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Chapter 5 - Electro-clinical Syndromes and Epilepsies in the Neonatal Period, Infancy, and Childhood

Published online by Cambridge University Press:  11 October 2019

By
Jules E. C. Constantinou
Edited by
Vibhangini S. Wasade  and
Marianna V. Spanaki
Vibhangini S. Wasade
Affiliation:
Henry Ford Medical Group HFHS, Michigan
Marianna V. Spanaki
Affiliation:
Wayne State University, Michigan
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Summary

Apart from age of presentation, the electro-clinical syndromes are elaborated by a distinctive and recognizable set of features including the type of seizure(s) and the electrographic traits which aggregate together.1 Imaging findings can be considered. Neurodevelopmental and psychiatric comorbidities of varying degree are often associated. Causation may be included in the classification system. According to the 2017 position paper of the ILAE Commission for Classification and Terminology, the etiology of epilepsy may be structural, genetic, infectious, metabolic, or immune. Causation may also be unknown (formerly cryptogenic).2 Idiopathic and self-limited (formerly benign) epilepsies occur in children with a normal neurological examination and normal neuro-imaging, in whom there may be a familial predisposition. The term idiopathic (as opposed to genetic) is still preferred by some in respect of four well-recognized idiopathic generalized epilepsy syndromes (IGEs): childhood absence epilepsy (CAE), juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and generalized tonic–clonic seizures alone (formerly generalized tonic–clonic seizures on awakening). Although monogenic or more complex genetic or environmental susceptibility factors may be implicated in these epilepsies, the mechanisms are not always fully elucidated. The attribution to genetic causation may incorrectly suggest a high rate of inheritance. Benign focal epilepsies, such as benign or childhood epilepsy with centrotemporal spikes (CECTS) and the occipital lobe epilepsies of Panayiotopoulos and Gastaut are, again according to the position paper, termed self-limited as the term benign does not seem to fully address the developmental impact of these transient epilepsies.2 The epileptic encephalopathies, recognized as a distinct category, comprise a polymorphous group of epilepsy syndromes in which the epileptic activity itself contributes to cognitive and behavioral impairments above and beyond what might be expected from the underlying pathology alone.1 There is abundant epileptiform activity and inherently there is the idea that limiting or suppressing the activity will improve the neurodevelopmental outlook.

Type
Chapter
Information
Understanding Epilepsy
A Study Guide for the Boards
 , pp. 77 - 109
Publisher: Cambridge University Press
Print publication year: 2019

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References

Berg, AT, Berkovic, SF, Brodie, MJ, et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005–2009. Epilepsia. 2010;51(4):676–685.Google Scholar
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;58(4):512–521.CrossRefGoogle ScholarPubMed
Scheffer, IE, Harkin, LA, Dibbens, LM, Mulley, JC, Berkovic, SF. Neonatal epilepsy syndromes and generalized epilepsy with febrile seizures plus (GEFS+). Epilepsia. 2005;46(Suppl 10):41–47.Google Scholar
Hirsch, E, Velez, A, Sellal, F, et al. Electroclinical signs of benign neonatal familial convulsions. Ann Neurol. 1993;34(6):835–841.Google Scholar
Plouin, P, Neubauer, BA. Benign familial and non-familial neonatal seizures. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:77–88.Google Scholar
Yamamoto, H, Okumura, A, Fukuda, M. Epilepsies and epileptic syndromes starting in the neonatal period. Brain Dev. 2011;33(3):213–220.Google Scholar
Specchio, N, Vigevano, F. The spectrum of benign infantile seizures. Epilepsy Res. 2006;70(Suppl 1):S156–167.Google Scholar
Vigevano, F. Benign familial infantile seizures. Brain Dev. 2005;27(3):172–177.Google Scholar
Zara, F, Specchio, N, Striano, P, et al. Genetic testing in benign familial epilepsies of the first year of life: clinical and diagnostic significance. Epilepsia. 2013;54(3):425–436.CrossRefGoogle ScholarPubMed
Berkovic, SF, Heron, SE, Giordano, L, et al. Benign familial neonatal-infantile seizures: characterization of a new sodium channelopathy. Ann Neurol. 2004;55(4):550–557.Google Scholar
