Vol 8, No 3 (2018)

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LECTURES AND REVIEWS

Epidemiologic studies of myasthenia gravis: literature review

Alekseeva T.M., Kryuchkova V.V., Stuchevskaya T.R., Khalmurzina A.N.

Abstract

The review of domestic and foreign literature devoted to epidemiological studies of myasthenia gravis has been reviewed. The article presents data on the prevalence and incidence of myasthenia gravis in several regions of Russia and abroad. The prevalence of myasthenia gravis in the world varies, according to different authors, in a very wide range – from 2.17 to 32.0 per 100 thousand people. There are few epidemiological studies of myasthenia gravis in large cities and regions of Russia. Meanwhile, studies of the prevalence and incidence of myasthenia gravis are a necessary stage in the work to increase the effectiveness of diagnosis and treatment of this pathology, to determine the need for specialized types of medical care. The results of these studies can be used to optimize the management of myasthenia gravis patients.
Neuromuscular Diseases. 2018;8(3):12-18
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Falls in the stationary for patients with cardiovascular diseases of the senior age and polypharmacy

Ilina E.S., Bogova O.T., Sinitsina I.I., Puzin S.N., Sychev D.A.

Abstract

Background. The fall in the elderly is observed much more often than in younger people, and according to statistics the number of people over 65 years who have suffered a fall during the year reaches 30 %, while the probability of injury. Most of the falls in elderly patients does not lead to injury, but the developing at the same time unfavorable psychological discomfort can sharply limit the usual activity, lead to the formation of dependence on outside help, disadaptation in the home and form a fear of a possible fall. Falls, accompanied by the development of trauma, are observed in 10–15 % of cases.

The objective was to study the relationship between polymorbidity, polypragmasia and the development of the fall in elderly patients cardiological profile.

Results. Polymorbidity prevailed in the group of patients who suffered a fall in the hospital (8.9 ± 2.6 vs 7.5 ± 1.5 in men. 8.5 ± 2.2 vs 7.2 ± 1.6 in women). In patients undergoing a decline, it was also noted, and polypharmacy to a greater extent than cohort patients without falling (9.4 ± 1.9 vs 78.4 ± 2.2 in men. and 9.6 ± 2.7 vs 9.1 ± 2.5 for women). In addition, patients who suffered a fall in the hospital by age were older than patients in the comparison group (86 ± 6 vs 85 ± 5 in men, 87 ± 5 vs 84 ± 5 in women). We also revealed the tendency of the predominance of women in the subgroup of patients with the incidence and development of trauma.

Conclusion. Minimizing the amount of drugs received, as the fight against polypragmasy can help reduce the incidence of falls in elderly patients. 

Neuromuscular Diseases. 2018;8(3):19-27
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ORIGINAL REPORTS

Clinical and genetic characteristics of new allele variants of the Mowat–Wilson syndrome caused by ZEB2 gene mutations

Akimova I.A., Markova T.V., Konovalov F.A., Antonets A.V., Dadali E.L.

Abstract

To date, a large number of monogenic diseases and syndromes, in the clinical picture of which there are convulsions, a psycho-speech development delay and dysmorphic features have been described. One of the hereditary syndromes with a specific phenotype is the Movat–Wilson syndrome. To diagnose the syndrome, a set of survey methods was used: genealogical analysis, neurological examination, evaluation of intellectual development with the help of psychological tests, and sequencing of the new generation exome. As a result of sequencing exome on the panel of genes responsible for the emergence of hereditary epilepsy, two patients of different sex at the age of 10 and 5 years were identified with previously not described mutations in the ZEB2 gene in the heterozygous state. Clinical manifestations of the disease in these patients were of varying degrees of severity, which can be explained in terms of the functional significance of the changes detected. The variety of clinical manifestations of the same disease leads to considerable difficulties in diagnosing, however, due to the introduction of the nextgeneration sequencing in medical practice, the effectiveness of diagnosing hereditary diseases and syndromes, the verification of which has been difficult for a long time, has increased significantly.
Neuromuscular Diseases. 2018;8(3):28-33
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Chronic pain, depression and cognitive impairment: a close relationship

Latysheva N.V., Filatova E.G., Osipova D.V.

