Dados do Trabalho

Título

ELECTRICAL STATUS EPILEPTICUS IN SLEEP (ESES): A CASE SERIES OF 10 PATIENTS

Introdução

Electrical Status Epilepticus in Sleep (ESES) is a rare electrographic pattern in less than 1% of epilepsy cases, characterized by significant epileptiform activity during non-REM sleep, often leading to epilepsy and encephalopathy. This study presents the clinical characteristics of 10 ESES patients.

Objetivo

This study aims to delineate the clinical profiles of 10 children diagnosed with ESES, highlighting the impact of various anti-seizure medications (ASM) and the associated neurological outcomes.

Método

Undergraduate scientific research project (CAAE: 334383220.9.0000.5558), a descriptive, retrospective cross-sectional study, based on the collection of data from the medical records of 10 patients with ESES and some degree of clinical neurological deterioration, cognitive engagement and / or behavioral disorder, treated at the Pediatric Neurology outpatient clinics of the University Hospital of Brasília. The following aspects were evaluated: gender, age at which ESES was identified, electrographic pattern prior to ESES, etiology, duration of ESES, type of epileptic seizures, Anti-Seizure Medications (ASM) used at the time of diagnosis and in the control.

Resultados

There were 7 male patients, with a mean age of 6.9 years (2 to 11 years). Eight patients had an EEG with predominantly Benign Childhood Epilepsy with Centro-Temporal Spikes (BCECTS). The etiology was defined in five patients (1 with frontal gliosis; 1 with pachygyria, 1 with perisylvian polymicrogyria, 1 with temporal venous anomaly and 1 with sequelae lesion in the thalamus). The ESES had a mean duration of 2.1 years (4 months to 3.6 years), being higher in the group of patients with structural damage (2.3 x 1.9 years). There was a predominance of focal motor seizures (6 patients). On average, 3.8 ASM (1 to 8) were used in mono- or polytherapy. At the time of diagnosis, the most commonly used ASM was valproic acid (VA) (7 patients). Seizure control was obtained in 8 of 10 patients, and 6 patients maintained VA, associated with clobazam in 4 and levetiracetam in 2. Corticosteroid therapy was used in 50% of the sample, for a mean period of 2.8 months.

Conclusão

The study confirmed typical ESES demographics and symptoms, noting prevalent brain injuries and focal seizures. The efficacy of valproic acid over carbamazepine suggests alternative therapies could be more effective, highlighting the influence of intrinsic factors in ESES pathophysiology and the need for further research on personalized treatments.

Referências

Ng, R., & Hodges, E. (2020). Neurocognitive profiles of pediatric patients with ESES, generalized epilepsy, or focal epilepsy. Epilepsy Research, 167, 106351. https://doi.org/10.1016/j.eplepsyres.2020.106351​:contentReference[oaicite:0]{index=0}

Bonanni, P., Negrin, S., Volzone, A., Zanotta, N., Epifanio, R., Zucca, C., et al. (2017). Electrical status epilepticus during sleep in Mowat-Wilson syndrome. Brain Dev., 39, 727–734. https://doi.org/10.1016/j.braindev.2017.04.011

Tassinari, C.A., Rubboli, G., Volpi, L., Meletti, S., d’Orsi, G., Franca, M., et al. (2000). Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia. Clin Neurophysiol., 111(Suppl 2), S94–102. https://doi.org/10.1016/S1388-2457(00)00366-4

Carmi, N., Lev, D., Leshinsky-Silver, E., Anikster, Y., Blumkin, L., Kivity, S., et al. (2015). Atypical presentation of Costeff syndrome—Severe psychomotor involvement and electrical status epilepticus during slow-wave sleep. Eur J Paediatr Neurol., 19, 733–736. https://doi.org/10.1016/j.ejpn.2015.06.001

Zanni, G., Barresi, S., Cohen, R., Specchio, N., Basel-Vanagaite, L., Valente, E.M., et al. (2014). A novel mutation in the endosomal Na+/H+ exchanger NHE6 (SLC9A6) causes Christianson syndrome with electrical status epilepticus during slow-wave sleep (ESES). Epilepsy Res., 108, 811–815. https://doi.org/10.1016/j.eplepsyres.2014.02.015

Siniatchkin, M., Groening, K., Moehring, J., Moeller, F., Boor, R., Brodbeck, V., et al. (2010). Neuronal networks in children with continuous spikes and waves during slow sleep. Brain, 133, 2798–2813. https://doi.org/10.1093/brain/awq223

Palavras Chave

epilepsy; eses; Pediatric Epilepsy