FOCAL EPILEPTIC MYOCLONUS IN KOZHEVNIKOV–RASMUSSEN SYNDROME
https://doi.org/10.17650/2073-8803-2016-11-1-14-22
Abstract
The article presents the clinical, electroencephalographic (EEG) and neuroimaging features, as well as the results of treatment of patients with focal epileptic myoclonus (FEM) with the Kozhevnikov–Rasmussen syndrome (KRS). FEM in KRS-patients was identified in 11 cases, accounting for 0.9 % of all the cases of epilepsy with the onset of seizures up to 18 years (n = 1261). The age at onset of KRS ranged from 3 to 21 years (average – 9.2 ± 5.7 years). In the active period of the disease of all patients in the clinical picture the active FEM appeared and increasing frequency of the secondary generalized seizures (SGS). In addition SGS and FEM, the clinical picture of the disease in most patients (91 %) the focal motor (clonic) and the somatosensory focal seizures were observed. As the disease progressed, the FEM became more pronounced in frequency and intensity, seized more muscle groups, localizing mainly in the muscles of the trunk and limbs. The typical EEG pattern of FEM patients with KRS was regional epileptiform activity that occurs in the structure of the continued regional slowing localizing maximum of the fronto-central-temporal region. During the magnetic resonance tomography of the brain in dynamics all the patients observed the increase in total cortical hemiatrophy. In all the cases, the appointment of antiepileptic therapy resulted in a slowing of the FEM, however, a complete remission was reached at none of the patients. Two patients were made surgical treatment of epilepsy. In one case remission of epileptic seizures was observed after right-side hemispherotomy. Our study showed that FEM is very resistant type of epileptic seizures. This fact calls for the identification of the FEM at the early stages of the disease with the purpose to improve the prognosis, as well as for an earlier surgical treatment.
About the Authors
N. E. Kvaskova
Svt. Luka’s Institute of Child Neurology and Epilepsy
Russian Federation
6 Svetlaya St., Puchkovo Village, Pervomayskoe Settlement, Moscow, 143396
Russian Federation
6 Svetlaya St., Puchkovo Village, Pervomayskoe Settlement, Moscow, 143396
K. Yu. Mukhin
Svt. Luka’s Institute of Child Neurology and Epilepsy
Russian Federation
6 Svetlaya St., Puchkovo Village, Pervomayskoe Settlement, Moscow, 143396
Russian Federation
6 Svetlaya St., Puchkovo Village, Pervomayskoe Settlement, Moscow, 143396
References
1. Гуляева С.Е. Эпилепсия Кожевникова. Владивосток: Уссури, 2005. 262 с. [Gulyaevа S.Е. Kozhevnikov’s epilepsy. Vladivostok: Ussuri, 2005. 262 p. (In Russ.)].
2. Зенков Л.Р. Эпилепсия Кожевникова или «синдром Расмуссена»? Неврологический журнал 2001;(2):58–63. [Zenkov L.R. Kozhevnikov’s epilepsy or Rasmussen syndrome? Nevrologicheskiy zhurnal = Neurological Journal 2001;(2):58–63. (In Russ.)].
3. Карлов В.А. Судорожный и бессудорожный эпилептический статус. М., 2007. 81 с. [Karlov V.А. Сonvulsive and non-convulsive status epileptiсus. Мoscow, 2007. 81 p. (In Russ.)].
4. Карлов В.А. Фармакорезистентность и толерантность при эпилепсии. Журнал неврологии и психиатрии им. С.С. Корсакова 2008;108(10):75–80. [Karlov V.А. Farmacoresistance and tolerance at epilepsy. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2008;108(10):75–80. (In Russ.)].
5. Карлов В.А. Эпилепсия у детей и взрослых женщин и мужчин. Руководство для врачей. М.: Медицина, 2010. 717 с. [Karlov V.А. Epilepsy at children and adult women and men. Guidelines for physicians. Мoscow: Меditsina, 2010. 717 p. (In Russ.)].
6. Кожевников А.Я. Особый вид кортикальной эпилепсии. Медицинское обозрение 1894;42:97–118. [Kozhevnikov А.Ya. Special kind of the cortical epilepsy. Meditsinskoe obozrenie = Меdical Survey 1894;42:97–118. (In Russ.)].
7. Котов А.С., Рудакова И.Г., Мухин К.Ю. и др. Энцефалит Расмуссена. Описание двух клинических случаев. Русский журнал детской неврологии 2009;4(2):42–50. [Kotov А.S., Rudakovа I.G., Мukhin K.Yu. et al. Rasmussen encephalitis. Description of two clinical cases. Russkiy zhurnal detskoy nevrologii = Russian Journal of Child Neurology 2009;4(2):42–50. (In Russ.)].
