Russian Journal of Child NeurologyRussian Journal of Child NeurologyРусский журнал детской неврологии2073-88032412-9178Publishing House ABV Press16310.17650/2073-8803-2016-11-3-8-21ORIGINAL REPORTSОРИГИНАЛЬНЫЕ СТАТЬИLANDAU–KLEFFNER SYNDROME (ACQUIRED EPILEPTIC APHASIA) WITH A FOCUS ON ELECTROENCEPHALOGRAPHIC CRITERIAСиндром Ландау–Клеффнера (приобретенная эпилептическая афазия) с фокусом на электроэнцефалографические критерииMukhinK. Yu.МухинК. Ю.
Russian Federation

6 Svetlaya St., Puchkovo Village, Pervomayskoe Settlement, Moscow, 143396

143396, Москва, пос. Первомайское, дер. Пучково, ул. Светлая, 6

center@epileptologist.ruSvt. Luka's Institute of Child Neurology and EpilepsyООО «Институт детской неврологии и эпилепсии им. Святителя Луки»150720161138213011201630112016Copyright ©; 2016, ABV-PressCopyright ©; 2016, АБВ-пресс2016ABV-PressАБВ-прессhttps://rjdn.abvpress.ru/jour/about/editorialPolicieshttps://rjdn.abvpress.ru/jour/article/view/163

Landau–Kleffner syndrome (LKS) is a disease from the group of age-dependent epileptic encephalopathies with the phenomenon of prolonged spike–wave activity in slow-wave sleep, which is manifested by acquired sensorimotor aphasia, impaired higher mental functions concurrent with various epileptic seizures and diffuse electroencephalographic (EEG) epileptiform changes in the absence of cerebral structural changes. In 1957, W. M. Landau, a pediatric neurologist, and F. R. Kleffner, a logopedist, first described the clinical presentation of the disease in 6 patients. The author associated the occurrence of aphasia with epileptiform EEG activity rather than with epileptic seizures, i. e. they per se formulated the modern concept of epileptic encephalopathy. There has been currently evidence that prolonged diffuse epileptiform activity has a damaging effect on a child’s developing brain, which is seen in impairments of speech and other higher mental functions. The age of patients with manifestations of LKS (3–5 years) is a critical period for the development of speech function. According to the concept of H. Doose (2003), congenital brain maturation disorder underlies LKS. The paper considers ideas on the origin of the LKS, including the possible genetic bases of diseases, epidemiological aspects, clinical and EEG manifestations with a detailed description ictal and interictal EEG changes, and treatment approaches, including the author’s data. The disease is characterized by an electroclinical triad: seizures (may be absent or may be single in a history), impaired higher mental functions (with a predominance of aphasic disorders), epileptiform EEG activity (a combination of regional and diffuse patterns with an increase during sleep).

epilepsyepileptic encephalopathyphenomenon of prolonged spike–wave electroencephalographic activity in slow-wave sleep syndromeLandau–Kleffner syndromeetiologyclinical presentationdiagnosistreatmentэпилепсияэпилептическая энцефалопатияфеномен продолженной пик-волновой активности на электроэнце- фалограмме в фазу медленного снасиндром Ландау–Клеффнераэтиологияклиникадиагностикалечение1. Александрова Н.Ш. Детские афазии и синдром Ландау–Клеффнера в свете пластичности мозга. Журнал неврологии и психиатрии им. С.С. Корсакова 2004;104(6):54–8. [Aleksandrova N.Sh. Children aphasias and Landau–Kleffner syndrome in the light of brain flexibility. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2004;104(6):54–8. (In Russ.)].