Essential tremor (ET) is the most common movement disorder, the main manifestation of which is progressive postural-kinetic trembling of the limbs, its pattern most often involving the hands. Recent data indicate a higher frequency of ET in combination with various neurological symptoms, which in the new classification is usually classified as essential tremor plus (ET+). The purpose is to identify and determine the characteristics of intragroup division and changes in neurocognitive status among various forms of ET+ in comparison with ET patients. Material and methods. At the Republic Consultative-diagnostic Center for extra-pyramid pathology and botulin therapy in Kazan, a prospective study was carried out on 116 patients with essential tremor, using clinical, anamnestic and statistical methods of data collection and analysis. Results. Neurocognitive phenotypic variants of ET have been established. It has been proven that all patients with ET, even in the absence of a global decline in intelligence, are characterized by changes in attention, speech fluency, memory, and executive functions; dementia was detected in 28% of patients included in the study. For a reliable diagnosis of ET, in addition to assessing the standard neurological status, it is necessary to assess the severity of tremor using the FTM scale, assessing ataxia using the SARA scale, and assessing the cognitive status using the MOCA test.

Tremor is one of the most common motor symptoms in Parkinson’s disease (PD) and affects up to 75% of patients. The tremolous form of PD is heterogeneous in clinical manifestations. Along with classic rest tremor, patients with PD may experience postural and kinetic types of the tremor, which reflects the multimodal mechanism of its formation. Classifications of PD are constantly changing, including within the tremor-dominant form. The varied responses to levodopa also reflect the role of multiple underlying pathophysiological processes. New, non-antiparkinson drugs are being studied. Evidence of the effectiveness of advanced neurosurgical, non-invasive treatment methods is not always convincing; there are no large-scale comparative studies assessing their effectiveness in patients with tremor-dominant forms of PD.

The article is of a review nature and is devoted to tremor, one of the maladaptive and difficult-to-treat symptoms of Parkinson's disease (PD). Along with the classic rest tremor, patients with PD may experience tremor of other modalities: postural tremor, kinetic tremor, which reflects a multimodal mechanism of tremor formation involving multiple neurotransmitter systems. The unpredictable response to therapeutic options, the ambiguous response to levodopa, also reflects the role of multiple underlying pathophysiological processes. Among the drug methods of tremor correction, preference is given to dopamine receptor agonists - due to the spectrum of their pharmaceutical action, high efficiency in relation to all leading motor and a number of non-motor manifestations. The evidence for advanced neurosurgical, non-invasive modalities is mixed, and there are insufficient comparative studies to assess their efficacy in patients with tremor-dominant forms of PD.

This article discusses the issues of muscular dystonia pathogenesis that are relevant to modern medicine, as well as the possibility of using the pathogenetic concept as a substrate to supplement the classification criteria. Reviewing the international publications on etiopathophysiology, genetics and classification, the article summarizes the main mechanisms for the occurrence of a pathological process at different levels of the nervous system. Despite the frequent clinical similarity of the symptoms of various forms of muscular dystonia, these symptoms may be the result of dysfunction of completely different genetic and neurophysiological mechanisms. The article focuses on the importance of studying the etiological and pathogenetic mechanisms and their further implementation in clinical practice in order to develop more accurate treatment techniques aimed at eliminating the specific causes of the development of specific forms of muscular dystonia.

Essential tremor (ET) is the most common movement disorder, the main manifestation of which is progressive postural-kinetic tremor of the extremities, the pattern most often involving the hands. According to the latest classification, 2 different groups of patients with essential tremor are distinguished: patients with tremor (ET) and patients in whose clinic there are additional symptoms in addition to tremor (ET-plus syndrome, ET-P). The purpose was to identify and compare cognitive impairment in patients with essential tremor and essential tremor plus syndrome. Material and methods. A prospective study of 41 patients with essential tremor was performed at the Center for Movement Disorders and Botulinum Therapy of the Republic of Tatarstan using clinical, anamnestic and statistical methods for collecting and analyzing data. Results. Patients of both groups were comparable in terms of the disease duration, the frequency of family cases occurrence, and gender. While changes in cognitive functions are heterogeneous in different groups, ET-P patients show changes in visual-spatial functions, speech and short-term memory, which forms an amnestic type of intellectual impairment. The heterogeneity of the clinic of ET-P patients may indicate the involvement of various brain structures, in addition to the cerebellum, in the pathophysiological process.

