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Russian neurological journal

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Vol 29, No 1 (2024)
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REVIEWS

4-13 1167
Abstract

Paraneoplastic neurological syndromes (PNNS) are neurological disorders due to autoimmune attack on the nervous system, induced by a tumor remote from these structures. The existence of relatively stable associations between the neurological syndrome, the type of antineuronal antibodies (Ab) and the nature of tumor has been proven. At the same time, there are no pathognomonic combinations of this kind: the same antibodies can be detected in diff erent types of tumors and in diff erent syndromes. In addition, level of reliability of the diagnosis of PNNS is considered, taking into account the phenotype of the neurological disorder, the presence of certain antibodies in the blood serum, the verifi cation of oncopathology and the duration of the observation for the patient.

14-18 701
Abstract

Acute disseminated encephalomyelitis (ADEM) is a common acute multifocal autoimmune infl ammatory demyelinating disease of the central nervous system (CNS) with a predominant lesion of the white matter of the brain, brainstem and optic nerves, the conductors of the spinal cord, less often — gray matter of the CNS. ADEM is usually a self-limiting condition with spontaneous improvement and a favorable prognosis. Along with the possibility of a severe course in the classical form of ADEM, the development of hyperacute forms with a much more unfavorable prognosis is possible. Such variants of the course of ADEM are acute hemorrhagic leukoencephalitis (AHLE), acute hemorrhagic leukoencephalomyelitis and acute necrotizing hemorrhagic leukoencephalitis of Hurst, associated with the development of a cascade of hyperacute uncontrolled autoimmune reactions. The severity of these conditions (grouped under the term OHLE) is associated with rapid progression, brainstem involvement, large lesions is due to the development of acute edematous necrosis and hemorrhage with a high possibility of disability or death. The aggressive and severe course of АHLE requires the immediate initiation of decongestant and immunosuppressive therapy.

19-24 600
Abstract

Alternating hemiplegia (AH) is a rare and little researched disease. AH is characterized with early episodes of hemiplegia (from a few minutes to a few days), abnormal movements and cognition, and probable development of neurological defi ciency. Studies of genetic basis have led to discovery of mutations in gen ATP1A3, which codes alfa-3 subunit of Na+/K+-ATPase. For research, genetic data are considered most important prognostic information. Its diagnostics is diffi cult due to similarity of some symptoms to epilepsy and absence of deviations at neuro-visualisation. This article is an attempt to generalize world scientifi c experience and to determine most important research derections.

CLINICAL RESEARCHES AND CASE REPORTS

25-35 928
Abstract

Introduction. Multiple sclerosis (MS) is a chronic autoimmune and neurodegenerative disease of the central nervous system (CNS). High-dose immunosuppressive therapy with autologous hematopoietic stem cell transplantation (HDIT–AHSCT) is the most eff ective method of treating MS, which has a positive eff ect on the quality of life of patients and inhibition of the progression of disability.

The aim of the study was to analyze the dynamics of quality of life in patients with MS who underwent the HDIT– AHSCT method in groups with diff erent clinical effi  cacy of this method.

Material and methods. The single-center observational study included patients with MS (n = 21, mean age 35.5 ± 6.2 (28–50) years; men/women — 11/10; remitting MS (RMS) n = 16, secondary-progressive (SPMS) n = 4, primaryprogressive (PPMS) n = 1), after HDIT–AHSCT (cyclophosphamide-rituximab conditioning regimen). Assessment of the quality of life (SF-36, FAMS, CSP-MS42, HADS) and the degree of disability in all patients was carried out before and 12 months after HDIT–AHSCT.

Results. 12 months after HDIT–AHSCT, more than 50% of patients showed signifi cant improvement in quality of life according to the scales of viability and social functioning of the SF-36 questionnaire, as well as according to the scales of general well-being and additional problems of the FAMS questionnaire. 57% of patients had a signifi cant improvement in the overall FAMS score. According to the CSP-MS42 questionnaire, a decrease in weakness (p < 0.05), feelings of constant fatigue and rapid fatigue during exercise was revealed. After transplantation, there was a slight decrease in anxiety (p = 0.91) and a signifi cant decrease in depression (p = 0.011).

