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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="en"><front><journal-meta><journal-id journal-id-type="publisher-id">nnp</journal-id><journal-title-group><journal-title xml:lang="en">Neurology, Neuropsychiatry, Psychosomatics</journal-title><trans-title-group xml:lang="ru"><trans-title>Неврология, нейропсихиатрия, психосоматика</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2074-2711</issn><issn pub-type="epub">2310-1342</issn><publisher><publisher-name>"IMA-Press", LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.14412/2074-2711-2019-4-77-81</article-id><article-id custom-type="elpub" pub-id-type="custom">nnp-1213</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="en"><subject>ORIGINAL INVESTIGATIONS</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ И МЕТОДИКИ</subject></subj-group></article-categories><title-group><article-title>Long-term follow-up in patients with catastrophic epilepsies</article-title><trans-title-group xml:lang="ru"><trans-title>Многолетний катамнез у пациентов с катастрофическими формами эпилепсии</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>Фирсов</surname><given-names>К. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Firsov</surname><given-names>K. V.</given-names></name></name-alternatives><bio xml:lang="ru"/><bio xml:lang="en"/><email xlink:type="simple">firsovkonst@yandex.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>Котов</surname><given-names>А. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Kotov</surname><given-names>A. S.</given-names></name></name-alternatives><bio xml:lang="ru"/><bio xml:lang="en"/><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ГБУЗ МО «Московский областной научно-исследовательский клинический институт им. М.Ф. Владимирского»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>M.F. Vladimirsky Moscow Regional Research Clinical Institute</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2019</year></pub-date><pub-date pub-type="epub"><day>07</day><month>12</month><year>2019</year></pub-date><volume>11</volume><issue>4</issue><fpage>77</fpage><lpage>81</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Firsov K.V., Kotov A.S., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Фирсов К.В., Котов А.С.</copyright-holder><copyright-holder xml:lang="en">Firsov K.V., Kotov A.S.</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://nnp.ima-press.net/nnp/article/view/1213">https://nnp.ima-press.net/nnp/article/view/1213</self-uri><abstract><p>Objective: to investigate the features of the course and long-term outcome of catastrophic epilepsies. Patients and methods. A total of 130 patients (62 (47.7%) men and 68 (52.3%) women) aged 21 to 78 years were examined. The follow-up was 1 year to 14 years. The inclusion criteria for the investigation were above 18 years of age; severe epilepsy with daily seizures considered as catastrophic epilepsy. The exclusion criteria were a history of nonepileptic seizures of any etiology, as well as noncompliance. The examination included history data collection; seizure diary analysis; clinical and neurological examination; routine EEG and/or EEG-video monitoring; brain magnetic resonance imaging; and laboratory tests. Results and discussion. The onset of epilepsy immediately with catastrophic seizures was observed in 28 (21.5%) patients; this onset was most frequently seen in 18 of the 28 children. At the same time, catastrophic seizures did not appear immediately, but it took some time for them to occur after the onset of active epilepsy in 102 (78.5%) patients. Moreover, the onset of catastrophic seizures could not be associated with any external influences or errors in 58 (56.9%) of these patients taking the drugs. Transition to catastrophic epilepsy could occur both abruptly in 11 (10.8%) patients and gradually in 91 (89.2%). After treatment correction, 16.8% of patients achieved remission at the time of study completion; the frequency of seizures decreased by 50% or more in 27.1% of cases; the effect was absent in 56.1%. Conclusion. Based on the findings, it can be concluded that in catastrophic epilepsy, the probability exists for spontaneous occurrence and remission of destructive seizures, frequently regardless of the therapy. The onset of the disease and that of catastrophic seizures can be spread in time. The efficiency of treatment remains low in patients with catastrophic epilepsies.</p></abstract><trans-abstract xml:lang="ru"><p>Цель исследования – изучение особенностей течения и отдаленного исхода катастрофических эпилепсий. Пациенты и методы. Обследовано 130 пациентов: 62 (47,7%) мужчины и 68 (52,3%) женщин в возрасте от 21 до 78 лет. Катамнез составил от 1 года до 14 лет. Критериями включения в исследование были возраст старше 18 лет; тяжелое течение эпилепсии с ежедневными приступами, рассматриваемое как катастрофическая эпилепсия. Критериями исключения являлись наличие в анамнезе неэпилептических приступов любой этиологии, некомплаентность. Обследование включало сбор анамнеза, анализ дневника приступов, клинический и неврологический осмотр, рутинную электроэнцефалографию (ЭЭГ) и/или ЭЭГ-видеомониторинг, магнитно-резонансную томографию головного мозга, лабораторные анализы. Результаты и обсуждение. Дебют эпилепсии сразу с катастрофических приступов отмечался у 28 (21,5%) больных, чаще всего такое начало наблюдалось у детей (у 18 из 28). В то же время у 102 (78,5%) пациентов катастрофические приступы появились не сразу, а через некоторое время после начала активной эпилепсии. При этом у 58 (56,9%) из этих 102 пациентов начало катастрофических приступов нельзя было связать с какими-либо внешними воздействиями или погрешностями в приеме препаратов. Переход к катастрофической эпилепсии мог происходить как резко – у 11 (10,8%) пациентов, так и постепенно – у 91 (89,2%). После коррекции лечения ремиссия на момент завершения исследования была достигнута у 16,8% пациентов; снижение частоты приступов на 50% и более – у 27,1%; эффект отсутствовал у 56,1%. Заключение. Можно сделать вывод, что при катастрофической эпилепсии имеется вероятность спонтанного возникновения и ремиссии разрушительных приступов, часто независимо от проводимой терапии. Дебют заболевания и начало катастрофических приступов могут быть разнесены во времени. Эффективность лечения пациентов с катастрофическими формами эпилепсии остается невысокой.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>эпилепсия</kwd><kwd>катастрофическая эпилепсия</kwd><kwd>эпилептическая энцефалопатия</kwd><kwd>ремиссия</kwd><kwd>рецидив</kwd><kwd>диагностика</kwd><kwd>лечение</kwd><kwd>прогноз</kwd><kwd>электроэнцефалография</kwd><kwd>магнитно-резонансная томография</kwd></kwd-group><kwd-group xml:lang="en"><kwd>epilepsy</kwd><kwd>catastrophic epilepsy</kwd><kwd>epileptic encephalopathy</kwd><kwd>remission</kwd><kwd>recurrence</kwd><kwd>diagnosis</kwd><kwd>treatment</kwd><kwd>prognosis</kwd><kwd>electroencephalography</kwd><kwd>magnetic resonance imaging</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">Howard MA, Baraban SC. Catastrophic Epilepsies of Childhood. 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