Amantadine has begun to be used as a possible alternative in COVID-19 therapy to mitigate its effects. There is anecdotal evidence that patients with Parkinson's disease (PD) treated with amantadine and who test positive for COVID-19 often do not develop clinical manifestations of COVID-19. Objective : to compare the clinical course of COVID-19 in patients with PD who took or did not take amantadine sulfate. Patients and methods . A prospective continuous study included 142 patients with PD who were treated in Republican Clinical Diagnostic Center for Extrapyramidal Pathology and Botulinum Therapy in Kazan from October 2021 to January 2022. Patients filled out a proprietary internally developed questionnaire. Results and discussion . Out of 142 individuals with PD COVID-19 occurred in 77 (54.2%), of which 52.0% had a mild course, 39.0% had a moderate course, 2.6% had a severe course, and in 6.5% the severity of the disease has not been established. Deterioration after COVID-19 infection was noted by 36% of patients: the appearance or increase in motor fluctuations (41%), increased tremor, stiffness or slowness (31%), the appearance of "exhaustion" of the effect of a single dose of levodopa (13%), the appearance or increased dyskinesia (21%), hallucinations (3.5%). Patients taking amantadine sulfate had PD much longer (11.5±5.62 years versus 5.12±3.24 years) and had a more pronounced (III–IV) stage of the disease. These patients were more likely to experience mild COVID-19 (in 60.87% of cases), in contrast to patients not receiving amantadine sulfate (only in 48.15% of cases). There was no correlation between the severity of COVID-19 and levodopa intake. Conclusion . The results of the study showed that patients with PD taking amantadine sulfate are more likely to have a mild course of COVID-19.

FTD is a group of neurodegenerative diseases with progressive deterioration of behavioral and speech disorders, morphologically associated with pathology of the frontal or temporal lobes. International clinical trials have made it possible to define modern diagnostic criteria for various subtypes of clinically «possible/probable» FTD. Our article is devoted to one of the rare subtypes of frontotemporal dementia (FTD), corticobasal syndrome (CBD), in which we presented a review of current data with a demonstration of clinical observation. A clinical case of a patient with a patient with speech disorders and memory impairment is presented. A 60-year-old man at the time of the outpatient visit had been complaining of speech impairment for two years, a slight decrease in memory for current events. Neurological and neuropsychological studies revealed two leading clinical syndromes in the patient: «frontal» syndrome with impaired higher cortical functions in the form of efferent motor aphasia, impaired writing and reading with visual-spatial agnosia and dysgraphia, «frontal» signs (positive «palm-mouth «and» grasping «reflexes); «Corticobasal syndrome» with pronounced dynamic, optic-kinesthetic dyspraxia, dermolexia, apraxia of closing the eyes, «alien» hand syndrome with symptoms of levitation and intermanual conflict. MRI diagnostics revealed changes characteristic of neurodegeneration of the frontotemporal type (atrophy of the frontal and temporal lobes prevails). Taking into account complaints, anamnesis of the disease, identified clinical syndromes and structural changes according to MRI data, the patient was diagnosed with a clinically «probable» FTD. Determination and accurate diagnosis of FTD subtypes will help the neurologist in managing these patients with the appointment of the correct pharmacologic treatment. In FTD, in contrast to AD patients, the administration of cholinesterase inhibitors does not lead to a positive therapeutic effect a positive therapeutic effect and, therefore, is not advisable. The standards of patient therapy should include recommendations for antipsychotic therapy, the use of antidepressants (SSRIs) and anxiolytics with nootropic effects for the correction of affective and behavioral disorders.

Tourette’s syndrome, the most common cause of tics, manifests itself in a wide range of motor and behavioral disorders and is found in men about three times more often than in women. The purpose — to establish the comorbidity of neuropsychiatric disorders in patients with tic hyperkinesis. Material and methods. The study included 111 patients with tic hyperkinesis of various ages and sex, who were outpatiently observed at the Clinical and Diagnostic Center for Movement Disorders and Botulinum Therapy of the Republic of Tatarstan from 2011 to 2014. Results and conclusions. In the group of patients we studied, tics were more common in males (in 71.6% of cases), and patients with Tourette’s syndrome were numerically predominant, taking into account the visits to the Clinical and Diagnostic Center for Movement Disorders and Botulinum Therapy of the Republic of Tatarstan. Clinical manifestations of obsessive-compulsive disorders were found not only in patients with Tourette’s syndrome, but they are characteristic of 53.2% of patients with tic hyperkinesis of various severity and phenomenology. Also, regardless of the structural and phenomenological characteristics of tic hyperkinesis, they were almost always associated with attention deficit hyperactivity disorder with a predominance of hyperactivity.

