CLINICAL AND ELECTROENCEPHALOGRAPHIC SEMIOLOGY OF MYOCLONIC-ATONIC SEIZURES

Abstract: myoclonic-atonic seizures (MAS) – short epileptic generalized seizures in the form of sudden falling spells (or drop attacks). Inour study were vealed MAS in 1.3% of all cases of epilepsy with onset of seizures before 18 years old (n=1261). Predominance of man among the patients was revealed (76.5% men versus 23.5% woman). The most often in the patients with MAS myoclonic-astatic epilepsy (MAE) was diagnosed – 41,2% of cases. Symptomatic and cryptogenic forms of focal epilepsy were diagnosed in 35.2% of the patients. Lennox-Gastaut syndrome was revealed in 23.6% of cases. Onset of epilepsy with MAS varied widely – from 9 month to 6 year soflife; middle age of onset was 3.4 years±1.28 years. In all the cases MAS occurred in combination with other types of seizures: there can be any of 9 differenttypes of seizures. The most often myoclonic seizures (70.6% of cases), generalized convulsive seizures (47.1%) and absences (47.1%) were registered. Remission was achieved in 64.7% cases of epilepsy, associated with MAS. Reduction of seizure frequency ≥50% on antiepileptic treatment was achieved in 29.4% of the patients. Only in one patient (5.9%) the therapy was not effective. Our study demonstrated different efficacyof antiepileptic drugs in the treatment of different epileptic syndromes, associated with MAS.

myoclonic-atonic seizures, myatonic, myoclonic-astatic, Doose syndrome, myoclonic-astatic epilepsy, symptomatic epilepsy, secondary bilateral synchrony

1. Миронов М.Б., Мухин К.Ю. Исходы и трансформация фебрильных приступов у детей по данным Института детской неврологии и эпилепсии имени Святителя Луки. Русский журнал детской неврологии, 2012, том VII, выпуск 4. Стр.3-16.

2. Миронов М.Б., Мухин К.Ю., Петрухин А.С., Холин А.А. Контроль эффективности лечения пациентов с юношескими формами идиопатической генерализованной эпилепсии и состояние «псевдоремиссии». Журнал неврологии и психиатрии им. С.С. Корсакова. 2005; 105(8): 24-28.

3. Мухин К.Ю. Эпилепсия с миоклоническиастатическими приступами (синдром Дозе). В кн. под ред. К.Ю. Мухина, А.С. Петрухина. Идиопатические формы эпилепсии: систематика, диагностика, терапия. М. 2000; 150-157.

4. Мухин К.Ю., Миронов М.Б., Тысячина М.Д., Алиханов А.А., Петрухин А.С. Электро-клиническая характеристика больных симптоматической фокальной эпилепсией с феноменом вторичной билатеральной синхронизации на ЭЭГ. Русский журнал детской неврологии. 2006; 1 (1): 6-17.

5. Мухин К.Ю., Петрухин А.С., Рыкова Е.А. Генерализованная эпилепсия с миоклонически-астатическими приступами: диагностика и терапия. Неврологический журнал. 1996; 1 (1): 18-21.

6. Arzimanoglou A., Resnick T. All children who experience epileptic falls donot necessarily have Lennox-Gastaut syndrome but many do. Epileptic Disord. 2011; 13: 3-13.

7. Blume W.T., Pillay N. Electroencephalographic and clinical correlates of secondary bilateral synchrony. Epilepsia. 1985; 26 (6): 636-641.

8. Bonanni P., Parmeggiani L., Guerrini R. Different neurophysiologic patterns of myoclonus characterize Lennox-Gastaut syndrome and myoclonic astatic epilepsy. Epilepsia. 2002; 43: 609-615.

9. Deng J., Zhang Y.H., Liu X.Y., Yang Z.X. исоавт. Electroclinical features of myoclonicatonic epilepsy. Zhonghua Er Ke Za Zhi (China). 2011; 49 (8): 577-582.

