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Long-term follow-up in patients with catastrophic epilepsies

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Objective: to investigate the features of the course and long-term outcome of catastrophic epilepsies.
Patients and methods. A total of 130 patients (62 (47.7%) men and 68 (52.3%) women) aged 21 to 78 years were examined. The follow-up was 1 year to 14 years. The inclusion criteria for the investigation were above 18 years of age; severe epilepsy with daily seizures considered as catastrophic epilepsy. The exclusion criteria were a history of nonepileptic seizures of any etiology, as well as noncompliance. The examination included history data collection; seizure diary analysis; clinical and neurological examination; routine EEG and/or EEG-video monitoring; brain magnetic resonance imaging; and laboratory tests.
Results and discussion. The onset of epilepsy immediately with catastrophic seizures was observed in 28 (21.5%) patients; this onset was most frequently seen in 18 of the 28 children. At the same time, catastrophic seizures did not appear immediately, but it took some time for them to occur after the onset of active epilepsy in 102 (78.5%) patients. Moreover, the onset of catastrophic seizures could not be associated with any external influences or errors in 58 (56.9%) of these patients taking the drugs. Transition to catastrophic epilepsy could occur both abruptly in 11 (10.8%) patients and gradually in 91 (89.2%). After treatment correction, 16.8% of patients achieved remission at the time of study completion; the frequency of seizures decreased by 50% or more in 27.1% of cases; the effect was absent in 56.1%.
Conclusion. Based on the findings, it can be concluded that in catastrophic epilepsy, the probability exists for spontaneous occurrence and remission of destructive seizures, frequently regardless of the therapy. The onset of the disease and that of catastrophic seizures can be spread in time. The efficiency of treatment remains low in patients with catastrophic epilepsies.

About the Authors

K. V. Firsov
M.F. Vladimirsky Moscow Regional Research Clinical Institute
Russian Federation
61/2, Shchepkin St., Build. 1, Moscow 129110

A. S. Kotov
M.F. Vladimirsky Moscow Regional Research Clinical Institute
Russian Federation
61/2, Shchepkin St., Build. 1, Moscow 129110


1. Howard MA, Baraban SC. Catastrophic Epilepsies of Childhood. Annu Rev Neurosci. 2017 Jul 25;40:149-166. doi: 10.1146/annurev-neuro-072116-031250.

2. Kuzniecky R. Catastrophic focal epilepsy. Epilepsia. 2004;45 Suppl 4:2-3.

3. Fisher RS, Cross JH, French JA, et al. Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):522-530. doi: 10.1111/epi.13670. Epub 2017 Mar 8.

4. Scheffer IE, Berkovic S, Capovilla G, et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia. 2017 Apr;58(4):512-521. doi: 10.1111/epi.13709. Epub 2017 Mar 8.

5. Avakyan GN, Blinov DV, Lebedeva AV, et al. Klassifikatsiya epilepsii Classification of epilepsy the International League against epilepsy: a revised and updated 2017. Epilepsiya i paroksizmal'nye sostoyaniya. 2017;9(1):6-25. (In Russ.)

6. Kotov AS, Tolstova NV. On the issue of idiopathic generalized and cryptogenic focal epilepsy in adolescents and young adults. Nevrologicheskii zhurnal. 2012;(1):21-5. (In Russ.)].

7. Petrukhin AS, Mukhin KYu, Blagosklonova NK, Alikhanov AA. Epileptologiya detskogo vozrasta [Epileptology of childhood]. Moscow; 2000. 623 p.

8. Editorial. Recommendations of the expert Council on neurophysiology of the Russian antiepileptic League on routine EEG. Epilepsiya i paroksizmal'nye sostoyaniya. 2016; 8(4):99-108. (In Russ.)

9. Lombardo AJ, Kuzniecky R, Powers RE, Brown GB. Altered brain sodium channel transcript levels in human epilepsy. Brain Res Mol Brain Res. 1996 Jan;35(1-2):84-90.

10. Lö scher W, Schmidt D. Experimental and clinical evidence for loss of effect (tolerance) during prolonged treatment with antiepileptic drugs. Epilepsia. 2006 Aug;47(8):1253-84.

11. Vreugdenhil M, van Veelen CW, van Rijen PC, et al. Effect of valproic acid on sodium currents in cortical neurons from patients with pharmaco-resistant temporal lobe epilepsy. Epilepsy Res. 1998 Sep;32(1-2):309-20.

12. Avanzini G. Is tolerance to antiepileptic drugs clinically relevant? Epilepsia. 2006 Aug; 47(8):1285-7.

13. Remy S, Gabriel S, Urban BW, et al. A novel mechanism underlying drug resistance in chronic epilepsy. Ann Neurol. 2003 Apr;53(4): 469-79.

14. Von Oertzen J, Urbach H, Jungbluth S, et al. Standard magnetic resonance imaging is inadequate for patients with refractory focal epilepsy. J Neurol Neurosurg Psychiatry. 2002 Dec;73(6):643-7.

15. Baxter P, Clarke A, Cross H, et al. Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status. Seizure. 2003 Sep;12(6):379-87.

16. Nariai H, Duberstein S, Shinnar S. Treatment of Epileptic Encephalopathies: Current State of the Art. J Child Neurol. 2018 Jan;33(1): 41-54. doi: 10.1177/0883073817690290. Epub 2017 Jan 30.

17. Villanueva VE, Serratosa JM. The course of the catastrophic epilepsies. Rev Neurol. 2002 Mar; 16-31;34(6):501-5.

18. Park JT, Fernandez-Baca Vaca G, Tangen RB, et al. Noninvasive Presurgical Data for OneStage Leucotomy in Catastrophic Epilepsy. World Neurosurg. 2018 Aug;116:268-273. doi: 10.1016/j.wneu.2018.05.182. Epub 2018 Jun 1.

19. Grioni D, Landi A, Gasperini S, et al. Vagal Nerve Stimulation in the Treatment of DrugResistant Epileptic Encephalopathies in Inborn Errors of Metabolism: Report of 2 Cases. Child Neurol Open. 2015 Oct 25;2(4):2329048X15612432. doi: 10.1177/2329048X15612432.eCollection 2015 Oct-Dec.

20. Blumer D, Davies K, Alexander A, Morgan S. Major Psychiatric Disorders Subsequent to Treating Epilepsy by Vagus Nerve Stimulation. Epilepsy Behav. 2001 Oct;2(5): 466-472.

21. Rudakova IG, Belova YuA, Kotov AS. Pharmacoresistant epilepsy is treatable. Vestnik epileptologii. 2013;(1):3-7. (In Russ.)

22. Kitaeva VE, Kotov AS. The effectiveness of treatment of patients with a prolonged course of medial temporal lobe epilepsy. Epilepsiya i paroksizmal'nye sostoyaniya. 2018; 10(3):31-7. (In Russ.)

23. Kotov SV, Rudakova IG, Morozova OS. The influence of modern antiepileptic drugs on the quality of life of patients with epilepsy. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova. 2008;108(3):36. (InRuss.)

24. Glauser TA. Following catastrophic epilepsy patients from childhood to adulthood. Epilepsia. 2004;45 Suppl 5:23-6.


For citations:

Firsov K.V., Kotov A.S. Long-term follow-up in patients with catastrophic epilepsies. Neurology, Neuropsychiatry, Psychosomatics. 2019;11(4):77-81. (In Russ.)

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