More than one self-limited epilepsy of childhood in the same patient: A multicenter study

Fortini, Sebastián; Espeche, Alberto; Galicchio, Santiago; Cersósimo, Ricardo; Chacón, Santiago; Gallo, Adolfo; Gamboni, Beatriz; Adi, Javier; Fasulo, Lorena; Semprino, Marcos; Cachia, Pedro; Caraballo, Roberto

doi:10.1016/j.eplepsyres.2021.106768

Cited by 6 publications

(6 citation statements)

References 41 publications

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“…A third conceptual change relates to the rare progress of SELECTS to EE-SWAS (ESES/LKS) ( 4 ). It is theoretically interesting, as it offers a model of epileptic progression.…”

Section: Discussionmentioning

confidence: 99%

“…SELECTS may progress to EE-SWAS in <10% of cases ( 4 ), but there are no data about the transformation ratio to atypical SELECTS. It resembles the encephalopathic forms in manifesting poorer neuropsychological performance and higher frequencies/densities of HFO.…”

Section: The Electrophysiological Mechanism Of the Transition From Se...mentioning

confidence: 99%

“…Originally, this included three types (RE, PS, and GOE), but the recent ILAE classification involved a fourth member, POE. Those epilepsies can mutually transform into each other and occur in a common age window (4). The age of onset is between 3 and 10 years, with a peak around 6-7 years in 90% of cases, and recovery occurs before the age of 15-16 years (5).…”

Section: Changing Interpretations Of the Spectrummentioning

confidence: 99%

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Self-limited childhood epilepsies are disorders of the perisylvian communication system, carrying the risk of progress to epileptic encephalopathies—Critical review

Halász

Szücs

2023

Front. Neurol.

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“Sleep plasticity is a double-edged sword: a powerful machinery of neural build-up, with a risk to epileptic derailment.”We aimed to review the types of self-limited focal epilepsies...“i.e. keep as two separate paragraphs” We aimed to review the types of self-limited focal epilepsies: (1) self-limited focal childhood epilepsy with centrotemporal spikes, (2) atypical Rolandic epilepsy, and (3) electrical status epilepticus in sleep with mental consequences, including Landau–Kleffner-type acquired aphasia, showing their spectral relationship and discussing the debated topics. Our endeavor is to support the system epilepsy concept in this group of epilepsies, using them as models for epileptogenesis in general. The spectral continuity of the involved conditions is evidenced by several features: language impairment, the overarching presence of centrotemporal spikes and ripples (with changing electromorphology across the spectrum), the essential timely and spatial independence of interictal epileptic discharges from seizures, NREM sleep relatedness, and the existence of the intermediate-severity “atypical” forms. These epilepsies might be the consequences of a genetically determined transitory developmental failure, reflected by widespread neuropsychological symptoms originating from the perisylvian network that have distinct time and space relations from secondary epilepsy itself. The involved epilepsies carry the risk of progression to severe, potentially irreversible encephalopathic forms.

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“…A third conceptual change relates to the rare progress of SELECTS to EE-SWAS (ESES/LKS) ( 4 ). It is theoretically interesting, as it offers a model of epileptic progression.…”

Section: Discussionmentioning

confidence: 99%

Section: The Electrophysiological Mechanism Of the Transition From Se...mentioning

confidence: 99%

Section: Changing Interpretations Of the Spectrummentioning

confidence: 99%

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Self-limited childhood epilepsies are disorders of the perisylvian communication system, carrying the risk of progress to epileptic encephalopathies—Critical review

Halász

Szücs

2023

Front. Neurol.