Abstract

Over a half of chronic pain (CP) patients present with cognitive complaints, which increase their disability and impact quality of life. The paper reviews objective impairments in memory, attention, processing speed and executive function demonstrated in the CP population. The paper also reviews common pathology underlying cognitive impairment and CP: neuroplasticity in the shared brain areas, neurotransmitter and other molecular mechanisms. Common mechanisms in CP and depression precipitating cognitive impairment are also discussed. The paper also compares the potential of different antidepressants to improve cognitive functions in depression and CP.
Neuromuscular Diseases. 2018;8(3):34-42
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Validation of the Leeds Assessment of Neuropathic Symptoms and Signs Pain Scale (LANSS) in Russia

Yusupova D.G., Suponeva N.A., Zimin A.A., Zaytsev A.B., Bennett M., Belova N.V., Gushcha A.O., Chechotkin A.O., Gatina G.A., Polekhina N.V., Bundhun P., Ashrafov V.M., Piradov M.A.

Abstract

Background. Objectivization of the characteristics of neuropathic pain is an important component of differential diagnosis in various diseases. Scales and questionnaires are topical diagnostic tools for assessing subjective symptoms for patients and health professionals.

Objective to development and validation of the Russian version of the the Leeds Assessment of Neuropathic Symptoms and Signs Pain Scale (LANSS).

Materials and methods. The study included two stages: linguocultural ratification and assessment of the psychometric properties of the developed Russian version.

Results. The study showed a high level of reliability, validity and sensitivity of the developed Russian-language version of the scale. Conclusion. By the results of the study, the official validated Russian-language version of the scale is presented and recommended for use in work with Russian-speaking patients. 

Neuromuscular Diseases. 2018;8(3):43-50
pages 43-50 views
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CLINICAL DISCUSSION

Clinical case study of Kearns–Sayre syndrome: diagnosis, methods of treatment

Vlasenko S.V., Ponomarenko E.N., Kushnir G.M., Korsunskaya L.L., Larina N.V., Shalanin V.V., Usulceva N.I.

Abstract

The article outlines the current knowledge of the etiology, pathogenesis, clinical features and diagnostic criteria of one of the forms of mitochondrial encephalomyopathy – the Kearns–Sayre syndrome. The observation of a patient with an incomplete case of the Kearns–Sayre syndrome is presented. The complexity of diagnosis and the range of differential diagnostic search as well as approaches to treatment with the use of neurotrophic factors are widely discussed in the research.
Neuromuscular Diseases. 2018;8(3):51-57
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Features of high-dose intravenous immunotherapy administration in patients with reduced IgA level in neurological practice: literature review and description of a clinical case

Rizvanova A.S., Belova N.V., Raskurazhev A.A., Grishina D.A., Litvinov N.I., Kutepov D.E., Ryabinkina Y.V., Latysheva T.V., Suponeva N.A.

Abstract

Intravenous high-dose immunotherapy is one of the highly effective proven treatments for a number of autoimmune diseases of the nervous system. Nowadays there is enough knowledge about the spectrum of side effects and approaches to their prevention and monitoring. Most of them are leveled by improving the technology of the production process, but anaphylactic reactions remain one of the uncontrolled, albeit rare, undesirable reactions. The development of allergic reactions is associated with the presence of antibodies to immunoglobulin class A (IgA) in the patient, which is manifested by a decrease in the level of IgA during routine immunological examination. The article provides a review of the literature on the prevalence of IgA deficiency, its causes. A modern view of the need for routine testing of IgA level before the course of intravenous immunotherapy, approaches to reduce the risk of developing serious adverse reactions in such cases is described. A clinical case of treatment of a patient with chronic inflammatory demyelinating polyneuropathy and a reduced level of IgA is presented.
Neuromuscular Diseases. 2018;8(3):58-64
pages 58-64 views
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LETTER TO THE EDITOR

Response to the article “Bickerstaff brainstem encephalitis, acute transverse myelitis, and acute motor axonal neuropathy: diagnostic and treatment challenges in patients with concomitant syndromes. Clinical observation”

Suponeva N.A., Bakulin I.S., Rizvanova A.S., Konovalov R.N., Konovalov Y.E.

Abstract

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Neuromuscular Diseases. 2018;8(3):65-67
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Reply to the letter to the editor

Editorial a.

Abstract

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Neuromuscular Diseases. 2018;8(3):68-69
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INFORMATION FOR AUTHORS

Information for Authors

Editorial a.

Abstract

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Neuromuscular Diseases. 2018;8(3):70
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