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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">Russian Journal of Child Neurology</journal-id><journal-title-group><journal-title xml:lang="en">Russian Journal of Child Neurology</journal-title><trans-title-group xml:lang="ru"><trans-title>Русский журнал детской неврологии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2073-8803</issn><issn publication-format="electronic">2412-9178</issn><publisher><publisher-name xml:lang="en">Publishing House ABV Press</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">185</article-id><article-id pub-id-type="doi">10.17650/2073-8803-2016-11-4-54-60</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>CLINICAL OBSERVATIONS</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>КЛИНИЧЕСКИЕ НАБЛЮДЕНИЯ</subject></subj-group><subj-group subj-group-type="article-type"><subject></subject></subj-group></article-categories><title-group><article-title xml:lang="en">EFFICACY OF RUFINAMIDE IN THE TREATMENT OF DRUG-RESISTANT FOCAL EPILEPSIES IN PAEDIATRIC PRACTICE</article-title><trans-title-group xml:lang="ru"><trans-title>Эффективность руфинамида в лечении фармакорезистентной фокальной эпилепсии в педиатрической практике</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Shchederkina</surname><given-names>I. O.</given-names></name><name xml:lang="ru"><surname>Щедеркина</surname><given-names>И. О.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>1/9 4th Dobryninskiy Pereulok, Moscow 119049;</p><p>1 Ostrovityanova St., Moscow, 117997</p></bio><bio xml:lang="ru"><p>119049 Москва, 4-й Добрынинский переулок, 1/9;</p><p>117997 Москва, ул. Островитянова, 1</p></bio><email>schederkina@bk.ru</email><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Orlova</surname><given-names>K. A.</given-names></name><name xml:lang="ru"><surname>Орлова</surname><given-names>К. А.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>1/9 4th Dobryninskiy Pereulok, Moscow 119049;</p><p>1 Ostrovityanova St., Moscow, 117997</p></bio><bio xml:lang="ru"><p>119049 Москва, 4-й Добрынинский переулок, 1/9;</p><p>117997 Москва, ул. Островитянова, 1</p></bio><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff2"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Koltunov</surname><given-names>I. E.</given-names></name><name xml:lang="ru"><surname>Колтунов</surname><given-names>И. Е.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>1/9 4th Dobryninskiy Pereulok, Moscow 119049;</p><p>6 Miklukho-Maklaya St., Moscow 117198</p></bio><bio xml:lang="ru"><p>119049 Москва, 4-й Добрынинский переулок, 1/9;</p><p>117198 Москва, ул. Миклухо-Маклая, 6</p></bio><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff3"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Dorofeeva</surname><given-names>M. Yu.</given-names></name><name xml:lang="ru"><surname>Дорофеева</surname><given-names>М. Ю.</given-names></name></name-alternatives><address><country country="RU">Russian Federation</country></address><bio xml:lang="en"><p>2 Taldomskaya St., Moscow 125412</p></bio><bio xml:lang="ru"><p>125412 Москва, ул. Талдомская, 2</p></bio><xref ref-type="aff" rid="aff4"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">Morozov Children’s City Clinical Hospital, Moscow Healthcare Department</institution></aff><aff><institution xml:lang="ru">ГБУЗ «Морозовская детская городская клиническая больница Департамента здравоохранения г. Москвы»</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia</institution></aff><aff><institution xml:lang="ru">ФГБОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава России</institution></aff></aff-alternatives><aff-alternatives id="aff3"><aff><institution xml:lang="en">Peoples’ Friendship University of Russia</institution></aff><aff><institution xml:lang="ru">ФГАОУ ВО «Российский университет дружбы народов»</institution></aff></aff-alternatives><aff-alternatives id="aff4"><aff><institution xml:lang="en">Acad. Yu.E. Vel’tishchev Research Clinical Institute of Pediatrics, N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia</institution></aff><aff><institution xml:lang="ru">ОСП «Научно-исследовательский клинический институт педиатрии им. академика Ю.