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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">10.24884/1607-4181-2018-25-3-89-95</article-id><article-id custom-type="elpub" pub-id-type="custom">uzspbgmu-505</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>В ПОМОЩЬ ПРАКТИЧЕСКОМУ ВРАЧУ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>PRACTICAL GUIDELINES</subject></subj-group></article-categories><title-group><article-title>СИНДРОМ  «ЗАПЕРТОГО  ЧЕЛОВЕКА»  ВСЛЕДСТВИЕ   МНОЖЕСТВЕННЫХ  ИНФАРКТОВ  СТВОЛА  ГОЛОВНОГО   МОЗГА  И  ОЧАГОВ  ДЕМИЕЛИНИЗАЦИИ  ПОСЛЕ  ЛУЧЕВОЙ  ТЕРАПИИ  АДЕНОМЫ  ГИПОФИЗА  С  АКРОМЕГАЛИЕЙ</article-title><trans-title-group xml:lang="en"><trans-title>LOCKED-IN  SYNDROME  DUE  TO  MULTIPLE  BRAIN  STEM  INFARCTIONS  AND  FOCI  OF  DEMYELINATION  AFTER  RADIATION  THERAPY   OF PITUITARY  ADENOMA  WITH  ACROMEGALY</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>Заславский</surname><given-names>Л. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Zaslavskii</surname><given-names>L. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>194291,  Санкт-Петербург, пр. Луначарского, д. 45-49.</p></bio><bio xml:lang="en"><p>45-49 Lunacharskogo street, St. Petersburg, Russia, 194291.</p></bio><email xlink:type="simple">Doctorleonid@gmail.com</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>Скорнякова</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Skorniakova</surname><given-names>E. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>194291,  Санкт-Петербург, пр. Луначарского, д. 45-49.</p></bio><bio xml:lang="en"><p>45-49 Lunacharskogo street, St. Petersburg, Russia, 194291.</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Заславская</surname><given-names>О. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Zaslavskaia</surname><given-names>O. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Санкт-Петербург.</p></bio><bio xml:lang="en"><p>St. Petersburg.</p></bio><xref ref-type="aff" rid="aff-3"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Феофанова</surname><given-names>С. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Feofanova</surname><given-names>S. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>194291,  Санкт-Петербург, пр. Луначарского, д. 45-49.</p></bio><bio xml:lang="en"><p>45-49 Lunacharskogo street, St. Petersburg, Russia, 194291.</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ломтева</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Lomteva</surname><given-names>E. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>194291,  Санкт-Петербург, пр. Луначарского, д. 45-49.</p></bio><bio xml:lang="en"><p>45-49 Lunacharskogo street, St. Petersburg, Russia, 194291.</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Оточкин</surname><given-names>В. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Otochkin</surname><given-names>V. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>194291,  Санкт-Петербург, пр. Луначарского, д. 45-49.</p></bio><bio xml:lang="en"><p>45-49 Lunacharskogo street, St. Petersburg, Russia, 194291.</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Борисов</surname><given-names>М. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Borisov</surname><given-names>M. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>194291,  Санкт-Петербург, пр. Луначарского, д. 45-49.</p></bio><bio xml:lang="en"><p>45-49 Lunacharskogo street, St. Petersburg, Russia, 194291.</p></bio><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Государственное бюджетное учреждение здравоохранения «Ленинградская областная клиническая больница»;  Федеральное государственное бюджетное образовательное учреждение высшего образования «Первый Санкт-Петербургский государственный медицинский университет имени академика И. П. Павлова» Министерства здравоохранения Российской Федерации.