Русский журнал детской неврологии. 2023; 18: 36-43
Современные подходы к диагностике и лечению синдрома Леннокса–Гасто (обзор литературы)
https://doi.org/10.17650/2073-8803-2023-18-4-36-43Аннотация
Несмотря на значительные успехи, достигнутые в эпилептологии, резистентные к терапии эпилепсии составляют примерно 30 % среди всех форм этого заболевания. К таким заболеваниям относится в том числе синдром ЛенноксаГасто – классическая энцефалопатия развития и эпилептическая с дебютом в детском возрасте, характеризующаяся резистентностью к терапии, тяжелым течением и неблагоприятным прогнозом. У пациентов этой категории сохраняет высокую актуальность поиск новых эффективных антиэпилептических препаратов, особенно в тех случаях, когда многочисленные комбинации антиэпилептических препаратов не дают эффекта, оперативное лечение невозможно и альтернативные методы (стимуляция блуждающего нерва и кетогенная диета) малоэффективны. Авторы представляют обзор литературы, посвященной современной дефиниции и диагностическим критериям синдрома Леннокса–Гасто, методам диагностики и лечению этой формы эпилепсии, имеющей тяжелое течение и в целом неблагоприятный прогноз.
Список литературы
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26. Juhász C. Prefrontal cortex in the driving seat of epileptic networks in Lennox–Gastaut syndrome. Neurology 2019;16;93(3):91, 92.
27. Kerr M., Kluger G., Philip S. Evolution and management of Lennox–Gastaut syndrome through adolescence and into adulthood: are seizures always the primary issue? Epileptic Disord 2011;13(Suppl 1): S15–S26. DOI: 10.1684/epd.2011.0409
28. Kluger G., Glauser T., Krauss G. et al. Adjunctive rufinamide in Lennox–Gastaut syndrome: A long-term, open-label extension study. Acta Neurol Scand 2010;122(3):202–8. DOI: 10.1111/j.1600-0404.2010.01334.x
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37. Nwosu G., Reddy S.B., Riordan H.R.M., Kang J.-Q. Variable expression of GABAA receptor subunit gamma 2 mutation in a nuclear family displaying developmental and encephalopathic phenotype. Int J Mol Sci 2022;23:9683. DOI: 10.3390/ijms23179683
38. Ostendorf A.P., Ng Y.T. Treatment-resistant Lennox–Gastaut syndrome: Therapeutic trends, challenges and future directions. Neuropsychiatr Dis Treat 2017;13:1131–40. DOI: 10.2147/NDT.S115996
39. Panayiotopoulos C.P. A Clinical Guide TO Epileptic Syndromes and Their Treatment: Based on the ILAE Classifications and Practice Parameter Guidelines. 2nd edn. London: Springer, 2010. 654 p.
40. Panayotopoulos С.P. Principles of therapy in the epilepsies. In: A Clinical Guide to Epileptic Syndromes and Their Treatment. London: Springer, 2007. Pp. 155–184.
41. Samanta D. Management of Lennox–Gastaut syndrome beyond childhood: A comprehensive review. Epilepsy Behav 2021;114(Pt A): 107612. DOI: 10.1016/j.yebeh.2020.107612
42. Sankar R., Chez M., Pina-Garza J.E. et al. Proposed anti-seizure medication combinations with rufinamide in the treatment of Lennox– Gastaut syndrome: Narrative review and expert opinion. Seizure 2023;110:42–57. DOI: 10.1016/j.seizure.2023.05.018
43. Scheffer I.E., Berkovic S., Capovilla G. et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia 2017;58(4):512–21.
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45. Specchio N., Wirrell E.C., Scheffer I.E. et al. ILAE classification and definition of epilepsy syndromes with onset in childhood: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022;63(6):1398–442.
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47. Wirrell E.С., Nabbout R., Scheffer I. et al. Methodology for classification and definition of epilepsy syndromes: Report of the ILAE Task Force on Nosology and Definitions. Epilepsia 2022;63(6):1333–48.
Russian Journal of Child Neurology. 2023; 18: 36-43
The modern approaches to the diagnostics and treatment of Lennox–Gastaut syndrome (literature review)
https://doi.org/10.17650/2073-8803-2023-18-4-36-43Abstract
Despite significant advances made in epileptology, treatment-resistant epilepsy accounts for approximately 30 % of all forms of this disease. Such diseases include, among others, Lennox–Gastaut syndrome – a classic developmental and epileptic encephalopathy with onset in childhood, characterized by resistance to therapy, severe course and poor prognosis. For patients in this category, the search for new effective antiepileptic drugs remains highly relevant, especially in cases where numerous combinations of antiepileptic drugs do not produce an effect, surgical treatment is impossible, and alternative methods (vagus nerve stimulation and ketogenic diet) are ineffective. The authors present a review of the literature on the modern definition and diagnostic criteria of Lennox–Gastaut syndrome, diagnostic methods and treatment of this form of epilepsy, which has a severe course and a generally unfavorable prognosis.
