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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">uzspbgmu</journal-id><journal-title-group><journal-title xml:lang="ru">Учёные записки Первого Санкт-Петербургского государственного медицинского университета имени академика И. П. Павлова</journal-title><trans-title-group xml:lang="en"><trans-title>The Scientific Notes of the Pavlov University</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1607-4181</issn><issn pub-type="epub">2541-8807</issn><publisher><publisher-name>Academician I.P. Pavlov First St. Petersburg State Medical University</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.24884/1607-4181-2023-30-3-84-91</article-id><article-id custom-type="elpub" pub-id-type="custom">uzspbgmu-1002</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>НАБЛЮДЕНИЯ ИЗ ПРАКТИКИ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>OBSERVATION FROM PRACTICE</subject></subj-group></article-categories><title-group><article-title>Транзиторная глобальная амнезия у пациента с церебральной аутосомно-доминантной артериопатией с подкорковыми инфарктами и лейкоэнцефалопатией (ЦАДАСИЛ)</article-title><trans-title-group xml:lang="en"><trans-title>Transient global amnesia in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts, and leukoencephalopathy (CADASIL)</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-5601-5899</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Кирьянов</surname><given-names>Ю. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Kyrianov</surname><given-names>Yu. М.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Кирьянов Юрий Михайлович, врач-невролог высшей квалификационной категории, аспирант кафедры неврологии</p><p>197022, Санкт-Петербург, ул. Льва Толстого, д. 6-8</p></bio><bio xml:lang="en"><p>Kyrianov Urii M., Neurologist of the Highest Qualification Category, Postgraduate Student of the Department of Neurology</p><p>6-8, L’va Tolstogo str., Saint Petersburg, 197022</p></bio><email xlink:type="simple">dontwritehere@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-6715-8203</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Тотолян</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Totolyan</surname><given-names>N. А.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Тотолян Наталья Агафоновна, доктор медицинских наук, профессор, профессор кафедры неврологии</p><p>197022, Санкт-Петербург, ул. Льва Толстого, д. 6-8</p></bio><bio xml:lang="en"><p>Totolyan Natalia A., Dr. of Sci. (Med.), Professor, Professor of the Department of Neurology</p><p>Saint Petersburg</p><p> </p></bio><email xlink:type="simple">ntotolyan@mail.ru</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное образовательное учреждение высшего образования «Первый Санкт-Петербургский государственный&#13;
медицинский университет имени академика И. П. Павлова» Министерства здравоохранения Российской Федерации; Федеральное государственное бюджетное образовательное учреждение высшего образования «Военно-медицинская академия имени С. М. Кирова» Министерства обороны Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Pavlov University; Military Medical Academy</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное образовательное учреждение высшего образования «Первый Санкт-Петербургский государственный&#13;
медицинский университет имени академика И. П. Павлова» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Pavlov University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2023</year></pub-date><pub-date pub-type="epub"><day>19</day><month>10</month><year>2023</year></pub-date><volume>30</volume><issue>3</issue><fpage>84</fpage><lpage>91</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Кирьянов Ю.М., Тотолян Н.А., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Кирьянов Ю.М., Тотолян Н.А.</copyright-holder><copyright-holder xml:lang="en">Kyrianov Y.М., Totolyan N.А.</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.sci-notes.ru/jour/article/view/1002">https://www.sci-notes.ru/jour/article/view/1002</self-uri><abstract><p>Транзиторная глобальная амнезия (ТГА) характеризуется внезапным развитием преходящих нарушений всех видов памяти с утратой возможности запоминания, антероградной и иногда ретроградной амнезией и воспроизведением происходящих событий, при сохранности сознания, с повторяющимися вопросами, произносимыми в это время пациентом, продолжительностью менее 24 часов (обычно 1–8 часов). При этом состоянии страдают медиальные отделы височных долей и гиппокамповы извилины. Патогенез развития ТГА включает развитие венозных расстройств по типу механизма Вальсальвы, другие нарушения кровообращения и мигрень. В статье приводится обзор литературы по развитию ТГА у пациентов с рассеянным склерозом и церебральной аутосомно-доминантной артериопатией с подкорковыми инфарктами и лейкоэнцефалопатией (ЦАДАСИЛ), что нехарактерно для этих пациентов, и описание редкого наблюдения за пациенткой с установленным диагнозом ЦАДАСИЛ, у которой впервые развившийся острый сосудистый ишемический эпизод протекал в виде транзиторной глобальной амнезии. Диагноз ЦАДАСИЛ был у нее установлен ранее на основании наличия в анамнезе мигрени, характерных для заболевания изменений на МРТ головного мозга, а также молекулярно-генетического анализа, выявившего гетерозиготную мутацию в гене NOTCH3: мутация р.207 R&gt;R/C. В неврологическом статусе очаговых симптомов выявлено не было. При МРТ исследовании головного мозга в режиме диффузии (DWI) был выявлен характерный для ТГА точечный мелкий очаг поражения в области левой гиппокамповой извилины. Несмотря на частое описание мигрени и подкорковых инсультов при ЦАДАСИЛ, описания в литературе ТГА у таких пациентов единичные. Также единичны описания ТГА у пациентов с рассеянным склерозом (РС).</p></abstract><trans-abstract xml:lang="en"><p>Transient global amnesia (TGA) is characterized by a sudden onset of total memory dysfunction, and loss of the ability to memorize information, followed by anterograde, and sometimes, retrograde amnesia, and reproduction of current events with patient’s consciousness alert, accompanied by one’s repetitive questioning during this very episode that lasts less than 24 hours (usually 1-8 hours). The process involves mesial temporal lobes and hippocampi. Pathogenesis of TGA includes venous congestion with Valsalva-like activities, other vascular dysfunction, and migraine. The article contains literature review on the development of TGA in patients with Multiple Sclerosis and Cerebral Autosomal Dominant Arteriopathy and Subcortical Infarcts and Leukoencephalopathy (CADASIL), which is not common for such patients, and describes a rare clinical case of confirmed CADASIL, in whom first acute vascular ischemic attack presented by TGA developed. In this patient, CADASIL was diagnosed earlier, and was based on the presence of migraine in anamnesis, common for CADASIL patients MRI abnormalities, and the result of molecular-genetic analysis, which showed gene NOTCH3 heterozygous mutation: р.207 R&gt;R/C. Neurological examination revealed no focal neurological signs. Brain MRI in diffusion option (DWI) revealed a small pinpoint ischemic focus in left hyppocampus, which is characteristic for TGA. Despite of frequent review of migraine and subcortical ischemic events in these patients, the history of TGA is solely reported in literature. Reports of TGA development in patients with multiple sclerosis are also sole.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>транзиторная глобальная амнезия</kwd><kwd>ЦАДАСИЛ</kwd><kwd>рассеянный склероз</kwd><kwd>инсульт в области гиппокампа</kwd></kwd-group><kwd-group xml:lang="en"><kwd>transient global amnesia</kwd><kwd>Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)</kwd><kwd>multiple sclerosis4 hippocampus stroke</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">Григорьева В. Н., Нестерова В. Н., Сорокина Т. А. Транзиторная глобальная амнезия в практике невролога приемно-диагностического отделения сосудистого центра // Неврологический журнал. – 2014. – № 3. – С. 13–20.</mixed-citation><mixed-citation xml:lang="en">Grigoryeva V. N., Nesterova V. N., Sorokina T.A. 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