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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">Annals of Clinical and Experimental Neurology</journal-id><journal-title-group><journal-title xml:lang="en">Annals of Clinical and Experimental Neurology</journal-title><trans-title-group xml:lang="ru"><trans-title>Анналы клинической и экспериментальной неврологии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2075-5473</issn><issn publication-format="electronic">2409-2533</issn><publisher><publisher-name xml:lang="en">Eco-Vector</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">242</article-id><article-id pub-id-type="doi">10.17816/psaic242</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Original articles</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Оригинальные статьи</subject></subj-group><subj-group subj-group-type="article-type"><subject>Unknown</subject></subj-group></article-categories><title-group><article-title xml:lang="en">The role of cyclosporin in the treatment of severe myasthenia: a clinical study of 51 patients</article-title><trans-title-group xml:lang="ru"><trans-title>Роль циклоспорина в лечении тяжелых форм миастении: клиническое исследование 51 больного</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Shcherbakova</surname><given-names>N. I.</given-names></name><name xml:lang="ru"><surname>Щербакова</surname><given-names>Н. И.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>shnata@inbox.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Pavlova</surname><given-names>E. М.</given-names></name><name xml:lang="ru"><surname>Павлова</surname><given-names>E. М.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>shnata@inbox.ru</email><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Lantsova</surname><given-names>V. B.</given-names></name><name xml:lang="ru"><surname>Ланцова</surname><given-names>В. Б.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>shnata@inbox.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Savitskaya</surname><given-names>N. G.</given-names></name><name xml:lang="ru"><surname>Савицкая</surname><given-names>Н. Г.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>shnata@inbox.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Pavlov</surname><given-names>E. V.</given-names></name><name xml:lang="ru"><surname>Павлов</surname><given-names>E. В.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>shnata@inbox.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Retinskaya</surname><given-names>I. G.</given-names></name><name xml:lang="ru"><surname>Ретинская</surname><given-names>И. Г.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>shnata@inbox.ru</email><xref ref-type="aff" rid="aff1"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Zakutnaya</surname><given-names>V. N.</given-names></name><name xml:lang="ru"><surname>Закутняя</surname><given-names>В. Н.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><email>shnata@inbox.ru</email><xref ref-type="aff" rid="aff1"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Research Center of Neurology, Russian Academy of Medical Sciences</institution></aff><aff><institution xml:lang="ru">ФГБУ «Научный центр неврологии» РАМН</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">The A.I. Burnazian Federal Medical Biophysical Center, Federal Medical Biological Agency</institution></aff><aff><institution xml:lang="ru">Федеральный медицинский биофизический центр им. А.И. Бурназяна ФМБА России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2013-06-09" publication-format="electronic"><day>09</day><month>06</month><year>2013</year></pub-date><volume>7</volume><issue>2</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>4</fpage><lpage>10</lpage><history><date date-type="received" iso-8601-date="2017-02-02"><day>02</day><month>02</month><year>2017</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2013, Shcherbakova N.I., Pavlova E.