EPILEPTIC SPASMS

Epileptic spasms are epileptic seizures with sudden flexion/extension or of the mixed flexion and extension type, mainly involving the proximal and truncal muscles, that are normally longer than myoclonic seizures but shorter than tonic seizures, and last for about 1 second. For diagnostics of epileptic spasms, it is necessary that they are combined with ictal and interictal epileptiform patterns on electroencephalography (EEG). The first detailed clinical description of seizures of the infantile spasms type was provided by English pediatrician W.J. West in 1841. The term of infantile spasms is limited with age and means epileptic spasms that occur to children in early infancy, usually up to 1 y.o. Infantile spasms cannot be synonymous to the West syndrome. Infantile spasms are a type of epileptic seizures and West syndrome is a form of epilepsy that is usually manifested through hypsarrhythmia on the EEG and mental retardation, apart from infantile spasms. Epileptic spasms is the term broader than infantile spasms. Committee of the International League Against Epilepsy (ILAE) recommends exactly the “epileptic spasms” term, as this type of seizures is not a prerogative of the West syndrome and can be observed in children older than 1 y.o. and even in adults. The authors provided a detailed review of modern references devoted to epileptic spasms including the history of the issue, determination of the term, and position of epileptic spasms in modern classification systems, approaches to diagnostics including differential diagnosis, treatment, and prognosis.

1. Алиханов А.А. Эпилептические энцефалопатии раннего детского возраста. В кн.: Эпилептология детского возраста. Под ред. А.С. Петрухина. М.: Медицина, 2000. С. 203–26. [Alikhanov A.A. Epileptic

2. encephalopathies of early infancy In: Epileptology of childhood. A.S. Petrukhin (ed.). Moscow: Medicine, 2000. Pp. 203–26. (In Russ.)].

3. Бадалян Л.О., Темин П.А., Никанорова М.Ю. и др. Инфантильные спазмы. Журнал неврологии и психиатрии им. С.С. Корсакова 1989;89(12):90–5. [Badalyan L.O., Tyomin P.A., Nikanorova M.Yu. et al. Infantile spasms. S.S. Korsakov Journal of Neurology and Psychiatrics 1989;89(12):90–5. (In Russ.)].

4. Глухова Л.Ю. Дифференциальный диагноз эпилепсии со схожими пароксизмальными состояниями и синдромами. В кн.: Эпилептические синдромы. Диагностика и терапия. Под ред. К.Ю. Мухина, А.С. Петрухина, М.Б. Миронова. М., 2008. С. 191–210. [Glukhova L.Yu. Differential diagnosis of epilepsy with similar paroxysmal states and syndromes. In: Epileptic syndromes. Diagnostics and therapy. K.Yu. Mukhin, A.S. Petrukhin, M.B. Mironov (eds.). Мoscow, 2008. Pp. 191–210. (In Russ.)].

5. Миронов М.Б., Мухин К.Ю., Кременчугская М.Р., Петрухин А.С. Видео- ЭЭГ-мониторинг в диагностике эпилептического негативного миоклонуса. Русский журнал детской неврологии 2009;4(2):51–9. [Mironov M.B., Mukhin K.Yu., Kremenchugskaya M.R., Petrukhin A.S. Video EEG Monitoring in diagnostics of epileptic negative mioclonus. Russian Journal of Child Neurology 2009;4(2):51–9. (In Russ.)].

6. Мухин К.Ю. Синдром Леннокса–Гасто. В кн.: Мухин К.Ю., Петрухин А.С., Холин А.А. Эпилептические энцефалопатии и схожие синдромы у детей. М.: АртСервис Лтд, 2011. С. 176–224. [Mukhin K.Yu. Lennox–Gastaut syndrome. In: Mukhin K.Yu., Petrukhin A.S., Kholin A.A. Epileptic encephalopathies and similar syndromes of children. Moscow: ArtService Ltd, 2011. Pp. 176–224. (In Russ.)].