Александрова Н.Ш. Детские афазии и синдром Ландау–Клеффнера в свете пластичности мозга. Журнал неврологии и психиатрии им. С.С. Корсакова 2004;104(6):54–8. [Aleksandrova N.Sh. Children aphasias and Landau–Kleffner syndrome in the light of brain flexibility. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2004;104(6):54–8. (In Russ.)].2. Зенков Л.Р. Бессудорожные эпилептические энцефалопатии с психиатрическими, коммуникативными и поведенческими расстройствами. Вестник эпилептологии 2004; (2):7–11. [Zenkov L.R. Epileptic encephalopathies with psychiatric, communication and behaviour disorders. Vestnik epileptologii = Bulletin of Epileptology 2004;(2):7–11. (In Russ.)].Зенков Л.Р. Бессудорожные эпилептические энцефалопатии с психиатрическими, коммуникативными и поведенческими расстройствами. Вестник эпилептологии 2004; (2):7–11. [Zenkov L.R. Epileptic encephalopathies with psychiatric, communication and behaviour disorders. Vestnik epileptologii = Bulletin of Epileptology 2004;(2):7–11. (In Russ.)].3. Карлов В.А., Гнездицкий В.В. Бифокальная атипичная роландическая эпилепсия с апраксией языка. Журнал неврологии и психиатрии им. С.С. Корсакова 2004;104(12):4– 9. [Karlov V.A., Gnezditskiy V.V. Bifocal atypical rolandic epilepsy with lingual apraxia. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2004;104(12):4–9. (In Russ.)].Карлов В.А., Гнездицкий В.В. Бифокальная атипичная роландическая эпилепсия с апраксией языка. Журнал неврологии и психиатрии им. С.С. Корсакова 2004;104(12):4– 9. [Karlov V.A., Gnezditskiy V.V. Bifocal atypical rolandic epilepsy with lingual apraxia. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2004;104(12):4–9. (In Russ.)].4. Мухин К.Ю., Холин А.А., Петрухин А.С. и др. Электро-клиническая характеристика синдрома Ландау–Клеффнера. Журнал неврологии и психиатрии им. С.С. Корсакова 2003;103(9):16–27. [Mukhin K.Yu., Kholin A.A., Petrukhin А.S. et al. Electroclinical characteristis of Landau–Kleffner syndrome. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2003;103(9): 16–27. (In Russ.)].Мухин К.Ю., Холин А.А., Петрухин А.С. и др. Электро-клиническая характеристика синдрома Ландау–Клеффнера. Журнал неврологии и психиатрии им. С.С. Корсакова 2003;103(9):16–27. [Mukhin K.Yu., Kholin A.A., Petrukhin А.S. et al. Electroclinical characteristis of Landau–Kleffner syndrome. Zhurnal nevrologii i psikhiatrii im. S.S. Korsakova = S.S. Korsakov Journal of Neurology and Psychiatry 2003;103(9): 16–27. (In Russ.)].5. Мухин К.Ю. Синдром Ландау–Клеффнера. В кн.: Эпилепсия. Атлас электро- клинической диагностики. Под ред. К.Ю. Мухина, А.С. Петрухина, Л.Ю. Глуховой. М.: Альварес Паблишинг, 2004. С. 337–45. [Mukhin K.Yu. Landau– Kleffner syndrome. In: Epilepsy. Atlas of electroclinical diagnosis. Ed. by K.Yu. Mukhin, A.S. Petrukhin, L.Yu. Glukhova. Мoscow: Alvares Pаblishing, 2004. Pp. 337–45. (In Russ.)].Мухин К.Ю. Синдром Ландау–Клеффнера. В кн.: Эпилепсия. Атлас электро- клинической диагностики. Под ред. К.Ю. Мухина, А.С. Петрухина, Л.Ю. Глуховой. М.: Альварес Паблишинг, 2004. С. 337–45. [Mukhin K.Yu. Landau– Kleffner syndrome. In: Epilepsy. Atlas of electroclinical diagnosis. Ed. by K.Yu. Mukhin, A.S. Petrukhin, L.Yu. Glukhova. Мoscow: Alvares Pаblishing, 2004. Pp. 337–45. (In Russ.)].6. Мухин К.Ю., Холин А.А. Синдром Ландау–Клеффнера (приобретенная эпилептическая афазия). В кн.: Эпилептические энцефалопатии и схожие синдромы у детей. Под ред. К.Ю. Мухина, А.С. Петрухина, А.