Beal, JC, Cherian, K, Moshe, SL. Early-onset epileptic encephalopathies: Ohtahara syndrome and early myoclonic encephalopathy. Pediatr Neurol. 2012;47(5):317–323.Google Scholar
Ohtahara, S, Yamatogi, Y. Epileptic encephalopathies in early infancy with suppression-burst. J Clin Neurophysiol. 2003;20(6):398–407.CrossRefGoogle ScholarPubMed
Djukic, A, Lado, FA, Shinnar, S, Moshe, SL. Are early myoclonic encephalopathy (EME) and the Ohtahara syndrome (EIEE) independent of each other? Epilepsy Res. 2006;70(Suppl 1):S68–76.Google Scholar
Ohtahara, S, Yamatogi, Y. Ohtahara syndrome: with special reference to its developmental aspects for differentiating from early myoclonic encephalopathy. Epilepsy Res. 2006;70(Suppl 1):S58–67.CrossRefGoogle ScholarPubMed
Mizrahi, EM, Milh, M. Early severe neonatal and infantile epilepsies. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:89–98.Google Scholar
Pavone, P, Spalice, A, Polizzi, A, Parisi, P, Ruggieri, M. Ohtahara syndrome with emphasis on recent genetic discovery. Brain Dev. 2012;34(6):459–468.Google Scholar
Gursoy, S, Ercal, D. Diagnostic approach to genetic causes of early-onset epileptic encephalopathy. J Child Neurol. 2016;31(4):523–532.Google Scholar
Coppola, G. Malignant migrating partial seizures in infancy: an epilepsy syndrome of unknown etiology. Epilepsia. 2009;50(Suppl 5):49–51.CrossRefGoogle ScholarPubMed
McTague, A, Appleton, R, Avula, S, et al. Migrating partial seizures of infancy: expansion of the electroclinical, radiological and pathological disease spectrum. Brain. 2013;136(Pt 5):1578–1591.CrossRefGoogle ScholarPubMed
Marsh, E, Melamed, SE, Barron, T, Clancy, RR. Migrating partial seizures in infancy: expanding the phenotype of a rare seizure syndrome. Epilepsia. 2005;46(4):568–572.CrossRefGoogle ScholarPubMed
Merdariu, D, Delanoe, C, Mahfoufi, N, Bellavoine, V, Auvin, S. Malignant migrating partial seizures of infancy controlled by stiripentol and clonazepam. Brain Dev. 2013;35(2):177–180.Google Scholar
Trevathan, E. Infantile spasms and Lennox-Gastaut syndrome. J Child Neurol. 2002;17(Suppl 2):2S9–2S22.Google Scholar
Pavone, P, Striano, P, Falsaperla, R, Pavone, L, Ruggieri, M. Infantile spasms syndrome, West syndrome and related phenotypes: what we know in 2013. Brain Dev. 2014;36(9):739–751.Google Scholar
Pellock, JM, Hrachovy, R, Shinnar, S, et al. Infantile spasms: a U.S. consensus report. Epilepsia. 2010;51(10):2175–2189.Google Scholar
Riikonen, R. Epidemiological data of West syndrome in Finland. Brain Dev. 2001;23(7):539–541.Google Scholar
Hrachovy, RA, Frost, JD Jr. Infantile epileptic encephalopathy with hypsarrhythmia (infantile spasms/West syndrome). J Clin Neurophysiol. 2003;20(6):408–425.Google Scholar
Fusco, L, Chiron, C, Trivisano, M, Vigevano, F, Chugani, HT. Infantile spasms. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:99–113.Google Scholar
Kramer, U, Sue, WC, Mikati, MA. Focal features in West syndrome indicating candidacy for surgery. Pediatr Neurol. 1997;16(3):213–217.Google Scholar
Osborne, JP, Lux, AL, Edwards, SW, et al. The underlying etiology of infantile spasms (West syndrome): information from the United Kingdom Infantile Spasms Study (UKISS) on contemporary causes and their classification. Epilepsia. 2010;51(10):2168–2174.Google Scholar
Wirrell, EC, Shellhaas, RA, Joshi, C, et al. How should children with West syndrome be efficiently and accurately investigated?: results from the National Infantile Spasms Consortium. Epilepsia. 2015;56(4):617–625.Google Scholar
Dulac, O. What is West syndrome? Brain Dev. 2001;23(7):447–452.Google Scholar
Fusco, L, Vigevano, F. Ictal clinical electroencephalographic findings of spasms in West syndrome. Epilepsia. 1993;34(4):671–678.CrossRefGoogle ScholarPubMed
Drury, I, Beydoun, A, Garofalo, EA, Henry, TR. Asymmetric hypsarrhythmia: clinical electroencephalographic and radiological findings. Epilepsia. 1995;36(1):41–47.Google Scholar
Gaily, E, Liukkonen, E, Paetau, R, Rekola, R, Granstrom, ML. Infantile spasms: diagnosis and assessment of treatment response by video-EEG. Dev Med Child Neurol. 2001;43(10):658–667.Google Scholar
Oka, M, Kobayashi, K, Akiyama, T, Ogino, T, Oka, E. A study of spike-density on EEG in West syndrome. Brain Dev. 2004;26(2):105–112.Google Scholar
