Main Article Content
Abstract
Objectives: To evaluate the spatial distribution of the epileptiform activity in electrical status epilepticus in sleep (ESES) and to correlate data from electroencephalograms (EEGs) with clinical and neuroimaging variables.
Methods: From 2008 to 2015, 162 reports (1.01%) out of 16,000 EEGs, from 23 patients, showed ESES. We selected one representative EEG per patient. Clinical data was collected retrospectively. Neuroimaging examinations were reviewed. The EEGs were classified as generalized ESES (ESESg) and focal ESES (ESESf) according to the distribution of epileptiform discharges.
Results: From the 23 patients, 5 were classified as ESESg and 18 as ESESf. In ESESf, there was a prevalence of focal epileptic discharges in the centrotemporal regions. Abnormal neuroimaging occurred in 100% of the patients with ESESg and in 38.9% of the patients with ESESf (p=0.037). Other clinical data did not show significant differences between the groups. All patients with ESESg had structural etiology, while only 39% of patients with ESESf had structural etiology and the remaining 61% potentially genetic epilepsies of the rolandic spectrum.
Conclusion: ESESg occurred predominantly in patients with structural lesions, while most patients with ESESf had normal neuroimaging scans and electrical dysfunction mainly in the rolandic region.
Significance: ESESg seems to occur mostly in structural epilepsies. Distinctly, ESESf occurs in epileptic syndromes within the functional spectrum of rolandic epilepsy.
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Copyright (c) 2021 Raphael R. Almeida, Ana Carolina Coan, Marilisa M Guerreiro
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References
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- Tassinari CA, Cantalupo G, Dalla Bernardina B, et al. Encephalopathy related to status epilepticus during slow sleep (ESES) including Landau-Kleffner syndrome. In: Bureau M, Genton P, Dravet C, Delgado-Escueta AV, Tassinari CA, Thomas P, Wolf P, (Eds.) Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th ed. Montrouge: John Libbey Eurotex Ltd; 2012:255-75.
- Pal DK, Ferrie C, Addis L, et al. Idiopathic focal epilepsies: the “lost tribe”. Epileptic Disord 2016; 18(3):252-88.
- 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):676-85.
- Sanchéz Fernández I, Peters JM, Akhondi-Asl A, Klehm J, Warfield SK, Loddenkemper T. Reduced thalamic volume in patients with Electrical Status Epilepticus in Sleep. Epilepsy Res 2017; 130:74-80.
- Fernández IS, Peters J, Takeoka M, et al. Patients with electrical status epilepticus in sleep share similar clinical features regardless of their focal or generalized sleep potentiation of epileptiform activity. J Child Neurol 2013; 28(1):83-9.
- Teixeira KC, Cendes F, Guerreiro CA, Guerreiro MM. Focal electrical status (FES): a new finding associated with polymicrogyria. J Clin Neurophysiol 2009; 26(3):155-9.
- Guzzetta F, Battaglia D, Veredice C, et al. Early thalamic injury associated with epilepsy and continuous spike-wave during slow sleep. Epilepsia 2005; 46(6):889-900.
- Loddenkemper T, Fernández IS, Peters J. Continuous spike and waves during sleep and electrical status epilepticus in sleep. J Clin Neurophysiol 2011; 28(2):154-64.
- Uliel-Sibony S, Kramer U. Benign childhood epilepsy with centro-temporal spikes (BCECTSs), electrical status epilepticus in sleep (ESES), and academic decline — how aggressive should we be? Epilepsy Behav 2015; 44:117–20.
- American Clinical Neurophysiology Society. Guideline 5: Guidelines for standard electrode position nomenclature. J Clin Neurophysiol 2006;23(2):107-10.
- Chen XQ, Zhang WN, Hu LY, Liu MJ, Zou LP. Syndrome of electrical status epilepticus during sleep: epileptic encephalopathy related to brain development. Pediatr Neurol 2016; 56:35-41.
- Kevelam SHG, Jansen FE, Binsbergen E, et al. Copy number variations in patients with electrical status epilepticus in sleep. J Child Neurol 2012; 27(2):178-82.
- Tuchman R. CSWS-related autistic regression versus autistic regression without CSWS. Epilepsia 2009; 50(7):18-20.
- 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.
- Van Hirtum-Das M, Licht EA, Koh S, Wu JY, Shields WD, Sankar R. Children with ESES: variability in the syndrome. Epilepsy Res 2006;70 (Suppl 1):248-58.
- Peng SJ, Hsin YL. Altered structural and functional thalamocortical networks in secondarily generalized extratemporal lobe seizures. Neuroimage Clin 2016; 13:55-61.
- Kersbergen KJ, de Vries LS, Leijten FS, et al. Neonatal thalamic hemorrhage is strongly associated with electrical status epilepticus in slow wave sleep. Epilepsia 2013; 54(4):733-40.
- Sanchéz Fernández I, Loddenkemper T, Peters JM, Kothare SV. Electrical status epilepticus in sleep: clinical presentation and pathophysiology. Pediatr Neurol 2012; 47(6): 390-410.
