Currently, due to the lack of specific etiotropic therapy, rituximab is widely used for the treatment of most autoimmune diseases of the central and peripheral nervous system. Rituximab is a chimeric monoclonal antibody with specificity for CD20, the antigen found on the surface of normal and malignant B-lymphocytes. It is used mainly in hematological practice. It is used off-label for the treatment of neurological diseases. The world literature describes the use of rituximab for the treatment of such pathologies as autoimmune encephalitis, neuromyelitis optica spectrum disorder, multiple sclerosis, primary angiitis of the central nervous system, immune-mediated inflammatory polyneuropathy, myasthenia gravis, refractory to basic immunosuppressive therapy. This article provides an overview of the world literature on the use of rituximab in neurological practice, describes our own experience of its use on the basis of the Department of Neurology № 1 of Pavlov University (Saint Petersburg, Russia).

Aim. To describe best practices in using human normal immunoglobulin in patients with immune-mediated neurological disorders according to the data of one clinical center.Materials and methods. From 2016 to 2021, 20 patients with various autoimmune disorders of the peripheral and central nervous system were treated with human normal immunoglobulin at the Neurology Unit No.1 of Pavlov First Saint Petersburg State Medical University. Treatment efficacy was assessed by changes in the neurological examination data according to specialized scales for specific diseases or clinical manifestations (INCAT, QMGS, MoCA, EDSS). Safety of the therapy was assessed considering the instructions to the drug.Results. In the vast majority of patients, treatment allowed to stabilize the course of the disease or was accompanied by pronounced regression.Conclusion. The considered clinical cases of the use of human normal immunoglobulin preparations demonstrate the possibility of their use in the treatment of a number of autoimmune neurological diseases for unregistered indications.

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.

Background. The review analyzes a possible rare complication of COVID-19 in the form of spondylodiscitis, including with developed epiduritis, in patients who have undergone COVID-19 with severe pneumonia, respiratory failure and systemic inflammatory response syndrome (SIRS). Clinical Case Description. Based on our own clinical observations, an approach to the diagnosis and treatment of three patients is described, each of whom had SIRS, severe fever, a significant increase in laboratory markers of inflammation (C - reactive protein (CRP), leukocytosis, erythrocyte sedimentation rate (ESR), fibrinogen, procalcitonin , ferretin), the addition of bacterial pneumonia, pronounced disorders of coagulation hemostasis, the development of spondylodiscitis, despite the wide range of previous antibiotic therapy, acute pain in the lumbar spine (LSP) with features of "red flags". In the first patient, against the background of massive antibiotic therapy, revisions of purulent foci, glucocorticosteroid (GCS) therapy, and surgical treatment, there was a significant positive trend in the form of pain relief. The second patient showed positive dynamics against the background of conservative antibiotic therapy. The third patient, with a paravertebral abscess at the level of developed spondylodiscitis, received massive antibiotic therapy in combination with GCS, and was operated on to decompress the spinal cord. Conclusion. The authors note that spondylodiscitis and epiduritis may be some of the possible complications of COVID-19 and / or the result of the unwanted action of drugs used to treat this disease. In this regard, timely diagnosis and treatment of this pathology in COVID - 19 seems to be very relevant