Mackay, MT, Weiss, SK, Adams-Webber, T, et al. Practice parameter: medical treatment of infantile spasms. Report of the American Academy of Neurology and the Child Neurology Society. Neurology. 2004;62(10):1668–1681.CrossRefGoogle ScholarPubMed
Wilmshurst, JM, Gaillard, WD, Vinayan, KP, et al. Summary of recommendations for the management of infantile seizures: task force report for the ILAE Commission of Pediatrics. Epilepsia. 2015;56(8):1185–1197.Google Scholar
Hrachovy, RA, Frost, JD Jr., Glaze, DG. High-dose, long-duration versus low-dose, short-duration corticotropin therapy for infantile spasms. J Pediatr. 1994;124(5 Pt 1):803–806.Google Scholar
Wheless, JW, Clarke, DF, Carpenter, D. Treatment of pediatric epilepsy: expert opinion, 2005. J Child Neurol. 2005;20(Suppl 1):S1–56; quiz S59–60.CrossRefGoogle ScholarPubMed
Vigevano, F, Cilio, MR. Vigabatrin versus ACTH as first-line treatment for infantile spasms: a randomized, prospective study. Epilepsia. 1997;38(12):1270–1274.Google Scholar
Brodie, SE. Screening for vigabatrin (Sabril®) retinal toxicity in children. Ophthalmic Genet. 2011;32(4):193–195.Google Scholar
Gaily, E, Jonsson, H, Lappi, M. Visual fields at school-age in children treated with vigabatrin in infancy. Epilepsia. 2009;50(2):206–216.Google Scholar
Pearl, PL, Vezina, LG, Saneto, RP, et al. Cerebral MRI abnormalities associated with vigabatrin therapy. Epilepsia. 2009;50(2):184–194.Google Scholar
Lux, AL, Edwards, SW, Hancock, E, et al. The United Kingdom Infantile Spasms Study comparing vigabatrin with prednisolone or tetracosactide at 14 days: a multicentre, randomised controlled trial. Lancet. 2004;364(9447):1773–1778.Google Scholar
Lux, AL, Edwards, SW, Hancock, E, et al. The United Kingdom Infantile Spasms Study (UKISS) comparing hormone treatment with vigabatrin on developmental and epilepsy outcomes to age 14 months: a multicentre randomised trial. Lancet Neurol. 2005;4(11):712–717.Google Scholar
Darke, K, Edwards, SW, Hancock, E, et al. Developmental and epilepsy outcomes at age 4 years in the UKISS trial comparing hormonal treatments to vigabatrin for infantile spasms: a multi-centre randomised trial. Arch Dis Child. 2010;95(5):382–386.Google Scholar
Chugani, HT. Pathophysiology of infantile spasms. Adv Exp Med Biol. 2002;497:111–121.Google Scholar
Chugani, HT, Shewmon, DA, Shields, WD, et al. Surgery for intractable infantile spasms: neuroimaging perspectives. Epilepsia. 1993;34(4):764–771.Google Scholar
Wong, M, Trevathan, E. Infantile spasms. Pediatr Neurol. 2001;24(2):89–98.Google Scholar
Kivity, S, Lerman, P, Ariel, R, et al. Long-term cognitive outcomes of a cohort of children with cryptogenic infantile spasms treated with high-dose adrenocorticotropic hormone. Epilepsia. 2004;45(3):255–262.Google Scholar
Kossoff, EH. Infantile spasms. Neurologist. 2010;16(2):69–75.Google Scholar
Marini, C, Scheffer, IE, Nabbout, R, et al. The genetics of Dravet syndrome. Epilepsia. 2011;52(Suppl 2):24–29.Google Scholar
Meisler, MH, Kearney, JA. Sodium channel mutations in epilepsy and other neurological disorders. J Clin Invest. 2005;115(8):2010–2017.Google Scholar
Wu, YW, Sullivan, J, McDaniel, SS, et al. Incidence of Dravet syndrome in a US population. Pediatrics. 2015;136(5):e1310–1315.Google Scholar
Dravet, C. The core Dravet syndrome phenotype. Epilepsia. 2011;52(Suppl 2):3–9.Google Scholar
Arzimanoglou, A. Dravet syndrome: from electroclinical characteristics to molecular biology. Epilepsia. 2009;50(Suppl 8):3–9.Google Scholar
Wirrell, EC, Laux, L, Donner, E, et al. Optimizing the diagnosis and management of Dravet syndrome: recommendations from a North American consensus panel. Pediatr Neurol. 2017;68:18–34 e13.Google Scholar
Oguni, H, Hayashi, K, Awaya, Y, Fukuyama, Y, Osawa, M. Severe myoclonic epilepsy in infants – a review based on the Tokyo Women’s Medical University series of 84 cases. Brain Dev. 2001;23(7):736–748.Google Scholar
Guerrini, R, Aicardi, J. Epileptic encephalopathies with myoclonic seizures in infants and children (severe myoclonic epilepsy and myoclonic-astatic epilepsy). J Clin Neurophysiol. 2003;20(6):449–461.Google Scholar
Gataullina, S, Dulac, O. From genotype to phenotype in Dravet disease. Seizure. 2017;44:58–64.Google Scholar
Dravet, C, Bureau, M, Oguni, H, Cokar, O, Guerrini, R. Dravet syndrome (severe myoclonic epilepsy in infancy). In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:125–156.Google Scholar
Bureau, M, Dalla Bernardina, B. Electroencephalographic characteristics of Dravet syndrome. Epilepsia. 2011;52(Suppl 2):13–23.Google Scholar
