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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">helmholtzeyeinstitute</journal-id><journal-title-group><journal-title xml:lang="ru">Российский офтальмологический журнал</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Ophthalmological Journal</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2072-0076</issn><issn pub-type="epub">2587-5760</issn><publisher><publisher-name>Real time Publishers</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.21516/2072-0076-2019-12-1-56-63</article-id><article-id custom-type="elpub" pub-id-type="custom">helmholtzeyeinstitute-223</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>КЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CLINICAL STUDIES</subject></subj-group></article-categories><title-group><article-title>Структурные изменения сетчатки и хориоидеи при болезни Гентингтона</article-title><trans-title-group xml:lang="en"><trans-title>Retinal and choroidal morphological changes in Huntington's disease</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Светозарский</surname><given-names>С. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Svetozarskiy</surname><given-names>S. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>аспирант кафедры глазных болезней</p><p>603000, Н. Новгород, пл. Минина и Пожарского, д. 10/1</p></bio><bio xml:lang="en"><p>Ph.D. student, department of eye diseases</p><p>10/1, Minin and Pozharsky Square, Nizhny Novgorod, 603005, Russia</p></bio><email xlink:type="simple">svetozarskij@rambler.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Копишинская</surname><given-names>С. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Kopishinskaya</surname><given-names>S. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>канд. мед. наук, доцент кафедры неврологии, психиатрии и наркологии</p><p>603000, Н. Новгород, пл. Минина и Пожарского, д. 10/1</p></bio><bio xml:lang="en"><p>Cand. Med. Sci., associate professor, department of neurology, psychiatry and addiction medicine, postgraduate faculty</p><p>10/1, Minin and Pozharsky Square, Nizhny Novgorod, 603005, Russia</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Сметанкин</surname><given-names>И. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Smetankin</surname><given-names>I. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д-р мед. наук, заведующий кафедрой глазных болезней</p><p>603000, Н. Новгород, пл. Минина и Пожарского, д. 10/1</p></bio><bio xml:lang="en"><p>Dr. Med. Sci., head of chair of ophthalmology</p><p>10/1, Minin and Pozharsky Square, Nizhny Novgorod, 603005, Russia</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБОУ ВО «Приволжский исследовательский медицинский университет»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Privolzhsky Research Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2019</year></pub-date><pub-date pub-type="epub"><day>15</day><month>03</month><year>2019</year></pub-date><volume>12</volume><issue>1</issue><fpage>56</fpage><lpage>63</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Светозарский С.Н., Копишинская С.В., Сметанкин И.Г., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Светозарский С.Н., Копишинская С.В., Сметанкин И.Г.</copyright-holder><copyright-holder xml:lang="en">Svetozarskiy S.N., Kopishinskaya S.V., Smetankin I.G.</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://roj.igb.ru/jour/article/view/223">https://roj.igb.ru/jour/article/view/223</self-uri><abstract><p>Цель исследования — изучение структур хориоидеи и сетчатки у пациентов с болезнью Гентингтона (БГ) с помощью оптической когерентной томографии (ОКТ) и анализ связи исследуемых показателей с клиническими характеристиками. Материал и методы. В исследовании участвовали две группы пациентов: 44 пациента (средний возраст — 37,6 ± 10,2 года) с БГ и 31 (средний возраст — 37,3 ± 10,8 года) практически здоровый доброволец. В основной группе 21 пациент находился на преманифестной стадии, 23 — на манифестной стадии БГ. Группы были сопоставимы по возрасту, полу, уровню внутриглазного давления и клинической рефракции. Все пациенты прошли детальное неврологическое и офтальмологическое обследование, включавшее ОКТ сетчатки. Оценивали толщину хориоидеи в области фовеа, толщину сетчатки в 9 областях макулярной зоны, толщину комплекса ганглиозных клеток сетчатки (ГКС) и слоя нервных волокон сетчатки (СНВС) перипапиллярной области в 4 квадрантах. У пациентов с БГ оценивали количество ЦАГ-повторов (цитозин — аденин — гуанин) в гене гентингтина, длительность заболевания и балл по двигательной шкале Унифицированной шкалы оценки БГ (UHDRS). Результаты. У пациентов основной группы количество ЦАГ-повторов в гене гентингтина варьировало от 37 до 56 (44,3 ± 3,8), балл по двигательной шкале UHDRS составил 36,3 ± 29,7, длительность заболевания — 13,7 ± 7,2 года. По данным ОКТ, при БГ выявлено снижение толщины хориоидеи субфовеально, комплекса ГКС, средней толщины СНВС и толщины СНВС в височном, нижнем и назальном квадранте, общей толщины сетчатки в наружном височном секторе. Кроме того, обнаружена обратная корреляция между длительностью заболевания, количеством баллов по шкале UHDRS и рядом ОКТ-параметров. Заключение. Полученные результаты подтверждают перспективность применения ретинотомографических параметров в качестве биомаркера для ранней диагностики и мониторинга прогрессии нейродегенеративного процесса. Топография изменений указывает на специфический паттерн ретинальной нейродегенерации при БГ.</p></abstract><trans-abstract xml:lang="en"><p>Purpose: to investigate the choroidal and retinal morphology in Huntington's disease (HD) using optical coherence tomography (OCT) and to analyze how the parameters studied correlate with the clinical data. Material and methods. The study included two groups of subjects, (1) 44 HD patients, averagely aged 37.6 ± 10.2 yrs, and (2) 31 healthy volunteers, averagely aged 37.3 ± 10.8 yrs. The groups had matching age, sex distribution, intraocular pressure and mean refractive error. In the study group, 21 patients had pre-manifest and 23, manifest HD stage. All patients underwent a thorough neurological and ophthalmic examination which included retinal OCT. The foveal choroidal thickness, retinal thickness in 9 areas of the macular zone, retinal ganglion cells complex (GCC) and peripapillary retinal nerve fiber layer thickness (RNFL) were evaluated in 4 quadrants. CAG repeat expansion size (cytosine-adenine-guanine) in the huntingtin gene, the disease duration and Unified HD Rating Scale motor scores (UHDRS) were evaluated for HD patients. Results. The range of the CAG repeat expansion size in the study group was 37–56 repeats (44.3 ± 3.8), the UHDRS motor score was 36.3 ± 29.7, disease duration was 13.7 ± 7.2 years. OCT revealed a significant decrease in the foveal choroidal thickness, GCC complex thickness, average, temporal, inferior and nasal RNFL thickness and total retinal thickness in the external temporal area in HD patients as compared to the controls. In addition, an inverse correlation between the disease duration, UHDRS Motor Score and a number of OCT parameters was found. Conclusion. The results confirm the promising potential of retinal tomographic parameters as a biomarker for early diagnosis and monitoring of the neurodegenerative process progression. The topography of retinal thickness reduction indicates a specific pattern of retinal neurodegeneration in HD. </p></trans-abstract><kwd-group xml:lang="ru"><kwd>оптическая когерентная томография</kwd><kwd>болезнь Гентингтона</kwd><kwd>нейродегенерация</kwd><kwd>зрительный нерв</kwd><kwd>биомаркер</kwd></kwd-group><kwd-group xml:lang="en"><kwd>optical coherence tomography</kwd><kwd>Huntington's disease</kwd><kwd>neurodegeneration</kwd><kwd>optic nerve</kwd><kwd>biomarker</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">McColgan P., Tabrizi S. Huntington's disease: a clinical review. Eur. J. 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