Е. Вельтищева» ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава России</institution></aff></aff-alternatives><pub-date date-type="pub" iso-8601-date="2016-12-15" publication-format="electronic"><day>15</day><month>12</month><year>2016</year></pub-date><volume>11</volume><issue>4</issue><issue-title xml:lang="en"/><issue-title xml:lang="ru"/><fpage>54</fpage><lpage>60</lpage><history><date date-type="received" iso-8601-date="2017-02-28"><day>28</day><month>02</month><year>2017</year></date><date date-type="accepted" iso-8601-date="2017-02-28"><day>28</day><month>02</month><year>2017</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2016, ABV-Press</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2016, АБВ-пресс</copyright-statement><copyright-year>2016</copyright-year><copyright-holder xml:lang="en">ABV-Press</copyright-holder><copyright-holder xml:lang="ru">АБВ-пресс</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://rjdn.abvpress.ru/jour/about/editorialPolicies</ali:license_ref></license></permissions><self-uri xlink:href="https://rjdn.abvpress.ru/jour/article/view/185">https://rjdn.abvpress.ru/jour/article/view/185</self-uri><abstract xml:lang="en"><p>Among drug-resistant epilepsies, epileptic syndromes, characterized by combination of several types of seizures, are considered to be the most difficult in terms of treatment. Lennox–Gastaut syndrome is one of them. It manifests with polymorphic seizures (tonic axial, myatonic, atypical absence seizures, status epilepticus of minor motor seizures, myoclonic, generalized convulsive, and focal seizures). This is a heterogeneous disease, represented by a complex of clinical and electroencephalographic manifestations with various etiology. Current review is devoted to a novel antiepileptic drug rufinamide, which has a new mechanism of action. The drug has been registered in Russia in 2015. The authors also describe their own experience of rufinamide usage in the treatment of drug-resistant focal epilepsy as a part of multicomponent therapy for polymorphic seizures. One patient achieved clinical remission for 16 months; the second one had more than 50 % decrease in seizures frequency with a remission of drop-attacks.</p></abstract><trans-abstract xml:lang="ru"><p/></trans-abstract><kwd-group xml:lang="en"><kwd>drug-resistant epilepsy</kwd><kwd>polymorphic seizures</kwd><kwd>Lennox–Gastaut syndrome</kwd><kwd>rufinamide</kwd><kwd>Inovelon</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>фармакорезистентная эпилепсия</kwd><kwd>полиморфные приступы</kwd><kwd>синдром Леннокса–Гасто</kwd><kwd>руфинамид</kwd><kwd>Иновелон</kwd></kwd-group><funding-group/></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><citation-alternatives><mixed-citation xml:lang="en">1. Инструкция по медицинскому применению препарата Иновелон (руфинамид) ЛП-002841. [Guidelines for medical use of Inovelon (rufinamide) LP-002841. (In Russ.)].</mixed-citation><mixed-citation xml:lang="ru">Инструкция по медицинскому применению препарата Иновелон (руфинамид) ЛП-002841. [Guidelines for medical use of Inovelon (rufinamide) LP-002841. (In Russ.)].</mixed-citation></citation-alternatives></ref><ref id="B2"><label>2.</label><citation-alternatives><mixed-citation xml:lang="en">2. Карлов В.А., Мухин К.Ю., Гузева В.И. Современные подходы к критериям выбора фармакотерапии эпилепсии в детском возрасте. Русский журнал детской неврологии 2013;8(4)–2014;9(1):61–2. [Karlov V.A., Mukhin K.Yu., Guzeva V.I. Modern approaches to drug selection in the therapy of epilepsy in children. Russkiy zhurnal detskoy nevrologii = Russian Journal of Child Neurology 2013;8(4)–2014;9(1):61–2. (In Russ.)].</mixed-citation><mixed-citation xml:lang="ru">Карлов В.А., Мухин К.Ю., Гузева В.И. Современные подходы к критериям выбора фармакотерапии эпилепсии в детском возрасте. Русский журнал детской неврологии 2013;8(4)–2014;9(1):61–2. [Karlov V.A., Mukhin K.Yu., Guzeva V.I. Modern approaches to drug selection in the therapy of epilepsy in children. Russkiy zhurnal detskoy nevrologii = Russian Journal of Child Neurology 2013;8(4)–2014;9(1):61–2. (In Russ.)].