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Sulthiame add-on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES).
Epilepsia. 2012 Jul; 53(7):1156-61.E

Abstract

PURPOSE

In children with symptomatic or idiopathic focal epilepsies, their disease may evolve into an epileptic encephalopathy related to continuous spike and wave during slow sleep (CSWS) or electrical status epilepticus during slow sleep (ESES). ESES syndrome implies serious risks of neuropsychologic impairment, and its treatment has frequently been disappointing. The aim of this study is to present our experience using sulthiame as add-on treatment in 53 patients with ESES syndrome that was refractory to other antiepileptic drugs (AEDs).

METHODS

Neurologic examinations, cerebral magnetic resonance imaging (MRI), and repeated prolonged sleep electroencephalography (EEG) studies were performed in all cases. Data about school achievements and or neuropsychological evaluations were obtained repeatedly during the follow-up of 1.5-16 years. Sulthiame was added in doses ranging between 5 and 30 mg/kg/day.

KEY FINDINGS

Since add-on of sulthiame, 10 of 28 patients in the symptomatic group became seizure free: 4 patients with normal EEG studies and 6 with residual spikes. Nine of 28 patients showed a significant reduction in number of seizures and presented spikes but no ESES on EEG. The other nine cases showed neither clinical nor EEG improvement. A striking result was that 3 of 11 children with unilateral polymicrogyria and ESES syndrome became seizure free, and in another six a significant improvement in frequency of seizures and in EEG abnormalities seemed to be related to the add-on of sulthiame. Twenty-one of the 25 patients in the idiopathic group became seizure free and without ESES in <3 months after add on of sulthiame. In two of the patients the changes were seen in a few days.

SIGNIFICANCE

We understand that sulthiame may be effective as add-on treatment in children with ESES syndrome.

Authors+Show Affiliations

Department of Neurology, Juan P. Garrahan Pediatric Hospital, Buenos Aires, Argentina. natalio@fejerman.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

22509732

Citation

Fejerman, Natalio, et al. "Sulthiame Add-on Therapy in Children With Focal Epilepsies Associated With Encephalopathy Related to Electrical Status Epilepticus During Slow Sleep (ESES)." Epilepsia, vol. 53, no. 7, 2012, pp. 1156-61.
Fejerman N, Caraballo R, Cersósimo R, et al. Sulthiame add-on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES). Epilepsia. 2012;53(7):1156-61.
Fejerman, N., Caraballo, R., Cersósimo, R., Ferraro, S. M., Galicchio, S., & Amartino, H. (2012). Sulthiame add-on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES). Epilepsia, 53(7), 1156-61. https://doi.org/10.1111/j.1528-1167.2012.03458.x
Fejerman N, et al. Sulthiame Add-on Therapy in Children With Focal Epilepsies Associated With Encephalopathy Related to Electrical Status Epilepticus During Slow Sleep (ESES). Epilepsia. 2012;53(7):1156-61. PubMed PMID: 22509732.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Sulthiame add-on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES). AU - Fejerman,Natalio, AU - Caraballo,Roberto, AU - Cersósimo,Ricardo, AU - Ferraro,Stella Maris, AU - Galicchio,Santiago, AU - Amartino,Hernán, Y1 - 2012/04/17/ PY - 2012/4/19/entrez PY - 2012/4/19/pubmed PY - 2012/9/15/medline SP - 1156 EP - 61 JF - Epilepsia JO - Epilepsia VL - 53 IS - 7 N2 - PURPOSE: In children with symptomatic or idiopathic focal epilepsies, their disease may evolve into an epileptic encephalopathy related to continuous spike and wave during slow sleep (CSWS) or electrical status epilepticus during slow sleep (ESES). ESES syndrome implies serious risks of neuropsychologic impairment, and its treatment has frequently been disappointing. The aim of this study is to present our experience using sulthiame as add-on treatment in 53 patients with ESES syndrome that was refractory to other antiepileptic drugs (AEDs). METHODS: Neurologic examinations, cerebral magnetic resonance imaging (MRI), and repeated prolonged sleep electroencephalography (EEG) studies were performed in all cases. Data about school achievements and or neuropsychological evaluations were obtained repeatedly during the follow-up of 1.5-16 years. Sulthiame was added in doses ranging between 5 and 30 mg/kg/day. KEY FINDINGS: Since add-on of sulthiame, 10 of 28 patients in the symptomatic group became seizure free: 4 patients with normal EEG studies and 6 with residual spikes. Nine of 28 patients showed a significant reduction in number of seizures and presented spikes but no ESES on EEG. The other nine cases showed neither clinical nor EEG improvement. A striking result was that 3 of 11 children with unilateral polymicrogyria and ESES syndrome became seizure free, and in another six a significant improvement in frequency of seizures and in EEG abnormalities seemed to be related to the add-on of sulthiame. Twenty-one of the 25 patients in the idiopathic group became seizure free and without ESES in <3 months after add on of sulthiame. In two of the patients the changes were seen in a few days. SIGNIFICANCE: We understand that sulthiame may be effective as add-on treatment in children with ESES syndrome. SN - 1528-1167 UR - https://www.unboundmedicine.com/medline/citation/22509732/Sulthiame_add_on_therapy_in_children_with_focal_epilepsies_associated_with_encephalopathy_related_to_electrical_status_epilepticus_during_slow_sleep__ESES__ L2 - https://doi.org/10.1111/j.1528-1167.2012.03458.x DB - PRIME DP - Unbound Medicine ER -