Cognitive disorders are one of the most important manifestations of various cerebral pathologies. Among all the nosological forms that occur with the formation of dementia, Alzheimer’s disease is the most common. At various stages of historical development, scientists conducted numerous studies aimed at establishing effective means of its therapy. In ancient times, the emphasis was on the use of such means as baths, diets, wine, massage, mental training. In addition, various rites and rituals were practiced. A large role was given to the use of decoctions, tinctures based on medicinal plants, often with the addition of minerals and animal products. In the XIX century, physical care, being in a family environment, attempts to involve in everyday activities, restrictive measures became widespread. In the process of the development of sciences at the beginning of the XX century, pharmacological preparations began to be used. Initially, these were sedatives and diuretics based on bromine and iodine. Then vasodilating and other “vascular” means came into practice. As data on the pathogenesis of the disease accumulated, drugs that affect cholinergic transmission and other mediator systems were developed. There have also been attempts to use drugs with neurotrophic, antioxidant, and anti-inflammatory properties. The most modern direction of therapy, taking into account the main pathobiochemical changes formed in the brain in Alzheimer’s disease, is an antiamyloid strategy aimed at modifying the course of the disease.

Recovering the walking ability is one of the major goals in rehabilitation of poststroke patients. Locomotor training should be preceded by identifying pathological gait characteristics and functional gait assessment. The spectrum of rehabilitation methods for restoration of walking skills is quite wide and includes conventional training, training on a treadmill without body weight support and with body weight support, using of electromechanical and robotic devices, additional use of biofeedback, virtual reality, orthoses, electrical muscle stimulation, strength training and balance training. The review provides a brief description of these rehabilitation methods in view of evidence-based medicine. The individual choice of procedures depends on the severity, duration and characteristics of walking defiits, the patient’s preferences and limitations associated with his concomitant diseases.

The article discusses the pathogenesis and manifestations of the cardio-cerebral syndrome in patients with heart failure (HF). HF is one of the significant problems of older adults due to its high prevalence. The mechanisms of the effect of HF on the development of acute and chronic cerebral ischemia, often accompanied by cognitive impairment and mood disorders, are considered. For an optimal integrated approach to managing patients with cardiocerebral syndrome, it is advisable to involve cardiologists, neurologists, and psychiatrists.

Introduction. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is one of the most common genetic causes of small-vessel cerebral diseases.

Objective. The aim of our study was to examine the frequency and severity of phenotypic spectrum in patients with CADASIL, including the study of the prevalence of the NOTCH3 gene mutations in patients with suspected CADASIL.

Material and methods. Sanger sequencing of exons 2–7, 11 of NOTCH3 gene was conducted in 314 patients with suspected CADASIL (confirmed by anamnesis and magnetic resonance imaging (MRI)). Clinical and MRI data were collected and analyzed for 14 patients with CADASIL.

Results. NOTCH3 gene aberrations in exons 2–7, 11 were detected in 34 of 314 examined patients, that is 11% of all cases. The most frequent aberrations are localized in exon 4 (70.4%), exon 3 and exon 6 (8.8%) of the NOTCH3 gene. A detailed analysis of clinical and instrumental data was conducted in 14 cases of confirmed CADASIL with pathogenic mutations.

Conclusion. The age of manifestation of CADASIL in the Russian population varies significantly. Patients without a previous history of TIA/stroke may have an atypical course of the disease, including cerebellar ataxia and epilepsy. MRI pattern of the CADASIL patients of the studied cohort showed no severe damage of external capsules and temporal lobes. Spinal cord lesion are not to be excluded as a CADASIL symptom.

Anti-CGRP monoclonal antibodies and particularly Erenumab become the first migraine-specific treatment and have demonstrated high efficacy and safety in the randomized clinical trials.

Aim. The aim of this study is the prospective evaluation of the efficacy and safety of Erenumab in patients with episodic migraine (EM) and chronic migraine (CM).

Material and мethods. We recruited patients aged 18–85 diagnosed with EM and CM according to the International Classification of Headache Disorders, 3rd edition. All patients were from November 2020 to December 2021. Demographic data, history of migraine and previous migraine treatment were collected for each patient. All patients filled out scales and questionnaires, headache frequency dynamics was followed up monthly with headache diaries.