</institution><country>Россия</country></aff><aff xml:lang="en"><institution>State Budgetary Institution of Healthcare «Leningrad Regional Clinical Hospital»; Pavlov University.</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Государственное бюджетное учреждение здравоохранения «Ленинградская областная клиническая больница».</institution><country>Russian Federation</country></aff><aff xml:lang="en"><institution>State Budgetary Institution of Healthcare «Leningrad Regional Clinical Hospital».</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное образовательное учреждение высшего образования «Первый Санкт-Петербургский государственный медицинский университет имени академика И. П. Павлова» Министерства здравоохранения Российской Федерации.</institution><country>Russian Federation</country></aff><aff xml:lang="en"><institution>Pavlov University.</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2018</year></pub-date><pub-date pub-type="epub"><day>30</day><month>09</month><year>2018</year></pub-date><volume>25</volume><issue>3</issue><fpage>89</fpage><lpage>95</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Заславский Л.Г., Скорнякова Е.А., Заславская О.Л., Феофанова С.Г., Ломтева Е.А., Оточкин В.В., Борисов М.В., 2018</copyright-statement><copyright-year>2018</copyright-year><copyright-holder xml:lang="ru">Заславский Л.Г., Скорнякова Е.А., Заславская О.Л., Феофанова С.Г., Ломтева Е.А., Оточкин В.В., Борисов М.В.</copyright-holder><copyright-holder xml:lang="en">Zaslavskii L.G., Skorniakova E.A., Zaslavskaia O.L., Feofanova S.G., Lomteva E.A., Otochkin V.V., Borisov M.V.</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/505">https://www.sci-notes.ru/jour/article/view/505</self-uri><abstract><p> Cиндром «запертого человека» (СЗЧ) является редким неврологическим расстройством, обычно возникающим в результате поражения моста мозга, чаще всего вследствие инсульта. При СЗЧ возникает паралич скелетных мышц (дыхательные, лицевые, глотки, языка, а также конечностей), т. е. утрачивается способность к речи, мимике, произвольным движениям, дыханию. Акромегалия – заболевание, при котором наблюдается повышение уровня гормона роста (GH), инсулиноподобного фактора роста 1 (IGF-1), и развивается в большинстве случаев при аденомах гипофиза. При лечении аденомы гипофиза применяют нейрохирургические методики, медикаментозную и лучевую терапию (ЛТ). В представленном клиническом наблюдении у 33-летней женщины через 2 месяца после дистанционной ЛТ опухоли гипофиза, проявлявшейся акромегалией, появилась мышечная слабость, тошнота, рвота, нарушения дыхания. Развилось подостро коматозное состояние. При магнитно-резонансной томографии головного мозга выявлено многоочаговое поражение медиобазальных отделов обоих полушарий, передних отделов мозолистого тела и ствола. Дифференциальный диагноз включал острую демиелинизацию (SD, PML), вирусный энцефалит и васкулит. Пациентка получила пульс-терапию Метилпреднизолоном, плазиообмен. Сознание прояснилось, но самостоятельное дыхание не восстановилось, оставалась тетраплегия. Аутоиммунные заболевания и инфекционные болезни были исключены. Обнаружена мутантная гомозигота PAI-1-675 4G\4G. В данном случае, вероятно, патогенетическими факторами развития множественных инфарктов и очагов демиелинизации выступили как сама акромегалия, приводящая к эндотелиальной дисфункции, так и ЛТ с ее доказанным патологическим воздействием на сосудистую стенку и фибринолитическую систему. Множественным инфарктам способствовала и выявленная тромбофилия. Возможное сочетание патогенетических факторов развития инфарктов головного мозга следует учитывать при прогнозировании осложнений ЛТ.