7. Мухин К.Ю., Миронов М.Б., Боровиков К.С., Петрухин А.С. Фокальная эпилепсия детского возраста со структурными изменениями в мозге и доброкачественными эпилептиформными паттернами на ЭЭГ (ФЭДСИМ–ДЭПД) (предварительные результаты). Русский журнал детской неврологии 2010;5(1):3–18. [Mukhin K.Yu., Mironov M.B., Borovikov K.S., Petrukhin A.S. Focal epilepsy of hildhood with structural changes in the brain and benign epileptiform EEG patterns (FECSCB-BEDC) (preliminary results). Russian Journal of Child Neurology 2010;5(1):3–18. (In Russ.)].

8. Мухин К.Ю., Миронов М.Б., Тысячина М.Д. и др. Электро-клиническая характеристика больных симптоматической фокальной эпилепсией с феноменом вторичной билатеральной синхронизации на ЭЭГ. Русский журнал детской неврологии 2006;1(1):6–17. [Mukhin K.Yu., Mironov M.B., Tysyachina M.D. et al. Electroclinical characteristics of patients with symptomatic focal epilepsy with the phenomenon of secondary bilateral synchrony on the EEG. Russian Journal of Child Neurology 2006;1(1):6–17. (In Russ.)].

9. Мухин К.Ю., Холин А.А. Синдром Веста. Лечение и прогноз. В кн.: Мухин К.Ю., Петрухин А.С., Холин А.А. Эпилептические энцефалопатии и схожие синдромы у детей. М.: АртСервис Лтд, 2011. С. 134–56. [Mukhin K.Yu., Kholin A.A. West syndrome. Treatment and prognosis. In: Mukhin K.Yu., Petrukhin A.S., Kholin A.A. Epileptic encephalopathies and similar syndromes of children. Moscow: ArtService Ltd, 2011. Pp. 134–56. (In Russ.)].

10. Холин А.А., Мухин К.Ю. Синдром Веста. Этиология, электро-клинические характеристики и дифференциальный диагноз. В кн.: Мухин К.Ю., Петрухин А.С., Холин А.А. Эпилептические энцефалопатии и схожие синдромы у детей. М.: Арт-Сервис Лтд, 2011. С. 95–133. [Kholin A.A., Mukhin K.Yu. West syndrome. Etiology, electroclinical characteristics, and differential diagnosis. In: Mukhin K.Yu., Petrukhin A.S., Kholin A.A. Epileptic encephalopathies and similar syndromes of children. Moscow: ArtService Ltd, 2011. Pp. 95–133. (In Russ.)].

11. Arzimanoglou A., Guerrini R., Aicardi J. Aicardi’s epilepsy in children. 3rd ed. Philadelphia: Lippincott–Raven, 2004. Pр. 38–50.

12. Beaumanoir A., Mira L. Secondary bilateral synchrony: significant EEG pattern in frontal lobe seizures. In: Frontal lobe seizures and epilepsies in children. A. Beaumanoir, F. Andermann, P. Chauvel et al. (eds.). Paris: John Libbey Eurotext, 2003. Pр. 195–205.

13. Berg A.T., Berkovic S.F., Brodie M.J. Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Сlassification and Тerminology, 2005–2009. Epilepsia 2010;51(4):676–85.

14. Blume W.T. Lennox–Gastaut syndrome and secondary bilateral synchrony: a comparison. In: Epileptic seizures and syndromes. P. Wolf (еd.). London, 1994. Pр. 285–97.

15. Blume W.T., Luders H.O., Mizrahi Е. et al. Glossary of descriptive terminology for ictal semiology: report of the ILAE task force on classification and terminology. Epilepsia 2001;42(9):1212–8.

16. Cerullo A., Marini C., Carcanglu R. et al. Clinical and video-polygraphic features of epileptic spasms in adults with cortical migration disorder. Epileptic Disord 1999;1(1):27–33.

17. Chugani H.T., Asano E., Sood S. Surgical treatment of West syndrome. In: Progress in epileptic spasms and West syndrome. F. Guzzetta, B. Dalla Bernardina, R. Guerrini (еds.). UK: J.L., 2007. Pр. 143–51.

18. de Menezes M.A., Rho J.M. Clinical and electrographic features of epileptic spasms persisting beyond the second year of life. Epilepsia 2002;43(6):623–30.

