patient. The onset of the disease was characterized by polyneuropathy accompanied by changes in the M-protein index, organomegaly in the absence of bone-destructive changes, which made the diagnosis difficult. Additional clinical signs included fever, edema (ascites, exudative pleurisy), and hypoproteinemia, changes in peripheral blood (moderate leukocytosis with a decrease in the level of erythrocytes and platelets, which is atypical), kidney and cardiovascular damage. Despite the specific therapy, the patient’s condition was complicated by the development of infectious complications and was fatal. This clinical observation illustrates the need for a careful differential search for a possible cause of non-polyneuropathy of unknown genesis.

A medical case of an acute demyelinating process in cerebrum caused by taking levamisole is presented. The issues of pathogenesis and differential diagnosis of levamisole-induced leukoencephalopathy with acute disseminated encephalomyelitis, multiple sclerosis, progressive multifocal leukoencephalopathy, and cerebral lymphoma are discussed.

Cerebral insufficiency accompanies all conditions associated with the development of critical states arising from acute diseases, including infectious ones. The causes of cerebral disorders in critical states, regardless of their genesis, are multifactorial and are characterized by progression of cerebral blood flow insufficiency, cerebral ischemia, which results in the formation of critical state encephalopathies with neurological deficit, as well as a decrease in cognitive functions in the outcome of the disease. The article defines the concept of encephalopathy of a critical state, deals with the main causes and mechanisms leading to its development, and clinical manifestations. The data of our own study of 30 children with critical state encephalopathies in various infectious diseases are presented. Neurofunctional changes in patients with critical state encephalopathies are described. Recommendations on the tactics of maintaining reconvalescents with encephalopathies of critical condition are given.