- Veggiotti P, Beccaria F, Guerrini R, Capovilla G, Lanzi G. Continuous spike-and-wave activity during slow-wave sleep: syndrome or EEG pattern? Epilepsia 1999; 40(11):1593-601.
- Fortini S, Corredera L, Pastrana AL, et al. Encephalopathy with hemi-status epilepticus during sleep or hemi-continuous spikes and waves during slow sleep syndrome: a study of 21 patients. Seizure 2013; 22(7):565-71.
- Caraballo RH, Fortini S, Flesler S, et al. Encephalopathy with status epilepticus during sleep: unusual EEG patterns. Seizure 2015; 25:117-25.
References
Patry G, Lyagoubi S, Tassinari CA. Subclinical “electrical status epilepticus” induced by sleep in children. Arch Neurol 1971; 24:242-52.
Tassinari CA, Cantalupo G, Dalla Bernardina B, et al. Encephalopathy related to status epilepticus during slow sleep (ESES) including Landau-Kleffner syndrome. In: Bureau M, Genton P, Dravet C, Delgado-Escueta AV, Tassinari CA, Thomas P, Wolf P, (Eds.) Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th ed. Montrouge: John Libbey Eurotex Ltd; 2012:255-75.
Pal DK, Ferrie C, Addis L, et al. Idiopathic focal epilepsies: the “lost tribe”. Epileptic Disord 2016; 18(3):252-88.
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):676-85.
Sanchéz Fernández I, Peters JM, Akhondi-Asl A, Klehm J, Warfield SK, Loddenkemper T. Reduced thalamic volume in patients with Electrical Status Epilepticus in Sleep. Epilepsy Res 2017; 130:74-80.
Fernández IS, Peters J, Takeoka M, et al. Patients with electrical status epilepticus in sleep share similar clinical features regardless of their focal or generalized sleep potentiation of epileptiform activity. J Child Neurol 2013; 28(1):83-9.
Teixeira KC, Cendes F, Guerreiro CA, Guerreiro MM. Focal electrical status (FES): a new finding associated with polymicrogyria. J Clin Neurophysiol 2009; 26(3):155-9.
Guzzetta F, Battaglia D, Veredice C, et al. Early thalamic injury associated with epilepsy and continuous spike-wave during slow sleep. Epilepsia 2005; 46(6):889-900.
Loddenkemper T, Fernández IS, Peters J. Continuous spike and waves during sleep and electrical status epilepticus in sleep. J Clin Neurophysiol 2011; 28(2):154-64.
Uliel-Sibony S, Kramer U. Benign childhood epilepsy with centro-temporal spikes (BCECTSs), electrical status epilepticus in sleep (ESES), and academic decline — how aggressive should we be? Epilepsy Behav 2015; 44:117–20.
American Clinical Neurophysiology Society. Guideline 5: Guidelines for standard electrode position nomenclature. J Clin Neurophysiol 2006;23(2):107-10.
Chen XQ, Zhang WN, Hu LY, Liu MJ, Zou LP. Syndrome of electrical status epilepticus during sleep: epileptic encephalopathy related to brain development. Pediatr Neurol 2016; 56:35-41.
Kevelam SHG, Jansen FE, Binsbergen E, et al. Copy number variations in patients with electrical status epilepticus in sleep. J Child Neurol 2012; 27(2):178-82.
Tuchman R. CSWS-related autistic regression versus autistic regression without CSWS. Epilepsia 2009; 50(7):18-20.
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.
Van Hirtum-Das M, Licht EA, Koh S, Wu JY, Shields WD, Sankar R. Children with ESES: variability in the syndrome. Epilepsy Res 2006;70 (Suppl 1):248-58.
Peng SJ, Hsin YL. Altered structural and functional thalamocortical networks in secondarily generalized extratemporal lobe seizures. Neuroimage Clin 2016; 13:55-61.
Kersbergen KJ, de Vries LS, Leijten FS, et al. Neonatal thalamic hemorrhage is strongly associated with electrical status epilepticus in slow wave sleep. Epilepsia 2013; 54(4):733-40.
Sanchéz Fernández I, Loddenkemper T, Peters JM, Kothare SV. Electrical status epilepticus in sleep: clinical presentation and pathophysiology. Pediatr Neurol 2012; 47(6): 390-410.
Veggiotti P, Beccaria F, Guerrini R, Capovilla G, Lanzi G. Continuous spike-and-wave activity during slow-wave sleep: syndrome or EEG pattern? Epilepsia 1999; 40(11):1593-601.
Fortini S, Corredera L, Pastrana AL, et al. Encephalopathy with hemi-status epilepticus during sleep or hemi-continuous spikes and waves during slow sleep syndrome: a study of 21 patients. Seizure 2013; 22(7):565-71.
Caraballo RH, Fortini S, Flesler S, et al. Encephalopathy with status epilepticus during sleep: unusual EEG patterns. Seizure 2015; 25:117-25.