8. Мухин К.Ю. Энцефалит Кожевникова - Расмуссена. В кн.: Мухин К.Ю., Петрухин А.С., Холин А.А. Эпилептические энцефалопатии и схожие синдромы у детей. М.: АртСервис ЛТД, 2011. С. 278–99. [Мukhin K.Yu. Kozhevnikov–Rasmussen encephalitis. In: Мukhin K.Yu., Petrukhin А.S., Kholin А.А. Epileptic encephalopathies and related syndromes at childhood. Мoscow: АrtServis LTD, 2011. Pp. 278–99. (In Russ.)].
9. Мухин К.Ю., Миронов М.Б., Кваскова Н.Е. и др. Эпилепсия Кожевникова у детей. Русский журнал детской неврологии 2006;1(2):19–29. [Мukhin K.Yu., Мironov М.B., Kvaskovа N.Е. et al. Kozhevnikov’s childhood epilepsy. Russkiy zhurnal detskoy nevrologii = Russian Journal of Child Neurology 2006;1(2):19–29. (In Russ.)].
10. Мухин К.Ю., Пилия С.В., Волкова Э.Ю. и др. Применение высоких доз пирацетама в лечении синдрома кожевниковской эпилепсии. Журнал неврологии и психиатрии им. С.С. Корсакова 2007;107(3):30–6. [Мukhin K.Yu., Piliya S.V., Volkovа E.Yu. et al. Application of high pieracetem dosages for the treatment of Kozhevnikov’s epilepsy. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2007;107(3): 30–6. (In Russ.)].
11. Bancaud J. Kojewnikow’s syndrome (epilepsia partialis continua) in children. In: Epileptic syndrome in infancy, childhood and adolescence. Ed. by J. Roger. 2nd edn. London: John Libbey, 1992. Pp. 363–79.
12. Bien C.G., Elger C.E. Epilepsia partialis continua: semiology and differential diagnoses. Epileptic Disord 2008;10(1):3–7.
13. Bien C.G., Elger C.E. Recent insights into Rasmussen encephalitis. Nervenarzt 2005;76(12):1470, 1472–4, 1477–80, 1484–7.
14. Bien C.G., Widman G., Urbach H. et al. The natural history of Rasmussen’s encephalitis. Brain 2002;125(Pt 8):1751–9.
15. Caviness J.N. Treatment of myoclonus. Neurotherapeutics 2014;11(1):188–200.
16. Chinchilla D., Dulac O., Robain O. et al. Reappraisal of Rasmussen’s syndrome with special emphasis on treatment with high doses of steroids. J Neurol Neurosurg Psychiatry 1994;57(11):1325–33.
17. Eileen P.G. Current review in clinical science. Struggling with Rasmussen’s Syndrome. Epilepsy Currents 2006;6(1):20–1.
18. 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.
19. Engel J. Jr. Report of the ILAE сlassification сore group. Epilepsia 2006;47(9):1558–68.
20. Granata T., Andermann F. Rasmussen encephalitis. Handb Clin Neurol 2013;111:511–9.
21. Gueririni R., Aicardi J., Anderman F. et al. Myoclonus and epilepsy. In: Epilepsy and movement disorders. Cambridge: University Press, 2002. Рр. 165–210.
22. Guerrini R., Takahashi T. Myoclonus and epilepsy. Handb Clin Neurol 2013;111:667–79.
23. 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.
24. Myoclonus and epilepsy in childhood. Commission on Pediatric Epilepsy of the International League Against Epilepsy. Epilepsia 1997;38(11):1251–4.
25. Proposal for revised classification of epilepsies and epileptic syndromes. Сommission on Classification and Terminology of the International League Against Epilepsy. Epilepsia 1989;30(4):389–99.
26. Rasmussen T., Andermann F. Rasmussen’s syndrome: symptomatology of chronic encephalitis and seizures: 35-year experience with 51 cases. In: Epilepsy surgery. Ed. by H. Lüders. N.-Y.: Raven Press, 1991. Pp. 173–8.
27. Rasmussen T., Obszewski J., Lloyd-Smith D. Focal seizures due to chronic localized encephalitis. Neurology (Minneap) 1958;8(6):435–45.
28. Takahashi Y., Mori H., Mishina M. et al. Autoantibodies and cell-mediated autoimmunity to NMDA-type GluRepsilon2 in patients with Rasmussen’s encephalitis and chronic progressive epilepsia partialis continua. Epilepsia 2005;46 Suppl 5:152–8.
29. Walker M.C., Schorge S., Kullmann D.M. еt al. Gene therapy in status epilepticus. Epilepsia 2013;54 Suppl 6:43–5.
Review
For citations:
Kvaskova N.E., Mukhin K.Yu. FOCAL EPILEPTIC MYOCLONUS IN KOZHEVNIKOV–RASMUSSEN SYNDROME. Russian Journal of Child Neurology. 2016;11(1):14-22. (In Russ.) https://doi.org/10.17650/2073-8803-2016-11-1-14-22
Views:
1685
Email this article 