Wolff, M, Casse-Perrot, C, Dravet, C. Severe myoclonic epilepsy of infants (Dravet syndrome): natural history and neuropsychological findings. Epilepsia. 2006;47(Suppl 2):45–48.Google Scholar
Genton, P, Velizarova, R, Dravet, C. Dravet syndrome: the long-term outcome. Epilepsia. 2011;52(Suppl 2):44–49.Google Scholar
Sakauchi, M, Oguni, H, Kato, I, et al. Mortality in Dravet syndrome: search for risk factors in Japanese patients. Epilepsia. 2011;52(Suppl 2):50–54.Google Scholar
Scheffer, IE, Berkovic, SF. Generalized epilepsy with febrile seizures plus: a genetic disorder with heterogeneous clinical phenotypes. Brain. 1997;120(Pt 3):479–490.Google Scholar
Camfield, P, Camfield, C. Febrile seizures and genetic epilepsy with febrile seizures plus (GEFS+). Epileptic Disord. 2015;17(2):124–133.Google Scholar
Scheffer, IE, Zhang, YH, Jansen, FE, Dibbens, L. Dravet syndrome or genetic (generalized) epilepsy with febrile seizures plus? Brain Dev. 2009;31(5):394–400.Google Scholar
De Jonghe, P. Molecular genetics of Dravet syndrome. Dev Med Child Neurol. 2011;53(Suppl 2):7–10.Google Scholar
Dravet, C, Bureau, M, Genton, P. Benign myoclonic epilepsy of infancy: electroclinical symptomatology and differential diagnosis from the other types of generalized epilepsy of infancy. Epilepsy Res Suppl. 1992;6:131–135.Google Scholar
Zuberi, SM, O’Regan, ME. Developmental outcome in benign myoclonic epilepsy in infancy and reflex myoclonic epilepsy in infancy: a literature review and six new cases. Epilepsy Res. 2006;70(Suppl 1):S110–115.Google Scholar
Hirano, Y, Oguni, H, Funatsuka, M, Imai, K, Osawa, M. Differentiation of myoclonic seizures in epileptic syndromes: a video-polygraphic study of 26 patients. Epilepsia. 2009;50(6):1525–1535.Google Scholar
Auvin, S, Pandit, F, De Bellecize, J, et al. Benign myoclonic epilepsy in infants: electroclinical features and long-term follow-up of 34 patients. Epilepsia. 2006;47(2):387–393.Google Scholar
Darra, F, Fiorini, E, Zoccante, L, et al. Benign myoclonic epilepsy in infancy (BMEI): a longitudinal electroclinical study of 22 cases. Epilepsia. 2006;47(Suppl 5):31–35.Google Scholar
Caraballo, RH, Flesler, S, Pasteris, MC, et al. Myoclonic epilepsy in infancy: an electroclinical study and long-term follow-up of 38 patients. Epilepsia. 2013;54(9):1605–1612.Google Scholar
Matricardi, S, Verrotti, A, Chiarelli, F, Cerminara, C, Curatolo, P. Current advances in childhood absence epilepsy. Pediatr Neurol. 2014;50(3):205–212.Google Scholar
Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia. 1989;30(4):389–399.Google Scholar
Panayiotopoulos, CP. Absence epilepsies. In: Engel, J, Pedley, TA, eds., Epilepsy: A Comprehensive Textbook. Philadelphia: Lippincott-Raven; 1998:2327–2346.Google Scholar
Sadleir, LG, Farrell, K, Smith, S, Connolly, MB, Scheffer, IE. Electroclinical features of absence seizures in childhood absence epilepsy. Neurology. 2006;67(3):413–418.CrossRefGoogle ScholarPubMed
Panayiotopoulos, CP, Obeid, T, Waheed, G. Differentiation of typical absence seizures in epileptic syndromes: a video EEG study of 224 seizures in 20 patients. Brain. 1989;112(Pt 4):1039–1056.Google Scholar
Medina, MT, Bureau, M, Hirsch, E, Panayiotopoulos, CP. Childhood absence epilepsy. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:277–296.Google Scholar
Penry, JK, Porter, RJ, Dreifuss, RE. Simultaneous recording of absence seizures with video tape and electroencephalography. A study of 374 seizures in 48 patients. Brain. 1975;98(3):427–440.CrossRefGoogle ScholarPubMed
Sadleir, LG, Scheffer, IE, Smith, S, Connolly, MB, Farrell, K. Automatisms in absence seizures in children with idiopathic generalized epilepsy. Arch Neurol. 2009;66(6):729–734.Google Scholar
Riviello, JJ Jr., Foley, CM. The epileptiform significance of intermittent rhythmic delta activity in childhood. J Child Neurol. 1992;7(2):156–160.Google Scholar
Guilhoto, LM, Manreza, ML, Yacubian, EM. Occipital intermittent rhythmic delta activity in absence epilepsy. Arq Neuropsiquiatr. 2006;64(2A):193–197.Google Scholar
Panayiotopoulos, CP, Engel, J Jr. Childhood absence epilepsy. http://www.medlink.com/article/childhood_absence_epilepsy. Accessed March 15, 2019.Google Scholar
Wirrell, EC, Camfield, CS, Camfield, PR, Gordon, KE, Dooley, JM. Long-term prognosis of typical childhood absence epilepsy: remission or progression to juvenile myoclonic epilepsy. Neurology. 1996;47(4):912–918.Google Scholar
