Sulthiame add-on treatment in children with epileptic encephalopathy with status epilepticus: an efficacy analysis in etiologic subgroups

Kanmaz, Seda; Şimşek, Erdem; Serin, Hepsen Mine; Yılmaz, Sanem; Aktan, Gül; Tekgül, Hasan; Gökben, Sarenur

doi:10.1007/s10072-020-04526-y

Cited by 8 publications

(10 citation statements)

References 32 publications

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“…STM acts through central carbonic anhydrase inhibition. It causes an extracellular proton concentration rise, which inhibits inward currents mediated by NMDAR and calcium currents through voltage-gated channels, consequently reducing neuronal excitatory function [9]. It also exerts a sodium channel-blocking effect in isolated hippocampal neurons, resulting in degradation of repetitive action potential generation [9].…”

Section: Discussionmentioning

confidence: 99%

“…GRIN2A gene encodes a GluN2A protein, believed to be the most relevant GluN2 subunit of N-methyl-D-aspartate receptors (NMDAR) [7]. Sulthiame (STM), a sulfonamide derivative, was found to be efficient in focal and generalized epilepsies in 1960 [8,9]. It causes a global depression of intrinsic neuronal excitability, especially acting over NMDAR [8,9].…”

Section: Introductionmentioning

confidence: 99%

“…Sulthiame (STM), a sulfonamide derivative, was found to be efficient in focal and generalized epilepsies in 1960 [8,9]. It causes a global depression of intrinsic neuronal excitability, especially acting over NMDAR [8,9]. Although abandoned during the mid-1970s due to toxicity when used in combination with phenytoin, lately it has reemerged as monotherapy in SeLECTS or as an add-on therapy in EE-SWAS/DEE-SWAS [8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

“…It causes a global depression of intrinsic neuronal excitability, especially acting over NMDAR [8,9]. Although abandoned during the mid-1970s due to toxicity when used in combination with phenytoin, lately it has reemerged as monotherapy in SeLECTS or as an add-on therapy in EE-SWAS/DEE-SWAS [8][9][10][11]. It has also been found to be effective in the treatment of refractory epilepsies [8].…”

Section: Introductionmentioning

confidence: 99%

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Successful Treatment of a Child With Epileptic Encephalopathy With Spike-Wave Activation in Sleep and GRIN2A Variant Using Sulthiame

Pereira-Nunes¹,

Sousa²,

Fonseca³

et al. 2023

Cureus

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Epileptic encephalopathy with spike-wave activation in sleep (EE-SWAS) and developmental EE-SWAS (DEE-SWAS) are characterized by variable combinations of cognitive, language, behavioral, and/or motor regression associated with continuous or near-continuous diffuse spike-and-wave complexes during sleep. Glutamate ionotropic receptor NMDA type subunit 2A (GRIN2A) variants have been associated with EE-SWAS. It encodes the most relevant GluN2 subunit of the N-methyl-D-aspartate receptor (NMDAR). Sulthiame reduces NMDAR-mediated neuronal excitability and has been progressively used as monotherapy in self-limited epilepsy with centrotemporal spikes (SeLECTS) or as add-on therapy in EE-SWAS/DEE-SWAS. A five-year-old female, with family history of epilepsy, was initially diagnosed with SeLECTS and medicated with valproic acid (VPA). One year later, she presented a focal to bilateral tonic-clonic seizure during sleep and learning difficulty. The electroencephalogram revealed continuous spike-and-wave during sleep leading to the diagnosis of EE-SWAS. Prednisolone was effective, but there was repeated recurrence after its discontinuation and associated adverse effects. As an alternative, sulthiame was added to VPA. Four years later, she remains clinically stable. Genetic testing revealed a GRIN2A missense variant, C.3228C>A (p.Asn1076Lys). Sulthiame appeared effective in this recurrent EE-SWAS child, who presented a GRIN2A missense variant with possible NMDAR gain-of-function and adverse effects of corticosteroids. Functional studies of GRIN2A variants might become a future tool for individualized therapies.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Successful Treatment of a Child With Epileptic Encephalopathy With Spike-Wave Activation in Sleep and GRIN2A Variant Using Sulthiame

