Misleading Effects of Clonazepam in Symptomatic Electrical Status Epilepticus During Sleep Syndrome

Bahi‐Buisson, Nadia; Savini, Rosanna; Eisermann, M.; Bulteau, Christine; Dulac, Olivier; Hertz-Pannier, Lucie; Chiron, Catherine

doi:10.1016/j.pediatrneurol.2005.08.014

Cited by 9 publications

(5 citation statements)

References 11 publications

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“…Improvement of interictal epileptiform activity in continuous spikes and waves during sleep may also be accompanied by improvement in the clinical features 7, 9, 42 . However, published data are contradictory 6 and the correlation of interictal epileptiform activity with cognitive features is not always consistent 18, 20, 22, 43 . Our case series provides long-term follow-up of the epileptiform activity in patients with continuous spikes and waves during sleep.…”

Section: Discussionmentioning

confidence: 96%

Long-Term Response to High-Dose Diazepam Treatment in Continuous Spikes and Waves During Sleep

Fernández¹,

Peters²,

An³

et al. 2013

Pediatric Neurology

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Section: Discussionmentioning

confidence: 96%

Long-Term Response to High-Dose Diazepam Treatment in Continuous Spikes and Waves During Sleep

Fernández¹,

Peters²,

An³

et al. 2013

Pediatric Neurology

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“…There is evidence in the literature supporting a beneficial effect of treatment on seizure frequency and severity [7, 19, 26, 36, 66–70] and epileptiform activity [19, 21, 71, 72]. Several studies suggest that long-term neurocognitive function can significantly improve once epileptiform discharges are reduced, and this effect has been related to treatment with antiepileptic drugs [3, 6, 7, 17, 19, 73–77].…”

Section: Managementmentioning

confidence: 99%

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

Fernández

Chapman

Peters

et al. 2013

Epilepsy Research and Treatment

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Continuous spikes and waves during sleep (CSWS) is an epileptic encephalopathy characterized in most patients by (1) difficult to control seizures, (2) interictal epileptiform activity that becomes prominent during sleep leading to an electroencephalogram (EEG) pattern of electrical status epilepticus in sleep (ESES), and (3) neurocognitive regression. In this paper, we will summarize current epidemiological, clinical, and EEG knowledge on CSWS and will provide suggestions for treatment. CSWS typically presents with seizures around 2–4 years of age. Neurocognitive regression occurs around 5-6 years of age, and it is accompanied by subacute worsening of EEG abnormalities and seizures. At approximately 6–9 years of age, there is a gradual resolution of seizures and EEG abnormalities, but the neurocognitive deficits persist in most patients. The cause of CSWS is unknown, but early developmental lesions play a major role in approximately half of the patients, and genetic associations have recently been described. High-dose benzodiazepines and corticosteroids have been successfully used to treat clinical and electroencephalographic features. Corticosteroids are often reserved for refractory disease because of adverse events. Valproate, ethosuximide, levetiracetam, sulthiame, and lamotrigine have been also used with some success. Epilepsy surgery may be considered in a few selected patients.

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“…Thus, the increasing interest in supporting the genetic risk of CNV for epilepsies allows better examination of the CNV carriers of unexplained cases, thus helping clinical reporting [38]. The treatment of ESES, ABPE, and LKS may include old and new anti-epileptic drugs, alone or in combination with valproic acid, ethosuximide, sulthiame, and steroids, and immunoglobulins [39]. Ketogenic diet and surgery with multiple subpial transactions led to good results in terms of both reduction in epileptic seizures and cognitive development [40][41].…”

Section: Resultsmentioning

confidence: 99%

Atypical benign partial epilepsy and a new variant of SLC35A3 gene plus 2p25.1 duplication. Phenotypic-Genotypic correlation?

Parano¹,

Pavone²,

Pappalardo³

et al. 2021

Preprint

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Background Atypical Benign Partial Epilepsy (ABPE), recognized also as pseudo-Lennox syndrome, is an uncommon form of epilepsy characterized by generalized minor seizures such as atonic, absences, or myoclonic seizures, and electroencephalographic pattern of focal or multifocal sharp waves with activation of epileptiform discharges during sleep. ABPE is indicated as a variant of ESES (ILAE classification 2017). ESES is a clinical entity that is characterized by encephalopathy with cognitive/ behavioral regression and EEG pattern of electrical status epilepticus during slow sleep. Fine and gross motor, language and social/behavioral impairment are associated symptoms, which may have reversible or persistent course. ABPE has been ascribed to the group of the “epilepsy aphasia spectrum” disorders, which includes also Rolandic Epilepsy, Landau-Kleffner syndrome, and electrical status epilepticus during sleep/continuous spike-wave during sleep. We report a young boy with a previous mild motor and language delay, who at 2-year-old presented with recurrent atonic seizures and an EEG pattern consisting of continuous spike and waves during sleep. Methods Next Generation Sequencing and microarray technology were used to investigate the molecular background of the proband, and the findings were then analyzed and integrated with clinical data. Results The child has been followed up to 7 years of age showing a progressive, complete EEG resolution and a normalization of the previous motor and cognitive impairment in association to the levetiracetam treatment. Genetic diagnosis displayed a novel heterozygous mutation c.310G>A of the SLC35A3 gene and a partial duplication of the short arm of chromosome 2, which might have pathogenic correlation with the neurological signs presented by the child. Conclusions Data generated by a genomic approach disclose a more comprehensive view of the genotype-phenotype correlation analysis for the novel pathogenic variant and ABPE. The relationship between the phenotypic manifestations of the child and genetic data is discussed.

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Misleading Effects of Clonazepam in Symptomatic Electrical Status Epilepticus During Sleep Syndrome

Cited by 9 publications

References 11 publications

Long-Term Response to High-Dose Diazepam Treatment in Continuous Spikes and Waves During Sleep

Long-Term Response to High-Dose Diazepam Treatment in Continuous Spikes and Waves During Sleep

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

Atypical benign partial epilepsy and a new variant of SLC35A3 gene plus 2p25.1 duplication. Phenotypic-Genotypic correlation?

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