А. Холина. М.: АртСервис ЛТД, 2011. С. 343–65. [Mulhin K.Yu., Kholin А.А. Landau–Kleffner syndrome (acquired epileptic aphasia). In: Epileptic encephalopathies and similar syndromes in children. Ed. by K.Yu. Mukhin, A.S. Petrukhin, A.A. Kholin. Мoscow: ArtServis Ltd, 2011. Pp. 343–65. (In Russ.)].Мухин К.Ю., Холин А.А. Синдром Ландау–Клеффнера (приобретенная эпилептическая афазия). В кн.: Эпилептические энцефалопатии и схожие синдромы у детей. Под ред. К.Ю. Мухина, А.С. Петрухина, А.А. Холина. М.: АртСервис ЛТД, 2011. С. 343–65. [Mulhin K.Yu., Kholin А.А. Landau–Kleffner syndrome (acquired epileptic aphasia). In: Epileptic encephalopathies and similar syndromes in children. Ed. by K.Yu. Mukhin, A.S. Petrukhin, A.A. Kholin. Мoscow: ArtServis Ltd, 2011. Pp. 343–65. (In Russ.)].7. Никанорова М.Ю. Синдром Ландау–Клеффнера (приобретенная эпилептическая афазия). Вестник практической неврологии 1998;(4):114–6. [Nikanorova M.Yu. Landau– Kleffner syndrome (acquired epileptic aphasia). Vestnik prakticheskoy nevrologii = Bulletin of Practical Neurology 1998;(4): 114–6. (In Russ.)].Никанорова М.Ю. Синдром Ландау–Клеффнера (приобретенная эпилептическая афазия). Вестник практической неврологии 1998;(4):114–6. [Nikanorova M.Yu. Landau– Kleffner syndrome (acquired epileptic aphasia). Vestnik prakticheskoy nevrologii = Bulletin of Practical Neurology 1998;(4): 114–6. (In Russ.)].8. Arzimanoglou A., Guerrini R., Aicardi J. Aicardi’s epilepsy in children. 3rd edn. Philadelphia: Lippincott, 2004. Pp. 176–87.Arzimanoglou A., Guerrini R., Aicardi J. Aicardi’s epilepsy in children. 3rd edn. Philadelphia: Lippincott, 2004. Pp. 176–87.9. Atkins M., Nikanorova M. A prospective study of levetiracetam efficacy in epileptic syndromes with continuous spikes-waves during slow sleep. Seizure 2011;20(8):635–9. DOI: 10.1016/j.seizure.2011.06.007. PMID: 21752673.Atkins M., Nikanorova M. A prospective study of levetiracetam efficacy in epileptic syndromes with continuous spikes-waves during slow sleep. Seizure 2011;20(8):635–9. DOI: 10.1016/j.seizure.2011.06.007. PMID: 21752673.10. Beaumanoir A. The Landau–Kleffner syndrome. In: Epileptic syndromes in infancy, childhood and adolescence. Ed. by J. Roger, M. Bureau, Ch. Dravet et al. London: John Libbey, 1992. Pp. 231–44.Beaumanoir A. The Landau–Kleffner syndrome. In: Epileptic syndromes in infancy, childhood and adolescence. Ed. by J. Roger, M. Bureau, Ch. Dravet et al. London: John Libbey, 1992. Pp. 231–44.11. Blagosklonova N.K., Velichko M.A. Neurophysiological mechanisms of the Landau– Kleffner syndrome. Epilepsia 1999;40(Suppl 2):164–5.Blagosklonova N.K., Velichko M.A. Neurophysiological mechanisms of the Landau– Kleffner syndrome. Epilepsia 1999;40(Suppl 2):164–5.12. Buzatu M., Bulteau C., Altuzarra C. et al. Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia 2009;50(Suppl 7): 68–72. DOI: 10.1111/j.1528-1167.2009.02224.x. PMID: 19682056.Buzatu M., Bulteau C., Altuzarra C. et al. Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia 2009;50(Suppl 7): 68–72. DOI: 10.1111/j.1528-1167.2009.02224.x. PMID: 19682056.13. Covanis A. Landau–Kleffner syndrome. In: Atlas of Epilepsies. Vol. 2. Ed. by C.P. Panayiotopoulos. London: Springer, 2010. Pp. 905–11.Covanis A. Landau–Kleffner syndrome. In: Atlas of Epilepsies. Vol. 2. Ed. by C.P. Panayiotopoulos. London: Springer, 2010. Pp. 905–11.14. Cross J.H., Neville B.G. The surgical treatment of Landau–Kleffner syndrome. Epilepsia 2009;50(Suppl 7):63–7. DOI: 10.1111/j.1528-1167.2009.02223.x. PMID: 19682055.Cross J.H., Neville B.G. The surgical treatment of Landau–Kleffner syndrome. Epilepsia 2009;50(Suppl 7):63–7. DOI: 10.1111/j.1528-1167.2009.02223.x. PMID: 19682055.15. Deonna T., Prelaz-Girod A.C., Mayor-Dubois C., Roulet-Perez E. Sign language in Landau– Kleffner syndrome. Epilepsia;2009:50(Suppl 7):77–82. DOI: 10.1111/j.15281167.2009.02226.x. PMID: 19682058.Deonna T., Prelaz-Girod A.C., Mayor-Dubois C., Roulet-Perez E. Sign language in Landau– Kleffner syndrome. Epilepsia;2009:50(Suppl 7):77–82. DOI: 10.1111/j.15281167.2009.02226.x. PMID: 19682058.16. Doose H.P. EEG in childhood epilepsy. Hamburg: John Libbey, 2003. Pp. 202–10.Doose H.P. EEG in childhood epilepsy. Hamburg: John Libbey, 2003. Pp. 202–10.17. Engel J. Jr. 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. PMID: 11422340.Engel J. Jr. 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. PMID: 11422340.18. Fainberg N., Harper A., Tchapyjnikov D., Mikati M.A. Response to immunotherapy in a patient with Landau–Kleffner syndrome and GRIN2A mutation. Epileptic Disord 2016;18(1):97–100. DOI: 10.1684/epd.2016.0791.PMID: 26806548.Fainberg N., Harper A., Tchapyjnikov D., Mikati M.A. Response to immunotherapy in a patient with Landau–Kleffner syndrome and GRIN2A mutation. Epileptic Disord 2016;18(1):97–100. DOI: 10.1684/epd.2016.0791.PMID: 26806548.19. Fejerman N., Caraballo R., Dalla Bernardina B. Atypical evolutions of benign focal epilepsies in children. In: Benign focal epilepsies in infancy, childhood and adolescence. Ed. by N. Fejerman, R.H. Caraballo. Montrouge: JL Eurotext, 2007. Pp. 179–219.Fejerman N., Caraballo R., Dalla Bernardina B. Atypical evolutions of benign focal epilepsies in children. In: Benign focal epilepsies in infancy, childhood and adolescence. Ed. by N. Fejerman, R.H. Caraballo. Montrouge: JL Eurotext, 2007. Pp. 179–219.20. Genton P., Guerrini R. What differentiates Landau–Kleffner syndrome from the syndrome of continuous spikes and waves during slow sleep? Arch Neurol 1993;50(8):1008– 9. PMID: 7692838.Genton P., Guerrini R. What differentiates Landau–Kleffner syndrome from the syndrome of continuous spikes and waves during slow sleep? Arch Neurol 1993;50(8):1008– 9. PMID: 7692838.21. Guerrini R., Marini C. Epileptic encephalopathies. In: Epilepsy and epileptic seizures. Ed. by S. Shorvon, R. Guerrini, M. Cook, S. Lhatoo. UK: Oxford University Press, 2013. Pp. 177– 85.Guerrini R., Marini C. Epileptic encephalopathies. In: Epilepsy and epileptic seizures. Ed. by S. Shorvon, R. Guerrini, M. Cook, S. Lhatoo. UK: Oxford University Press, 2013. Pp. 177– 85.22. Imataka G., Arisaka O. Serial EEG study in a girl with Landau–Kleffner syndrome associated with continuous spikes and waves during slow sleep. Eur Rev Med Pharmacol Sci 2014;18(15):2145–7. PMID: 25070819.Imataka G., Arisaka O. Serial EEG study in a girl with Landau–Kleffner syndrome associated with continuous spikes and waves during slow sleep. Eur Rev Med Pharmacol Sci 2014;18(15):2145–7. PMID: 25070819.23. Kanazawa O., Tohyama J., Akasaka N. et al. Relationship between clinical characteristics and magnetoencephalographic findings in typical and atypical benign childhood epilepsy with centrotemporal spikes. Epilepsia 2005;46(Suppl 1):72.Kanazawa O., Tohyama J., Akasaka N. et al. Relationship between clinical characteristics and magnetoencephalographic findings in typical and atypical benign childhood