Results. After three months of treatment reduction marked reduction of headache frequency was observed, and 53.5% of patients became ≥ 50% responders. After six months of treatment a further increase in the effect was observed: 57% of patients had at least a two-fold reduction in headache frequency, 74% of patients became ≥ 30% responders. Constipation was observed in 3.8% of patients.

Conclusion. Erenumab is safe and effective in the treatment of moderate- and high-frequency EM, CM and medication-overuse headache.

Epilepsy surgery is effective in 30–85% of patients with drug-resistant epilepsy. However, risk factors associated with favorable and unfavorable outcomes of epilepsy surgery need to be further evaluated. We present the outcome of the large epilepsy surgery cohort in Russian Federation.

Purpose: evaluation of risk factors of favorable and unfavorable long-term outcomes in the Russian cohort of drugresistant patients with epilepsy.

Material and methods. Three hundred and eight patients with structural drug-resistant epilepsy were operated by the neurosurgery team of Moscow State University of Medicine and Dentistry. Presurgical investigations and surgeries were performed by this team between 01.01.2014 and 31.12.2020. All patients underwent neurological and neuropsychological evaluation, seizure semiology assessment, neuroimaging and neurophysiological examination. Histological analysis of resected tissues was performed. Results of surgery were assessed according to J. Engel (1993) at 6, 12, 24, 48 and 60 months after surgery. Risk factors, associated with favorable (Engel I–II) and unfavorable (Engel III–IV) outcomes were evaluated.

Results. Underwent 308 primary resection procedures, placement of a vagus nerve stimulator were in 41 patients, 9 patients have been repeated by resection procedures and 8 — underwent radiosurgical treatment. 256 (83%) patients were MR-positive, 53 (17%) — MR-negative. Temporal lesions were revealed in 186 (60%) patients, extratemporal — in 8 (3%), bilateral temporal — in 15 (5%), combination of temporal and extra-temporal — in 81 (26%), multifocal — in 16 (5%), generalized form in 2 (1%). In 12 months after surgery there were favorable outcomes (Engel I + II) in 85% of patients (n = 148), in 24 months — 70% (n = 127). In patients with MRI positive and negative lesions the result of surgery after 12 months was comparable. Postoperative complications were diagnosed in 6 (1.9%) patients, there were no mortality. Temporal plus epilepsy (p = 0.009), multifocal (p = 0.008) and bilateral lesions (p = 0.006) were the most significant risk factors of unfavorable surgery outcomes.

Conclusion. The presented results confirm the efficiency of surgical treatment of drug-resistant epilepsy. Temporal plus form epilepsy, multifocal and bilateral lesions were the most significant risk factors of unfavorable surgery outcomes.

Sudden-onset sleep (SOS) is one of the manifestations of excessive daytime sleepiness (ЕDS) in Parkinson’s disease (PD).

Aim: сlarify the clinical and polysomnographic characteristics of patients with SOS at the expanded (2 and 3 stages according to Hoehn–Yahr) stages of PD.

Material and methods. 42 patients (18 men and 24 women, average age 66.5 ± 7.9 years, duration of PD 8.9 ± 4.6 years, Hoehn–Yahr stage of PD 2.6 ± 0.5) who received combined dopaminergic therapy with levodopa and dopamine agonist were examined. 32 patients had ЕDS, 10 patients did not have it. The Unified Parkinson’s disease rating scale (UPDRS parts II, III in “on” state of dopaminergic medication), the diary of the assessment of the waking period, the Epworth Sleepiness Scale, the Parkinson Disease Sleep Scale I (PDSS I), the Sсales for Outcomes of Parkinsons disease-Cognition (SCOPA-Cog), the Beck Depression Inventory, the State-Trait Anxiety Inventory, the Apathy Scale, the Stroop test. Objective sleep-wake assessment: standard nocturnal video-polysomnography (PSG), multiple sleep latency test (MSLT).

Results. SOS was detected in 69% of patients with complaints of ЕDS. Patients with SOS had a shorter latency of falling asleep according to MSLT data (8.1 ± 5.9 min versus 14.2 ± 3.9 min in patients without of ЕDS, p < 0.05).Patients with SOS had a high severity of motor function disorders, apathy and depression (p < 0.05). Рatients with SOS had no significant differences in the main PSG characteristics of night sleep.

Conclusion. SOS can be considered as one of the manifestations of a more severe course of PD. A decrease in the effectiveness of night sleep and a change in its structure are not a determining factor in the development of SOS.