М., Lantsova V.B., Savitskaya N.G., Pavlov E.V., Retinskaya I.G., Zakutnaya V.N.</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2013, Shcherbakova N.I., Pavlova E.М., Lantsova V.B., Savitskaya N.G., Pavlov E.V., Retinskaya I.G., Zakutnaya V.N.</copyright-statement><copyright-year>2013</copyright-year><copyright-holder xml:lang="en">Shcherbakova N.I., Pavlova E.М., Lantsova V.B., Savitskaya N.G., Pavlov E.V., Retinskaya I.G., Zakutnaya V.N.</copyright-holder><copyright-holder xml:lang="ru">Shcherbakova N.I., Pavlova E.М., Lantsova V.B., Savitskaya N.G., Pavlov E.V., Retinskaya I.G., Zakutnaya V.N.</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://annaly-nevrologii.com/pathID/article/view/242">https://annaly-nevrologii.com/pathID/article/view/242</self-uri><abstract xml:lang="en"><p> </p><p>About 15–20% of myasthenic patients are refractory to standard steroid therapy. The present study was aimed at evaluation of efficacy and analysis of the algorithm of use of a novel immunosuppressant of natural origin, cyclosporine A, in the treatment of severe refractory forms of myasthenia gravis. In this work, for the first time on a large group of myasthenic patients (51 patients who became ill at 5–80 years of age), mechanisms of action of cyclosporine (sandimmun orally 2.5–5 mg/kg) on main levels of neuro-muscular junction were studied, and new confirmation of selective immune-modulating activity of cyclosporine (without total decrease of patient’s immune system) was presented. High efficacy and good tolerability of the drug in different pathogenic subtypes of myasthenia gravis were shown. Cyclosporin may be recommended in refractory steroid-dependent myasthenia, as well as in cases of combination of myasthenia with thymoma, autoimmune disorders and immunodeficiency viral infections.</p>  <p> </p> <p> </p></abstract><trans-abstract xml:lang="ru"><p>Около 15–20% пациентов с миастенией рефрактерны к стандартной стероидной терапии. Настоящее исследование посвящено оценке эффективности и анализу алгоритма применения нового иммуносупрессанта природного происхождения – циклоспорина А – в лечении тяжелых рефрактерных форм миастении. В работе впервые на большой группе пациентов с миастенией (51 больной, заболевший в возрасте от 5 до 80 лет) изучены механизмы воздействия циклоспорина (препарат сандиммун внутрь в дозе 2,5–5 мг/кг) на основные звенья нервно-мышечной передачи, представлены новые доказательства избирательной иммуномодулирующей активности циклоспорина, без угнетения всей иммунной системы больного. Установлены высокая эффективность и хорошая переносимость препарата при разных патогенетических подтипах миастении. Циклоспорин может быть рекомендован при рефрактерной стероидзависимой миастении, а также при комбинации миастении с тимомой, аутоиммунными заболеваниями и вирусными иммунодефицитными состояниями.</p></trans-abstract><kwd-group xml:lang="en"><kwd>cyclosporine А</kwd><kwd>refractory steroid-dependent myasthenia gravis</kwd><kwd>myastheniс crises</kwd><kwd>treatment</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>циклоспорин А</kwd><kwd>рефрактерная стероидозависимая миастения</kwd><kwd>миастенические кризы</kwd><kwd>лечение</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Гехт Б.М., Ильина Н.А. Нервно-мышечные болезни. М.:Медицина,1982.</mixed-citation></ref><ref id="B2"><label>2.</label><mixed-citation>Кузин М.И., Гехт Б.М. Миастения. М.: Медицина, 1996.</mixed-citation></ref><ref id="B3"><label>3.</label><mixed-citation>Сепп Е.К., Ланцова В.Б. Миастения. М.: АНО Учебный центр «Невромед-Клиника», 2008.</mixed-citation></ref><ref id="B4"><label>4.</label><mixed-citation>Щербакова Н.И., Павлова Е.М., Санадзе А.Г. и др. Клинический случай миастенического криза: возможные механизмы развития, особенности клинического течения и тактика ведения. Неврол. журн. 2010; 3: 35–41.</mixed-citation></ref><ref id="B5"><label>5.