Conclusion. The expected dynamics was established: in patients with clinical improvement, there was an improvement in the quality of life for most indicators; in patients with stabilization, most quality of life indicators were similar before and after HDIT–AHSCT; with clinical deterioration after HDIT–AHSCT, quality of life parameters worsened. These data indicate the expediency of further optimization of patient selection criteria and various stages of the HDIT–AHSCT in order to obtain optimal clinical results and, accordingly, improve the quality of life of patients.

36-45 570
Abstract

Frailty is a key geriatric syndrome characterized by an age-associated diminution of the physiological reserve and functions of many body systems leading to increased vulnerability of the elderly people to the eff ects of endo- and exogenous factors. The combination of frailty, cognitive impairment, anxiety and depression is a signifi cant clinical predictor for an unfavorable prognosis.

Purpose. Identifi cation and study of the relationship of cognitive impairment, anxiety, depression and frailty assessment indexes in female patients over 60 years old, observed on an outpatient basis.

Material and methods. The study included 80 women over 60 years old. All participants were divided into 4 groups: non-frail (10 women), pre-frail (44 women), mildly frail (20 women), more-frail (6 women). A comprehensive clinical examination was carried out: Montreal cognitive assessment (MoCA), Mini-Mental State Examination (MMSE), frontal assessment battery (FAB), phonetic association test, semantic association test, Beck anxiety inventory, geriatric depression scale–15 (GDS-15), questionnaire “Age is no barrier”, Frailty index (FI, Hoover et al., 2013).

Results. All women in 4 groups did not signifi cantly diff er in age. There was no statistically signifi cant diff erence in the MMSE, FAB data and mild cognitive impairment (MCI) prevalence among the groups. Lower rates of MoCA scale were associated with higher probability of frailty (odds ratio (OR) = 0.78; 95 confi dence interval (CI) CI: 0.63–0.96, p = 0.016). The cut-off  level of 26 and lower according MoCA has sensitivity 69% and specifi city 50% to detect of high frailty risk (according FI). Questionnaire “Age is no barrier” has a sensitivity of 86% and a specifi city of 53% to detect prefrailty (score 1–2) and a sensitivity of 69% and a specifi city of 87% to detect the presence of frailty (score ≥ 3) regarding FI. Combined sensitivity of MoCA (≤ 26 score) and questionnaire “Age is no barrier” (≥ 3 score) of 78%, combined specifi city of 69%. Higher rates of Beck anxiety inventory and GDS-15 were associated with frailty (OR = 1.12; 95 CI: 1.02–1.22, p = 0.0134 and OR = 1.39; 95 CI: 1.05–1.84, p = 0.0201, respectively). A higher index of the Beck anxiety inventory was also associated with mild cognitive impairment according MoCA (OR = 1.12; 95% CI: 1.04–1.2, p = 0.003). Arterial hypertension was signifi cantly more common for women with FS (χ2 = 4.23, p = 0.04).

Conclusion. There was no signifi cant association between MMSE, FAB, MCI prevalence and the severity of frailty in senior women due to the insuffi  cient study power (< 80%). MoCA cut-off  ≤ 26 score is associated with higher burden of frailty. The questionnaire «Age is no barrier» is an acceptable screening method for determining the necessity of additional geriatric examination of independently living women over 60 years old. Higher rates of the Beck anxiety inventory, GDS-15, BMI, lower rates of MoCA scale and arterial hypertension are associated with high rates of FS and may be predictors for the frailty progression.

46-51 608
Abstract

Multiple sclerosis (MS) is a chronic autoimmune, infl ammatory and degenerative disease of the central nervous system and is the most common neurological disorder in young people.

Purpose: to determine the clinical and epidemiological features of multiple sclerosis usig the example of the Stavropol region (SR).

Materials and methods. A retrospective analysis of 655 medical records of patients for 2021–2022, hospitalized in the neurological department of a multidisciplinary hospital with a diagnosis of MS, was carried out. The age, gender, place of residence of the patient, age of onset of the disease, type of MS, degree of disability, and rate of disease progression were assessed. Statistical processing was carried out using Microsoft Excel and Statistica 10 software.