epilepsy with centrotemporal spikes. Epilepsia 2005;46(Suppl 1):72.24. Kleffner F.R., Landau W.M. The Landau–Kleffner syndrome. Epilepsia 2009;50(Suppl 7):3. DOI: 10.1111/j.1528-1167.2009.02208.x. PMID: 19682040.Kleffner F.R., Landau W.M. The Landau–Kleffner syndrome. Epilepsia 2009;50(Suppl 7):3. DOI: 10.1111/j.1528-1167.2009.02208.x. PMID: 19682040.25. Lagae L. Rational treatment options with AEDs and ketogenic diet in Landau–Kleffner syndrome: still waiting after all these years. Epi lepsia 2009;50(Suppl 7):59–62. DOI: 10.1111/j.1528-1167.2009.02222.x. PMID: 19682054.Lagae L. Rational treatment options with AEDs and ketogenic diet in Landau–Kleffner syndrome: still waiting after all these years. Epi lepsia 2009;50(Suppl 7):59–62. DOI: 10.1111/j.1528-1167.2009.02222.x. PMID: 19682054.26. Landau W.M., Kleffner F.R. Syndrome of acquired aphasia with convulsive disorder in children. Neurology 1957;7(4):523–30. PMID: 13451887.Landau W.M., Kleffner F.R. Syndrome of acquired aphasia with convulsive disorder in children. Neurology 1957;7(4):523–30. PMID: 13451887.27. Lesca G., Rudolf G., Bruneau N. et al. GRIN2A mutations in acquired epileptic aphasia and related childhood focal epilepsies and encephalopathies with speech and language dysfunction. Nat Genet 2013;45(9):1061–6. DOI: 10.1038/ng.2726. PMID: 23933820.Lesca G., Rudolf G., Bruneau N. et al. GRIN2A mutations in acquired epileptic aphasia and related childhood focal epilepsies and encephalopathies with speech and language dysfunction. Nat Genet 2013;45(9):1061–6. DOI: 10.1038/ng.2726. PMID: 23933820.28. Libenson M. Practical approach to electroencephalography. Philadelphia: Saunders Elsevier, 2010. Pp. 256–7.Libenson M. Practical approach to electroencephalography. Philadelphia: Saunders Elsevier, 2010. Pp. 256–7.29. Massa R., de Saint-Martin A., Hirsch E. et al. Landau–Kleffner syndrome: sleep EEG characteristics at onset. Clin Neurophysiol 2000;111(Suppl 2):S87–93. PMID: 10996560.Massa R., de Saint-Martin A., Hirsch E. et al. Landau–Kleffner syndrome: sleep EEG characteristics at onset. Clin Neurophysiol 2000;111(Suppl 2):S87–93. PMID: 10996560.30. Metz-Lutz M.N. The assessment of auditory function in CSWS: lessons from long-term outcome. Epilepsia 2009;50(Suppl 7):73–6. DOI: 10.1111/j.1528-1167.2009.02225.x. PMID: 19682057.Metz-Lutz M.N. The assessment of auditory function in CSWS: lessons from long-term outcome. Epilepsia 2009;50(Suppl 7):73–6. DOI: 10.1111/j.1528-1167.2009.02225.x. PMID: 19682057.31. Mikati M.A., El-Bitar M.K., Najjar M.W. et al. A child with refractory complex partial seizures, right temporal ganglioglioma, contralateral continuous electrical status epilepticus, and a secondary Landau–Kleffner autistic syndrome. Epilepsy Behav 2009;14(2):411–7. DOI: 10.1016/j.yebeh.2008.06.006. PMID: 18602026.Mikati M.A., El-Bitar M.K., Najjar M.W. et al. A child with refractory complex partial seizures, right temporal ganglioglioma, contralateral continuous electrical status epilepticus, and a secondary Landau–Kleffner autistic syndrome. Epilepsy Behav 2009;14(2):411–7. DOI: 10.1016/j.yebeh.2008.06.006. PMID: 18602026.32. Mukhin K.Yu., Kholin A.A. Landau–Kleffner syndrome (acquired epileptic aphasia). In: Epileptic encephalopathies and related syndromes in children. Ed. by K.Yu. Mukhin, A.A. Kholin, M.B. Mironov et al. Paris: John Libbey Eurotext, 2014. 