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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">657</article-id><article-id pub-id-type="doi">10.17650/2222-8721-2025-15-2-37-47</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>ORIGINAL REPORTS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">Factors influencing the progression of amyotrophic lateral sclerosis: prospective cohort study</article-title><trans-title-group xml:lang="ru"><trans-title>Факторы, влияющие на прогрессирование бокового амиотрофического склероза: проспективное когортное исследование</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0076-2593</contrib-id><name-alternatives><name xml:lang="en"><surname>Smirnova</surname><given-names>A. Yu.</given-names></name><name xml:lang="ru"><surname>Смирнова</surname><given-names>А. Ю.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>Anna Yuryevna Smirnova,</p><p>2, Akkuratova St., Saint Petersburg 197341.</p></bio><bio xml:lang="ru"><p>Анна Юрьевна Смирнова,</p><p>197341 Санкт-Петербург, ул. Аккуратова, 2.</p></bio><email>dr.a.smirnova@gmail.com</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6244-0867</contrib-id><name-alternatives><name xml:lang="en"><surname>Druzhinin</surname><given-names>D. S.</given-names></name><name xml:lang="ru"><surname>Дружинин</surname><given-names>Д. С.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>8, Build. 2, Trubetskaya St., Moscow 119991.</p></bio><bio xml:lang="ru"><p>119991 Москва, ул. Трубецкая, 8, стр. 2.</p></bio><xref ref-type="aff" rid="aff2"/></contrib><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>2, Akkuratova St., Saint Petersburg 197341.</p></bio><bio xml:lang="ru"><p>197341 Санкт-Петербург, ул. Аккуратова, 2.</p></bio><xref ref-type="aff" rid="aff1"/></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>2, Akkuratova St., Saint Petersburg 197341.</p></bio><bio xml:lang="ru"><p>197341 Санкт-Петербург, ул. Аккуратова, 2.</p></bio><xref ref-type="aff" rid="aff1"/></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. B. A. Алмaзoвa» Минздрава России</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia</institution></aff><aff><institution xml:lang="ru">ФГАОУ ВО Первый Московский государственный медицинский университет им. И. М. Сеченова (Сеченовский Университет) Минздрава России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2025-09-23" publication-format="electronic"><day>23</day><month>09</month><year>2025</year></pub-date><volume>15</volume><issue>2</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>37</fpage><lpage>47</lpage><history><date date-type="received" iso-8601-date="2025-09-22"><day>22</day><month>09</month><year>2025</year></date><date date-type="accepted" iso-8601-date="2025-09-22"><day>22</day><month>09</month><year>2025</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2025, Smirnova A.Y., Druzhinin D.S., Alekseeva T.M., Demeshonok V.S.</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2025, Смирнова А.Ю., Дружинин Д.С., Алексеева Т.М., Демешонок В.С.</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="en">Smirnova A.Y., Druzhinin D.S., Alekseeva T.M., Demeshonok V.S.</copyright-holder><copyright-holder xml:lang="ru">Смирнова А.Ю., Дружинин Д.С., Алексеева Т.М., 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/657">https://nmb.abvpress.ru/jour/article/view/657</self-uri><abstract xml:lang="en"><p><bold>Aim. </bold>Using factor analysis, identify and rank the parameters potentially influencing the course of amyotrophic lateral sclerosis (ALS) according to their significance.</p><p><bold>Materials and methods.</bold> A prospective cohort study of 70 patients with ALS diagnosed according to the Gold Coast criteria (2020) was performed. We studied basic socio-demographic indicators, education and social status, geography of living, anamnestic data about the disease (appearance of the first symptoms, onset, etc.), clinical picture (symptoms in the main muscle groups), progression of ALSFRS-R score, environmental risk factors (including 23 features, presented in gradation from the lowest to the highest expression).</p><p><bold>Results.</bold> The mean age of the patients was 60.62 ± 10.2 years. The predominant proportion of patients was female,</p><p>61.4 % (<italic>n = </italic>43), the proportion of males was 38.6 % (<italic>n = </italic>27). The mean time from onset of symptoms to inclusion in the study and questionnaire was 12 months (95 % confidence interval 9.00–21.50). Low level of education, onset from bulbar symptoms and neck muscle involvement, severity of oral automatism reflexes, difficulty in walking and standing, contact with heavy metals, occupational sports and high stress level were correlated with rapid progression of ALS, while high level of physical activity, frequent caffeine consumption, antioxidant intake, vitamin D deficiency were observed in slow progression of ALS.</p><p><bold>Conclusion.</bold> Our data confirm that ALS is a clinically and pathogenetically heterogeneous disease, whose rate of progression is influenced by a wide range of clinical, anamnestic, and environmental factors.</p></abstract><trans-abstract xml:lang="ru"><p><bold>Цель исследования </bold>– посредством факторного анализа выявить и ранжировать по степени значимости параметры, потенциально влияющие на течение бокового амиотрофического склероза (БАС).</p><p><bold>Материалы и методы.</bold> Проведено проспективное когортное исследование 70 пациентов с диагнозом БАС, соответствующих критериям Gold Coast (2020). Изучены основные социально-демографические показатели, сведения об образовании и социальном положении, география проживания, анамнестические сведения о болезни (появление первых симптомов, дебют и пр.), клиническая картина (симптомы по основным группам мышц), динамика оценки по шкале ALSFRS-R, факторы окружающей среды (23 признака, представленных в градации от наименьшей выраженности к наибольшей).</p><p><bold>Результаты.</bold> Средний возраст пациентов – 60,62 ± 10,2 года. Среди пациентов преобладали женщины – 61,4 % (<italic>n</italic><italic> = </italic>43), доля мужчин – 38,6 % (<italic>n</italic><italic> = </italic>27). Среднее время от начала симптомов до включения в исследование и анкетирования – 12 мес (95 % доверительный интервал 9,00–21,50). С быстрым прогрессированием БАС коррелировали низкий уровень образования, дебют с бульбарных симптомов и вовлечения мышц шеи, выраженность рефлексов орального автоматизма, затруднение при ходьбе и вставании, контакт с тяжелыми металлами, занятие профессиональным спортом и высокий уровень стресса, тогда как высокий уровень физической нагрузки, частое употребление кофе, прием антиоксидантов, дефицит витамина D отмечались при медленном прогрессировании БАС.</p><p><bold>Выводы.</bold> Полученные данные подтверждают, что БАС представляет собой клинически и патогенетически гетерогенное заболевание, на скорость прогрессирования которого влияет большое количество клинико-анамнестических и средовых факторов.</p></trans-abstract><kwd-group xml:lang="en"><kwd>amyotrophic lateral sclerosis</kwd><kwd>pattern of progression</kwd><kwd>prognostic factor</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>боковой амиотрофический склероз</kwd><kwd>характер течения</kwd><kwd>прогностический фактор</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">Van Es M. 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