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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">epilepsia</journal-id><journal-title-group><journal-title xml:lang="en">Epilepsy and paroxysmal conditions</journal-title><trans-title-group xml:lang="ru"><trans-title>Эпилепсия и пароксизмальные состояния</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2077-8333</issn><issn pub-type="epub">2311-4088</issn><publisher><publisher-name>IRBIS LLC</publisher-name></publisher></journal-meta><article-meta><article-id custom-type="elpub" pub-id-type="custom">epilepsia-38</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 ARTICLES</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОРИГИНАЛЬНЫЕ СТАТЬИ</subject></subj-group></article-categories><title-group><article-title>FOCAL MOTOR SEIZURES WITH HYPERKINETIC AUTOMATISMS IN PEDIATRIC PRACTICE</article-title><trans-title-group xml:lang="ru"><trans-title>ФОКАЛЬНЫЕ ПРИСТУПЫ С ГИПЕРКИНЕТИЧЕСКИМИ (ГИПЕРМОТОРНЫМИ) АВТОМАТИЗМАМИ В ПЕДИАТРИЧЕСКОЙ ПРАКТИКЕ</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>Мухин</surname><given-names>К. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Mukhin</surname><given-names>K. Yu.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Миронов</surname><given-names>М. Б.</given-names></name><name name-style="western" xml:lang="en"><surname>Mironov</surname><given-names>M. B.</given-names></name></name-alternatives><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Институт детской неврологии и эпилепсии имени Святителя Луки, Москва</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Svt. Luka’s Institute of Child Neurology and Epilepsy, Moscow</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2014</year></pub-date><pub-date pub-type="epub"><day>02</day><month>06</month><year>2016</year></pub-date><volume>6</volume><issue>2</issue><fpage>19</fpage><lpage>28</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Mukhin K.Y., Mironov M.B., 2016</copyright-statement><copyright-year>2016</copyright-year><copyright-holder xml:lang="ru">Мухин К.Ю., Миронов М.Б.</copyright-holder><copyright-holder xml:lang="en">Mukhin K.Y., Mironov M.B.</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.epilepsia.su/jour/article/view/38">https://www.epilepsia.su/jour/article/view/38</self-uri><abstract><p>Abstract: focal motor seizures with hyperkinetic automatisms (FSHA) – seizures with involves predominantly proximal limb or axial muscles producing irregular sequential ballistic movements. In our study we revealed FSHA in 1.5% of all cases of epilepsy with onset of seizures before 18 years old (n=1261). Onset of epilepsy with FSHA varied widely – from 9 month to 18 years of life; middle age of onset was 6.3 years ± 5.9 years. FSHA occurred in combination with 8 different types of seizures. Remission was achieved in 42.1% cases of epilepsy, associated with FSHA. Reduction of seizure frequency ≥50% on antiepileptic treatment was achieved in 42.1% of the patients.</p></abstract><trans-abstract xml:lang="ru"><p>Фокальные эпилептические приступы с гиперкинетическими автоматизмами (ФПГА) – приступы, характеризующиеся чрезмерными движениями, локализованными в основном в проксимальных отделах конечностей и в области туловища. Цель исследования – изучение клинико-электроэнцефалографических, нейровизуализационных особенностей и эффективности АЭП у пациентов с фокальными приступами с гиперкинетическими автоматизмами. Результаты: ФПГА выявлены у 19 пациентов, что составило 1,5% случаев среди всех больных эпилепсией с дебютом приступов до 18 лет. Отмечалось незначительное преобладание в группе пациентов женского пола – 52,6% случаев. Возраст дебюта эпилепсии варьировал от 9 месяцев жизни до 18 лет (в среднем 6,3 лет ± 5,9 лет). Изолированные гипермоторные