Diagnosis, treatment and rehabilitation of patients with facial neuropathy (FN) is a significant medical problem. The aim of the work was to create, implement and evaluate the effectiveness of an internal algorithm for working with patients suffering from FN.

Material and methods. The study included 53 patients: group 1 (n = 27) — treatment in the first 2 weeks from the moment of development of symptoms, group 2 (n = 11) — for more than 2 weeks to 3 months, group 3 (n = 15) — for more than 1 year. The algorithm of diagnosis and treatment were created in the N.V. Sklifosovskii Research Institute of Emergency Medical Care. It includes a multidisciplinary approach, teamwork and continuity.

Results. In patients with FN diff erent genesis, at the beginning of treatment in the acute period, complete restoration of the function of the facial nerve and facial muscles occurred in 74% of cases in the period from 2 weeks to 9 months, the period depended on the severity of clinical manifestations (House–Brackmann scale) (r = 0.539, p = 0.014), the degree of nerve damage according to electroneuromyography (ENMG) (r = 0.595, p = 0.006), the severity of complications (contractures and synkinesias) (r = 0.524, p = 0.018). In patients with idiopathic FN, at the beginning of treatment in the acute period, complete recovery was observed in 81.3% of cases. At the beginning of treatment of patients with a disease period of more than a year, conservative tactics led to an improvement in 67% of cases.

There was an incomplete correspondence between the clinical (House–Brackmann scale) and degree of nerve damage according to ENMG (r = 0.620, p = 0.004). ENMG should be performed when the House–Brackmann is VI degree. In cases of a decrease in the amplitude of the M-response by 90% or more compared to the healthy side, needle myography (IMG) is indicated. In the absence of motor unit potentials (PDE), it is necessary to consider the issue of surgical treatment, which is indicated in the acute period of FN to every 10 patients, and after a year in cases of lack of recovery — to every second.

FN should be considered a progressive disease due to the development of complications, such as the consequences of lagophthalmos, contracture, synkinesia. Lagophthalmos occurs in 73.5% of patients with various consequences — from dry eye syndrome to corneal ulcers, so it is important to use local protective and regenerating drugs early to prevent the development of keratopathies, even with a mild degree of lagophthalmos. Hypertonus of facial muscles is formed already on the 7th day of the disease on the healthy side of the face in an average of 67% of patients, and in the absence of timely treatment — in more than 90% of patients. Synkinesia develops in the period from 4 months to a year in every fifth patient. Timely detection and correction of complications prevents their further development and leads to a complete restoration of the facial nerve function.

The success of recovery in FN depends more on identifying the risks of complications and carrying out their prevention. Risk factors for the development of contractures and synkinesias are: prosoplegia, facial nerve damage by more than 90% according to ENMG and gross exercises. FN, despite the different causes of its development, clinically has similar sanogenetic mechanisms, which should be used in rehabilitation.

The prevalence of various types of headaches in onco-haematological patients has been understudied so far. The aim of the study was to assess the prevalence of various types of primary and secondary headaches in oncohaematological patients before haematopoietic stem cell transplantation.

Material and methods. We conducted a retrospective study of 65 adult (≥ 18 years old) onco-haematological patients. The history of primary and secondary types of headaches, total number of headache types in an individual, the relationship between the presence of headache and onco-haematological disease features, as well as the sensitivity and specificity of ID Migraine questionnaire were assessed.

Results. A history of headache was found in 36 patients (55.4%). Among primary headaches, migraine was the most common (24.6%; n = 16). Among secondary headaches, post-dural puncture headache was the most prevalent (15.4%; n = 10). Almost a quarter of patients had 2 or more headache types (2 types were found in 16.92% of patients, 3 types were found in 6.15% of patients, 4 types were found in 1.54% of patients). No feature of oncohaematological disease was found to have a significant association with the presence of headache or a significant correlation with the number of headache types in an individual. The female sex increased the OR for the presence of primary headache (OR 0.23; 95% CI 0.06–0.86; p = 0.02). Sensitivity and specificity of ID Migraine™ questionnaire were found to be 90.9% (95% CI 58.7–99.8) and 58.3% (95% CI 27.7–84.8), respectively.

Conclusion. More than half of onco-haematological patients referred for haematopoietic stem cell transplantation had a history of headache, the most common type being migraine. In our sample, screening with ID Migraine questionnaire had high sensitivity, but low specificity.