Results. The incidence of MS during this period was 22.7 per 100,000 population. The share of women was 70.2%, men — 29.8%. Average age — 40.04 ± 10.8 years. The vast majority of cases of onset of MS (83.2%) occur between the ages of 21 and 50 years — 82.8% of women and 84% of men. When analyzing the severity of neurological defi cit, the average score on the extended Kurtzke scale of disability was 3.53 ± 1.7 points. The rate of progression was 1.21 ± 1.1 points per year, which corresponds to a fast pace. Among those hospitalized, 65.4% have a disability group: third — 41.9% (275 patients), second — 21.4% (140 patients), fi rst — 2.1% (14 patients).

Conclusion. The Stavropol Territory is one of the regions with a moderate risk of developing multiple sclerosis. The results of the work may be useful in planning medical and social care in certain territories of this region.

52-58 655
Abstract

The article presents a long-term catamnestic observation (15 years) of a patient with Landau–Kleff ner syndrome (LCS), in its debut manifested by epileptic paroxysms, total aphasia, cognitive and behavioral impairment, on EEG spike-wave complexes. MRI in epileptic mode was without pathology. He received levetiracetam and hormone therapy (glucocorticosteroids, ACTH analogues) with regression of epileptic seizures and epileptic discharges on EEG and pronounced positive dynamics of speech and other cognitive functions. After 15 years, the patient had a confi dent cognitive defi cit, speech disorders were almost absent.

59-66 452
Abstract

Justifi cation. Due to the high prevalence of diseases of the visual organs (cataracts, glaucoma, etc.) and the large number of surgical interventions performed annually in elderly and senile people, the prevention of in-hospital ischemic stroke (IHS) is an urgent task.

The purpose of the work. To study the risk factors of development, characteristics of care and outcomes of IHS in ophthalmic patients.

Material and methods. The study was performed in the period from 01.01.2022 to 31.12.2022 on the basis of ophthalmological departments specializing in the performance of planned vitreoretinal surgical interventions, two large multidisciplinary hospitals in St. Petersburg.

Results. It has been established that perioperative ischemic stroke (included in the structure of the IHS) is a rare complication of minimally invasive ophthalmic operations. Its share was 0.07% (n = 5) of the total number of patients who underwent elective ophthalmological intervention during the year; the total share of patients with IHS (taking into account 4 cases of ischemic stroke in the preoperative period) was 0.13%. Most strokes (n = 5; 55.6%) belonged to the cardioembolic subtype, the proportion of using reperfusion techniques was high and amounted to 33.3% (2 endovascular interventions, 1 systemic thrombolysis); the proportion of adverse outcomes was 22.2% (n = 2). A distinctive characteristic of patients with IHS was a combination of high comorbidity with insuffi  cient antithrombotic prophylaxis, which consisted in the cancellation of antiplatelet agents and anticoagulants in the preoperative period.

Conclusion. Current recommendations on perioperative management of patients indicate the need to continue taking antiplatelet and anticoagulant drugs for most ophthalmic operations, due to the predominance of threats of thromboembolic complications over the risk of retrobulbar bleeding.

LECTURE

67-76 603
Abstract

Objective: to study morphofunctional features of the sacrococcygeal region structures, their syntopy to neighboring anatomical formations and internal organs, as well as associated pathological processes and anomalies of their development to improve the search for the sacralgia and coccygodynia causes.
Material and мethods: data from electronic resources e-Library.ru, CyberLeninck, Cochrane Library, Google Academy, Global Health, Medline, Elsevier, Scopus, Web of Science and PubMed were used.
Results. The sacrococcygeal region is a place of concentration of numerous nerve branches and plexuses innervating not only the pelvic organs but also the lower extremities. Even a small change in the form and function of any sacrococcygeal region components violates their correct syntopy and leads to the pathogenetic mechanisms, the leading manifestation of which is pain. The basis of the most pain syndrome cases is skeletal-muscular or nonspecifi c pain, in which the facet joints, sacroiliac joints, spinal muscles, piriformis muscle and ligaments are identifi ed as anatomical pain impulsation sources. Connective tissue dysplasia syndrome is a serious risk factor for musculoskeletal ligament failure, joint instability, and pain syndrome chronifi zation at working age. Neuropatic pain caused by compression of the spinal root or “cauda equina” by disc herniation or spondylolisthesis is much less common. The least common pain in the sacroiliac region is caused by specifi c causes, including developmental abnormalities, injuries, internal organ diseases, tumors, infectious and autoimmune processes.

 


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ISSN 2658-7947 (Print)
ISSN 2686-7192 (Online)