Russian Journal of Child NeurologyRussian Journal of Child NeurologyРусский журнал детской неврологии2073-88032412-9178Publishing House ABV Press2510.17650/2073-8803-2014-9-3-6-12ORIGINAL REPORTSОРИГИНАЛЬНЫЕ СТАТЬИFOCAL EPILEPTIC MYOCLONUS: DIFFERENTIAL DIAGNOSIS AND PROGNOSISФокальный эпилептический миоклонус: дифференциальный диагноз и прогнозKvaskovaN. E.КвасковаН. Е.
Russian Federation
nekvaskova@mail.ruMukhinK. Yu.МухинК. Ю.
Russian Federation
MironovM. B.МироновМ. Б.
Russian Federation
Svt. Luka’s Institute of Child Neurology and Epilepsy, MoscowООО «Институт детской неврологии и эпилепсии им. Святителя Луки», Москва15072014936121804201518042015Copyright ©; 2014, ABV-PressCopyright ©; 2014, АБВ-пресс2014ABV-PressАБВ-прессhttps://rjdn.abvpress.ru/jour/about/editorialPolicieshttps://rjdn.abvpress.ru/jour/article/view/25

The paper describes the clinical, electroencephalographic (EEG), and neuroimaging features of focal epileptic myoclonus (FEM) and the results of therapy in patients with this type of seizure. The latter was identified in 2.5 % of the cases of epilepsy with onset beyond the age of 18 years (n = 1261). The age of onset of FЕМ was from 5 months to 23 years (mean 8.1 ± 1.6 years). The addition of secondary generalized seizures was stated in 90.3% of cases; that of focal motor seizures, epileptic aura, and negative FЕМ was seen in 64.5, 25.8, and 12.8 %, respectively. FЕМ was most commonly located in the flexor muscles of the upper extremities (22.6 %) and facial and upper limb muscles (25.6 %). The regional epileptoformactivity maximally located in the frontocentroparietal leads is a typical EEG pattern of FЕМ in all the patients. Magnetic resonance imaging most frequently revealed diffuse cortical and subcortical atrophy (29 %) and total cortical hemiatrophy (32 %). There was no case of complete remission of FЕМ. Monotherapy with antiepileptic drugs (AEDs) resulted in reduction of FЕМ in only 6.4 % of cases. During duo therapy, there was more than a 50 % reduction in FЕМ in 67.7 %. Our investigation has indicated that active FЕМ is a seizure type resistant to AEDs. This fact necessitates the detection of FЕМ at its early stages in order to predict the course of the disease and search for new therapies for epileptic syndromes associated with this type of seizures.

epilepsyfocal epileptic myoclonusKozhenvikov’s epilepsyKozhevnikov–Rasmussen’s encephalitisvideoelectroencephalographic monitoringэпилепсияфокальный эпилептический миоклонусэпилепсия Кожевниковаэнцефалит Кожевникова–Расмуссенавидеоэлектроэнцефалографический мониторинг1. Карлов В.А. Фармакорезистентность и толерантность при эпилепсии. Журн неврол психиатр 2008;108(10):75–80.Карлов В.А. Фармакорезистентность и толерантность при эпилепсии. Журн неврол психиатр 2008;108(10):75–80.2. Карлов В.А. Эпилепсия у детей и взрослых женщин и мужчин. Руководство для врачей. М.: Медицина, 2010. 717 с.Карлов В.А. Эпилепсия у детей и взрослых женщин и мужчин. Руководство для врачей. М.: Медицина, 2010. 717 с.3. Котов А.С., Рудакова И.Г., Мухин К.Ю. и др. Энцефалит Расмуссена. Описание двух клинических случаев. Рус журн дет неврол 2009;4(2):42–50.Котов А.С., Рудакова И.Г., Мухин К.Ю. и др. Энцефалит Расмуссена. Описание двух клинических случаев. Рус журн дет неврол 2009;4(2):42–50.4. Мухин К.Ю., Кваскова Н.Е., Миронов М.Б. и др. Кожевниковская эпилепсия при клещевом русском весенне-летнем энцефалите. Детская больница 2011;2:30–4.Мухин К.Ю., Кваскова Н.Е., Миронов М.Б. и др. Кожевниковская эпилепсия при клещевом русском весенне-летнем энцефалите. Детская больница 2011;2:30–4.5. Мухин К.Ю., Миронов М.Б., Кваскова Н.Е. и др. Эпилепсия Кожевникова у детей. Рус журн дет неврол 2006;1(2):19–29.Мухин К.Ю., Миронов М.Б., Кваскова Н.Е. и др. Эпилепсия Кожевникова у детей. Рус журн дет неврол 2006;1(2):19–29.6. Мухин К.Ю., Петрухин А.С., Холин А.