10. Doose H., Gerken H., Leonhardt R., Volzke E., Volz C. Centrencephalic myoclonic–astatic petit mal. Clinical and genetic investigations.Neuropediatrie. 1970; 2: 59-78.

11. Doose H. EEG in Childhood Epilepsy. Initial Presentation and Long-term Follow-up. Hamburg, John LibbeyEurotext. 2003; 138-149.

12. Doose H. Myoclonic astatic epilepsy of early childhood. In: Epileptic syndromes in infancy, childhood and adolescence. Eds. J.Roger, M. Bureau, Ch. Dravet et al. London, 1992; 103-114.

13. Guerrini R., Mari F., Dravet Ch. Idiopathic myoclonic epilepsies in infancy and early childhood. In: Eds. M. Bureau, P. Genton, C. Dravet, A.V. Delgado-Escueta, C.A. Tassinari, P. Tomas, P. Wolf. Epileptic Syndromes in infancy, childhood and adolescence. – 5th edition with video. Paris. 2012; 157-173.

14. Guerrini R., Parmeggiani L., Bonanni P., Kaminska A., Dulac O. Myoclonic – astatic epilepsy. In: Eds. J. Roger, M. Bureau, C. Dravet, P. Genton, C.A. Tassinari, P. Wolf. Epileptic Syndromes in infancy, childhood and adolescence. 4th edition with video – John LibbeyEurotext Ltd. Montrouge. 2005; 115 -124.

15. Holthausen H., Fogarasi A., Arzimanoglou A., Kahane Ph. Structural (symptomatic) focal epilepsies of childhood. In: Eds. M. Bureau, P. Genton, C. Dravet, A.V. Delgado-Escueta, C.A. Tassinari, P. Tomas, P. Wolf. Epileptic Syndromes in infancy, childhood and adolescence. 5th edition with video. Paris. 2012; 455-505.

16. ILAE report. Commission on terminology and classification.Epilepsia. 2001; 42 (6): 796-803.

17. Jiruska P., Marusic P., Jefferys J.G., Krsek P., Cmejla R., Sebronova V., Komarek V. Sturge-Weber syndrome: a favorable surgical outcome in a case with contralateral seizure onset and myoclonic-astatic seizures. Epileptic.Disord. 2011;13 (1): 76-81.

18. Kaminska A., Ickowicz A., Plouin P., Bru M.F., Dellatolas G., Dulac O. Delineation of cryptogenic Lennox-Gastaut syndrome and myoclonic-astatic epilepsy using multiple correspondence analysis. Epilepsy Res. 1999; 36: 15-29.

19. Kelley S.A., Kossoff E.H. Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress. Dev. Med. Child Neurol. 2010; 52(11): 988-993.

20. Lugaresi E., Pazzaglia P., Frank L. Evolution and prognosis of primary generalized epilepsies of the petit mal absence type. In: Eds. E. Lugaresi. Evolution and prognosis of epilepsies. Bologna, AuloGagg. 1973; 3-22.

21. Mukhin K.Yu., Medvedev M.I., Petrukhin A.S. Epilepsy with myoclonic – astatic seizures: diagnostic criterion and treatment. Brain & Development. 1998; 20 (6): 481.

22. Panayiotopoulos C.T. Idiopathic Generalized Epilepsies. N.Y. 2012; 5-11.

23. Proposal for revised classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. 1989 Jul-Aug;30(4):389-99.

24. Stephani U. The Natural History of Myoclonic Astatic Epilepsy (Doose Syndrome) and Lennox-Gastaut Syndrome.Epilepsia. 2006; 47(2): 53-55.

25. Trivisano M., Specchio N., Cappelletti S., Di Ciommo V., Claps D., Specchio L.M., Vigevano F., Fusco L. Myoclonic astatic epilepsy: an age-dependent epileptic syndrome with favorable seizure outcome but variable cognitive evolution. Epilepsy Res. 2011; 97(1-2): 133-141.