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<article article-type="research-article" dtd-version="1.3" 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" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">mes</journal-id><journal-title-group><journal-title xml:lang="ru">Экстремальная биомедицина</journal-title><trans-title-group xml:lang="en"><trans-title>Extreme Medicine</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">3033-8964</issn><issn pub-type="epub">3033-8972</issn><publisher><publisher-name>Centre for Strategic Planning of the Federal Medical and Biological Agency</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.47183/mes.2025-310</article-id><article-id custom-type="elpub" pub-id-type="custom">mes-310</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ГЛАВНАЯ ТЕМА: АКТУАЛЬНЫЕ ВОПРОСЫ КЛИНИЧЕСКОЙ НЕВРОЛОГИИ И ИНСТРУМЕНТАЛЬНОЙ ДИАГНОСТИКИ ЗАБОЛЕВАНИЙ НЕРВНОЙ СИСТЕМЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>MAIN TOPIC: TOPICAL ISSUES OF CLINICAL NEUROLOGY &amp; NEUROLOGICAL DISORDER INSTRUMENTAL DIAGNOSTICS</subject></subj-group></article-categories><title-group><article-title>Особенности дифференциального диагноза быстропрогрессирующей болезни Альцгеймера</article-title><trans-title-group xml:lang="en"><trans-title>Differential diagnosis features of rapidly progressive Alzheimer’s disease</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2797-7877</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мартынов</surname><given-names>М. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Martynov</surname><given-names>M. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Мартынов Михаил Юрьевич, д-р мед. наук, член-корреспондент РАН, профессор</p><p>Москва</p></bio><bio xml:lang="en"><p>Mikhail Yu. Martynov, Dr. Sci. (Med.), Corresponding Member of the RAS, Professor</p><p>Moscow</p></bio><email xlink:type="simple">m-martin@inbox.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6327-3546</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Боголепова</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Bogolepova</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Боголепова Анна Николаевна, д-р мед. наук, профессор</p><p>Москва</p></bio><bio xml:lang="en"><p>Annа N. Bogolepova, Dr. Sci. (Med.), Professor</p><p>Moscow</p></bio><email xlink:type="simple">annabogolepova@yandex.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-8606-5715</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Махнович</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Makhnovich</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Махнович Екатерина Владимировна, канд. мед. наук</p><p>Москва</p></bio><bio xml:lang="en"><p>Ekaterina V. Makhnovich, Cand. Sci. (Med.)</p><p>Moscow</p></bio><email xlink:type="simple">Ekaterinamakhnovich@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-0828-9868</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Коваленко</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kovalenko</surname><given-names>E. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Коваленко Екатерина Андреевна, канд. мед. наук</p><p>Москва</p></bio><bio xml:lang="en"><p>Ekaterina A. Kovalenko, Cand. Sci. (Med.)</p><p>Moscow</p></bio><email xlink:type="simple">ekaterinakov90@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Федеральный центр мозга и нейротехнологий Федерального медико-биологического агентства; Российский национальный исследовательский медицинский университет им. Н.И. Пирогова</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Federal Center of Brain Research and Neurotechnologies; Pirogov Russian National Research Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2025</year></pub-date><pub-date pub-type="epub"><day>26</day><month>06</month><year>2025</year></pub-date><volume>27</volume><issue>2</issue><fpage>152</fpage><lpage>160</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Мартынов М.Ю., Боголепова А.Н., Махнович Е.В., Коваленко Е.А., 2025</copyright-statement><copyright-year>2025</copyright-year><copyright-holder xml:lang="ru">Мартынов М.Ю., Боголепова А.Н., Махнович Е.В., Коваленко Е.А.</copyright-holder><copyright-holder xml:lang="en">Martynov M.Y., Bogolepova A.N., Makhnovich E.V., Kovalenko E.A.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://www.extrememedicine.ru/jour/article/view/310">https://www.extrememedicine.ru/jour/article/view/310</self-uri><abstract><sec><title>Введение</title><p>Введение. Спектр патологий и состояний, которые могут приводить к развитию быстропрогрессирующей деменции (БПД), достаточно обширен. Наиболее распространенной причиной развития деменции является болезнь Альцгеймера (БА), однако существуют и другие патологии, которые, в отличие от БА, являются излечимыми, и при верной постановке диагноза возможно достижение полного регресса патологической симптоматики. Вышесказанное повышает значимость дифференциальной диагностики быстропрогрессирующей БА с другими причинами БПД.