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“…The concept of the epilepsy-aphasia spectrum includes some epileptic syndromes (SeLECTS, atypical benign partial epilepsy (ABPE), LKS, and epileptic encephalopathy with CSWS) that have in common the association between epilepsy, language disorders, and centro-temporal spikes shown through EEG [241]. The theory of a spectrum that unites these conditions, suggested by the shared morphology of EEG paroxysmal abnormalities and by the possible evolution from one into another syndrome (e.g., SeLECTS into CSWS), is present in many works in the literature, albeit with some differentiations among authors [270][271][272][273][274][275][276][277]. The results obtained through genetic techniques such as copy number variation, exome sequencing, and linkage suggest a genetic etiology for these conditions [272,278,279].…”

Section: Etiopathogenetic Factors Of Csws (Including Eses and Lks)mentioning

confidence: 99%

Continuous Spike–Waves during Slow Sleep Today: An Update

Posar,

Visconti

2024

Children

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In the context of childhood epilepsy, the concept of continuous spike–waves during slow sleep (CSWS) includes several childhood-onset heterogeneous conditions that share electroencephalograms (EEGs) characterized by a high frequency of paroxysmal abnormalities during sleep, which have negative effects on the cognitive development and behavior of the child. These negative effects may have the characteristics of a clear regression or of a slowdown in development. Seizures are very often present, but not constantly. The above makes it clear why CSWS have been included in epileptic encephalopathies, in which, by definition, frequent EEG paroxysmal abnormalities have an unfavorable impact on cognitive functions, including socio-communicative skills, causing autistic features, even regardless of the presence of clinically overt seizures. Although several decades have passed since the original descriptions of the electroclinical condition of CSWS, there are still many areas that are little-known and deserve to be further studied, including the EEG diagnostic criteria, the most effective electrophysiological parameter for monitoring the role of the thalamus in CSWS pathogenesis, its long-term evolution, the nosographic location of Landau–Kleffner syndrome, standardized neuropsychological and behavioral assessments, and pharmacological and non-pharmacological therapies.

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“…At the most severe end of the same continuum, this link between IED and cognition is illustrated by the concept of epileptic encephalopathy with continuous spike and waves during sleep (EE-CSWS), defined as severe global or task-specific cognitive regression associated with almost continuous and diffuse IED during sleep. Because this encephalopathy may arise as a complication of SeLFE, these two entities are part of the same spectrum [2][3][4]. Interestingly, cases of Landau-Kleffner syndrome (i.e., an EE-CSWS with specific language regression in the form of auditory agnosia) are reported in patients without seizure [5].…”

Section: Introductionmentioning

confidence: 99%

How the Spreading and Intensity of Interictal Epileptic Activity Are Associated with Visuo-Spatial Skills in Children with Self-Limited Focal Epilepsy with Centro-Temporal Spikes

Dontaine,

Rouge,

Urbain

et al. 2023

Brain Sciences

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This paper investigates brain–behaviour associations between interictal epileptic discharges and cognitive performance in a population of children with self-limited focal epilepsy with centro-temporal spikes (SeLECTS). Sixteen patients with SeLECTS underwent an extensive neuropsychological assessment, including verbal short-term and episodic memory, non-verbal short-term memory, attentional abilities and executive function. Two quantitative EEG indices were analysed, i.e., the Spike Wave Index (SWI) and the Spike Wave Frequency (SWF), and one qualitative EEG index, i.e., the EEG score, was used to evaluate the spreading of focal SW to other parts of the brain. We investigated associations between EEG indices and neuropsychological performance with non-parametric Spearman correlation analyses, including correction for multiple comparisons. The results showed a significant negative correlation between (i) the awake EEG score and the Block Tapping Test, a visuo-spatial short-term memory task, and (ii) the sleep SWI and the Tower of London, a visuo-spatial planning task (pcorr < 0.05). These findings suggest that, in addition to the usual quantitative EEG indices, the EEG analysis should include the qualitative EEG score evaluating the spreading of focal SW to other parts of the brain and that neuropsychological assessment should include visuo-spatial skills.

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More than one self-limited epilepsy of childhood in the same patient: A multicenter study

Cited by 6 publications

References 41 publications

Self-limited childhood epilepsies are disorders of the perisylvian communication system, carrying the risk of progress to epileptic encephalopathies—Critical review

Self-limited childhood epilepsies are disorders of the perisylvian communication system, carrying the risk of progress to epileptic encephalopathies—Critical review

Continuous Spike–Waves during Slow Sleep Today: An Update

How the Spreading and Intensity of Interictal Epileptic Activity Are Associated with Visuo-Spatial Skills in Children with Self-Limited Focal Epilepsy with Centro-Temporal Spikes

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