Grosso, S, Galimberti, D, Vezzosi, P, et al. Childhood absence epilepsy: evolution and prognostic factors. Epilepsia. 2005;46(11):1796–1801.Google Scholar
Callenbach, PM, Bouma, PA, Geerts, AT, et al. Long-term outcome of childhood absence epilepsy: Dutch Study of Epilepsy in Childhood. Epilepsy Res. 2009;83(2–3):249–256.Google Scholar
Wirrell, E, Camfield, C, Camfield, P, Dooley, J. Prognostic significance of failure of the initial antiepileptic drug in children with absence epilepsy. Epilepsia. 2001;42(6):760–763.Google Scholar
Koutroumanidis, M, Bourvari, G, Tan, SV. Idiopathic generalized epilepsies: clinical and electroencephalogram diagnosis and treatment. Expert Rev Neurother. 2005;5(6):753–767.Google Scholar
Trinka, E, Baumgartner, S, Unterberger, I, et al. Long-term prognosis for childhood and juvenile absence epilepsy. J Neurol. 2004;251(10):1235–1241.Google Scholar
Shinnar, S, Cnaan, A, Hu, F, et al. Long-term outcomes of generalized tonic-clonic seizures in a childhood absence epilepsy trial. Neurology. 2015;85(13):1108–1114.Google Scholar
Glauser, TA, Cnaan, A, Shinnar, S, et al. Ethosuximide, valproic acid, and lamotrigine in childhood absence epilepsy. N Engl J Med. 2010;362(9):790–799.Google Scholar
Masur, D, Shinnar, S, Cnaan, A, et al. Pretreatment cognitive deficits and treatment effects on attention in childhood absence epilepsy. Neurology. 2013;81(18):1572–1580.Google Scholar
Wirrell, EC, Camfield, CS, Camfield, PR, et al. Long-term psychosocial outcome in typical absence epilepsy: sometimes a wolf in sheeps’ clothing. Arch Pediatr Adolesc Med. 1997;151(2):152–158.Google Scholar
Bureau, M, Tassinari, CA. Myoclonic absences and absences with myoclonias. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy and Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:297–304.Google Scholar
Larsson, K, Eeg-Olofsson, O. A population based study of epilepsy in children from a Swedish county. Eur J Paediatr Neurol. 2006;10(3):107–113.Google Scholar
Panayiotopoulos, CP, Michael, M, Sanders, S, Valeta, T, Koutroumanidis, M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain. 2008;131(Pt 9):2264–2286.Google Scholar
Ma, CK, Chan, KY. Benign childhood epilepsy with centrotemporal spikes: a study of 50 Chinese children. Brain Dev. 2003;25(6):390–395.Google Scholar
Panayiotopoulos, CP, Bureau, M, Caraballo, RH, Bernardina, DB, Valeta, T. Idiopathic focal epilepsies in childhood. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:218–254.Google Scholar
Parakh, M, Katewa, V. A review of the not so benign- benign childhood epilepsy with centrotemporal spikes. J Neurol Neurophysiol. 2015;6:314.Google Scholar
Guerrini, R, Pellacani, S. Benign childhood focal epilepsies. Epilepsia. 2012;53(Suppl 4):9–18.Google Scholar
Panayiotopoulos, CP. Benign Childhood Partial Seizures and Related Epileptic Syndromes. London: John Libbey; 1999.Google Scholar
Arzimanoglou, A, Guerrini, R, Aicardi, J, eds. Aicardi’s Epilepsy in Children. 3rd edn. Philadelphia: Lippincott Williams & Wilkins; 2004.Google Scholar
Lerman, P. Benign childhood epilepsy with centro temporal spikes (BECTS). In: Engel, J, Pedley, TA, Aicardi, J, eds., Epilepsy: A Comprehensive Textbook. Philadelphia: Lippincott-Raven; 1998:2307–2314.Google Scholar
Legarda, S, Jayakar, P, Duchowny, M, Alvarez, L, Resnick, T. Benign rolandic epilepsy: high central and low central subgroups. Epilepsia. 1994;35(6):1125–1129.Google Scholar
Luders, H, Lesser, RP, Dinner, DS, Morris, HH III. Benign focal epilepsies of childhood. In: Luders, H, Lesser, RP, eds., Epilepsy: Electroclinical Syndromes. London: Springer-Verlag; 1987:303–346.Google Scholar
Jung, KY, Kim, JM, Kim, DW. Patterns of interictal spike propagation across the central sulcus in benign rolandic epilepsy. Clin Electroencephalogr. 2003;34(3):153–157.Google Scholar
Boor, R, Jacobs, J, Hinzmann, A, et al. Combined spike-related functional MRI and multiple source analysis in the non-invasive spike localization of benign rolandic epilepsy. Clin Neurophysiol. 2007;118(4):901–909.Google Scholar
Huiskamp, G, van Der Meij, W, van Huffelen, A, van Nieuwenhuizen, O. High resolution spatio-temporal EEG-MEG analysis of rolandic spikes. J Clin Neurophysiol. 2004;21(2):84–95.Google Scholar
Holmes, GL. Benign focal epilepsies of childhood. Epilepsia. 1993;34(Suppl 3):S49–61.Google Scholar