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22. Genton P., Dravet Ch. The Lennox–Gastaut syndrome. In: Comprehensive Epileptology. 2nd edn. Eds.: J. Engel, T.A. Pedley. Philadelphia: Lippincott–Raven, 2007. Pr. 2417–2427.
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26. Juhász C. Prefrontal cortex in the driving seat of epileptic networks in Lennox–Gastaut syndrome. Neurology 2019;16;93(3):91, 92.
27. Kerr M., Kluger G., Philip S. Evolution and management of Lennox–Gastaut syndrome through adolescence and into adulthood: are seizures always the primary issue? Epileptic Disord 2011;13(Suppl 1): S15–S26. DOI: 10.1684/epd.2011.0409
28. Kluger G., Glauser T., Krauss G. et al. Adjunctive rufinamide in Lennox–Gastaut syndrome: A long-term, open-label extension study. Acta Neurol Scand 2010;122(3):202–8. DOI: 10.1111/j.1600-0404.2010.01334.x
29. Kluger G., Bauer B. Role of rufinamide in the management of Lennox–Gastaut syndrome (childhood epileptic encephalopathy). Neuropsychiatr Dis Treat 2007;3(1):3–11.
30. Koutroumanidis M. The role of EEG in the diagnosis and classification of the epilepsy syndromes: A tool for clinical practice by the ILAE Neurophysiology Task Force. Paris: John Libbey Eurotext, 2018. Pp. 221–226.
31. Kwan P., Brodie M.J. Refractory epilepsy: Mechanisms and solutions. Expert Rev Neurother 2006;6(3):397R406.
32. Mastrangelo M. Lennox–Gastaut syndrome: A state of the art review. Neuropediatrics 2017;48(3):143–51.
33. Montouris G., Aboumatar S., Burdette D. et al. Expert opinion: Proposed diagnostic and treatment algorithms for Lennox–Gastaut syndrome in adult patients. Epilepsy Behav 2020;110:107146.
34. Montouris G.D., Wheless J.W., Glauser T.A. The efficacy and tolerability of pharmacologic treatment options for Lennox–Gastaut syndrome. Epilepsia 2014;55(Suppl 4):10–20. DOI: 10.1111/epi.12732
35. Morita D.A., Glauser T. Lennox–Gastaut Syndrome. In: Pediatric Epilepsy. 4rd edn. New York: Demos, 2017. Pp. 451–466.
36. Mukhin K.Yu. Lennox–Gastaut syndrome. In: Epileptic Encephalopathies. Paris: John Libbey, 2014. Pp. 127–158
37. Nwosu G., Reddy S.B., Riordan H.R.M., Kang J.-Q. Variable expression of GABAA receptor subunit gamma 2 mutation in a nuclear family displaying developmental and encephalopathic phenotype. Int J Mol Sci 2022;23:9683. DOI: 10.3390/ijms23179683
38. Ostendorf A.P., Ng Y.T. Treatment-resistant Lennox–Gastaut syndrome: Therapeutic trends, challenges and future directions. Neuropsychiatr Dis Treat 2017;13:1131–40. DOI: 10.2147/NDT.S115996
39. Panayiotopoulos C.P. A Clinical Guide TO Epileptic Syndromes and Their Treatment: Based on the ILAE Classifications and Practice Parameter Guidelines. 2nd edn. London: Springer, 2010. 654 p.
40. Panayotopoulos S.P. Principles of therapy in the epilepsies. In: A Clinical Guide to Epileptic Syndromes and Their Treatment. London: Springer, 2007. Pp. 155–184.
41. Samanta D. Management of Lennox–Gastaut syndrome beyond childhood: A comprehensive review. Epilepsy Behav 2021;114(Pt A): 107612. DOI: 10.1016/j.yebeh.2020.107612
42. Sankar R., Chez M., Pina-Garza J.E. et al. Proposed anti-seizure medication combinations with rufinamide in the treatment of Lennox– Gastaut syndrome: Narrative review and expert opinion. Seizure 2023;110:42–57. DOI: 10.1016/j.seizure.2023.05.018
43. Scheffer I.E., Berkovic S., Capovilla G. et al. ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology. Epilepsia 2017;58(4):512–21.
44. Shorvon S. The causes of epilepsy. In: Epilepsy and Epileptic seizures. Eds.: S. Shorvon, R. Guerrini, M. Cook, S. Lhatoo. Oxford: Oxford University Press, 2013. Pp. 61–69.
45. Specchio N., Wirrell E.C., Scheffer I.E. et al. ILAE classification and definition of epilepsy syndromes with onset in childhood: Position statement by the ILAE Task Force on Nosology and Definitions. Epilepsia 2022;63(6):1398–442.
46. Tomson T., Marson A., Boon P. et al. Valproate in the treatment of epilepsy in girls and women of childbearing potential. Epilepsia 2015;56(7):1006–19. DOI: 10.1111/epi.13021
47. Wirrell E.S., Nabbout R., Scheffer I. et al. Methodology for classification and definition of epilepsy syndromes: Report of the ILAE Task Force on Nosology and Definitions. Epilepsia 2022;63(6):1333–48.