</p></abstract><trans-abstract xml:lang="en"><p>Locked-in syndrome (LIS) is a rare neurological disorder, usually appears as a result of the pons cerebellar damage, mostly after the brain stroke. Locked-in syndrome is characterized by the paralysis of skeletal muscles (respiratory, facial, pharyngeal, lingual and muscles of the extremities). Patient is unable to speak and breath, facial expressions and voluntary movements are also impossible. Acromegaly is a disease that can be described by the increase of the growth hormone (GH) and Insulin-like growth factor (IGF-1) and develops in most cases due to the pituitary adenomas. Pituitary adenoma (PA) can be treated by neurosurgical techniques, pharmaceutical and radiation therapy (RT). We present a clinical case of 33-year-old woman with PA-caused acromegaly, that developed muscle weakness, nausea, vomit and respiratory disturbance in a 2 months after the radiation therapy. Subacute comatose state was developed in the patient. MRI of the brain revealed a multi-focal lesion of the media-basal regions on both sides, frontal corpus callosum and brain stem. Differential diagnosis included an acute demyelination (SD, PML), viral encephalitis and vasculitis. Treatment included methylprednisolone pulse therapy and plasmapheresis. The consciousness cleared up, but there was no spontaneous breathing, tetraplegia persisted. Autoimmune and infectious diseases was excluded. The homozygous mutation PAI-1-675 4G/4G was found. In this case, acromegaly induced endothelial dysfunction was the pathogenesis factor of multiple cerebral infarctions and demyelinating lesions, as well as RT and its proven pathological influence on the vascular wall and the fibrinolytic system. The revealed thrombophilia was also a factor of multiple cerebral infarctions. A Potential combination of pathogenic factors in the development of cerebral should be taken into account in predicting complications of RT.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>акромегалия</kwd><kwd>лучевая терапия</kwd><kwd>множественные инфаркты ствола головного мозга</kwd><kwd>синдром «запертого человека»</kwd></kwd-group><kwd-group xml:lang="en"><kwd>acromegaly</kwd><kwd>radiation therapy</kwd><kwd>multiple brain stem infarctions</kwd><kwd>Locked-in syndrome</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">Красильникова Л. А., Тютин Л. А. Ультразвуковая диагностика ангиопатий при противоопухолевой терапии // Вопр. онкол. - 2003. - Т. 49, №2 5. - С. 595-600.</mixed-citation><mixed-citation xml:lang="en">Krasil’nikova L. A., Tyutin L. A. Ultrasound diagno¬sis of angiopathy with anticancer therapy. Voprosy onkologii [Questions of Oncology], 2003, no 5, vol.49 pp. 595-6002.</mixed-citation></citation-alternatives></ref><ref id="cit2"><label>2</label><citation-alternatives><mixed-citation xml:lang="ru">Bauer G., Gerstenbrand F., Rumpl E. Varieties of the locked-in syndrome // J. Neurol. - 1979. - №2 221 (2). - Р 77-91.</mixed-citation><mixed-citation xml:lang="en">Bauer G., Gerstenbrand F., Rumpl E. Varieties of the locked-in syndrome. J. Neurol. 1979;221(2):77-91.</mixed-citation></citation-alternatives></ref><ref id="cit3"><label>3</label><citation-alternatives><mixed-citation xml:lang="ru">Systemic complications of acromegaly: epidemiolo¬gy, pathogenesis, and management / А. Colao, D. Ferone, P Marzullo, G. Lombardi // Endocr. Rev. - 2004. - № 25. - Р 102-152. Doi: 10.1210/er. 2002-0022.</mixed-citation><mixed-citation xml:lang="en">Colao A, Ferone D, Marzullo P, Lombardi G (2004) System¬ic complications of acromegaly: epidemiology, pathogenesis, and management. Endocr Rev25:102-152. Doi:10.1210/er.2002-0022.</mixed-citation></citation-alternatives></ref><ref id="cit4"><label>4</label><citation-alternatives><mixed-citation xml:lang="ru">MolitchM. E. Diagnosis and Treatment of Pituitary Ade-nomas: A Review // JAMA. - 2017. - №2 317 (5). - Р 516-524.