Cavazzuti, GB, Cappella, L, Nalin, A. Longitudinal study of epileptiform EEG patterns in normal children. Epilepsia. 1980;21(1):43–55.Google Scholar
Loiseau, P, Beaussart, M. The seizures of benign childhood epilepsy with Rolandic paroxysmal discharges. Epilepsia. 1973;14(4):381–389.Google Scholar
Bouma, PA, Bovenkerk, AC, Westendorp, RG, Brouwer, OF. The course of benign partial epilepsy of childhood with centrotemporal spikes: a meta-analysis. Neurology. 1997;48(2):430–437.Google Scholar
Loiseau, P, Duche, B, Cordova, S, Dartigues, JF, Cohadon, S. Prognosis of benign childhood epilepsy with centrotemporal spikes: a follow-up study of 168 patients. Epilepsia. 1988;29(3):229–235.Google Scholar
Coppola, G, Franzoni, E, Verrotti, A, et al. Levetiracetam or oxcarbazepine as monotherapy in newly diagnosed benign epilepsy of childhood with centrotemporal spikes (BECTS): an open-label, parallel group trial. Brain Dev. 2007;29(5):281–284.Google Scholar
Lerman, P. Seizures induced or aggravated by anticonvulsants. Epilepsia. 1986;27(6):706–710.Google Scholar
Parmeggiani, L, Seri, S, Bonanni, P, Guerrini, R. Electrophysiological characterization of spontaneous and carbamazepine-induced epileptic negative myoclonus in benign childhood epilepsy with centro-temporal spikes. Clin Neurophysiol. 2004;115(1):50–58.Google Scholar
Clarke, T, Strug, LJ, Murphy, PL, et al. High risk of reading disability and speech sound disorder in rolandic epilepsy families: case-control study. Epilepsia. 2007;48(12):2258–2265.Google Scholar
Bulgheroni, S, Franceschetti, S, Vago, C, et al. Verbal dichotic listening performance and its relationship with EEG features in benign childhood epilepsy with centrotemporal spikes. Epilepsy Res. 2008;79(1):31–38.Google Scholar
Shields, WD, Snead, OC, 3rd. Benign epilepsy with centrotemporal spikes. Epilepsia. 2009;50(Suppl 8):10–15.Google Scholar
Ferrie, C, Caraballo, R, Covanis, A, et al. Panayiotopoulos syndrome: a consensus view. Dev Med Child Neurol. 2006;48(3):236–240.Google Scholar
Martinovic, Z. The new ILAE report on classification and evidence-based commentary on Panayiotopoulos syndrome and autonomic status epilepticus. Epilepsia. 2007;48(6):1215–1216.Google Scholar
Panayiotopoulos, CP. Vomiting as an ictal manifestation of epileptic seizures and syndromes. J Neurol Neurosurg Psychiatry. 1988;51(11):1448–1451.Google Scholar
Panayiotopoulos, CP. Panayiotopoulos Syndrome: A Common and Benign Childhood Epileptic Syndrome. Eastleigh, England: John Libbey; 2002.Google Scholar
Covanis, A. Panayiotopoulos syndrome: a benign childhood autonomic epilepsy frequently imitating encephalitis, syncope, migraine, sleep disorder, or gastroenteritis. Pediatrics. 2006;118(4):e1237–1243.Google Scholar
Oguni, H, Hayashi, K, Imai, K, et al. Study on the early-onset variant of benign childhood epilepsy with occipital paroxysms otherwise described as early-onset benign occipital seizure susceptibility syndrome. Epilepsia. 1999;40(7):1020–1030.Google Scholar
Dura-Trave, T, Yoldi-Petri, ME, Gallinas-Victoriano, F. Panayiotopoulos syndrome: epidemiological and clinical characteristics and outcome. Eur J Neurol. 2008;15(4):336–341.Google Scholar
Leal, AJ, Nunes, S, Dias, AI, et al. Analysis of the generators of epileptic activity in early-onset childhood benign occipital lobe epilepsy. Clin Neurophysiol. 2007;118(6):1341–1347.Google Scholar
Kikumoto, K, Yoshinaga, H, Oka, M, et al. EEG and seizure exacerbation induced by carbamazepine in Panayiotopoulos syndrome. Epileptic Disord. 2006;8(1):53–56.Google Scholar
Caraballo, RH, Cersosimo, RO, Fejerman, N. Childhood occipital epilepsy of Gastaut: a study of 33 patients. Epilepsia. 2008;49(2):288–297.Google Scholar
Sanchez Fernandez, I, Loddenkemper, T. Pediatric focal epilepsy syndromes. J Clin Neurophysiol. 2012;29(5):425–440.Google Scholar
Ferini-Strambi, L, Sansoni, V, Combi, R. Nocturnal frontal lobe epilepsy and the acetylcholine receptor. Neurologist. 2012;18(6):343–349.Google Scholar
Tinuper, P, Bisulli, F, Cross, JH, et al. Definition and diagnostic criteria of sleep-related hypermotor epilepsy. Neurology. 2016;86(19):1834–1842.Google Scholar
Picard, F, Scheffer, IE. Genetically determined focal epilepsies. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:349–361.Google Scholar
Tinuper, P, Bisulli, F. From nocturnal frontal lobe epilepsy to sleep-related hypermotor epilepsy: a 35-year diagnostic challenge. Seizure. 2017;44:87–92.Google Scholar