</mixed-citation></citation-alternatives></ref><ref id="B3"><label>3.</label><citation-alternatives><mixed-citation xml:lang="en">3. Мазин П.В., Шешунов И.В., Кислицын Ю.В., Мазина Н.К. Эффективность и безопасность лечения эпилепсии руфинамидом (по данным метаанализа). Журнал неврологии и психиатрии им. С.С. Корсакова 2016;8:40–3. [Mazin P.V., Sheshunov I.V., Kislitsyn Yu.V., Mazina N.K. Effectiveness and safety of rufinamide in the treatment of epilepsy (according to the data of meta-analysis). Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2016;8:40–3. (In Russ.)].</mixed-citation><mixed-citation xml:lang="ru">Мазин П.В., Шешунов И.В., Кислицын Ю.В., Мазина Н.К. Эффективность и безопасность лечения эпилепсии руфинамидом (по данным метаанализа). Журнал неврологии и психиатрии им. С.С. Корсакова 2016;8:40–3. [Mazin P.V., Sheshunov I.V., Kislitsyn Yu.V., Mazina N.K. Effectiveness and safety of rufinamide in the treatment of epilepsy (according to the data of meta-analysis). Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2016;8:40–3. (In Russ.)].</mixed-citation></citation-alternatives></ref><ref id="B4"><label>4.</label><citation-alternatives><mixed-citation xml:lang="en">4. Федеральное руководство по детской неврологии. Подред. В.И. Гузевой. М.: МК, 2016. 656 c. [Federal child neurology guidelines. Ed. by V.I. Guzeva. Moscow: MK, 2016. 656 p. (In Russ.)].</mixed-citation><mixed-citation xml:lang="ru">Федеральное руководство по детской неврологии. Подред. В.И. Гузевой. М.: МК, 2016. 656 c. [Federal child neurology guidelines. Ed. by V.I. Guzeva. Moscow: MK, 2016. 656 p. (In Russ.)].</mixed-citation></citation-alternatives></ref><ref id="B5"><label>5.</label><citation-alternatives><mixed-citation xml:lang="en">5. Al-Banji M.H., Zanr D.K., Jan M.M. Lennox–Gastaut syndrome. Manag Upd Neurosci (Riyadh) 2015;20(3):207–12.</mixed-citation><mixed-citation xml:lang="ru">Al-Banji M.H., Zanr D.K., Jan M.M. Lennox–Gastaut syndrome. Manag Upd Neurosci (Riyadh) 2015;20(3):207–12.</mixed-citation></citation-alternatives></ref><ref id="B6"><label>6.</label><citation-alternatives><mixed-citation xml:lang="en">6. Albini M., Morano A., Fanella M. et al. Effectiveness of rufinamide in treatment of idiopathic generalized epilepsy with atypical evolution: case report and rewiev of the literature. Clon EEG Neurosci 2016;47(2):162–6.</mixed-citation><mixed-citation xml:lang="ru">Albini M., Morano A., Fanella M. et al. Effectiveness of rufinamide in treatment of idiopathic generalized epilepsy with atypical evolution: case report and rewiev of the literature. Clon EEG Neurosci 2016;47(2):162–6.</mixed-citation></citation-alternatives></ref><ref id="B7"><label>7.</label><citation-alternatives><mixed-citation xml:lang="en">7. Arzimanoglou A., French J., Blume W.T. Lennox–Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol 2009;8:82–93.</mixed-citation><mixed-citation xml:lang="ru">Arzimanoglou A., French J., Blume W.T. Lennox–Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol 2009;8:82–93.</mixed-citation></citation-alternatives></ref><ref id="B8"><label>8.</label><citation-alternatives><mixed-citation xml:lang="en">8. Beaumanoir A., Dravet Ch. The Lennox–Gastaut syndrome. In: Epileptic symdromes in infancy, childhood and adolescence. 2th edn. Ed. by. J. Roger, M. Bureau, Ch. Dravet et al. London: John Libbey, 1992. Pp. 307–12.</mixed-citation><mixed-citation xml:lang="ru">Beaumanoir A., Dravet Ch. The Lennox–Gastaut syndrome. In: Epileptic symdromes in infancy, childhood and adolescence. 2th edn. Ed. by. J. Roger, M. Bureau, Ch. Dravet et al. London: John Libbey, 1992. Pp. 307–12.</mixed-citation></citation-alternatives></ref><ref id="B9"><label>9.</label><citation-alternatives><mixed-citation xml:lang="en">9. Coppola G., Besag F., Cusmai R. et al. Current role of rufinamide in the treatment of childhood epilepsy: literature review and treatment guidelines. Eur J Paediatr Neurol 2014;18(6):685–90.