</mixed-citation><mixed-citation xml:lang="en">Molitch ME. Diagnosis and Treatment of Pituitary Ad-enomas A Review. JAMA. 2017;317(5):516-524.</mixed-citation></citation-alternatives></ref><ref id="cit5"><label>5</label><citation-alternatives><mixed-citation xml:lang="ru">Amanda J. Walker, Jake Ruzevick, Ashkan A. Malayeri et al. Postradiation imaging changes in the CNS: how can we differentiate between treatment effect and disease progres¬sion? // Future Oncol. - 2014. - № 10 (7). - Р 1277-1297.</mixed-citation><mixed-citation xml:lang="en">Amanda J Walker, Jake Ruzevick, Ashkan A Malayeri et al. Postradiation imaging changes in the CNS: how can we differentiate between treatment effect and disease progres¬sion? Future Oncol. 2014 May; 10(7): 1277-1297.</mixed-citation></citation-alternatives></ref><ref id="cit6"><label>6</label><citation-alternatives><mixed-citation xml:lang="ru">Freeman J. E., Johnston P G., Voke J. M. Somnolence after prophylactic cranial irradiation in children with acute lymphoblas¬tic leukaemia // Br. Med. J. - 1973. - № 4 (5891). - Р 523-525.</mixed-citation><mixed-citation xml:lang="en">Freeman JE, Johnston PG, Voke JM. Somnolence after prophylactic cranial irradiation in children with acute lympho¬blastic leukaemia. Br Med J. 1973;4(5891):523-525.</mixed-citation></citation-alternatives></ref><ref id="cit7"><label>7</label><citation-alternatives><mixed-citation xml:lang="ru">Sheline G. E., Wara W. M., Smith V. Therapeutic irra¬diation and brain injury // Int. J. Radiat. Oncol. Biol. Phys. - 1980. - № 6 (9). - Р 1215-1228.</mixed-citation><mixed-citation xml:lang="en">Sheline GE, Wara WM, Smith V Therapeutic irradi¬ation and brain injury. Int J Radiat Oncol Biol Phys. 1980; 6(9):1215-1228.</mixed-citation></citation-alternatives></ref><ref id="cit8"><label>8</label><citation-alternatives><mixed-citation xml:lang="ru">Cerebral radiation necrosis following treatment of extra-cranial malignancies / J. P Glass, T. L. Hwang, M. E. Leavens, H. I. Libshitz // Cancer. - 1984. - № 54 (9). - Р 1966-1972.</mixed-citation><mixed-citation xml:lang="en">Glass JP, Hwang TL, Leavens ME, Libshitz HI. Cere¬bral radiation necrosis following treatment of extracranial malignancies. Cancer. 1984;54(9):1966-1972.</mixed-citation></citation-alternatives></ref><ref id="cit9"><label>9</label><citation-alternatives><mixed-citation xml:lang="ru">Leibel S. A., Sheline G. E. Tolerance of the Brain and Spinal Cord to Conventional Irradiation. -N.-Y, USA: Raven Press, 1991. - Р 211-239.</mixed-citation><mixed-citation xml:lang="en">Leibel SA, Sheline GE. Tolerance of the Brain and Spinal Cord to Conventional Irradiation. Raven Press; NY, USA: 1991: 211-239.</mixed-citation></citation-alternatives></ref><ref id="cit10"><label>10</label><citation-alternatives><mixed-citation xml:lang="ru">McDonald L. W., Hayes T. L. The role of capillaries in the pathogenesis of delayed radionecrosis of brain // Am. J. Pathol. - 1967. - № 50 (5). - Р 745-764.</mixed-citation><mixed-citation xml:lang="en">McDonald LW, Hayes TL. The role of capillaries in the pathogenesis of delayed radionecrosis of brain. Am J Pathol. 1967;50(5):745-764.</mixed-citation></citation-alternatives></ref><ref id="cit11"><label>11</label><citation-alternatives><mixed-citation xml:lang="ru">Burger P. C., Boyko O. B. The pathology of central nervous system radiation injury // Radiation Injury to the Ner¬vous System / eds by P H. Gutin, S. A. Leibel, G. E. Sheline. - N.-Y, USA: Raven, 1991. - P 191-208.</mixed-citation><mixed-citation xml:lang="en">Burger PC, Boyko OB. The pathology of central ner¬vous system radiation injury. In: Gutin PH, Leibel SA, Sheline GE, editors. Radiation Injury to the Nervous System. Raven; NY, USA: 1991: 191-208.