Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia

Tassinari, C. A.; Rubboli, Guido; Volpi, L.; Meletti, Stefano; D’Orsi, G.; Franca, Matteo; Sabetta, Annarita; Riguzzi, P.; Gardella, Elena; Zaniboni, Alberto; Michelucci, Roberto

doi:10.1016/s1388-2457(00)00408-9

Cited by 285 publications

(381 citation statements)

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“…Epilepsy syndromes with continuous spikes-and-waves during slow sleep (CSWS) are age-related epileptic encephalopathies characterized by the development of various types of psychomotor regression in close temporal concordance with the appearance of the electroencephalography (EEG) pattern of CSWS (Tassinari et al, 2000). This EEG pattern consists of sleep-related activation and diffusion of spike-wave (SW) discharges, usually during more than 85% of non-REM (rapid eye movement) sleep (Tassinari et al, 2000).…”

Section: Discussionmentioning

confidence: 99%

“…This EEG pattern consists of sleep-related activation and diffusion of spike-wave (SW) discharges, usually during more than 85% of non-REM (rapid eye movement) sleep (Tassinari et al, 2000). A minority of CSWS cases have been associated with cortical or thalamic lesions (symptomatic cases), whereas in the other cases, the etiology is unknown (De Tige et al, 2006).…”

Section: Discussionmentioning

confidence: 99%

“…These findings highlight that the psychomotor regression observed in CSWS syndromes is not only related to the neurophysiologic impairment at the site of the epileptic foci but also to epilepsy-induced neurophysiologic changes in distant connected brain areas. KEY WORDS: Continuous spikes-and-waves during slow sleep, [18F]-Fluorodeoxyglucose, Positron emission tomography, EEG combined fMRI, Remote inhibition, Surrounding inhibition.Epilepsy syndromes with continuous spikes-and-waves during slow sleep (CSWS) are age-related epileptic encephalopathies characterized by the development of various types of psychomotor regression in close temporal concordance with the appearance of the electroencephalography (EEG) pattern of CSWS (Tassinari et al, 2000). This EEG pattern consists of sleep-related activation and diffusion of spike-wave (SW) discharges, usually during more than 85% of non-REM (rapid eye movement) sleep (Tassinari et al, 2000).…”

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confidence: 99%

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Insights into the pathophysiology of psychomotor regression in CSWS syndromes from FDG‐PET and EEG‐fMRI

2009

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SUMMARYEpilepsy syndromes with continuous spikes-andwaves during slow sleep (CSWS) are age-related epileptic encephalopathies characterized by the development of various types of psychomotor regression in close temporal concordance with the appearance of the electroencephalography (EEG) pattern of CSWS. Functional cerebral imaging studies performed in children with CSWS have shown evidence for the existence of increase in metabolism or perfusion at the site of the epileptic focus, associated with decrease in metabolism or perfusion in distant and connected brain areas. Longitudinal [18F]-fluorodeoxyglucose-positron emission tomography (FDG-PET) studies and effective connectivity analyses have suggested the existence of a pathophysiologic link between increases and decreases in metabolism/perfusion that could be explained by the theory of remote inhibition. These findings highlight that the psychomotor regression observed in CSWS syndromes is not only related to the neurophysiologic impairment at the site of the epileptic foci but also to epilepsy-induced neurophysiologic changes in distant connected brain areas. KEY WORDS: Continuous spikes-and-waves during slow sleep, [18F]-Fluorodeoxyglucose, Positron emission tomography, EEG combined fMRI, Remote inhibition, Surrounding inhibition.Epilepsy syndromes with continuous spikes-and-waves during slow sleep (CSWS) are age-related epileptic encephalopathies characterized by the development of various types of psychomotor regression in close temporal concordance with the appearance of the electroencephalography (EEG) pattern of CSWS (Tassinari et al., 2000). This EEG pattern consists of sleep-related activation and diffusion of spike-wave (SW) discharges, usually during more than 85% of non-REM (rapid eye movement) sleep (Tassinari et al., 2000). A minority of CSWS cases have been associated with cortical or thalamic lesions (symptomatic cases), whereas in the other cases, the etiology is unknown (De Tige et al., 2006). In some of the latter cases, the existence of a continuum ranging from asymptomatic carriers of centrotemporal spikes to epilepsies with CSWS is suspected (De Tige et al., 2006).Epilepsy syndromes with CSWS are characterized by an acute phase defined by the emergence of psychomotor deficits, various types of seizures, and CSWS activity at around 3-8 years of age (Holmes & Lenck-Santini, 2006). This acute phase is followed by a recovery phase in which the patient's clinical condition improves together with the remission of the CSWS pattern. Recovery spontaneously occurs at around 15 years of age but it may be prompted by using antiepileptic drugs (AEDs) including corticosteroids (Holmes & Lenck-Santini, 2006). This biphasic evolution suggests that CSWS activity largely contributes to the psychomotor deficits observed in these patients (Holmes & Lenck-Santini, 2006). However, some authors still consider CSWS activity as an epiphenomenon reflecting the underlying brain pathology, rather than the direct cause of the psychomotor regression (Aldenkamp & Arend...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Insights into the pathophysiology of psychomotor regression in CSWS syndromes from FDG‐PET and EEG‐fMRI