А. Эпилептические энцефалопатии и схожие синдромы у детей. М.: АртСервис Лтд, 2011. 680 с.Мухин К.Ю., Петрухин А.С., Холин А.А. Эпилептические энцефалопатии и схожие синдромы у детей. М.: АртСервис Лтд, 2011. 680 с.7. Мухин К.Ю., Пилия С.В., Волкова Э.Ю. и др. Применение высоких доз пирацетама в лечении синдрома кожевниковской эпилепсии. Журн неврол психиатр 2007;107(3):30–6.Мухин К.Ю., Пилия С.В., Волкова Э.Ю. и др. Применение высоких доз пирацетама в лечении синдрома кожевниковской эпилепсии. Журн неврол психиатр 2007;107(3):30–6.8. Темин П.А., Казанцева Л.З. Наследственные нарушения нервно-психического развития детей: руководство для врачей. М.: Медицина, 2001. 432 с.Темин П.А., Казанцева Л.З. Наследственные нарушения нервно-психического развития детей: руководство для врачей. М.: Медицина, 2001. 432 с.9. Bancaud J., Bonis A., Talairach J. et al. Kojewnikow syndrome and somato-motor attacks (clinical, E.E.G., E.M.G. and S.E.E.G. study). Encephale 1970;59(5):391–438.Bancaud J., Bonis A., Talairach J. et al. Kojewnikow syndrome and somato-motor attacks (clinical, E.E.G., E.M.G. and S.E.E.G. study). Encephale 1970;59(5):391–438.10. Bien C.G., Elger C.E. Epilepsia partialis continua: semiology and differential diagnoses. Epileptic Disord 2008;10(1): 3–7.Bien C.G., Elger C.E. Epilepsia partialis continua: semiology and differential diagnoses. Epileptic Disord 2008;10(1): 3–7.11. Bulteau C., Otsuki T., Delalande O. Epilepsy surgery for hemispheric syndromes in infants: hemimegalencepahly and hemispheric cortical dysplasia. Brain Dev 2013;35(8):742–7.Bulteau C., Otsuki T., Delalande O. Epilepsy surgery for hemispheric syndromes in infants: hemimegalencepahly and hemispheric cortical dysplasia. Brain Dev 2013;35(8):742–7.12. Caraballo R.H., Fortini S., Cersosimo R. et al. Rasmussen syndrome: an Argentinean experience in 32 patients. Seizure 2013;22(5):360–7.Caraballo R.H., Fortini S., Cersosimo R. et al. Rasmussen syndrome: an Argentinean experience in 32 patients. Seizure 2013;22(5):360–7.13. Caviness J.N. Treatment of myoclonus. Neurotherapeutics 2014;11(1):188–200.Caviness J.N. Treatment of myoclonus. Neurotherapeutics 2014;11(1):188–200.14. Cockerell O.C., Rothwell J., Thompson P.D. et al. Clinical and physiological features of epilepsia partialis continua. Cases ascertained in the UK.Cockerell O.C., Rothwell J., Thompson P.D. et al. Clinical and physiological features of epilepsia partialis continua. Cases ascertained in the UK.15. Engel J. Jr. Report of the ILAE сlassification сore group. Epilepsia 2006;47(9):1558–68.Engel J. Jr. Report of the ILAE сlassification сore group. Epilepsia 2006;47(9):1558–68.16. Engel J. Jr.; International League Against Epilepsy (ILAE). 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.Engel J. Jr.; International League Against Epilepsy (ILAE). 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):796–803.17. Fattouch J., Di Bonaventura C., Di Gennaro G. et al. Electrical status epilepticus "invisible" to surface EEG in late-onset Rasmussen encephalitis. Epileptic Disord 2008;10(3):219–22.Fattouch J., Di Bonaventura C., Di Gennaro G. et al. Electrical status epilepticus "invisible" to surface EEG in late-onset Rasmussen encephalitis. Epileptic Disord 2008;10(3):219–22.18. Granata T., Andermann F. Rasmussen encephalitis. Handb Clin Neurol 2013;111:511–9.Granata T., Andermann F. Rasmussen encephalitis. Handb Clin Neurol 2013;111:511–9.19. Guerrini R., Takahashi T. Myoclonus and epilepsy. Handb Clin Neurol 2013;111:667–79.Guerrini R., Takahashi T. Myoclonus and epilepsy. Handb Clin Neurol 2013;111:667–79.20. Hamad A.P., Caboclo L.O., Centeno R. еt al. Hemispheric surgery for refractory epilepsy in children and adolescents: outcome regarding seizures, motor skills and adaptive function. Seizure 2013;22(9):752–6.Hamad A.P., Caboclo L.O., Centeno R. еt al. Hemispheric