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Hemispherotomy in children with electrical status epilepticus of sleep.
J Neurosurg Pediatr. 2017 Jan; 19(1):56-62.JN

Abstract

OBJECTIVE

Electrical status epilepticus of sleep (ESES) is a rare electrographic pattern associated with global regression, which is often poorly responsive to traditional epilepsy treatments and can have a devastating and permanent neurocognitive outcome. The authors analyzed clinical, electroencephalographic, and neuropsychological outcomes in 9 patients with refractory ESES treated with functional hemispherotomy to illustrate the wide clinical spectrum associated with the disease and explore the role of hemispherotomy in its treatment.

METHODS

During the period between 2003 and 2015, 80 patients underwent hemispherotomy at the authors' institution. Video electroencephalography (EEG) reports were reviewed for ESES or continuous spikes and waves during sleep (CSWS). Patients with preoperative ESES (> 85% slow-wave sleep occupied by spike waves), a unilateral structural lesion amenable to surgery, and more than 6 months of follow-up data were included in the analysis. Clinical data, EEG recordings, neuropsychological testing, and parental and clinician reports were retrospectively reviewed.

RESULTS

Nine patients were eligible for study inclusion. Age at seizure onset ranged from birth to 4.2 years (mean 1.9 years), age at ESES diagnosis ranged from 3.5 to 8.8 years (mean 6.0 years), and age at hemispherotomy ranged from 3.7 to 11.5 years (mean 6.8 years). All patients had drug-resistant epilepsy. The duration of epilepsy prior to hemispherotomy ranged from 2.7 to 8.9 years (mean ± SD, 5.0 ± 2.2 years). Engel Class I seizure outcome was observed in all 9 children, with a mean follow-up of 3.0 years (range 0.5-6.1 years). Hemispherotomy terminated ESES in 6 of 6 patients with available postoperative sleep EEG. All children had preoperative neuropsychological impairments. Developmental regression was halted postoperatively, but none of the children returned to their original pre-ESES baseline. Four children demonstrated academic gains, 2 of whom transitioned to mainstream classes.

CONCLUSIONS

Children with drug-resistant ESES and a unilateral structural lesion should be evaluated for hemispherotomy as they may experience the cessation of seizures, termination of ESES, and improvement in neuropsychological status.

Authors+Show Affiliations

Departments of 1 Neurology and.Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri.Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri.Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri.Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri.Departments of 1 Neurology and.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

27791702

Citation

Jeong, Anna, et al. "Hemispherotomy in Children With Electrical Status Epilepticus of Sleep." Journal of Neurosurgery. Pediatrics, vol. 19, no. 1, 2017, pp. 56-62.
Jeong A, Strahle J, Vellimana AK, et al. Hemispherotomy in children with electrical status epilepticus of sleep. J Neurosurg Pediatr. 2017;19(1):56-62.
Jeong, A., Strahle, J., Vellimana, A. K., Limbrick, D. D., Smyth, M. D., & Bertrand, M. (2017). Hemispherotomy in children with electrical status epilepticus of sleep. Journal of Neurosurgery. Pediatrics, 19(1), 56-62. https://doi.org/10.3171/2016.8.PEDS16319
Jeong A, et al. Hemispherotomy in Children With Electrical Status Epilepticus of Sleep. J Neurosurg Pediatr. 2017;19(1):56-62. PubMed PMID: 27791702.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hemispherotomy in children with electrical status epilepticus of sleep. AU - Jeong,Anna, AU - Strahle,Jennifer, AU - Vellimana,Ananth K, AU - Limbrick,David D,Jr AU - Smyth,Matthew D, AU - Bertrand,Mary, Y1 - 2016/10/28/ PY - 2016/10/30/pubmed PY - 2017/2/16/medline PY - 2016/10/30/entrez KW - CSWS KW - CSWS = continuous spikes and waves during sleep KW - EEG = electroencephalography KW - ESES KW - ESES = electrical status epilepticus of sleep KW - FSIQ = Full Scale IQ KW - PIQ = Performance IQ KW - VIQ = Verbal IQ KW - VMI = visual-motor integration KW - VNS = vagus nerve stimulator KW - WPPSI = Wechsler Preschool and Primary Scale of Intelligence KW - continuous spikes and waves during sleep KW - electrical status epilepticus of sleep KW - epilepsy surgery SP - 56 EP - 62 JF - Journal of neurosurgery. Pediatrics JO - J Neurosurg Pediatr VL - 19 IS - 1 N2 - OBJECTIVE Electrical status epilepticus of sleep (ESES) is a rare electrographic pattern associated with global regression, which is often poorly responsive to traditional epilepsy treatments and can have a devastating and permanent neurocognitive outcome. The authors analyzed clinical, electroencephalographic, and neuropsychological outcomes in 9 patients with refractory ESES treated with functional hemispherotomy to illustrate the wide clinical spectrum associated with the disease and explore the role of hemispherotomy in its treatment. METHODS During the period between 2003 and 2015, 80 patients underwent hemispherotomy at the authors' institution. Video electroencephalography (EEG) reports were reviewed for ESES or continuous spikes and waves during sleep (CSWS). Patients with preoperative ESES (> 85% slow-wave sleep occupied by spike waves), a unilateral structural lesion amenable to surgery, and more than 6 months of follow-up data were included in the analysis. Clinical data, EEG recordings, neuropsychological testing, and parental and clinician reports were retrospectively reviewed. RESULTS Nine patients were eligible for study inclusion. Age at seizure onset ranged from birth to 4.2 years (mean 1.9 years), age at ESES diagnosis ranged from 3.5 to 8.8 years (mean 6.0 years), and age at hemispherotomy ranged from 3.7 to 11.5 years (mean 6.8 years). All patients had drug-resistant epilepsy. The duration of epilepsy prior to hemispherotomy ranged from 2.7 to 8.9 years (mean ± SD, 5.0 ± 2.2 years). Engel Class I seizure outcome was observed in all 9 children, with a mean follow-up of 3.0 years (range 0.5-6.1 years). Hemispherotomy terminated ESES in 6 of 6 patients with available postoperative sleep EEG. All children had preoperative neuropsychological impairments. Developmental regression was halted postoperatively, but none of the children returned to their original pre-ESES baseline. Four children demonstrated academic gains, 2 of whom transitioned to mainstream classes. CONCLUSIONS Children with drug-resistant ESES and a unilateral structural lesion should be evaluated for hemispherotomy as they may experience the cessation of seizures, termination of ESES, and improvement in neuropsychological status. SN - 1933-0715 UR - https://www.unboundmedicine.com/medline/citation/27791702/Hemispherotomy_in_children_with_electrical_status_epilepticus_of_sleep_ L2 - https://thejns.org/doi/10.3171/2016.8.PEDS16319 DB - PRIME DP - Unbound Medicine ER -