2009

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“…1,2,25 In this regard the similarities are interesting between our results and the ones obtained in children with electrical status epilepticus during sleep (CSWS). 26 By means of EEG-fMRI coregistration, a common bilateral BOLD increase in the perisylvian regions (plus thalamus and cingulate cortex) was observed in CSWS patients, independent of the etiology and the initial spike focus of the single cases. 12 This finding supports the notion that the involvement of these cortical regions is crucial for the neuropsychological impairment and the behavioral regression that accompany this disorder.…”

Section: ] Syndromementioning

confidence: 91%

Epilepsy‐related brain networks in ring chromosome 20 syndrome: An EEG‐fMRI study

et al. 2014

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SUMMARYObjective: To identify the brain networks that are involved in the different electroencephalography (EEG) abnormalities in patients with ring chromosome 20 [r(20)] syndrome. We hypothesize the existence of both distinctive and common brain circuits for the paroxysmal high voltage sharp waves (hSWs), the seizures, and the slow-wave 3-7 Hz rhythm that characterize this condition. Methods: Thirteen patients with [r(20)] syndrome were studied by means of EEG simultaneously recorded with functional magnetic resonance imaging (EEG-fMRI). EEG traces were reviewed in order to detect the pathologic interictal (hSWs) and ictal activities; the 3-7 Hz theta-delta power was derived using a fast Fourier transform. A group-level analysis was performed for each type of EEG abnormality separately using a fixed-effect model and a conjunction analysis. Finally, a second-level random-effect model was applied considering together the different EEG abnormalities, without distinction between hSW, seizures, or theta-delta rhythms. Results: Subcontinuous theta-delta rhythm was recorded in seven patients, seizures in two, and hSWs in three patients. The main results are the following: (1) the slow-wave rhythm was related to blood oxygen level-dependent (BOLD) increases in the premotor, sensory-motor, and temporoparietal cortex, and to BOLD decrements involving the default mode (DMN) and the dorsal attention networks (DANs); (2) the ictalrelated BOLD changes showed an early involvement of the prefrontal lobe; (3) increases in BOLD signal over the basal ganglia, either for interictal and ictal activities, were observed; (4) a common pattern of positive BOLD changes in the bilateral perisylvian regions was found across the different EEG abnormalities. Significance: The BOLD increment in the perisylvian network and the decrease of the DMN and DAN could be the expression of the [r(20)] syndrome-related cognitive and behavioral deficits. The observed BOLD patterns are similar to the ones detected in other epileptic encephalopathies, suggesting that different epileptic disorders characterized by neurobehavioral regression are associated with dysfunction in similar brain networks.

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“…This situation is encountered in epileptic encephalopathy (EE) with continuous spike and waves during slow-wave sleep (CSWS). EE with CSWS is an age-related epilepsy that presents with neurocognitive regression and an EEG pattern characterized by strong activation of the epileptic activity during sleep, with spikes that tend to diffuse over the whole scalp, and typically occupy more than 85% of slow-wave-sleep time (Tassinari et al, 2000). Proper diagnosis of EE with CSWS is very important, since it requires specific treatment, including corticosteroids .…”

Section: Introductionmentioning

confidence: 99%

Cluster-based spike detection algorithm adapts to interpatient and intrapatient variation in spike morphology

Nonclercq

Foulon

Verheulpen

et al. 2012

Journal of Neuroscience Methods

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Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia

Cited by 285 publications

References 33 publications

Insights into the pathophysiology of psychomotor regression in CSWS syndromes from FDG‐PET and EEG‐fMRI

Insights into the pathophysiology of psychomotor regression in CSWS syndromes from FDG‐PET and EEG‐fMRI

Epilepsy‐related brain networks in ring chromosome 20 syndrome: An EEG‐fMRI study

Cluster-based spike detection algorithm adapts to interpatient and intrapatient variation in spike morphology

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