surgery for refractory epilepsy in children and adolescents: outcome regarding seizures, motor skills and adaptive function. Seizure 2013;22(9):752–6.21. Honda R., Kaido T., Sugai K. еt al. Longterm developmental outcome after early hemispherotomy for hemimegalencephaly in infants with epileptic encephalopathy. Epilepsy Behav 2013;29(1):30–5.Honda R., Kaido T., Sugai K. еt al. Longterm developmental outcome after early hemispherotomy for hemimegalencephaly in infants with epileptic encephalopathy. Epilepsy Behav 2013;29(1):30–5.22. Honda R., Saito Y., Nakagawa E. еt al. Focal cortical myoclonus in rolandic cortical dysplasia presenting as hemifacial twitching. Brain Dev 2012;34(10): 886–90.Honda R., Saito Y., Nakagawa E. еt al. Focal cortical myoclonus in rolandic cortical dysplasia presenting as hemifacial twitching. Brain Dev 2012;34(10): 886–90.23. Kojovic M., Cordivari C., Bhatia K. Myoclonic disorders: a practical approach for diagnosis and treatment. Ther Adv Neurol Disord 2011;4(1):47–62.Kojovic M., Cordivari C., Bhatia K. Myoclonic disorders: a practical approach for diagnosis and treatment. Ther Adv Neurol Disord 2011;4(1):47–62.24. Korman B., Krsek P., Duchowny M. et al. Early seizure onset and dysplastic lesion extent independently disrupt cognitive networks. Neurology 2013;81(8):745–51.Korman B., Krsek P., Duchowny M. et al. Early seizure onset and dysplastic lesion extent independently disrupt cognitive networks. Neurology 2013;81(8):745–51.25. Mameniskiene R., Bast T., Bentes C. et al. Clinical course and variability of non-Rasmussen, nonstroke motor and sensory epilepsiapartialis continua: a European survey and analysis of 65 cases. Epilepsia 2011;52(6):1168–76.Mameniskiene R., Bast T., Bentes C. et al. Clinical course and variability of non-Rasmussen, nonstroke motor and sensory epilepsiapartialis continua: a European survey and analysis of 65 cases. Epilepsia 2011;52(6):1168–76.26. Molyneux P.D., Barker R.A., Thom M. et al. Successful treatment of intractable epilepsia partialis continua with multiple subpial transections. J Neurol Neurosurg Psychiatry 1998;65(1):137–8.Molyneux P.D., Barker R.A., Thom M. et al. Successful treatment of intractable epilepsia partialis continua with multiple subpial transections. J Neurol Neurosurg Psychiatry 1998;65(1):137–8.27. Mukhin K.Y., Mameniskienė R., Mironov M.B. et аl. Epilepsia partialis continua in tick-borne Russian spring-summer encephalitis. Acta Neurol Scand 2012;125(5):345–52.Mukhin K.Y., Mameniskienė R., Mironov M.B. et аl. Epilepsia partialis continua in tick-borne Russian spring-summer encephalitis. Acta Neurol Scand 2012;125(5):345–52.28. Myoclonus and epilepsy in childhood Commission on Pediatric Epilepsy of the International League Against Epilepsy. Epilepsia 1997;38(11):1251–4.Myoclonus and epilepsy in childhood Commission on Pediatric Epilepsy of the International League Against Epilepsy. Epilepsia 1997;38(11):1251–4.29. Myoclonus and epilepsy. In: Epilepsy and movement disorders. R. Gueririni, J. Aicardi, F. Anderman et al. (eds.). Cambridge: University Press, 2002. Рр. 165–210.Myoclonus and epilepsy. In: Epilepsy and movement disorders. R. Gueririni, J. Aicardi, F. Anderman et al. (eds.). Cambridge: University Press, 2002. Рр. 165–210.30. Obeso J.A. Classification, clinical features and treatment of myoclonus. In: Movement Disorders. Neurologic principles and practice. R.L. Watts, W.C. Koller (eds.). New York: McGraw-Hill, 1997. Pp. 541–50.Obeso J.A. Classification, clinical features and treatment of myoclonus. In: Movement Disorders. Neurologic principles and practice. R.L. Watts, W.C. Koller (eds.). New York: McGraw-Hill, 1997. Pp. 541–50.31. Paiboonpol S. Epilepsia partialis continua as a manifestation of hyperglycemia. J Med Assoc Thai 2005;88(6):759–62.Paiboonpol S. Epilepsia partialis continua as a manifestation of hyperglycemia. J Med Assoc Thai 2005;88(6):759–62.32. Panayiotopoulos С.P. A сlinical guide to epileptic syndromes and their treatment. 