Epileptic Encephalopathies in Adults and Childhood

Kural, Zekiye; Özer, Ali Fahir

doi:10.1155/2012/205131

Cited by 6 publications

(10 citation statements)

References 119 publications

(106 reference statements)

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“…Epileptic encephalopathies (EE) are a heterogeneous group of epilepsy syndromes ( 14 , 15 ) in which epileptic activity leads to progressively greater levels of cognitive and behavioral impairment as it would be expected only as a result of the underlying structural or genetic pathology. According to ILAE guidelines (International League Against Epilepsy), common EE syndromes with characteristic electroclinical manifestations are Lennox-Gastaut Syndrome (LGS), Dravet Syndrome (DS), West Syndrome (WS) and EE with continuous spike-and-wave during sleep (CSWS).…”

Section: Introductionmentioning

confidence: 99%

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Efficacy, Retention, and Tolerability of Brivaracetam in Patients With Epileptic Encephalopathies: A Multicenter Cohort Study From Germany

et al. 2018

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Objective: To evaluate the efficacy and tolerability of brivaracetam (BRV) in a severely drug refractory cohort of patients with epileptic encephalopathies (EE).Method: A multicenter, retrospective cohort study recruiting all patients treated with EE who began treatment with BRV in an enrolling epilepsy center between 2016 and 2017.Results: Forty-four patients (27 male [61%], mean age 29 years, range 6 to 62) were treated with BRV. The retention rate was 65% at 3 months, 52% at 6 months and 41% at 12 months. A mean retention time of 5 months resulted in a cumulative exposure to BRV of 310 months. Three patients were seizure free during the baseline. At 3 months, 20 (45%, 20/44 as per intention-to-treat analysis considering all patients that started BRV including three who were seizure free during baseline) were either seizure free (n = 4; 9%, three of them already seizure-free at baseline) or reported at least 25% (n = 4; 9%) or 50% (n = 12; 27%) reduction in seizures. An increase in seizure frequency was reported in two (5%) patients, while there was no change in the seizure frequency of the other patients. A 50% long-term responder rate was apparent in 19 patients (43%), with two (5%) free from seizures for more than six months and in nine patients (20%, with one [2 %] free from seizures) for more than 12 months. Treatment-emergent adverse events were predominantly of psychobehavioural nature and were observed in 16%.Significance: In this retrospective analysis the rate of patients with a 50% seizure reduction under BRV proofed to be similar to those seen in regulatory trials for focal epilepsies. BRV appears to be safe and relatively well tolerated in EE and might be considered in patients with psychobehavioral adverse events while on levetiracetam.

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Section: Introductionmentioning

confidence: 99%

“…The majority of EE patients develop refractory epilepsies and suffer from relapsing seizures of heterogeneous semiologies. Frequent hospitalization associated with the need for extended medical and nursing care place major social, interpersonal, and economic burden on patients, caregivers and society ( 14 – 18 ).…”

Section: Introductionmentioning

confidence: 99%

Efficacy, Retention, and Tolerability of Brivaracetam in Patients With Epileptic Encephalopathies: A Multicenter Cohort Study From Germany

et al. 2018

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“…Pgp function was first found to be inhibited by Ca 2+ -channel blocker verapamil and hence it has been used to tackle drug resistance epilepsy [61,62]. According to a few clinical case reports, patients not responding to various combination treatments of drug showed significant reduction of seizures when verapamil was coadministered with antiepileptic drugs [63,64]. Previous studies have reported that verapamil was used in patients with refractory epilepsy and showed effects by inhibiting Pgp [65,66].…”

Section: Direct Pgp Inhibitionmentioning

confidence: 99%

Novel therapeutic targets for epilepsy intervention

Kambli

Bhatt

Oza

et al. 2017

Seizure

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Epilepsy is a common neurological disorder involving recurrent seizures and affecting about 1% of the population worldwide. Despite several antiepileptic drugs and effective therapies available for epilepsy, about 25% of the patients show therapeutic failure. Thus there exists an unmet need for newer antiepileptic drugs targeting newer targets with different mechanisms of action. Current research in epilepsy generally focuses on mechanisms that control neuronal excitability. Recently attention has been focussed on novel targets, their various interactions, and signalling cascades relating to epilepsy. This review summarizes several experimental and clinical findings from literature and explores potential targets which may play crucial role in epilepsy.

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“…Кроме того, описано существование не-скольких аллельных вариантов ЭЭ, возникающих при мутациях в одном и том же гене. Так, например, мутации в гене SCN1A могут приводить к возникно- вению не только cиндрома Драве, но и фебрильных судорог 3А-типа, гемиплегической мигрени и эпи-лепсии из группы генерализованных эпилепсий с фебрильными судорогами плюс [19]. C другой сто-роны, у больных с клиническими проявлениями синдрома Драве обнаруживаются мутации и в дру-гих генах: SСN9A, PCDH19, GABRG2.…”

Section: наследственные варианты ээunclassified