Children with ESES: Variability in the Syndrome

Hirtum-Das, Michèle Van; Licht, Eliot A.; Koh, Susan; Wu, Joyce; Shields, W. Donald; Sankar, Raman

doi:10.1016/j.eplepsyres.2006.01.020

Cited by 162 publications

(116 citation statements)

References 29 publications

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“…Similarly, Tassinari et al (2012) relies on the secondary bilateral synchrony mechanism which was assumed to work classically (Tükel and Jasper, 1952) by involving secondarily thalamic and thalamo-cortical circuitry. However, asymmetric, unilateral, or more focal/regional discharges were frequently reported (Bureau, 1995;Saltik et al, 2005;Van Hirtum-Das et al, 2006;Kramer et al, 2009;Liukkonen et al, 2010;Sánches Fernández et al, 2012). The sleep discharges in LKS were also found almost always asymmetric, with a dominant hemispheric and regional distribution congruently to the language disturbances.…”

Section: Terminological and Conceptual Chaos Around Eses Cswsmentioning

confidence: 99%

“…This cutoff value has been largely followed in the majority of publications. However several authors used lower cutoff values (Van Hirtum-Das, 2006;Inutsuka et al, 2006;Kevelam et al, 2012). In patients with clinically evident continuous discharges during sleep, a value of less than 85% is accepted in more than one third of cases, and the values varies 25-85% (Beaumanoir et al, 1995).…”

Section: Spatial Distribution Synchrony and The Amount Of The Dischamentioning

confidence: 99%

“…The required amount of discharges in ESES and the methods of estimating it are different; "a significant proportion" of the non-REM sleep is defined from 25% to 85% (Beaumanoir, 1995;Boel and Casaer, 1989;Bureau, 1995;Dalla Bernardina et al, 1978;Hirsch et al, 1990;Inutsuka et al, 2006;Laurette and Arfel, 1976;Loddenkemper et al, 2009;Patry et al, 1971;Saltik et al, 2005;Tassinari et al, 2000, Van Hirtum-Das et al, 2006Yan Liu and Wong, 2000;Yasuhara et al, 1991).…”

Section: Spatial Distribution Synchrony and The Amount Of The Dischamentioning

confidence: 99%

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Encephalopathy with Electrical Status Epilepticus in Slow Wave Sleep – a review with an emphasis on regional (perisylvian) aspects

Halász¹,

Hegyi²,

Siegler³

et al. 2014

Journal of Epileptology

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SUMMARYAim. The aim of this article is to review criticaly the Electrical Status Epilepticus in Slow Sleep (ESES) phenomenon from a neurophysiological mechanisms aspect as well as terminological and classification issues. Methods. The review includes all the relevant papers published during the last 43 years on the subject of ESES and Continous Spike -Wave in Sleep (CSWS).These papers were identified in various large databases via the internet. Rewiev and remarks. ESES/CSWS phenomena can be held as a common final pathway originating from different etiologies, including patients with early brain damage (probably involving thalamic structures), but also patients without structural pathology as in atypical evolution of idiopathic regional childhood hyperexcitability syndromes (with Rolandic epilepsy as a prototype). There are hints that genetic predisposition might be an important factor in the development of this process. The two large patient groups (lesional and non-lesional) show the same EEG evolution and encephalopathic cognitive consequences. The sleep EEG activation can be held as a common endophenotype. ESES represents an extreme sleep activation/potentiation of the local/ regional interictal discharges, enhancing them in frequency, territorial extension, intra and trans-hemispherial propagation, synchrony and continuity. This process is most probably not identical with the development of bilateral spike-wave pattern in "generalized" epilepsies which involves primarily or secondarily the thalamocortical system as it had been explored by Gloor (1979) for idiopathic generalized rpilepsy and Steriade and Amzica (2003) for different types of generalized spike and wave discharges. Conclusions and syndromological embedding of ESES.In an overwhelming majority of the investigated cases, the maps of the single discharges constituting sleep activation are identical; with focal/regional interictal spikes followed by slow closing wave, as it is seen in childhood regional age dependent hyperexcitability syndromes (prototype of the centro-temporal spikes of Rolandic epilepsy). The main mechanism of the developing cognitive impairment is most probably the consequence of interference with plastic function of slow wave sleep by obliterating synaptic decline during sleep. Presently, the consensus and co-operative research is highly obstacled by the terminological chaos, the controversial definitions and views around this still striking and enigmatic phenomenon.