2nd ed. London: Springer Ltd, 2010. 654 p.Panayiotopoulos С.P. A сlinical guide to epileptic syndromes and their treatment. 2nd ed. London: Springer Ltd, 2010. 654 p.33. Panayiotopoulos C.P. The new ILAE report on terminology and concepts for organization of epileptic seizures: a clinician's critical view and contribution. Epilepsia 2011;52(12): 2155–60.Panayiotopoulos C.P. The new ILAE report on terminology and concepts for organization of epileptic seizures: a clinician's critical view and contribution. Epilepsia 2011;52(12): 2155–60.34. Panayiotopoulos C.P. The new ILAE report on terminology and concepts for the organization of epilepsies: critical review and contribution. Epilepsia 2012;53(3):399–404.Panayiotopoulos C.P. The new ILAE report on terminology and concepts for the organization of epilepsies: critical review and contribution. Epilepsia 2012;53(3):399–404.35. Perez-Jimenez A., Garcia-Fernandez M., Santiago Mdel M., Fournier-Del Castillo MC. Video electroencephalographic diagnosis of epileptic and non-epileptic paroxysmal episodes in infants and children at the pre-school age. Rev Neurol 2012;54 Suppl 3:S59–66.Perez-Jimenez A., Garcia-Fernandez M., Santiago Mdel M., Fournier-Del Castillo MC. Video electroencephalographic diagnosis of epileptic and non-epileptic paroxysmal episodes in infants and children at the pre-school age. Rev Neurol 2012;54 Suppl 3:S59–66.36. Proposal for classification of epilepsies and epileptic syndrome Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1989;30(4):389–99.Proposal for classification of epilepsies and epileptic syndrome Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1989;30(4):389–99.37. Riqueta A., Auvina S., Cuisseta J.M. еt al. Epilepsia partialis continua and defects in the mitochondrial respiratory chain. Epilepsy Res 2008:78(1):1–6.Riqueta A., Auvina S., Cuisseta J.M. еt al. Epilepsia partialis continua and defects in the mitochondrial respiratory chain. Epilepsy Res 2008:78(1):1–6.38. Shain C., Ramgopal S., Fallil Z. еt al.; EPGP Investigators. Polymicrogyria-associated epilepsy: a multicenter phenotypic study from the Epilepsy Phenome/Genome Project. Epilepsia 2013;54(8):1368–75.Shain C., Ramgopal S., Fallil Z. еt al.; EPGP Investigators. Polymicrogyria-associated epilepsy: a multicenter phenotypic study from the Epilepsy Phenome/Genome Project. Epilepsia 2013;54(8):1368–75.39. So N.K., Gloor P. Electroencephalographic and electrocorticographic findings in chronic encephalitis of the Rasmussen type. In: Chronic encephalitis and epilepsy: Rasmussen’s syndrome. F. Andermann (ed.). Boston: Butterworth-Heinemann, 1991. 3745 р.So N.K., Gloor P. Electroencephalographic and electrocorticographic findings in chronic encephalitis of the Rasmussen type. In: Chronic encephalitis and epilepsy: Rasmussen’s syndrome. F. Andermann (ed.). Boston: Butterworth-Heinemann, 1991. 3745 р.40. Spalletti M., Comanducci A., Vagaggini A. еt al. Efficacy of lacosamide on seizures and myoclonus in a patient with epilepsia partialis continua. Epileptic Disord 2013;15(2):193–6.Spalletti M., Comanducci A., Vagaggini A. еt al. Efficacy of lacosamide on seizures and myoclonus in a patient with epilepsia partialis continua. Epileptic Disord 2013;15(2):193–6.41. Walker M.C., Schorge S., Kullmann D.M. еt al. Gene therapy in status epilepticus. Epilepsia 2013;54 Suppl 6:43–5.Walker M.C., Schorge S., Kullmann D.M. еt al. Gene therapy in status epilepticus. Epilepsia 2013;54 Suppl 6:43–5.42. Wang B., Cai F.C. Polyneuroelectrophysiological studies of myoclonus in children. Zhonghua Er Ke Za Zhi 2009;47(10):750–6.Wang B., Cai F.C. Polyneuroelectrophysiological studies of myoclonus in children. Zhonghua Er Ke Za Zhi 2009;47(10):750–6.