According to numerous worldwide studies, patients with Parkinson’s disease (PD) often experience sleep disturbances, the most common of which are insomnia and excessive daytime sleepiness. The purpose of this study was to determine the quantitative and qualitative profile of sleep disorders in patients with the newly diagnosed PD, stages 1 and 2 according to Hoehn and Yahr. The assessment was carried out using the SCOPA-SLEEP scale. The study involved 95 people: 56 patients with a newly diagnosed PD, who had not previously received treatment (main group), and 39 individuals from the control group. Results. The profile of nocturnal sleep disturbances in PD patients was characterized by various symptoms: difficulty falling asleep (62,5%), night awakening (51,8%), long lying awake (57,1%), early awakening (50,0%), feeling lack of sleep (46,4%). In women, irrespective of the form and stage of PD, disturbances in nighttime sleep were found more often. Daytime hypersomnia among healthy volunteers was observed only in 5 participants (12,8%). The profile of daytime hypersomnia in PD patients was diverse: unexpected falling asleep during the day (n = 15, 26,8%), falling asleep in a calm environment (n = 17, 30,4%) or while reading and watching TV (n = 18, 32,1%). Difficulties in maintaining wakefulness during the day were noted by 13 patients (n = 13, 23,2%) with PD, and 4 patients (7,2%) reported difficulties due to excessive daytime sleepiness. Thus, given the prevalence and profile of dyssomnias, sleep disturbances may be an important sign of PD.

AIM:Based on the current conceptions on the genesis of hyperkinetic syndromes in children and adolescents, to single out a group of patients with suggestive PANS, compare clinical and laboratory results and determine clinical/laboratory characteristics of this syndrome.MATERIAL AND METHODS:Sixty-nine patients with tics were studied using neurological examination, questionnaires and international scales for assessment of tics, obsessive-compulsive disorders and attention deficit hyperactivity disorder (ADHD). Laboratory tests included general blood tests, antistreptolysin O test, determination of rheumatoid factor, C-reactive protein, circulating immune complexes, nasopharyngeal wash for β-hemolytic streptococcus, antineuronal antibodies and immunoglobulins A, M, G, E, CD4, CD8-lymphocytes. The same tests were performed in the control group.RESULTS AND CONCLUSION:Clinical symptoms were different by the severity and phenomenology of tic hyperkineses in patients with PANS compared to the patients with tics without immune disorders. Most of the patients were diagnosed with Tourette syndrome. ADHD was the most common diagnosis in the PANS group. Its frequency was 2.5 higher in the male patients. A chronic focus of infection did no predict the development of PANS. Based on the laboratory results, one can assume that β-hemolytic streptococcus A infection, lower JgM levels and an elevated CD8+ lymphocytes predict the development of autoimmune mental and neurological disorders in the group of PANS patients.

There was given a definition of «alien» limb. There were revealed and considered the main forms of this phenomena with detailed coverage of the history of this syndrome. Differential diagnostics of this phenomenon was studied. Classification of «alien» limb syndrome was offered. A clinical supervision was presented.

The analysis of ultimate composition of oral fluid of 35 patients with Parkinson's disease was made. As to compare with the control group, in the oral fluid of patients there is an excess of some toxic microelements: Ba (by 36.5 times), Pb, Al, Cd, Ni (by 1,5 times), As (by 3 times). Essential microelements by contrast have a tendency to decline: there is a lack of Fe, Cr, Se — by 7-11%. The content of Co, Sb, V, Ag does not change, and the level of Ca, Mg, Zn is significantly increased. Disturbance of water-salt barrier is evident with a decrease of Na level and increase of K level.

The analysis of ultimate composition of oral fluid of 35 patients with Parkinson’s disease was made. As to compare with the control group, in the oral fluid of patients there is an excess of some toxic microelements: Ba (by 36.5 times), Pb, Al, Cd, Ni (by 1,5 times), As (by 3 times). Essential microelements by contrast have a tendency to decline: there is a lack of Fe, Cr, Se — by 7-11%. The content of Co, Sb, V, Ag does not change, and the level of Ca, Mg, Zn is significantly increased. Disturbance of water-salt barrier is evident with a decrease of Na level and increase of K level.Key words: oral fluid, ultimate composition, Parkinson’s disease, toxic and essential microelements.