Combi, R, Dalpra, L, Tenchini, ML, Ferini-Strambi, L. Autosomal dominant nocturnal frontal lobe epilepsy – a critical overview. J Neurol. 2004;251(8):923–934.Google Scholar
Arzimanoglou, A, French, J, Blume, WT, et al. Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol. 2009;8(1):82–93.Google Scholar
Trevathan, E, Murphy, CC, Yeargin-Allsopp, M. Prevalence and descriptive epidemiology of Lennox-Gastaut syndrome among Atlanta children. Epilepsia. 1997;38(12):1283–1288.Google Scholar
Hancock, EC, Cross, JH. Treatment of Lennox-Gastaut syndrome. Cochrane Database Syst Rev. 2013(2):CD003277.Google Scholar
Markand, ON. Lennox-Gastaut syndrome (childhood epileptic encephalopathy). J Clin Neurophysiol. 2003;20(6):426–441.Google Scholar
Camfield, P, Camfield, C. Long-term prognosis for symptomatic (secondarily) generalized epilepsies: a population-based study. Epilepsia. 2007;48(6):1128–1132.Google Scholar
Camfield, PR. Definition and natural history of Lennox-Gastaut syndrome. Epilepsia. 2011;52(Suppl 5):3–9.Google Scholar
Dulac, O, N’Guyen, T. The Lennox-Gastaut syndrome. Epilepsia. 1993;34(Suppl 7):S7–17.Google Scholar
Chatrian, GE, Lettich, E, Wilkus, RJ, Vallarta, J. Polygraphic and clinical observations on tonic-autonomic seizures. Electroencephalogr Clin Neurophysiol Suppl. 1982(35):101–124.Google Scholar
Crumrine, PK. Lennox-Gastaut syndrome. J Child Neurol. 2002;17(Suppl 1):S70–75.Google Scholar
Gastraut, H, Roger, J, Soulayrol, R, et al. Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise known as “petit mal variant”) or Lennox syndrome. Epilepsia. 1966;7(2):139–179.Google Scholar
Crespel, A, Gelisse, P, Nikanorova, M, Ferlazzo, E, Genton, P. Lennox-Gastaut syndrome. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:189–216.Google Scholar
Yaqub, BA. Electroclinical seizures in Lennox-Gastaut syndrome. Epilepsia. 1993;34(1):120–127.Google Scholar
Camfield, P, Camfield, C. Epileptic syndromes in childhood: clinical features, outcomes, and treatment. Epilepsia. 2002;43(Suppl 3):27–32.Google Scholar
Ikeno, T, Shigematsu, H, Miyakoshi, M, et al. An analytic study of epileptic falls. Epilepsia. 1985;26(6):612–621.Google Scholar
Egli, M, Mothersill, I, O’Kane, M, O’Kane, F. The axial spasm – the predominant type of drop seizure in patients with secondary generalized epilepsy. Epilepsia. 1985;26(5):401–415.Google Scholar
Markand, ON. Slow spike-wave activity in EEG and associated clinical features: often called ‘Lennox’ or “Lennox-Gastaut’ syndrome. Neurology. 1977;27(8):746–757.Google Scholar
Zupanc, ML. Clinical evaluation and diagnosis of severe epilepsy syndromes of early childhood. J Child Neurol. 2009;24(8 Suppl):6S–14S.Google Scholar
Blume, WT. Pathogenesis of Lennox-Gastaut syndrome: considerations and hypotheses. Epileptic Disord. 2001;3(4):183–196.Google Scholar
Genton, P, Dravet, C. Lennox-Gastaut syndrome. In: Engel, J, Pedley, TA, eds. Epilepsy: A Comprehensive Textbook. Philadelphia: Lippincott-Raven; 1998:2355–2366.Google Scholar
Ohtahara, S, Ohtsuka, Y, Kobayashi, K. Lennox-Gastaut syndrome: a new vista. Psychiatry Clin Neurosci. 1995;49(3):S179–183.Google Scholar
VanStraten, AF, Ng, YT. Update on the management of Lennox-Gastaut syndrome. Pediatr Neurol. 2012;47(3):153–161.Google Scholar
Bourgeois, BF, Douglass, LM, Sankar, R. Lennox-Gastaut syndrome: a consensus approach to differential diagnosis. Epilepsia. 2014;55(Suppl 4):4–9.Google Scholar
Ostendorf, AP, Ng, YT. Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions. Neuropsychiatr Dis Treat. 2017;13:1131–1140.Google Scholar
Lancman, G, Virk, M, Shao, H, et al. Vagus nerve stimulation vs. corpus callosotomy in the treatment of Lennox-Gastaut syndrome: a meta-analysis. Seizure. 2013;22(1):3–8.Google Scholar
Kelley, SA, Kossoff, EH. Doose syndrome (myoclonic-astatic epilepsy): 40 years of progress. Dev Med Child Neurol. 2010;52(11):988–993.Google Scholar
Oguni, H, Fukuyama, Y, Tanaka, T, et al. Myoclonic-astatic epilepsy of early childhood – clinical and EEG analysis of myoclonic-astatic seizures, and discussions on the nosology of the syndrome. Brain Dev. 2001;23(7):757–764.Google Scholar
Neubauer, BA, Hahn, A, Doose, H, Tuxhorn, I. Myoclonic-astatic epilepsy of early childhood – definition, course, nosography, and genetics. Adv Neurol. 2005;95:147–155.Google Scholar