приступы отмечались лишь у одного пациента (5,3%). Кроме ФПГА в клинической картине могли встречаться 8 различных типов приступов. Наиболее часто ФПГА сочетались со вторично-генерализованными судорожными, асимметричными тоническими и фокальными аутомоторными приступами. Гипермоторные приступы в 11 случаях (57,9%) отмечались только во сне. У 8 пациентов (42,1%) приступы отмечались как во сне, так и в бодрствовании. Ремиссия достигнута лишь в 42,1% случаев, снижение частоты приступов на 50% и более на фоне АЭП наблюдалось у 42,1% пациентов. Отсутствие эффекта отмечено в 15,8% случаев.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>эпилепсия</kwd><kwd>фокальные приступы с гиперкинетическими (гипермоторными) автоматизмами</kwd><kwd>фокальные приступы с гипермоторными автоматизмами</kwd><kwd>фокальные гипермоторные приступы</kwd><kwd>видео ЭЭГ мониторинг</kwd></kwd-group><kwd-group xml:lang="en"><kwd>epilepsy</kwd><kwd>focal motor seizures with hyperkinetic automatisms</kwd><kwd>video-EEG monitoring</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">Глухова Л.Ю. Мухин К.Ю. Аутосомно-доминантная лобная эпилепсия с ночными приступами. В кн.: Эпилепсия: атлас электро-клинической диагностики / К.Ю. Мухин, А.С. Петрухин, Л.Ю. Глухова. М. 2004; 314-321.</mixed-citation><mixed-citation xml:lang="en">Gluhova L.Ju. Muhin K.Ju. Autosomno-dominantnaja lobnaja jepilepsija s nochnymi pristupami. V kn.: Jepilepsija: atlas jelektro-klinicheskoj diagnostiki (Autosomal dominant nocturnal frontal lobe epilepsy with seizures. In the book: Epilepsy Atlas electro-clinical diagnosis / K.Ju. Muhin, A.S. Petruhin, L.Ju. Gluhova). Moscow. 2004; 314-321.</mixed-citation></citation-alternatives></ref><ref id="cit2"><label>2</label><citation-alternatives><mixed-citation xml:lang="ru">Глухова Л.Ю., Мухин К.Ю., Петрухин А.С., Миронов М.Б. Топамакс в лечении симптоматической лобной эпилепсии. Журнал неврол. психиатр. 2003; 103 (12): 51-52.</mixed-citation><mixed-citation xml:lang="en">Gluhova L.Ju., Muhin K.Ju., Petruhin A.S., Mironov M.B. Zhurnal nevrol. psihiatr. 2003; 103 (12): 51-52.</mixed-citation></citation-alternatives></ref><ref id="cit3"><label>3</label><citation-alternatives><mixed-citation xml:lang="ru">Карлов В.А. Эпилепсия у детей и взрослых женщин и мужчин. М. 2010;196-199.</mixed-citation><mixed-citation xml:lang="en">Karlov V.A. Jepilepsija u detej i vzroslyh zhen¬-shhin i muzhchin. Moscow. 2010; 196-199.</mixed-citation></citation-alternatives></ref><ref id="cit4"><label>4</label><citation-alternatives><mixed-citation xml:lang="ru">Мухин К.Ю. Аутосомно-доминантная лобная эпилепсия с ночными пароксизмами. В кн. Идиопатические формы эпилепсии: систематика, диагностика, терапия / К.Ю. Мухин, А.С. Петрухин. М. 2000; 209-216.</mixed-citation><mixed-citation xml:lang="en">Muhin K.Ju. Autosomno-dominantnaja lobnaja jepilepsija s nochnymi paroksizmami. V kn. Idiopaticheskie formy jepilepsii: sistematika, diagnostika, terapija (Autosomal dominant nocturnal frontal lobe epilepsy with paroxysms. In the book. Idiopathic forms of epilepsy taxonomy, diagnosis, therapy / K.Ju. Muhin, A.S. Petruhin). Moscow. 2000; 209-216.</mixed-citation></citation-alternatives></ref><ref id="cit5"><label>5</label><citation-alternatives><mixed-citation xml:lang="ru">Ambrosetto G., Santucci M. Sleep-related hypermotor seizures in aspartylglucosaminuria: a case report. Epilepsia. 2009; 50 (6): 1638-1640.</mixed-citation><mixed-citation xml:lang="en">Ambrosetto G., Santucci M. Sleep-related hypermotor seizures in aspartylglucosami¬nuria: a case report. Epilepsia. 2009; 50 (6): 1638-1640.</mixed-citation></citation-alternatives></ref><ref id="cit6"><label>6</label><citation-alternatives><mixed-citation xml:lang="ru">Engel J. Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: Report of the ILAE Task Force on Classification and Terminology. Epilepsia. 2001; 42 (6): 796-803.</mixed-citation><mixed-citation xml:lang="en">Engel J. Jr. A proposed diagnostic scheme for people with epileptic seizures and with epi-lepsy: Report of the ILAE Task Force on Classification and Terminology. Epilepsia. 2001; 42 (6): 796-803.</mixed-citation></citation-alternatives></ref><ref id="cit7"><label>7</label><citation-alternatives><mixed-citation xml:lang="ru">Holthausen H., Hoppe M. Hypermotor seizures. In: Epileptic Seizures: path physiology and clinical semiology. Eds. H.-O. Luders, S. Noachtar. Philadelphia. 2000; 439-448.</mixed-citation><mixed-citation xml:lang="en">Holthausen H., Hoppe M. Hypermotor seizu¬res. In: Epileptic Seizures: path physiology and clinical semiology. Eds. H.-O. Luders, S. Noachtar. Philadelphia. 2000; 439-448.</mixed-citation></citation-alternatives></ref><ref id="cit8"><label>8</label><citation-alternatives><mixed-citation xml:lang="ru">Kaido T., Otsuki T., Nakama H., Kaneko Y., Kubota Y., Sugai K., Saito O. Complex behavioral automatism arising from insular cortex. Epilepsy Behav. 2006; 8 (1): 315-319.</mixed-citation><mixed-citation xml:lang="en">Kaido T., Otsuki T., Nakama H., Kaneko Y., Kubota Y., Sugai K., Saito O. Complex be-havioral automatism arising from insular cortex. Epilepsy Behav. 2006; 8 (1): 315-319.</mixed-citation></citation-alternatives></ref><ref id="cit9"><label>9</label><citation-alternatives><mixed-citation xml:lang="ru">Luders H.-O., Burgess R., Noachtar S. Expanding the international classification of seizures to provide localization information. Neurology. 1993; 43: 1650-1655.</mixed-citation><mixed-citation xml:lang="en">Luders H.-O., Burgess R., Noachtar S. Expanding the international classification of sei-zures to provide localization information. Neurology. 1993; 43: 1650-1655.</mixed-citation></citation-alternatives></ref><ref id="cit10"><label>10</label><citation-alternatives><mixed-citation xml:lang="ru">Luders H.-O., Noachtar S. Epileptic seizures. Pathophysiology and clinical semiology. N.Y. 2000; 796.</mixed-citation><mixed-citation xml:lang="en">Luders H.-O., Noachtar S. Epileptic seizures. Pathophysiology and clinical semiology. N.Y. 2000; 796.</mixed-citation></citation-alternatives></ref><ref id="cit11"><label>11</label><citation-alternatives><mixed-citation xml:lang="ru">Lugaresi E, Cirignotta F. Hypnogenic paroxysmal dystonia: epileptic seizure or a new syndrome? Sleep. 1981; 4 (2): 129-38.</mixed-citation><mixed-citation xml:lang="en">Lugaresi E, Cirignotta F. Hypnogenic paroxysmal dystonia: epileptic seizure or a new syndrome? Sleep. 1981; 4 (2): 129-38.</mixed-citation></citation-alternatives></ref><ref id="cit12"><label>12</label><citation-alternatives><mixed-citation xml:lang="ru">Nishibayashi H., Ogura M., Taguchi M., Miki J., Uematsu Y., Itakura T. Nondominant parietotemporal cortical dysplasia manifesting as hypermotor seizures. Epilepsy Behav. 2009; 14 (4): 691-695.</mixed-citation><mixed-citation xml:lang="en">Nishibayashi H., Ogura M., Taguchi M., Miki J., Uematsu Y., Itakura T. Nondominant parietotemporal cortical dysplasia manifesting as hypermotor seizures. Epilepsy Behav. 2009; 14 (4): 691-695.</mixed-citation></citation-alternatives></ref><ref id="cit13"><label>13</label><citation-alternatives><mixed-citation xml:lang="ru">Nobili L., Francione S., Mai R. et. al. Nocturnal frontal lobe epilepsy: intracerebral recordings of paroxysmal motor attacks with increasing complexity. Sleep. 2003; 26: 883-886.</mixed-citation><mixed-citation xml:lang="en">Nobili L., Francione S., Mai R. et. al. Nocturnal frontal lobe epilepsy: intracerebral re-cordings of paroxysmal motor attacks with increasing complexity. Sleep. 