</mixed-citation></citation-alternatives></ref><ref id="cit12"><label>12</label><citation-alternatives><mixed-citation xml:lang="ru">Kurita H., Kawahara N., Asai A. et al. Radiation-in¬duced apoptosis of oligodendrocytes in the adult rat brain // Neurol. Res. - 2001. - № 23 (8). - Р 869-874.</mixed-citation><mixed-citation xml:lang="en">Kurita H, Kawahara N, Asai A, Ueki K, Shin M, Kirino T. Radiation-induced apoptosis of oligodendrocytes in the adult rat brain. Neurol Res. 2001;23(8):869-874.</mixed-citation></citation-alternatives></ref><ref id="cit13"><label>13</label><citation-alternatives><mixed-citation xml:lang="ru">Yoshii Y. Pathological review of late cerebral radione¬crosis // Brain. Tumor. Pathol. - 2008. - №2 25 (2). - Р 51-58.</mixed-citation><mixed-citation xml:lang="en">Yoshii Y Pathological review of late cerebral radione-crosis. Brain Tumor Pathol. 2008;25(2):51-58.</mixed-citation></citation-alternatives></ref><ref id="cit14"><label>14</label><citation-alternatives><mixed-citation xml:lang="ru">Sawaya R. E. The fibrinolytic enzymes in the biology // Fibrinolysis and the Central Nervous System / eds by R. E. Sa-waya. - PA, USA: Hanley &amp; Belfus, 1990. - P 106-126.</mixed-citation><mixed-citation xml:lang="en">Sawaya RE. The fibrinolytic enzymes in the biology. In: Sawaya RE, editor. Fibrinolysis and the Central Nervous System. Hanley &amp; Belfus; PA, USA: 1990: 106-126.</mixed-citation></citation-alternatives></ref><ref id="cit15"><label>15</label><citation-alternatives><mixed-citation xml:lang="ru">Milic M., Rees J. H. Acute demyelination following radiotherapy for glioma: a cautionary tale // Pract. Neurol. - 2017. - № 17 (1). - Р 35-38.</mixed-citation><mixed-citation xml:lang="en">Milic M, Rees JH. Acute demyelination following radiotherapy for glioma: a cautionary tale. Pract Neurol. 2017 Jan;17(1):35-38.</mixed-citation></citation-alternatives></ref><ref id="cit16"><label>16</label><citation-alternatives><mixed-citation xml:lang="ru">Sievers C., Sämann P. G., Dose T. et al. Macroscop¬ic brain architecture changes and white matter pathology in acromegaly: a clinicoradiological study // Pituitary. - 2009. - № 12 (3). - Р 177-185.</mixed-citation><mixed-citation xml:lang="en">C. Sievers, P. G. Sämann, T. Dose, C. Dimopoulou et al. Macroscopic brain architecture changes and white matter pathology in acromegaly: a clinicoradiological study. Pitu¬itary. 2009 Sep; 12(3): 177-185.</mixed-citation></citation-alternatives></ref><ref id="cit17"><label>17</label><citation-alternatives><mixed-citation xml:lang="ru">Sartori M. T., Wiman B., Vettore S. et al. 4G/5G poly-morphism of PAI-1 gene promoter and fibrino)lytic capacity in patients with deep vein thrombosis // Thromb. Haemost. - 1998. - № 80 (6). - Р 956-960.</mixed-citation><mixed-citation xml:lang="en">Sartori MT, Wiman B, Vettore S, et al. 4G/5G poly-morphism of PAI-1 gene promoter and fibrino)lytic capacity in patients with deep vein thrombosis. Thromb Haemost. 1998 Dec;80(6):956-60.</mixed-citation></citation-alternatives></ref><ref id="cit18"><label>18</label><citation-alternatives><mixed-citation xml:lang="ru">Van GoorM. L., Gómez García E., LeebeekF. et al. The plasminogen activator inhibitor (PAI-1 ) 4G/5G promoter poly-morphism and PAI-1 levels in ischemic stroke: a case-control study // Thromb. Haemost. - 2005. - № 93 (1). - Р 92-96.</mixed-citation><mixed-citation xml:lang="en">Van Goor ML, Gómez García E, Leebeek F, et al. The plasminogen activator inhibitor (PAI-1) 4G/5G pro¬moter polymorphism and PAI-1 levels in ischemic stroke. A case-control study. Thromb Haemost. 2005 Jan;93(1):92-6. Prog Brain Res. 2005;150:495-511.</mixed-citation></citation-alternatives></ref></ref-list><fn-group><fn fn-type="conflict"><p>The authors declare that there are no conflicts of interest present.</p></fn></fn-group></back></article>
