Taking into account the frequency of its course and the severity of consequences, facial nerve palsy (FNP) is an urgent problem of modern medicine. Bell palsy is an autopathic form of FNP. The article represents clinical picture and advisable diagnostic study of this disease. Pathophysiology of facial muscles palsy development is discussed. The study is based on the theory of “tertiary ischemia” due to anatomic features of the nerve blood supply. The eff ectiveness of corticosteroids use in the acute period of FNP is analyzed. The advisability of performing surgical decompression of the facial nerve in the acute period in a number of clinical cases is proved.

Monoclonal antibodies (mAT) to calcitonin-gene-related peptide (CGRP) have become the fi rst class of preventive medications specifically designed to treat migraines. The article examines the mechanism of action of mAT from the standpoint of a modern view of the pathophysiology of migraine, discusses the issues of immunogenicity and drug interaction. The review describes in detail the monoclonal antibody erenumab, which became available in Russia from February 2020. The action of erenumab is associated with blocking the CGRP receptor, since it is the only one of the existing mAT that competes with the CGRP molecule for binding to the receptor. This review summarizes the pharmacological characteristics of erenumab, presents in detail the results of clinical trials with an emphasis on the publications of the last year. The article also presents the role of this method of treatment in real clinical practice: the eff ectiveness and safety of erenumab in various forms of migraine are described in detail, the possibility of transition (“reverse transformation”) from chronic migraine to episodic form on erenumab therapy is shown, impressive results of treatment of intractable cases in resistant and refractory migraines are presented. Erenumab is the only one of the mAT drugs for which the results of five-year clinical observations have been published, which have proved the sustained effectiveness and safety in long-term use, as well as the economic feasibility of using this therapeutic approach.

Recently, there is more and more evidence of the presence of a cognitive defect of varying severity in the clinical picture of ALS. A rare form of the disease is the amyotrophic lateral sclerosis (ALS) — dementia complex, characterized by a combination of dementia (usually frontotemporal) with ALS symptoms. The profile of cognitive deficit in ALS includes impairment of executive functions, memory, speech and visual-spatial disorders. A literature review on this problem is presented with a description of the clinical observation of ALS–dementia syndrome (frontal variant of possible Alzheimer’s disease). A patient with a reliable diagnosis of ALS showed rapidly progressive cognitive impairments in the form of hippocampal memory impairments, speech, visual-spatial impairments, and defective executive functions, accompanied by behavioral changes (apathy, decreased criticism). Magnetic resonance imaging of the brain revealed significant atrophy of the hippocampus, frontal lobe cortex, and left temporal lobe. In the literature, there are practically no descriptions of patients with a clinical picture of a combination of AD and ALS. Difficulties in diagnosing this condition are discussed. The relationship between these neurodegenerative diseases is discussed. The presented literature data and the presented clinical observation confi rm the expediency of studying cognitive functions in patients with suspected or signifi cant ALS, on the one hand, and analysis of the state of the central and peripheral neurons in patients with neuropsychiatric disorders of the frontotemporal type, on the other hand, which can be useful for diagnostics and treatment and rehabilitation measures.

Nowadays, the novel coronavirus infection (COVID-19) pandemic is one of the most important global health problems. There is increasing evidence that COVID-19 affects central and peripheral nervous system as well. The paper presents a clinical case of a 47 old patient with the ApoE ε4 haplotype and family history of Alzheimer’s disease who developed cognitive impairment after acute COVID-19. Before the infection the patient has no cognitive complaints and preserved everyday activity. After novel coronavirus infection, which was observed in mild form, the patient had started to complain on constant excessive forgetfulness. Neuropsychological assessment confirmed the presence of pre-mild cognitive impairment of predominantly single domain amnestic type. However, brain MRI showed only subtle periventricular white matter changes usually attributed to small vessel disease. Memory complaints were observed for 3 months of follow up despite intensive cognitive training, optimization of lifestyle and therapy with choline alphoscerate. Probable links between coronavirus infectious and cognitive impairment manifestation are discussed. There is data that ApoE ε4 haplotype is associated with increase of microglia mediated neuro-inflammation and it can be significant for accelerating of progression of neurodegenerative diseases after COVID-19. Further follow up of the patient is necessary for determination of nosological diagnosis explaining manifested predominantly amnestic type pre-mild cognitive impairment.

Normotensive hydrocephalus (NTH) is a syndrome characterized by enlarged ventricles of the brain, gait disturbance, cognitive impairment, and incontinence. In the elderly with gait disturbances of unspecified etiology, NTH should always be excluded. It is especially difficult to diagnose NTH in patients with neurodegenerative diseases, primarily with idiopathic Parkinson’s disease (PD), and vice versa, to diagnose PD in patients with NTH. We report on an 80-year-old patient with a five-year history of NTH, manifested by the classic clinical triad of symptoms and the subsequent development of Parkinson’s syndrome 3 years after the debut of NTH. MRI of his brain revealed ventriculomegaly and transcranial sonography did hyperechogenicity of the substantia nigra on the left, with an area of 0.41 cm2, which made it possible to diagnose two comorbid diseases in the patient, namely, normotensive hydrocephalus and Parkinson’s disease.

The article describes two clinical cases of idiopathic intracranial hypertension, the first manifestation of which was the development of retroorbital headache and the visual disorders. Leading in the clinical picture of the disease in both cases was the detection of stagnant optic nerve discs on the fundus. In both patients, the vascular system of the brain was examined using duplex scanning of the neck and brain vessels, MR angiography and MR venography, and in one case — SCT angiography, a lumbar puncture was performed with the study of cerebrospinal fluid (CSF). An increase in CSF pressure was found, accompanied by changes in the large venous vessels of the skull and brain. This suggests a significant role of venous outflow disorders in the development of Pseudotumor cerebri syndrome.

Progressive muscular dystrophies are a clinically and genetically heterogeneous group of hereditary diseases characterized by a non-inflammatory primary lesion of skeletal muscles. Although hereditary myopathies can debutе at any age and can affect various muscle groups, most muscular dystrophies share common clinical features. In addition to molecular genetic methods, there are many other diagnostic methods that help to make a correct diagnosis (study of creatine kinase in blood serum, CT and MRI of the affected muscles; histological examination, immunoblotting and immunocytochemical study of a biopsy of the affected muscle, etc.). Currently, for many of these diseases therapeutic studies are underway and there are medicines for Duchenne muscular dystrophy registered in Russia (ataluren) and abroad (eteplirsen, golodirsen, viltolarsen). The lecture presents basic data of the clinical picture, diagnosis and treatment of the most common forms of progressive muscular dystrophies.