The article presents two clinical cases of 51 and 57-year-old patients living in regions endemic for tick-borne encephalitis in which the differential diagnosis between progressive supranuclear palsy (PSP) and the extremely rare parkinsonian variant of chronic tick-borne encephalitis (CTBE) resulted in a diagnostic challenge. In both cases there was a clinical presentation of progressive symmetric levodopa-resistant parkinsonism with vertical gaze palsy, cognitive impairment of the executive type, frontal lobe signs, focal dystonia and in one case — early onset of postural instability, which corresponds to the diagnosis of probable PSP. The parkinsonian variant of CTBE was supported by the lack of tick-borne encephalitis virus IgG-antibodies negativity in the blood for several years, intrathecal oligoclonal antibody synthesis in one patient, the absence of typical neuroimaging signs of PSP, and delayed disease onset after a tick bite. Difficulties in diagnosis and possible mechanisms of the pathogenesis of primary progressive CTBE, as well as the similarity of the clinical and pathomorphological presentation of PSP, von Economo encephalitis lethargica, and postencephalitic parkinsonism are discussed.

In patients with severe brain damage, the severity of the course/outcome of a chronic critical condition is associated with dysfunction of the intestinal microbiota, which can be assessed by the level of microbial metabolites circulating in the blood.

The purpose of the study. To establish a connection between the dynamics of a chronic critical condition developed as a result of severe brain damage and microbiota dysfunction.

Patients and methods. The study included 83 patients in chronic critical state (CCS) with the consequences of ischemic/ hemorrhagic stroke (n = 34), traumatic brain injury (n = 18), hypoxic brain damage (n = 7), neurosurgical interventions (n = 7), meningitis (n = 3); and 30 healthy people (control group). To assess the neurological status in dynamics, the stroke scales of the National Institutes of Health (NIHSS), the FOUR, Rankin scales, and the Rivermead Mobility Index were used. The taxonomic composition of the microbiota was evaluated by 16S rRNA sequencing and PCR. Biomarkers (by ELISA Kit) and aromatic microbial metabolites (by GC-MS) were monitored twice a week.

Results. The peculiarities of the taxonomic composition of the intestinal microbiota in long-term ill patients with brain damage, the predominance of conditionally pathogenic facultative anaerobes (Klebsiella spp., Proteus spp., Staphylococcus aureus) over the pool of strict anaerobes were revealed. The relationship between the dynamics of microbial metabolites in blood and gut of patients with the course and outcome of the disease has been established. Patients with positive clinical dynamics of CCS were characterized by a stable level or decrease in microbial metabolites during treatment. In the group of patients with negative clinical dynamics, an increase in the level of microbial metabolites, especially hydroxylated phenyl carboxylic acids, was revealed.

Conclusion. In patients with brain damage, the levels of microbial metabolites circulating in the blood (p-hydroxyphenylacetic, phenyl-lactic, p-hydroxyphenyl-lactic acids) reflect the degree of microbiota dysfunction and the severity of the course of CCS, which is of prognostic importance.

During the outbreak of coronavirus disease 2019 (COVID-19) clinicians are increasingly involved in the observation of possible neurological complications due to the infection of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Some cases of encephalitis following COVID‐19 pandemic have been reported in literature that can mean possible damage to the central nervous system related to the SARS-CoV-2 infection. Early on, this was thought to be due to the direct effects of the virus itself, but the possibility of immune system mechanisms being implicated was gradually raised in scientific literature. The reports of anti‐NMDA receptor encephalitis among patients with COVID‐19 at different ages may be signs of this condition. We report the case of 16-year teenager with concomitant Anti-N-Methyl-D-Aspartate Receptor (Anti-NMDAR) encephalitis and COVID‐19. This clinical case brings arguments on the possible relationship between SARS-CoV-2 infection and autoimmune encephalitis.

Important factors for reducing the risk of stroke, as well as predictors of effective rehabilitation of this group of patients are proper nutrition, regular physical activity, smoking cessation and normalization of body weight. Currently, there is no unambiguous data on the needs of patients who have suffered acute cerebral circulatory disorders for nutrients, which dictates the need to develop a personalized diet for them, which should be calculated taking into account age, gender and body mass index. The intake of energy, proteins, fats, carbohydrates, minerals and vitamins can be carried out not only as part of traditional, but also from specialized food products, including mixtures for etheral nutrition. The use of mixtures helps to reduce the risk of bedsores.