Sulthiame add‐on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES)

Abstract

Summary 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.

Encephalopathy with status epilepticus during sleep: Unusual EEG patterns

“…[21][22][23][24][25][26] Treatment of the ESES syndrome has frequently been disappointing, but different antiepileptic drug schemes have been proposed. 19,21,23,[27][28][29][30] In refractory cases, therapeutic alternatives such as corticosteroids, gamma-globulins, the ketogenic diet, and surgery have been tried. 31,32 The aim of this study was to describe the electroclinical characteristics, etiology, treatment, and prognosis of 17 patients with the typical clinical features of epileptic encephalopathy with ESES but with unusual EEG patterns and to evaluate if these cases are part of the ESES syndrome.…”

Acetazolamide for electrical status epilepticus in slow‐wave sleep

“…Sulthiame, a sulfonamide carbonic anhydrase inhibitor used outside the United States, is mainly an adjunctive therapy for epilepsy. It has been reported to be effective in treatment of children with refractory or atypical benign epilepsy with centro‐temporal spikes (BECTS) and other focal epilepsies . In these children, ESES can be seen, which raises the question of whether BECTS is a milder form on the clinical spectrum of ESES‐related disorders.…”

Idiopathic focal epilepsies: the “lost tribe”

“…Nevertheless, in a recent series of 28 symptomatic and 25 idiopathic cases of focal epilepsies associated with encephalopathy related to ESES receiving add‐on therapy with sulthiame, the results were quite encouraging, especially in children with a previous diagnosis of RE or PS (Fejerman et al ., ). It is interesting to note that a particular group of children with unilateral polymicrogyria may present with the same atypical clinical and EEG evolutions as the patients with IFE responding to therapy in the same way (Caraballo et al ., ).…”