2003; 26: 883-886.</mixed-citation></citation-alternatives></ref><ref id="cit14"><label>14</label><citation-alternatives><mixed-citation xml:lang="ru">Nobili L., Francione S., Mai R. et. al. Nocturnal frontal lobe epilepsy: intracerebral recordings of paroxysmal motor attacks with increasing complexity. Sleep. 2003; 26: 883-886.</mixed-citation><mixed-citation xml:lang="en">Nobili L., Francione S., Mai R. et. al. Nocturnal frontal lobe epilepsy: intracerebral re-cordings of paroxysmal motor attacks with increasing complexity. Sleep. 2003; 26: 883-886.</mixed-citation></citation-alternatives></ref><ref id="cit15"><label>15</label><citation-alternatives><mixed-citation xml:lang="ru">Oldani A., Zucconi M., Asselta R. et. al. Autosomal dominant nocturnal frontal lobe epilepsy. A video-polysomnographic and genetic appraisal of 40 patients and delineations of the epileptic syndrome. Brain. 1998; 121: 205-223.</mixed-citation><mixed-citation xml:lang="en">Oldani A., Zucconi M., Asselta R. et. al. Autosomal dominant nocturnal frontal lobe epi-lepsy. A video-polysomnographic and genetic appraisal of 40 patients and delineations of the epileptic syndrome. Brain. 1998; 121: 205-223.</mixed-citation></citation-alternatives></ref><ref id="cit16"><label>16</label><citation-alternatives><mixed-citation xml:lang="ru">Oldani A., Zucconi M., Asselta R. et. al. Autosomal dominant nocturnal frontal lobe epilepsy. A video-polysomnographic and genetic appraisal of 40 patients and delineations of the epileptic syndrome. Brain. 1998; 121: 205-223.</mixed-citation><mixed-citation xml:lang="en">Oldani A., Zucconi M., Asselta R. et. al. Autosomal dominant nocturnal frontal lobe epi-lepsy. A video-polysomnographic and genetic appraisal of 40 patients and delineations of the epileptic syndrome. Brain. 1998; 121: 205-223.</mixed-citation></citation-alternatives></ref><ref id="cit17"><label>17</label><citation-alternatives><mixed-citation xml:lang="ru">Raju G.P., Sarco D.P., Poduri A., Riviello J.J., Bergin A.M., Takeoka M. Oxcarbazepine in children with nocturnal frontal-lobe epilepsy. Pediatr. Neurol. 2007; 37 (5): 345-349.</mixed-citation><mixed-citation xml:lang="en">Raju G.P., Sarco D.P., Poduri A., Riviello J.J., Bergin A.M., Takeoka M. Oxcarbazepine in children with nocturnal frontal-lobe epilepsy. Pediatr. Neurol. 2007; 37 (5): 345-349.</mixed-citation></citation-alternatives></ref><ref id="cit18"><label>18</label><citation-alternatives><mixed-citation xml:lang="ru">Rheims S., Ryvlin P., Scherer C., Minotti L., Hoffmann D., Guenot M., Mauguiиre F., Benabid A.L., Kahane P. Analysis of clinical patterns and underlying epileptogenic zones of hypermotor seizures. Epilepsia. 2008; 49: 2030-2040.</mixed-citation><mixed-citation xml:lang="en">Rheims S., Ryvlin P., Scherer C., Minotti L., Hoffmann D., Guenot M., Mauguiиre F., Benabid A.L., Kahane P. Analysis of clinical patterns and underlying epileptogenic zones of hypermotor seizures. Epilepsia. 2008; 49: 2030-2040.</mixed-citation></citation-alternatives></ref><ref id="cit19"><label>19</label><citation-alternatives><mixed-citation xml:lang="ru">Ryvlin P., Minotti L., Demarquay G. et. al. Nocturnal hypermotor seizures, suggesting frontal lobe epilepsy, can originate in the insula. Epilepsia. 2006; 47: 755-765.</mixed-citation><mixed-citation xml:lang="en">Ryvlin P., Minotti L., Demarquay G. et. al. Nocturnal hypermotor seizures, suggesting frontal lobe epilepsy, can originate in the insula. Epilepsia. 2006; 47: 755-765.</mixed-citation></citation-alternatives></ref><ref id="cit20"><label>20</label><citation-alternatives><mixed-citation xml:lang="ru">Scheffer I., Bhatia K., Lopes-Cendes I. et. al. Autosomal dominant nocturnal frontal lobe epilepsy. A distinctive clinical disorder. Brain. 1995; 118: 61-73.