</label><mixed-citation>Abramsky O., Tarrab-Hazdai R., Aharonov A., Fuchs S. Immunosuppression of experimental autoimmune myasthenia gravis by hydrocortisone and azathioprine. J. Immunol. 1976; 117: 225–228.</mixed-citation></ref><ref id="B6"><label>6.</label><mixed-citation>Antonioni G., Bove R., Filippini C., Millefiorini M. Results of open trial of cyclosporine in a group of steroido-dependent myasthenic subjects. J. Clin. Neurol. Nerosurg. 1990; 92: 317–321.</mixed-citation></ref><ref id="B7"><label>7.</label><mixed-citation>Aoyama K., Umegae N., Takahashi K. et al. A case of myasthenia gravis with an invasive thymoma. J. Rinsho Shinkeigaku. 1998; 38: 465–467.</mixed-citation></ref><ref id="B8"><label>8.</label><mixed-citation>Barohn R.J., McIntire D., Herbelin L. et al. Reliability testing of the quantitative myasthenia gravis score. Ann. N. Y. Acad. Sci. 1998; 841: 769–772.</mixed-citation></ref><ref id="B9"><label>9.</label><mixed-citation>Deymeer F., Gungor-Tuncer O., Yilmaz V. et al. Clinical comparison of anti-MuSK- vs anti-AChR-positive and seronegative myasthenia gravis. Neurology 2007; 68: 609–611.</mixed-citation></ref><ref id="B10"><label>10.</label><mixed-citation>Frey F.J. Cyclosporine in autoimmune diseases. J. Schweiz Med. Wochenschr. 1990; 120: 772–786.</mixed-citation></ref><ref id="B11"><label>11.</label><mixed-citation>Giraud C., Pourrat O., Badia P. et al. Are long-term plasma exchanges and cyclosporin useful in management of severe chronic respiratory failure due to myasthenia gravis? Ann. Intern. Med. 1997; 148: 504–505.</mixed-citation></ref><ref id="B12"><label>12.</label><mixed-citation>Goulon M., Elkharrat D., Gajdos P. Treatment of severe myasthenia gravis with cyclosporin. A 12-month open trial. Presse Med. 1989; 18: 341–346.</mixed-citation></ref><ref id="B13"><label>13.</label><mixed-citation>Hart I.K., Sharshar T., Sathasivam S. Immunosuppressant drugs for myasthenia gravis. J. Neurol. Neurosurg. Psychiatry 2009; 80: 5–6.</mixed-citation></ref><ref id="B14"><label>14.</label><mixed-citation>Kurokawa T., Nishiyama T., Yamamoto R. et al. Anti-MuSK antibody positive myasthenia gravis with HIV infection successfully treated with cyclosporin: a case report. Rinsho Shinkeigaku 2008; 48: 666–669.</mixed-citation></ref><ref id="B15"><label>15.</label><mixed-citation>Lavrnic D., Vujic A., Rakocevic-Stojanovic V. et al. Cyclosporine in the treatment of myasthenia gravis. Acta Neurol. Scand. 2005; 111: 247–252.</mixed-citation></ref><ref id="B16"><label>16.</label><mixed-citation>Lefvert A.K., Matell G. Antibodies against human cholinergic receptor proteins in patients with myasthenia gravis: studies during immunosupressive treatment. Preliminary report Acta Neurol. Scand. 1977; 201: 181–182.</mixed-citation></ref><ref id="B17"><label>17.</label><mixed-citation>17.Manna R., Verrecchia E., Fonnesu C. et al. Cyclosporine A: good response for patients affected by autoimmune disorders and HCV infection? Eur. Rev. Med. Pharmacol. Sci. 2009; Suppl 1: 63–69.</mixed-citation></ref><ref id="B18"><label>18.</label><mixed-citation>18.Marchiori P.E., De Assis J.L., Scaff M. Failure of treatment of myasthenia gravis by cyclosporin-A: A case report. Arq. Neuropsiquiatr. 1989; 47: 91–93.</mixed-citation></ref><ref id="B19"><label>19.</label><mixed-citation>19. Sanders D.B., Siddiqi Z.A. Lessons from two trials of Mycopfenjlate Mofetil in myasthenia gravis. Ann. N. Y. Acad. Sci. 2008; 1132: 249–253.</mixed-citation></ref><ref id="B20"><label>20.</label><mixed-citation>20. Sanders D.B., Evoli A. Immunosuppressive therapies in myasthenia gravis. Autoimmunity 2010; 43: 1–8.</mixed-citation></ref><ref id="B21"><label>21.</label><mixed-citation>21. Sato A., Katsui T., Honma A. et al. A case of myasthenia gravis treated with cyclosporine and high-dose intravenouse immunoglobulin. J. Nippon Naiaka Gakkai Zasshi. 1996; 85: 279–281.</mixed-citation></ref></ref-list></back></article>