Guerrini, R, Mari, F, Dravet, C. Idiopathic myoclonic epilepsies in infancy and early childhood. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:157–173.Google Scholar
Kramer, U. Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review. J Child Neurol. 2008;23(7):785–790.Google Scholar
Panayiotopoulos, CP. Syndromes of idiopathic generalized epilepsies not recognized by the International League Against Epilepsy. Epilepsia. 2005;46(Suppl 9):57–66.Google Scholar
Striano, S, Capovilla, G, Sofia, V, et al. Eyelid myoclonia with absences (Jeavons syndrome): a well-defined idiopathic generalized epilepsy syndrome or a spectrum of photosensitive conditions? Epilepsia. 2009;50(Suppl 5):15–19.Google Scholar
Caraballo, RH, Fontana, E, Darra, F, et al. A study of 63 cases with eyelid myoclonia with or without absences: type of seizure or an epileptic syndrome? Seizure. 2009;18(6):440–445.Google Scholar
Nar Senol, P, Tezer, FI, Saygi, S. Eyelid myoclonia seizures in adults: an alternate look at the syndrome paradox. Epilepsy Behav. 2015;45:265–270.Google Scholar
Destina Yalcin, A, Forta, H, Kilic, E. Overlap cases of eyelid myoclonia with absences and juvenile myoclonic epilepsy. Seizure. 2006;15(6):359–365.Google Scholar
da Conceicao, PO, Guaranha, MS, Uchida, CG, et al. Blinking and eyelid myoclonia: characteristics and correlations of eyelid movements. Seizure. 2015;24:12–16.Google Scholar
Nickels, K, Wirrell, E. Electrical status epilepticus in sleep. Semin Pediatr Neurol. 2008;15(2):50–60.Google Scholar
Smith, MC, Hoeppner, TJ. 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):462–472.Google Scholar
Tuft, M, Arva, M, Bjornvold, M, Wilson, JA, Nakken, KO. Landau-Kleffner syndrome. Tidsskr Nor Laegeforen. 2015;135(22):2061–2064.Google Scholar
Tassinari, CA, Cantalupo, G, Dalla Bernadina, B, et al. Encephalopathy related to status epilepticus during slow wave sleep (ESES) including Landau-Kleffner syndrome. In: Bureau, M, Genton, P, Dravet, C, et al., eds., Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th edn. Montrouge, France: John Libbey Eurotext; 2012:255–275.Google Scholar
Stefanatos, G. Changing perspectives on Landau-Kleffner syndrome. Clin Neuropsychol. 2011;25(6):963–988.Google Scholar
Smith, MC. Landau-Kleffner syndrome and continuous spike and waves during slow sleep. In: Engel, J Jr., Pedley, TA, eds., Epilepsy: A Comprehensive Textbook. Philadelphia: Lippincott-Raven; 1998:2367–2377.Google Scholar
Shimada, T, Takemiya, T, Sugiura, H, Yamagata, K. Role of inflammatory mediators in the pathogenesis of epilepsy. Mediators Inflamm. 2014;2014:901902.Google Scholar
Loddenkemper, T, Fernandez, IS, Peters, JM. Continuous spike and waves during sleep and electrical status epilepticus in sleep. J Clin Neurophysiol. 2011;28(2):154–164.Google Scholar
Hughes, JR. A review of the relationships between Landau-Kleffner syndrome, electrical status epilepticus during sleep, and continuous spike-waves during sleep. Epilepsy Behav. 2011;20(2):247–253.Google Scholar
Sanchez Fernandez, I, Loddenkemper, T, Peters, JM, Kothare, SV. Electrical status epilepticus in sleep: clinical presentation and pathophysiology. Pediatr Neurol. 2012;47(6):390–410.Google Scholar
Tassinari, CA, Rubboli, G, Volpi, L, et al. Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia. Clin Neurophysiol. 2000;111(Suppl 2):S94–S102.Google Scholar
Guerrini, R, Genton, P, Bureau, M, et al. Multilobar polymicrogyria, intractable drop attack seizures, and sleep-related electrical status epilepticus. Neurology. 1998;51(2):504–512.Google Scholar
Smith, MC, Spitz, MC. Treatment strategies in Landau-Kleffner syndrome and paraictal psychiatric and cognitive disturbances. Epilepsy Behav. 2002;3(5S):24–29.Google Scholar
Buzatu, M, Bulteau, C, Altuzarra, C, Dulac, O, Van Bogaert, P. Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia. 2009;50(Suppl 7):68–72.Google Scholar
De Negri, M, Baglietto, MG, Battaglia, FM, et al. Treatment of electrical status epilepticus by short diazepam (DZP) cycles after DZP rectal bolus test. Brain Dev. 1995;17(5):330–333.Google Scholar
Morrell, F, Whisler, WW, Smith, MC, et al. Landau-Kleffner syndrome. Treatment with subpial intracortical transection. Brain. 1995;118(Pt 6):1529–1546.Google Scholar
Wolf, P. Basic principles of the ILAE syndrome classification. Epilepsy Res. 2006;70(Suppl 1):S20–26.Google Scholar

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