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<article xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="other" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Neuromuscular Diseases</journal-id><journal-title-group><journal-title xml:lang="en">Neuromuscular Diseases</journal-title><trans-title-group xml:lang="ru"><trans-title>Нервно-мышечные болезни</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2222-8721</issn><issn publication-format="electronic">2413-0443</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">300</article-id><article-id pub-id-type="doi">10.17650/2222-8721-2018-8-4-12-18</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>LECTURES AND REVIEWS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>ЛЕКЦИИ И ОБЗОРЫ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Amyotrophic lateral sclerosis: pathogenetic mechanisms and new approaches to pharmacotherapy (literature review)</article-title><trans-title-group xml:lang="ru"><trans-title>Боковой амиотрофический склероз: механизмы патогенеза и новые подходы к фармакотерапии (обзор литературы)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4441-1165</contrib-id><name-alternatives><name xml:lang="en"><surname>Alekseeva</surname><given-names>T. M.</given-names></name><name xml:lang="ru"><surname>Алексеева</surname><given-names>Т. М.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>2 Akkuratova St., Saint Petersburg 197341, Russia</italic></p></bio><bio xml:lang="ru"><p><italic>197341 Санкт-Петербург, ул. Аккуратова, 2</italic></p></bio><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Stuchevskaya</surname><given-names>T. R.</given-names></name><name xml:lang="ru"><surname>Стучевская</surname><given-names>Т. Р.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>5 Uchebny Lane, Saint Petersburg 194354, Russia</italic></p></bio><bio xml:lang="ru"><p><italic>194354 Санкт-Петербург, Учебный пер., 5</italic></p></bio><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3379-9069</contrib-id><name-alternatives><name xml:lang="en"><surname>Demeshonok</surname><given-names>V. S.</given-names></name><name xml:lang="ru"><surname>Демешонок</surname><given-names>В. С.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p><italic>47 Piskarevsky Ave., Saint Petersburg 195067, Russia</italic></p></bio><bio xml:lang="ru"><p>Вера Сергеевна Демешонок </p><p> </p><p>195067 Санкт-Петербург, просп. Пискаревский, 47</p></bio><email>vera3009.85@mail.ru</email><xref ref-type="aff" rid="aff3"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">V.A. Almazov National Medical Research Center, Ministry of Health of Russia</institution></aff><aff><institution xml:lang="ru">ФГБУ «Национальный медицинский исследовательский центр им. В.А. Алмазова» Минздрава России</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">City Multi-Speciality Hospital No. 2</institution></aff><aff><institution xml:lang="ru">СПб ГБУЗ «Городская многопрофильная больница № 2»</institution></aff></aff-alternatives><aff-alternatives id="aff3"><aff><institution xml:lang="en">North-Western State Medical University n. a. I.I. Mechnikov, Ministry of Health of Russia</institution></aff><aff><institution xml:lang="ru">ФГБОУ ВО «Северо-Западный государственный медицинский университет им. И.И. Мечникова» Минздрава России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2019-01-12" publication-format="electronic"><day>12</day><month>01</month><year>2019</year></pub-date><volume>8</volume><issue>4</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>12</fpage><lpage>18</lpage><history><date date-type="received" iso-8601-date="2019-01-11"><day>11</day><month>01</month><year>2019</year></date><date date-type="accepted" iso-8601-date="2019-01-11"><day>11</day><month>01</month><year>2019</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2019, Alekseeva T.M., Stuchevskaya T.R., Demeshonok V.S.</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2019, Алексеева Т.М., Стучевская Т.Р., Демешонок В.С.</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="en">Alekseeva T.M., Stuchevskaya T.R., Demeshonok V.S.</copyright-holder><copyright-holder xml:lang="ru">Алексеева Т.М., Стучевская Т.Р., Демешонок В.С.</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://nmb.abvpress.ru/jour/article/view/300">https://nmb.abvpress.ru/jour/article/view/300</self-uri><abstract xml:lang="en"><p><italic>Amyotrophic lateral sclerosis is a neurodegenerative disease, resulting in the loss of self-service and death of the middle-aged and elderly people. In the last 2 decades, significant progress has been made in the study of the pathogenesis of this disease. Two known drugs (riluzole and edaravone) have been approved by the Food and Drug Administration for treatment of amyotrophic lateral sclerosis. The efficacy of these drugs is extremely low, so clinical trials of new drugs are ongoing all over the world. This review discusses the current achievements and future directions of therapy of this disease. </italic></p></abstract><trans-abstract xml:lang="ru"><p><italic>Боковой амиотрофический склероз – тяжелое нейродегенеративное заболевание, обусловленное поражением двигательных нейронов головного и спинного мозга и приводящее к тяжелой инвалидизации и смерти через 3–5 лет с момента его дебюта. В последние 2 десятилетия достигнут значительный прогресс в изучении патогенетических механизмов развития данного заболевания. Рилузол и эдаравон, одобренные Управлением по контролю за качеством пищевых продуктов и лекарственных препаратов (Food and Drug Administration) в 2003 и 2017 гг. для лечения бокового амиотрофического склероза, оказались недостаточно эффективны, поэтому продолжается поиск новых препаратов. В статье обсуждаются современные достижения и некоторые перспективные направления патогенетической терапии бокового амиотрофического склероза.</italic></p></trans-abstract><kwd-group xml:lang="en"><kwd>motor neuron disease</kwd><kwd>amyotrophic lateral sclerosis</kwd><kwd>pathogenetic therapy</kwd><kwd>clinical trial</kwd><kwd>riluzole</kwd><kwd>edaravone</kwd><kwd>nuedexta</kwd><kwd>masitinib</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>болезнь моторных нейронов</kwd><kwd>боковой амиотрофический склероз</kwd><kwd>патогенетическая терапия</kwd><kwd>клинические исследования</kwd><kwd>рилузол</kwd><kwd>эдаравон</kwd><kwd>нудекста</kwd><kwd>маситиниб</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Logroscino G., Traynor B.J., Hardiman O. et al. Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry 2010;81(4):385–90. DOI: 10.1136/jnnp.2009.183525. 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