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Status epilepticus in the immature brain induces neuronal injury in the hippocampal formation, but the mode and mechanism of death are poorly understood. Our laboratory has recently investigated the role of caspase‐3, ‐8, and ‐9 in neuronal injury, using a lithium–pilocarpine model of status epilepticus in 2‐week‐old rat pups. Our results showed that dying neurons in the dentate gyrus and CA1‐subiculum...
We used the method of rapid hippocampal kindling to assess the potential antiepileptogenic efficacy of a number of anticonvulsant medications. This method afforded a higher throughput than methods based on traditional kindling or post–status epilepticus models of epileptogenesis. This “compressed epileptogenesis” model also permitted the study of age‐dependent pharmacologic targets, and distinguished...
Infantile spasms (IS) is a devastating epilepsy syndrome of childhood. IS occurs in 3–12‐month‐old infants and is characterized by spasms, interictal electroencephalography (EEG) hypsarrhythmia, and profound mental retardation. Hormonal therapy [adrenocorticotropic hormone (ACTH), corticosteroids] is frequently used, but its efficacy is tainted by severe side effects. For research of novel therapies,...
Neurosteroids such as allopregnanolone (THP) act as positive allosteric modulators of γ‐aminobutyric acid (GABA)A receptors and have exerted anticonvulsant properties. However, their role in the regulation of epileptogenesis is unclear. It has been shown that circulating levels of THP fluctuate during development and seizure episodes. Furthermore, both chronic administration of THP and its withdrawal...
Purpose: Epilepsy surgery in young children with focal lesions offers a unique opportunity to study the impact of severe seizures on cognitive development during a period of maximal brain plasticity, if immediate control can be obtained. We studied 11 children with early refractory epilepsy (median onset, 7.5 months) due to focal lesion who were rendered seizure‐free after surgery performed before...
Purpose: To elucidate the abnormality of interictal regional cerebral blood flow (rCBF) of West syndrome at the onset.Methods: Quantitative measurement of rCBF with an autoradiography method using N‐isopropyl‐(123I) p‐iodoamphetamine single photon emission computed tomography (SPECT) was performed on 14 infants with cryptogenic West syndrome. Regions of interest (ROIs) for rCBF were placed automatically...
Purpose: Activation of ionotropic γ‐aminobutyric acid type A (GABAA) receptors induces in immature neocortical neurons a membrane depolarization that may contribute to the higher epilepsy susceptibility in newborns. To elucidate whether depolarizing GABAergic responses enhance or attenuate epileptiform activity in the immature neocortex, we investigated the effect of agonists, antagonists, and positive...
Purpose: Expression of the protein subunits that make up the γ‐aminobutyric acid (GABA)A receptor pentamer is known to change during postnatal brain development in animal models. In the present study, analysis of cortical GABAA subunit expression was performed in control human tissue obtained from infancy through adolescence, and was compared to that from similarly aged children with intractable...
Although epilepsy is defined by the occurrence of spontaneous epileptic seizures, a large body of evidence indicates that epilepsy is linked to a spectrum of behavioral, psychiatric, and cognitive disorders as well as to sudden death. Explanations for these associations include the following: (1) The effects of structural lesions that may impair the functions subserved by the regions of the brain...
Slowing of cognitive skills represents one of the diagnostic criteria of Dravet syndrome. This Italian multicentric study aims at clarifying the roles of epilepsy and/or underlying genetic alteration in determining the cognitive outcome. The study includes infants that were either in follow‐up (retrospective study: 26 cases) and newly diagnosed (prospective study: in progress). Our multicentric study...
In view of previous reports of changes in seizure susceptibility in adult rats exposed to phenobarbital or diazepam as pups, we examined the effects of early life exposure to lamotrigine and phenytoin, two commonly used antiepileptic drugs (AEDs), for their effect on seizure threshold in adult rats. We found that pups exposed to lamotrigine for 6 days during the second postnatal week had a significantly...
The induction of neuronal apoptosis throughout many regions of the developing rat brain by phenobarbital and phenytoin, two drugs commonly used for the treatment of neonatal seizures, has been well documented. However, several limbic regions have not been included in previous analyses. Because drug‐induced damage to limbic brain regions in infancy could contribute to emotional and psychiatric sequelae,...
Purpose: Despite the consistent observation that γ‐aminobutyric acid A (GABAA) receptors mediate excitatory responses at perinatal stages, the role of the GABAergic system in the generation of neonatal epileptiform activity remains controversial. Therefore, we analyzed whether tonic and phasic GABAergic transmission had differential effects on neuronal excitability during early development.
Methods: ...
Purpose: Febrile seizures (FS) are the most common seizure type in children between the age of 6 months and 5 years. Although FS are largely benign, recurrent FS are a major risk factor for developing temporal lobe epilepsy (TLE) later in life. The mechanisms underlying FS are largely unknown; however, family and twin studies indicate that FS susceptibility is under complex genetic control. We have...
PurposeTo evaluate the seizure control and developmental outcomes after hemispherotomy for refractory epilepsy in childhood and to identify their predictive factors.
MethodsWe retrospectively studied the clinical courses and outcomes of 52 children with refractory epilepsy who underwent hemispherotomy in the Epilepsy Center Freiburg between 2002 and 2011.
Key FindingsMean age at epilepsy onset was...
PurposeDelayed diagnosis of early onset epilepsy is a potentially important and avoidable complication in epilepsy care. We examined the frequency of diagnostic delays in young children with newly presenting epilepsy, their developmental impact, and reasons for delays.
MethodsChildren who developed epilepsy before their third birthday were identified in a prospective community‐based cohort. An interval...
ObjectiveTo investigate the safety/tolerability and efficacy of long‐term adjunctive zonisamide and its impact on growth and development in children (6–18 years) with partial epilepsy.
MethodsOpen‐label extension of a phase III, placebo‐controlled trial. Started with double‐blind transition period (2–11 weeks), during which patients on zonisamide continued at the same dose and those on placebo switched...
Cognitive and developmental outcomes in patients with epileptic encephalopathy are hypothesized to result from an interplay between the underlying epileptic pathologic substrate and the acquired consequences of frequent and repetitive seizures and epileptiform discharges that often straddle the interictal and ictal boundaries. This article briefly reviews the evidence related to this assumption, presents...
Objective
Absence seizures in childhood absence epilepsy are initiated in the thalamocortical (TC) system. We investigated if these seizures result from altered development of the TC system before the appearance of seizures in mice containing a point mutation in γ‐aminobutyric acid A (GABAA) receptor γ2 subunits linked to childhood absence epilepsy (R43Q). Findings from conditional mutant mice indicate...
Objective
In 2011, the International League Against Epilepsy (ILAE) proposed a consensus classification system of focal cortical dysplasia (FCD) to distinguish clinicopathological subtypes, for example, “isolated” FCD type Ia–c and IIa–b, versus “associated” FCD type IIIa–d. The histopathological differentiation of FCD type I and III variants remains, however, a challenging issue in everyday practice...
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