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Clinical and electroencephalographic characteristics of some forms of epileptic encephalopathies in infants: The data of the authors' follow-up observations

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Objective: to study clinical symptoms and brain activity in new forms of infantile epilepsy, such as malignant migrating partial seizures in infancy (MMPSI) and Markand-Blume-Ohtahara syndrome (MBOS) and to elaborate their differential diagnostic criteria for the timely choice of treatment policy and for the prediction of the disease.
Patients and methods. Thirty-eight children aged 1.5 months to 3 years with MMPSI and MBOS were examined. Their medical history and neurological examination data, electroencephalographic and neuroimaging characteristics, and the efficiency of chosen anticonvulsant therapy were investigated.
Results and discussion. The investigators revealed the following characteristics of these forms: the causes of these syndromes were highly diverse and unspecific; the neurological status was characterized by a variety of symptoms with an obvious delay in psycho-prespeech development concurrent with a high rate of partial focal (in MMPSI) and generalized (in MBOS) status epilepticus; The EEC characteristic sign was MISF and
«lafa» patterns in the children with MBOS and continuous migrating partial ictal status epilepticus patterns in those with MMPSI. These forms of epileptic encephalopathies are extremely drug-resistant and characterized by a high risk for a fatal outcome.

About the Authors

I. D. Lemeshko
University Children's Clinical Hospital, I.M. Sechenov First Moscow State Medical University
Russian Federation

K. V. Voronkova
N.I. Pirogov Russian National Research Medical University
Russian Federation


1. Анохин ПК. Проблема центра и периферии в современной физиологии нервной деятельности. В кн.: Проблема центра и периферии в нервной деятельности. Горький; 1935. С. 9-70 [Anohin PK. The problem of centre and periphery in modern physiology of nervous activity. In: Problema tsentra i periferii v nervnoi deyatel'nosti [The problem of center and periphery in the neural activity]. Gor'kii; 1935. P. 9-70].

2. Аршавский ИА. Физиологические механизмы и закономерности индивидуального развития. Москва: Наука; 1982. 270 с. [Arshavsky IA. Fiziologicheskie mekhanizmy I zakonomernosti individual'nogo razvitiya [Physiological mechanisms and regularities of individual development]. Moscow: Nauka; 1982. P. 270].

3. Volpe JJ. Neurology of the Newborn. Philadelphia; 1987.

4. Ohtahara Sh. Special Lecture. Evolution of Epilepsies and Epileptic Syndromes. Moscow, 1998.

5. Петрухин АС, Пылаева ОА, Воронкова КВ. Аггравация эпилепсии под влиянием антиэпилептических препаратов. Журнал неврологии и психиатрии им. С.С. Корсакова. 2006;106(3):26-30 [Petrukhin AS, Pylayeva OA, Voronkova KV. The aggravation of epilepsy under the influence of anti-epileptic drugs. Zhurnal Nevrologii i Psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry. 2006;106(3):26-30 (In Russ.)].

6. Карлов ВА. Эпилепсия у детей и взрослых мужчин и женщин. Москва: Медицина; 2010. C.720 [Karlov VA. Epilepsiya u detei I vzroslykh muzhchin i zhenshchin [Epilepsy inchildren and adult men and women]. Moscow: Medicina; 2010. P. 720].

7. Ohtahara Sh, Yamatogi Y. Epileptic encephalopathies in early infancy with suppression - burst. J Clin Neurophysiol. 2003;20(6): 398-407. doi: 10.1097/00004691-200311000-00003

8. Мухин КЮ. Идиопатическая фокальная эпилепсия с псевдогенерализованными приступами – особая форма эпилепсии в детском возрасте. Русский журнал детской невролии. 2009;4(2):3-19 [Mukhin KYu. Idiopathic focal epilepsy with pseudogeneralisation attacks – a special form of epilepsy in childhood. Russkii Zhurnal Detskoi Nevrologii = Russian Journal of Child Neurology. 2009;4(2): 3-19 (In Russ.)].

9. Coppola G, Plouin P, Chiron C, et al. Migrating partial seizures in infancy: a malignant disorder with developmental arrest. Epilepsia. 1995;36(10):1017-24. doi: 10.1111/j.1528-1157.1995.tb00961.x

10. Dulac O. Malignant migrating partial seizures in infancy. In: Roger J, Bureau M, Dravet Ch, et al, eds. Epileptic Syndromes in Infancy, Childhood and Adolescence. 4th ed. London: John Libbey Eurotext Ltd.; 2005. P. 73-6.

11. Matsumoto A, Miyazaki S, Hayakawa C, et al. Epilepsy in severe motor and intellectual disabilities syndrome (SMIDS) – a clinical and electroencephalographic study of epileptic syndromes. Epilepsy Res. 2007;77(2-3):120-7. doi: 10.1016/j.eplepsyres.2007.09.006

12. Холин АА, Ильина ЕС, Колпакчи ЛМ и др. Злокачественные мигрирующие парциальные приступы младенчества. Клиническое наблюдение 6 случаев. Русский журнал детской неврологии. 2007;II(2):25-38 [Kholin AA, Ilyina ES, Kolpakchi LM, et al. Malignant migrating partial seizures of infancy. Clinical observation of 6 cases. Russkii Zhurnal Detskoi Nevrologii = Russian Journal of Child Neurology. 2007;II(2):25-38 (In Russ.)].

13. Воронкова КВ. Эволюция эпилепсии и трансформация эпилептических приступов: Автореф. дисс. … докт. мед. наук. Москва; 2007. 51 c. [Voronkova KV. Evolyutsiya epilepsii i transformatsiya epilepticheskikh pristupov: Avtoref. diss. … dokt. med. nauk [Evolution of epilepsy and the transformation of epileptic seizures: Authoref. Diss. … Doct. Med. Sci]. Moscow; 2007. 51 p.].

14. Холин АА, Михайлова СВ, Ильина ЕС и др. Эпилептический статус мигрирующих фокальных приступов при GM2-ганглиозидозе. Редкое клиническое наблюдение. Русский журнал детской неврологии. 2008;1(1):30-6 [Kholin AA, Mikhaylova SV, Ilyina ES, et al. Status epilepticus migrating focal seizures in GM2-gangliosidosis. A rare clinical observation. Russkii Zhurnal Detskoi Nevrologii = Russian Journal of Child Neurology. 2008;1(1):30-6 (In Russ)].


For citations:

Lemeshko I.D., Voronkova K.V. Clinical and electroencephalographic characteristics of some forms of epileptic encephalopathies in infants: The data of the authors' follow-up observations. Neurology, Neuropsychiatry, Psychosomatics. 2016;(1S):41-46. (In Russ.)

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