Pereira-Nunes¹,

Sousa²,

Fonseca³

et al. 2023

Cureus

View full text Add to dashboard Cite

show abstract

“…It might be because epilepsy was in a dynamic and constantly changing development process, so abnormalities occurred in daily cranial imaging examinations. According to the results of neuropathological studies, the continuous point activity abnormality of the central nervous system might result in irreversible neuronal damage and even death [ 19 ]. Children with recurrent clinical manifestations such as vomiting, nausea, vertigo, limb pain, headache, and abdominal pain, especially school-age patients, should be questioned on medical history in detail.…”

Section: Discussionmentioning

confidence: 99%

Children’s Neurological Status Epilepticus and Poor Prognostic Factors through Electroencephalogram Image under Composite Domain Analysis Algorithm

Gao

Liu

Zhao

et al. 2021

Journal of Healthcare Engineering

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This study aimed to analyze the application of composite domain analysis algorithm for electroencephalogram (EEG) images of children with epilepsy and to investigate the risk factors related to poor prognosis. 70 children with neurological epilepsy admitted to the hospital were selected as the research objects. Besides, the EEG of the children during the intermittent and seizure phases of epilepsy were collected, so as to establish a composite domain analysis algorithm model. Then, the model was applied in EEG analysis. The clinical disease type and prognosis of children were statistically analyzed, and the risk factors that affected the prognosis of children were investigated. The results showed that the EEG signal values of the detail coefficients (d51 and d52) and the approximate coefficient (c5) during the epileptic seizure period were higher markedly than the signal values of the epileptic intermittent period; the EEG signal of the epileptic intermittent period was a transient waveform, which appeared as sharp waves or spikes. The EEG signal of epileptic seizures was continuous, with a composite waveform of sharp waves and spikes, and the change amplitude of the wavelet envelope spectrum during epileptic seizures was also higher hugely than that of intermittent epilepsy. The accurate identification rate, specificity, and sensitivity of EEG analysis with the composite domain algorithm were higher than those without the algorithm. Among the five types of epileptic seizures in children, the proportion of systemic tonic-clonic status was the largest, and the proportion of myoclonic status was equal to that of complex partial epileptic status, both of which were relatively small. The proportion of children with a better prognosis was 75.71% (53/70), which was higher than those with a poor prognosis 24.29% (17/70). Abnormal imaging examination (odds ratio (OR) = 3.823 and 95% confidence interval (CI) = 1.643–8.897); seizure duration greater than 1 hour (OR = 1.855 and 95% CI = 1.076–3.199); C-reactive protein (CRP) (OR = 5.089 and 95% CI = 1.507–17.187); and abnormal blood glucose (OR = 3.077, 95%CI = 1.640–5.773) were all independent risk factors for poor prognosis (all P < 0.05 ). The composite domain analysis algorithm was helpful for clinicians to find the difference in the EEG signals between the epileptic seizure period and the epileptic intermittent period in a short time, thereby improving the doctor’s analysis of the results, which could reflect its marked superiority. In addition, abnormal imaging examinations, convulsion duration greater than 1 hour, CRP, and abnormal blood glucose were independent risk factors for poor prognosis in children. Therefore, the invasion of related risk factors could be reduced clinically by prognostic review with medical advice, attention to food safety and hygiene, and improvement of children’s immunity.

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Unmet needs in epileptic encephalopathy with spike-and-wave activation in sleep: A systematic review

Chapman,

Haubenberger,

Jen

et al. 2024

Epilepsy Research

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Sulthiame add-on treatment in children with epileptic encephalopathy with status epilepticus: an efficacy analysis in etiologic subgroups

Cited by 8 publications

References 32 publications

Successful Treatment of a Child With Epileptic Encephalopathy With Spike-Wave Activation in Sleep and GRIN2A Variant Using Sulthiame

Successful Treatment of a Child With Epileptic Encephalopathy With Spike-Wave Activation in Sleep and GRIN2A Variant Using Sulthiame

Children’s Neurological Status Epilepticus and Poor Prognostic Factors through Electroencephalogram Image under Composite Domain Analysis Algorithm

Unmet needs in epileptic encephalopathy with spike-and-wave activation in sleep: A systematic review

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