502 p.Mukhin K.Yu., Kholin A.A. Landau–Kleffner syndrome (acquired epileptic aphasia). In: Epileptic encephalopathies and related syndromes in children. Ed. by K.Yu. Mukhin, A.A. Kholin, M.B. Mironov et al. Paris: John Libbey Eurotext, 2014. 502 p.33. Paetau R. Magnetoencephalography in Landau–Kleffner syndrome. Epilepsia 2009;50(Suppl 7):51–4. DOI: 10.1111/j.1528-1167.2009.02220.x. PMID: 19682052.Paetau R. Magnetoencephalography in Landau–Kleffner syndrome. Epilepsia 2009;50(Suppl 7):51–4. DOI: 10.1111/j.1528-1167.2009.02220.x. PMID: 19682052.34. Praline J., Hommet C., Barthez M.A. et al. Outcome at adulthood of the continuous spikewaves during slow sleep and Landau–Kleffner syndromes. Epilepsia 2003;44(11):1434– 40. PMID: 14636352.Praline J., Hommet C., Barthez M.A. et al. Outcome at adulthood of the continuous spikewaves during slow sleep and Landau–Kleffner syndromes. Epilepsia 2003;44(11):1434– 40. PMID: 14636352.35. Reutlinger C., Helbig I., Gawelczyk B. et al. Deletions in 16p13 including GRIN2A in patients with intellectual disability, various dysmorphic features, and seizure disorders of the rolandic region. Epilepsia 2010;51(9):1870–3. DOI: 10.1111/j.1528-1167.2010.02555.x. PMID: 20384727.Reutlinger C., Helbig I., Gawelczyk B. et al. Deletions in 16p13 including GRIN2A in patients with intellectual disability, various dysmorphic features, and seizure disorders of the rolandic region. Epilepsia 2010;51(9):1870–3. DOI: 10.1111/j.1528-1167.2010.02555.x. PMID: 20384727.36. Riccio C.A., Vidrine S.M., Cohen M.J. et al. Neurocognitive and behavioral profiles of children with Landau–Kleffner syndrome. Appl Neuropsychol Child 2016:1–10. DOI: 10.1080/21622965.2016.1197127. PMID: 27355396.Riccio C.A., Vidrine S.M., Cohen M.J. et al. Neurocognitive and behavioral profiles of children with Landau–Kleffner syndrome. Appl Neuropsychol Child 2016:1–10. DOI: 10.1080/21622965.2016.1197127. PMID: 27355396.37. Riviello J.J., Hadjiloizou S. The Landau–Kleffner syndrome and epilepsy with continuous spike-waves during sleep. In: Pediatric epilepsy. 3rd ed. Ed. by J.M. Pellock, B.F.D. Bourgeois, W.E. Dodson. N.Y.: Demos, 2008. Pp. 351–8.Riviello J.J., Hadjiloizou S. The Landau–Kleffner syndrome and epilepsy with continuous spike-waves during sleep. In: Pediatric epilepsy. 3rd ed. Ed. by J.M. Pellock, B.F.D. Bourgeois, W.E. Dodson. N.Y.: Demos, 2008. Pp. 351–8.38. Roll P., Rudolf G., Pereira S. et al. SRPX2 mutations in disorders of language cortex and cognition. Hum Mol Genet 2006;15(7):1195– 207. DOI: 10.1093/hmg/ddl035. PMID: 16497722.Roll P., Rudolf G., Pereira S. et al. SRPX2 mutations in disorders of language cortex and cognition. Hum Mol Genet 2006;15(7):1195– 207. DOI: 10.1093/hmg/ddl035. PMID: 16497722.39. Rossi P.G., Parmeggiani A., Posar A. et al. Landau–Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES). Brain Dev 1999;21(2):90–8. PMID: 10206525.Rossi P.G., Parmeggiani A., Posar A. et al. Landau–Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES). Brain Dev 1999;21(2):90–8. PMID: 10206525.40. Seri S., Cerquiglini A., Pisani F. Spike-induced interference in auditory sensory processing in Landau–Kleffner syndrome. Eltcroencephalogr Clin Neurophysiol 1998;108(5):506–10. PMID: 9780021.Seri S., Cerquiglini A., Pisani F. Spike-induced interference in auditory sensory processing in Landau–Kleffner syndrome. Eltcroencephalogr Clin Neurophysiol 1998;108(5):506–10. PMID: 9780021.41. Smith M.C., Hoeppner T.J. Epileptic encephalopathy of late childhood: Landau–Kleffner syndrome