</mixed-citation><mixed-citation xml:lang="ru">Coppola G., Besag F., Cusmai R. et al. Current role of rufinamide in the treatment of childhood epilepsy: literature review and treatment guidelines. Eur J Paediatr Neurol 2014;18(6):685–90.</mixed-citation></citation-alternatives></ref><ref id="B10"><label>10.</label><citation-alternatives><mixed-citation xml:lang="en">10. Glauser T., Kluger G., Sachdeo R. et al. Rufinamide for generalized seizures associated with Lennox–Gastaut syndrome. Neurology 2008:70(21):641–5.</mixed-citation><mixed-citation xml:lang="ru">Glauser T., Kluger G., Sachdeo R. et al. Rufinamide for generalized seizures associated with Lennox–Gastaut syndrome. Neurology 2008:70(21):641–5.</mixed-citation></citation-alternatives></ref><ref id="B11"><label>11.</label><citation-alternatives><mixed-citation xml:lang="en">11. Guerrini R., Marini C. Epileptic encephalopathies. In: Epilepsy and epileptic seizures. Ed. by S. Shor-von, R. Guerrini, M. Cook, S. Lhatoo. UK: Oxford University Press, 2013. Pp. 177–80.</mixed-citation><mixed-citation xml:lang="ru">Guerrini R., Marini C. Epileptic encephalopathies. In: Epilepsy and epileptic seizures. Ed. by S. Shor-von, R. Guerrini, M. Cook, S. Lhatoo. UK: Oxford University Press, 2013. Pp. 177–80.</mixed-citation></citation-alternatives></ref><ref id="B12"><label>12.</label><citation-alternatives><mixed-citation xml:lang="en">12. Kim J.Y., Lee C.G., Yu H.J. et al. The efficacy and tolerability of rufinamide in intractable pediatric epilepsy. J Epilepsy Res 2012;2(2):33–7.</mixed-citation><mixed-citation xml:lang="ru">Kim J.Y., Lee C.G., Yu H.J. et al. The efficacy and tolerability of rufinamide in intractable pediatric epilepsy. J Epilepsy Res 2012;2(2):33–7.</mixed-citation></citation-alternatives></ref><ref id="B13"><label>13.</label><citation-alternatives><mixed-citation xml:lang="en">13. Kluger G., Glauser T., Krauss G. et al. Adjunctive rufinamide in Lennox–Gastaut syndrome: a long-term, openlabel extension study. Acta Neurol Scand 2010;122:202–8.</mixed-citation><mixed-citation xml:lang="ru">Kluger G., Glauser T., Krauss G. et al. Adjunctive rufinamide in Lennox–Gastaut syndrome: a long-term, openlabel extension study. Acta Neurol Scand 2010;122:202–8.</mixed-citation></citation-alternatives></ref><ref id="B14"><label>14.</label><citation-alternatives><mixed-citation xml:lang="en">14. Kwan P., Broudie M.J. Effectiveness of first antiepiltptic drug. Epilepsia 2001;42(10):1255–60.</mixed-citation><mixed-citation xml:lang="ru">Kwan P., Broudie M.J. Effectiveness of first antiepiltptic drug. Epilepsia 2001;42(10):1255–60.</mixed-citation></citation-alternatives></ref><ref id="B15"><label>15.</label><citation-alternatives><mixed-citation xml:lang="en">15. Schiller Y., Najjar Y. Quantifying the response to antiepileptic drugs: effect of past treatment history. Neurology 2008;70(1):54–65.</mixed-citation><mixed-citation xml:lang="ru">Schiller Y., Najjar Y. Quantifying the response to antiepileptic drugs: effect of past treatment history. Neurology 2008;70(1):54–65.</mixed-citation></citation-alternatives></ref><ref id="B16"><label>16.</label><citation-alternatives><mixed-citation xml:lang="en">16. Shorvon S. The epidemiology of epilepsy. In: Lecture notes. British brabch of International League against epilepsy. Ed. by J.S. Duncan, J.Q. Gill. Oxford: Keble College, 1995. Pp. 1–6.</mixed-citation><mixed-citation xml:lang="ru">Shorvon S. The epidemiology of epilepsy. In: Lecture notes. British brabch of International League against epilepsy. Ed. by J.S. Duncan, J.Q. Gill. Oxford: Keble College, 1995. Pp. 1–6.</mixed-citation></citation-alternatives></ref><ref id="B17"><label>17.</label><citation-alternatives><mixed-citation xml:lang="en">17. Wier H.A., Cerna A., So T.Y. Rufinamide for pediatric patients with Lennox–Gastaut syndrome: a comprehensive overview. Paediatric Drugs 2011;13(2):97–106.</mixed-citation><mixed-citation xml:lang="ru">Wier H.A., Cerna A., So T.Y. Rufinamide for pediatric patients with Lennox–Gastaut syndrome: a comprehensive overview. Paediatric Drugs 2011;13(2):97–106.</mixed-citation></citation-alternatives></ref></ref-list></back></article>