</p></sec><sec><title>Цель</title><p>Цель. Определить дифференциальные особенности быстропрогрессирующей БА и изучить основные причины, предрасполагающие к развитию БПД и не связанные с нейродегенеративной патологией.</p></sec><sec><title>Обсуждение</title><p>Обсуждение. Быстропрогрессирующая БА отличается от типичной БА скоростью когнитивного снижения: в среднем при быстропрогрессирующей БА отмечается потеря 3-х баллов или более по Краткой шкале оценки психического статуса в течение шести месяцев и более быстрое (за 2–3 года) достижение терминальной стадии заболевания, в то время как при типичной БА этот период длительнее и составляет порядка 8–10 лет. К другим основным причинам БПД относятся прионные заболевания, нейродегенеративные заболевания неприонной этиологии (в том числе быстропрогрессирующая БА), cосудистые, инфекционные, воспалительные и аутоиммунные, онкологические заболевания, метаболические и дефицитарные нарушения, эндокринные расстройства, токсические и ятрогенные нарушения, психические заболевания, цереброваскулярная патология.</p></sec><sec><title>Выводы</title><p>Выводы. Выявление причины БПД требует детального и всестороннего осмотра пациента с проведением различных лабораторных и инструментальных методов исследования, что является залогом верной постановки диагноза и дальнейшей успешной медикаментозной коррекции курабельных заболеваний. Основную роль в постановке диагноза быстропрогрессирующей БА и дифференциальной диагностике с другими причинами БПД играет позитронно-эмисионная томография головного мозга и уровень биомаркеров, таких как бета-амилоид и гиперфосфорилированный тау-протеин, в цереброспинальной жидкости.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Introduction</title><p>Introduction. The range of pathologies and conditions that can lead to the development of rapidly progressive dementia (RPD) is rather extensive. Alzheimer’s disease (AD) is considered the most common cause of dementia. However, there are other pathologies that, unlike AD, are curable, and, given accurate diagnosis, allow a complete regression of pathological symptoms to be achieved. This highlights the importance of differential diagnosis of rapidly progressing AD from other causes of RPD.</p></sec><sec><title>Objective</title><p>Objective. To determine the differential features of rapidly progressing AD and to study the main causes predisposing to the development of RPD but not related to neurodegenerative pathology.</p></sec><sec><title>Discussion</title><p>Discussion. Rapidly progressing AD differs from typical AD in the rate of cognitive decline. On average, rapidly progressing AD is associated with a loss of three points or more scores on the Mini-Mental State Examination (MMSE) test within six months and a faster (in 2–3 years) achievement of the terminal stage of the disease. In case of typical AD, this period is longer, lasting for about 8–10 years. Other major causes of RPD include prion diseases, neurodegenerative diseases of non-prion etiology (including rapidly progressing AD), vascular diseases, infectious diseases, inflammatory and autoimmune diseases, oncological diseases, metabolic and deficiency disorders, endocrine disorders, toxic and iatrogenic disorders, mental diseases, and cerebrovascular pathology.</p></sec><sec><title>Conclusions</title><p>Conclusions. Identification of the RPD cause requires a detailed and comprehensive examination of the patient using various laboratory and instrumental research methods, which is the key to accurate diagnosis and further successful drug correction of terminal diseases. Positron emission tomography of the brain and such biomarkers as beta-amyloid and hyperphosphorylated tau protein in the cerebrospinal fluid play a major role in the diagnosis of rapidly progressive AD and differential diagnosis from other RPD causes.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>быстропрогрессирующая болезнь Альцгеймера</kwd><kwd>быстрогрессирующая деменция</kwd><kwd>когнитивные нарушения</kwd><kwd>дифференциальная диагностика</kwd><kwd>цереброваскулярные заболевания</kwd><kwd>аутоиммунный энцефалит</kwd></kwd-group><kwd-group xml:lang="en"><kwd>rapidly progressing Alzheimer’s disease</kwd><kwd>rapidly progressive dementia</kwd><kwd>cognitive impairment</kwd><kwd>differential diagnosis</kwd><kwd>cerebrovascular diseases</kwd><kwd>autoimmune encephalitis</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Боголепова АН, Васенина ЕЕ, Гомзякова НА, Гусев ЕИ, Дудченко НГ и др. 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