and the syndrome of continuous spikes and waves during slow-wave sleep. J Clin Neurophysiol 2003;20(6):462–72. PMID: 14734935.Smith M.C., Hoeppner T.J. Epileptic encephalopathy of late childhood: Landau–Kleffner syndrome and the syndrome of continuous spikes and waves during slow-wave sleep. J Clin Neurophysiol 2003;20(6):462–72. PMID: 14734935.42. Soprano A.M., Garcia E.F., Caraballo R., Fejerman N. Acquired epileptic aphasia: neuropsychologic follow-up of 12 patients. Pediatr Neurol 1994;11(3):230–5. PMID: 7533496.Soprano A.M., Garcia E.F., Caraballo R., Fejerman N. Acquired epileptic aphasia: neuropsychologic follow-up of 12 patients. Pediatr Neurol 1994;11(3):230–5. PMID: 7533496.43. Szabó L., Nagy J., Kálmánchey R. Adrenocorticotropic hormone therapy in acquired childhood epileptic aphasia. Ideggyogy Sz 2008;61(11–12):409–16. PMID: 19070317.Szabó L., Nagy J., Kálmánchey R. Adrenocorticotropic hormone therapy in acquired childhood epileptic aphasia. Ideggyogy Sz 2008;61(11–12):409–16. PMID: 19070317.44. Takeoka M., Riviello J.J. Jr, Duffy F.H. et al. Bilateral volume reduction of the superior temporal areas in Landau–Kleffner syndrome. Epilepsy Behav 2005;6(2):167–73. Neurology 2004;63(7):1289–92. PMID: 15477555.Takeoka M., Riviello J.J. Jr, Duffy F.H. et al. Bilateral volume reduction of the superior temporal areas in Landau–Kleffner syndrome. Epilepsy Behav 2005;6(2):167–73. Neurology 2004;63(7):1289–92. PMID: 15477555.45. Tassinari C.A., Cantaluppo G., Dalla Bernardina B. et al. Encephalopathy related to status epilepticus during slow sleep (ESES) including Landau–Kleffner syndrome. In: Epileptic syndromes in infancy, childhood and adolescence. 5th edn. Ed. by M. Bureau, P. Genton, C. Dravet et al. Paris: John Libbey Eurotext, 2012. Pp. 255–75.Tassinari C.A., Cantaluppo G., Dalla Bernardina B. et al. Encephalopathy related to status epilepticus during slow sleep (ESES) including Landau–Kleffner syndrome. In: Epileptic syndromes in infancy, childhood and adolescence. 5th edn. Ed. by M. Bureau, P. Genton, C. Dravet et al. Paris: John Libbey Eurotext, 2012. Pp. 255–75.46. Van Bogaert P., Paquier P.F. Landau–Kleffner syndrome: 50 years after. Epilepsia 2009;50(Suppl 7):1–2. DOI: 10.1111/j.1528-1167.2009.02207.x. PMID: 19682039.Van Bogaert P., Paquier P.F. Landau–Kleffner syndrome: 50 years after. Epilepsia 2009;50(Suppl 7):1–2. DOI: 10.1111/j.1528-1167.2009.02207.x. PMID: 19682039.47. Van Bogaert P. Epileptic encephalopathy with continious spike-waves during slow-wave sleep including Landau–Kleffner syndrome: what determines the outcome? In: Outcome of childhood epilepsies. Ed. by W.F. Arts, A. Arzimanoglou, O.F. Brouwer et al. Montrouge: JL Eurotext, 2013. Pp. 141–8.Van Bogaert P. Epileptic encephalopathy with continious spike-waves during slow-wave sleep including Landau–Kleffner syndrome: what determines the outcome? In: Outcome of childhood epilepsies. Ed. by W.F. Arts, A. Arzimanoglou, O.F. Brouwer et al. Montrouge: JL Eurotext, 2013. Pp. 141–8.48. Veggiotti P., Pera M.C., Teutonico F. et al. Therapy of encephalopathy with status epilepticus during slow sleep (ESES/CSWS syndrome): an update. Epileptic Disord 2012;14(1):1–11. DOI: 10.1684/epd.2012.0482. PMID: 22426353.Veggiotti P., Pera M.C., Teutonico F. et al. Therapy of encephalopathy with status epilepticus during slow sleep (ESES/CSWS syndrome): an update. Epileptic Disord 2012;14(1):1–11. DOI: 10.1684/epd.2012.0482. PMID: 22426353.