19. Doose H. EEG in childhood epilepsy. Hamburg: John Libbey, 2003. P. 272–99.

20. d'Orsi G., Demaio V., Minervini M.G. Adult epileptic spasms: a clinical and videopolygraphic study. Epileptic Disord 2007;9(3):276–83.

21. Dulac O., Chiron C., Robain O. еt al. Infantile spasms: a pathophysiological hypothesis. Semin Pediatr Neurol 1994;1(2):83–9.

22. Engel J.; 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.

23. Engel J. Jr. Report of the ILAE сlassification сore group. Epilepsia 2006;47(9):1558–68.

24. Ferrari T.P., Hamad A.P., Caboclo L.O. et al. Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms. Epileptic Disord 2011;13(3):321–5.

25. Gobbi G., Bruno L., Pini A. et al. Periodic spasms: an unclassified type of epileptic seizures in childhood. Dev Med Child Neurol 1987;29(6):766–75.

26. Gobbi G., Frattini D., Boni A. et al. Spasms outside West syndrome. In: Progress in epileptic spasms and West syndrome. F. Guzzetta, B. Dalla Bernardina, R. Guerrini (eds.). UK: J.L., 2007. Pр. 71–82.

27. Gobbi G., Pini A., Parmeggiani A. еt al. Periodic spasms in cortical dysplasia. In: Dysplasias of Cerebral Cortex and Epilepsy. R. Guerrini еt al. (еds.). N.Y.: Lippincott– Raven, 1996. Pр. 311–21.

28. Guerrini R., Andermann F., Canapicchi R. et al. Dysplasias of cerebral cortex and epilepsy. Philadelphia: Lippincott–Raven, 1996. 461 р.

29. Guzzetta F., Dalla Bernardina B., Guerrini R. Progress in epileptic spasms and West syndrome. UK: J.L., 2007. 175 р.

30. Kaplan P.W., Fisher R.S. Imitators of Epilepsy. 2nd ed. N.Y.: Demos, 2005. 296 р.

31. Labate A., Colosimo E., Gambardella A. et al. Reflex periodic spasms induced by eating. Brain Dev 2006;28(3):170–4.

32. Lo W.D., Donat J.F., Wright F.S. Tonic and myoclonic seizures in Lennox-Gastaut syndrome mistaken as complex partial seizures. J Epilepsy 1991;4:211–5.

33. Ohtahara S., Yamatogi Y. Epileptic encephalopathies in early infancy with suppression-burst. J Clin Neurophysiol 2003;20(6):398–407.

34. Ohtsuka Y., Kobayashi K., Ogino T., Oka E. Spasms in clusters in epilepsies other than typical West syndrome. Brain Dev 2001;23(7):473–81.

35. Pachatz C., Fusco L., Vigevano F. Epileptic spasms and partial seizures as a single ictal event. Epilepsia 2003;44(5):693–700.

36. Panayiotopoulos C.P. A сlinical guide to epileptic syndromes and their treatment. London: Springer-Verlag, 2007. 578 р.

37. Pini A., Merlini L., Tomé F.M. et al. Merosin-negative congenital muscular dystrophy, occipital epilepsy with periodic spasms and focal cortical dysplasia. Report of three Italian cases in two families. Brain Dev 1996;18(4):316–22.

38. Tassinari C.A., Ambrosetto G. Tonic seizures in the Lennox–Gastaut syndrome: semiology and differential diagnosis. In: The Lennox–Gastaut syndrome. E. Niedermeyer, R. Degen (еds.). N.Y., 1988. Pр. 109–24.

39. Tinuper P., D'Orsi G., Bisulli F. et al. Malformation of cortical development in adult patients. Epileptic Disord 2003;5 Suppl 2:S85–90.

40. Vendrame M., Guilhoto L.M., Loddenkemper T. et al. Outcomes of epileptic spasms in patients aged less than 3 years: single-center United States experience. Pediatr Neurol 2012;46(5):276–80.

41. West W.J. On a particular form of infantile convulsions. Lancet 1841;1:724–5.

42. Zellweger H. Krampfe in kindersalter. Helvet Pediatr Acta 1948;Suppl 5:1.