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Russian neurological journal

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Vol 26, No 5 (2021)
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LITERATURE REVIEW

4-15 706
Abstract

Cognitive impairments (CI) are a serious problem in modern society, because they significantly reduce patients’ quality of life and tend to progress. Age-related diseases such as neurodegenerative — first of all Alzheimer’s disease (AD) and cerebrovascular disorders are key causes leading to CI. At present, approaches to treating these diseases have limited effectiveness in restoring cognitive functions, and do not change disease course, although they can slow cognitive decline.

Understanding the immunopathogenesis of neurodegenerative and cerebrovascular diseases defines new targets and approaches to their treatment. In addition, suppression of neuroinflammation is advisable in the cases of early nonclarified cognitive decline, when information from routine medical, laboratory and instrumental examination of patients is insufficient to identify the causes of CI.

This article summarizes current understanding of the immunopathogenesis of AD and chronic cerebral ischemia. The mechanism of neuroinflammation is presented as a cascade of sequential events that are closed in a self-perpetuating inflammatory response in the end. So called damage-associated molecular patterns, specific receptors that can bind them (pattern recognition receptors), intracellular signal transduction in microglia, cytokines and adhesion molecules are considered as potential points of application of immunomodulatory therapy. The review provides information on the current level of development of immunotherapy of AD, chronic cerebral ischemia and offers the prospect of its application.

CLINICAL RESEARCH AND SURVEILLANCE

16-23 1448
Abstract

Alzheimer’s disease (AD) is the most common cause of dementia in the population. Late onset AD has a classic clinical picture with short-term memory deficit, apraxia and agnosia. Patients with early-onset AD may have an atypical clinical picture which complicates diagnosis. Atypical AD variants include the logopenic variant of primary progressive aphasia, posterior cortical atrophy, behavioral, biparietal, and cortico-basal variants. These variants have pathomorphological signs similar to classical AD, but at an early stage they are characterized by focal atrophy which explains their clinical polymorphism. This article provides a review of the current literature on atypical types of AD and presents a clinical case of a 62-year-old patient in whom the disease debuted with prosopagnosia due to focal atrophy of the temporo-occipital regions of the non-dominant hemisphere.

24-29 688
Abstract

The article describes a clinical case of cerebral venous thrombosis involving the deep venous system in a 42-year-old patient suffering from acute lymphoblastic leukemia. As the patient’s condition progressively deteriorated despite anticoagulant therapy, endovascular treatment was attempted. Transvenous thrombectomy and local thrombolysis were performed, which made it possible to achieve only partial recanalization. Further prolonged administration of alteplase into the cerebral sinus through a microcatheter facilitated complete recanalization of the direct and transverse sinuses. The restoration of blood flow was accompanied by regression of neurological deficit. This case is discussed in the context of modern approaches to endovascular treatment of cerebral venous thrombosis.

30-38 883
Abstract

The article presents an analysis of the literature and a clinical case of a rare disease from the group of diseases of small vessels — cerebral autosomal dominant arteriopathy with subcortical infarction and leukoencephalopathy (CADASIL).

It is based on the deposition of osmiophilic granulation material in vessels of small and medium caliber. A mutation in the NOTCH3 gene on chromosome 19p13 leads to significant structural changes in the walls of small arteries due to impaired differentiation and maturation of smooth muscle cells.

CADASIL is characterized by four key symptoms: migraines, recurrent ischemic strokes, mental disorders, and cognitive decline. The clinical case study is presented from the standpoint of a multidisciplinary patient-oriented approach of joint work of neurologists and morphologists. On the basis of clinical and laboratory criteria, a probable diagnosis was made. To confirm it, a muscle biopsy was performed (a musculocutaneous flap from the inner surface of the thighs and forearms), in order to conduct light and electron microscopy. The details of the results of the morphological study, which made it possible to verify the patient’s diagnosis, are presented. Differential diagnostic judgments are presented and recommendations for genetic studies in the family, prognosis and treatment of the patient are given.

39-43 677
Abstract

The article contains a description of the clinical observation of a patient with Fahr’s syndrome. A review of the literature on this rare neurodegenerative disease, Fahr’s disease, and Fahr’s syndrome is presented. An observation of a 54-year-old patient Sh. with Fahr’s syndrome is presented. The authors of the article set themselves the goal of drawing attention to Fahr’s syndrome, which is the sphere of interests of doctors of various specialties. Raising the awareness of doctors about this disease will contribute to its timely diagnosis and treatment.

44-50 786
Abstract

Patients with chronic migraine are characterized by frequent severe headache attacks, polypharmacy (painkillers), insufficient effect of preventive therapy, and a decrease in the quality of life. The aim of a prospective open-label study was the evaluation of the efficacy and tolerance of Erenumab (Irinex) in the treatment of patients with chronic migraine in real clinical practice.

Material and methods. 48 patients with chronic migraine were clinically and neurologically examined (35.5 [19; 56]; diagnosis was established according to the criteria of ICHD classification and diagnosis of migraine. Medical monitoring period was 3 months. Clinical and neurological testing and subcutaneous administration of Erenumab (Irinex) 70 mg were performed once a month during the whole period.

Results. Safety, good tolerance and an obvious significant clinical effect were noted when using Erenumab (Irinex) 70 mg. It led to a significant improvement in clinical score and the course of chronic migraine, the effectiveness of rapid relief of symptoms and significant reduction in the need for painkillers in most patients. The best results were found after 3 months of therapy.

52-60 838
Abstract

The article concentrates on the experience of using the ketogenic diet (KD) in neurological practice in children with epilepsy resistant to anticonvulsant treatment. Prescription of KD in combination with drug therapy or without it allowed to achieve 100% control over seizures (confirmed by electroencephalographic study), significant progress in psycho-speech, cognitive, motor development in 21% of cases, which increased the chances of socialization of children and improved family life quality.

LECTURE

61-72 913
Abstract

The lecture presents information about the central and peripheral structures that provide innervation and regulation of the functions of the lower urinary tract — the bladder and urethra. The mechanisms of regulation of the functions of accumulation and retention of urine (reservoir function) and emptying of the bladder (evacuation function) are shown.

Neural control of urination is organized in the form of a hierarchical system. The spinal centers are the executive structures, and the cerebral centers are the controlling structures. Involuntary implementation of the act of urination occurs through segmental sympathetic and parasympathetic mechanisms. The regulation of these functions and voluntary urination are carried out by the central mechanisms of the brain — the actuation of the urination reflex is under strict volitional control, which makes it possible to plan the emptying of the bladder in a socially acceptable place and time.

The information on the clinical symptoms of neurogenic dysfunction of the lower urinary tract (neurogenic bladder) is reviewed. The features of clinical manifestations depending on the level of damage to neural structures and in various neurological diseases are shown. Methods for assessing urodynamics and treatment directions for neurogenic dysfunction of the lower urinary tract are presented.



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