</mixed-citation><mixed-citation xml:lang="en">Scheffer I., Bhatia K., Lopes-Cendes I. et. al. Autosomal dominant nocturnal frontal lobe epilepsy. A distinctive clinical disorder. Brain. 1995; 118: 61-73.</mixed-citation></citation-alternatives></ref><ref id="cit21"><label>21</label><citation-alternatives><mixed-citation xml:lang="ru">Staack A.M., Bilic S., Wendling A.S., Scholly J., Kraus U., Strobl K., Bodin F., Zentner J., Steinhoff B.J. Hyperkinetic seizures in patients with temporal lobe epilepsy: clinical features and outcome after temporal lobe resection. Epilepsia. 2011; 52 (8): 1439-1446.</mixed-citation><mixed-citation xml:lang="en">Staack A.M., Bilic S., Wendling A.S., Scholly J., Kraus U., Strobl K., Bodin F., Zentner J., Steinhoff B.J. Hyperkinetic seizures in patients with temporal lobe epilepsy: clinical features and outcome after temporal lobe resection. Epilepsia. 2011; 52 (8): 1439-1446.</mixed-citation></citation-alternatives></ref><ref id="cit22"><label>22</label><citation-alternatives><mixed-citation xml:lang="ru">Tao Y., Guojun Z., Yuping W. и соавт. Surgical treatment of patients with drug-resistant hypermotor seizures. Epilepsia. 2010; 51: 2124-2130.</mixed-citation><mixed-citation xml:lang="en">Tao Y., Guojun Z., Yuping W. i soavt. Surgical treatment of patients with drug-resistant hypermotor seizures. Epilepsia. 2010; 51: 2124-2130.</mixed-citation></citation-alternatives></ref><ref id="cit23"><label>23</label><citation-alternatives><mixed-citation xml:lang="ru">Tinuper P., Cerullo A., Cirignotta F., Cortelli P., Lugaresi E., Montagna P. Nocturnal paroxysmal dystonia with short-lasting attacks: three cases with evidence for an epileptic frontal lobe origin of seizures. Epilepsia. 1990 Sep-Oct; 31 (5): 549-56.</mixed-citation><mixed-citation xml:lang="en">Tinuper P., Cerullo A., Cirignotta F., Cortelli P., Lugaresi E., Montagna P. Nocturnal paroxysmal dystonia with short-lasting attacks: three cases with evidence for an epileptic frontal lobe origin of seizures. Epilepsia. 1990 Sep-Oct; 31 (5): 549-56.</mixed-citation></citation-alternatives></ref><ref id="cit24"><label>24</label><citation-alternatives><mixed-citation xml:lang="ru">Tinuper P. Lugaresi E., Vigevano F. et. al. Nocturnal frontal lobe epilepsy. In Eds.: R. Guerrini et. al. / Epilepsy and movement disorders. Cambridge. 2001; 97-109.</mixed-citation><mixed-citation xml:lang="en">Tinuper P. Lugaresi E., Vigevano F. et. al. Nocturnal frontal lobe epilepsy. In Eds.: R. Guerrini et. al. / Epilepsy and movement disorders. Cambridge. 2001; 97-109.</mixed-citation></citation-alternatives></ref><ref id="cit25"><label>25</label><citation-alternatives><mixed-citation xml:lang="ru">Vigevano F., Fusco L. Hypnic tonic postural seizures in healthy children provide evidence for a partial epileptic syndrome of frontal origin. Epilepsia. 1993; 34: 110-119.</mixed-citation><mixed-citation xml:lang="en">Vigevano F., Fusco L. Hypnic tonic postural seizures in healthy children provide evidence for a partial epileptic syndrome of frontal origin. Epilepsia. 1993; 34: 110-119.</mixed-citation></citation-alternatives></ref><ref id="cit26"><label>26</label><citation-alternatives><mixed-citation xml:lang="ru">Vignatelli L., Bisulli F., Provini F., Naldi I., Pittau F., Zaniboni A., Montagna P., Tinuper P. Interobserver reliability of video recording in the diagnosis of nocturnal frontal lobe seizures. Epilepsia. 2007; 48 (8): 1506-1511.</mixed-citation><mixed-citation xml:lang="en">Vignatelli L., Bisulli F., Provini F., Naldi I., Pittau F., Zaniboni A., Montagna P., Tinuper P. Interobserver reliability of video recording in the diagnosis of nocturnal frontal lobe seizures. Epilepsia. 2007; 48 (8): 1506-1511.</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>