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Section: Terminological and Conceptual Chaos Around Eses Cswsmentioning

confidence: 99%

Section: Spatial Distribution Synchrony and The Amount Of The Dischamentioning

confidence: 99%

Section: Spatial Distribution Synchrony and The Amount Of The Dischamentioning

confidence: 99%

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Encephalopathy with Electrical Status Epilepticus in Slow Wave Sleep – a review with an emphasis on regional (perisylvian) aspects

Halász¹,

Hegyi²,

Siegler³

et al. 2014

Journal of Epileptology

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“…The key clinical feature of LKS is the gradual or sudden inability to understand and use spoken language [58,59]. All children with LKS have EEG abnormalities, and most have continuous or near-continuous spikewaves during slow wave sleep (CSWS).…”

Section: Landau-kleffner Syndromementioning

confidence: 99%

Epileptic Encephalopathies: New Genes and New Pathways

Nieh

Sherr

2014

Neurotherapeutics

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Epileptic encephalopathies represent a group of devastating epileptic disorders that occur early in life and are often characterized by pharmaco-resistant epilepsy, persistent severe electroencephalographic abnormalities, and cognitive dysfunction or decline. Next generation sequencing technologies have increased the speed of gene discovery tremendously. Whereas ion channel genes were long considered to be the only significant group of genes implicated in the genetic epilepsies, a growing number of non-ion-channel genes are now being identified. As a subgroup of the genetically mediated epilepsies, epileptic encephalopathies are complex and heterogeneous disorders, making diagnosis and treatment decisions difficult. Recent exome sequencing data suggest that mutations causing epileptic encephalopathies are often sporadic, typically resulting from de novo dominant mutations in a single autosomal gene, although inherited autosomal recessive and X-linked forms also exist.In this review we provide a summary of the key features of several early-and mid-childhood onset epileptic encephalopathies including Ohtahara syndrome, Dravet syndrome, Infantile spasms and Lennox Gastaut syndrome. We review the recent next generation sequencing findings that may impact treatment choices. We also describe the use of conventional and newer anti-epileptic and hormonal medications in the various syndromes based on their genetic profile. At a biological level, developments in cellular reprogramming and genome editing represent a new direction in modeling these pediatric epilepsies and could be used in the development of novel and repurposed therapies.

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“…The SWI is defined as the total number of minutes of all spike and slow-wave abnormalities multiplied by 100 and divided by the total non-rapid eye movement sleep minutes (20). Various SWI criteria are proposed by different authors for ESES: 85% (42), 50% (43), 90% (22), 60% (33), 25% (44). The commonly used definition in clinic studies is "bilateral secondarily generalized 1.5 to 4.5 Hz spike-waves occuring during greater than 85% of slow wave sleep" (6,7).…”

Section: Eeg Criteriamentioning

confidence: 99%

Landau kleffner syndrome, electrical status epilepticus in sleep and autistic regression: an overview of literature / Landau kleffner sendromu, uykuda elektriksel status epileptikus ve otistik regresyon: Literatüre bir bakış

Ekinci¹,

Işık²,

Melek³

2012

Dusunen Adam

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Landau kleffner syndrome, electrical status epilepticus in sleep and autistic regression: an overview of literature Language regression in children is a challenging condition for clinicians since the diagnostic and therapeutic algorithms are not well established. Landau Kleffner syndrome (LKS), Electrical Status Epilepticus in Sleep (ESES) and Autistic Regression (AR) are three rare clinical entities in which langauage regression is present. LKS and ESES appear to be two points on the spectrum of epileptic syndromes characterized by severe paroxysmal EEG disturbance in sleep and regression. However, the available literature indicates that AR has many distinct features than LKS and ESES. For all of these three conditions, early recognition and treatment is crucial for favorable outcomes. Overnight EEG has an important diagnostic role for LKS and ESES. In this review, the clinical features, prognosis and treatment options of LKS, ESES and AR will be discussed.

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Children with ESES: Variability in the Syndrome

Cited by 162 publications

References 29 publications

Encephalopathy with Electrical Status Epilepticus in Slow Wave Sleep – a review with an emphasis on regional (perisylvian) aspects

Encephalopathy with Electrical Status Epilepticus in Slow Wave Sleep – a review with an emphasis on regional (perisylvian) aspects

Epileptic Encephalopathies: New Genes and New Pathways

Landau kleffner syndrome, electrical status epilepticus in sleep and autistic regression: an overview of literature / Landau kleffner sendromu, uykuda elektriksel status epileptikus ve otistik regresyon: Literatüre bir bakış

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