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Lennox‐Gastaut syndrome (LGS) has numerous causes, but only rarely has familial recurrence been observed. We studied a family in which three male members had severe epilepsy and intellectual disability. The proband had seizure onset at 7 years of age with atonic, myoclonic, atypical absence, and tonic seizures with slow spike‐wave on electroencephalography (EEG). One living sibling had a similar clinical...
Purpose: Ohtahara syndrome is one of the most severe and earliest forms of epilepsy and is frequently associated with brain malformations, such as hemimegalencephaly. Recently, longer expansion of the first polyalanine tract of ARX was found to be causative for Ohtahara syndrome without brain malformation, whereas premature termination mutations of ARX were found to cause severe brain malformations,...
Lennox‐Gastaut syndrome (LGS) is a rare epileptic encephalopathy with a peak age of onset of 3–5 years of age. Reported prevalence rates for LGS vary widely from 1–10% of all childhood epilepsies. Incidence rates are much lower. LGS is characterized by intractable, multiple, generalized seizure types and an interictal electroencephalogram showing bursts of slow spike‐and‐wave, paroxysmal bursts of...
Lennox‐Gastaut syndrome (LGS) is an intractable childhood‐onset epileptic encephalopathy. Seizure freedom is rare in LGS. One of the hallmarks of LGS is medical intractability, with generally poor response to antiepileptic drugs (AEDs). Nevertheless, several treatment options are available that can mitigate the severity of seizures and curtail their frequency. New AEDs have been validated in randomized,...
Purpose: Even if etiologies of Lennox‐Gastaut syndrome (LGS) are diverse, the multiple causes converge into a final common pathway that results in this specific epilepsy phenotype. There is little knowledge, however, about neuronal networks that may be a part of this pathway.
Methods: To investigate these networks, 11 children with LGS and 9 control children with multifocal epileptic activity were...
Purpose: We used transcranial magnetic stimulation (TMS) to investigate cortical excitability changes in Lennox‐Gastaut syndrome (LGS), anticipating we would find a marked increase in excitability compared to other patients with refractory epilepsies.
Methods: Eighteen patients with LGS were studied. Motor threshold (MT), short intracortical inhibition (paired pulse TMS at 2 and 5 msec interstimulus...
Epileptic encephalopathies (EEs) represent a group of severe epileptic disorders associated with cognitive and behavioral disturbances. The mechanisms of cognitive disability in EEs remain unclear. This review summarized neuroimaging studies that have tried to describe specific fingerprints of brain activation in EE. Although the epileptic activity can be generated individually in different brain...
The pathophysiology of epileptic encephalopathies has long been debated. Recently, some authors proposed the new concept of so‐called system epilepsies. This hypothesis postulates that system epilepsies are produced by the enduring propensity to generate seizures in different cerebral areas that, alone, are unable to create a specific electroclinical phenotype. This goes beyond the classical dichotomy...
PurposeLennox‐Gastaut syndrome (LGS) is a severe epileptic disorder with characteristic electroclinical features but diverse etiologies. The shared electroclinical characteristics suggest that common cerebral networks are involved in generating seizures. We sought to reveal these networks by comparing ictal and interictal single‐photon emission computed tomography (SPECT).
MethodsWe identified 10...
ObjectiveLennox‐Gastaut syndrome (LGS) is a severe epilepsy phenotype with characteristic electroclinical features despite diverse etiologies. We previously found common cerebral networks involved during slow spike‐and‐wave (SSW) and generalized paroxysmal fast activity (PFA), characteristic interictal discharges. Some patients have a Lennox‐Gastaut–like phenotype and cortical lesions. We wished to...
Lennox‐Gastaut syndrome (LGS) is a drug‐resistant epileptic encephalopathy of childhood with a heterogeneous etiology. Recently, genome‐wide association studies have led to the identification of new de novo mutations associated with this epileptic syndrome. Herein, we report an 8‐year‐old child with intellectual disability, severe postnatal microcephaly, Rett‐like features, and LGS, carrying a de...
ObjectiveLennox‐Gastaut syndrome, and the similar but less tightly defined Lennox‐Gastaut phenotype, describe patients with severe epilepsy, generalized epileptic discharges, and variable intellectual disability. Our previous functional neuroimaging studies suggest that abnormal diffuse association network activity underlies the epileptic discharges of this clinical phenotype. Herein we use a data‐driven...
The term epileptic encephalopathy (EE) denotes a process by which epileptic activity adversely affects brain function over and above the underlying etiology. Underlying mechanisms are poorly understood, but recent studies demonstrate that seizures and interictal epileptiform discharges can disrupt distributed neural networks that underpin cognitive functions, both temporarily and permanently. EE is...
ObjectiveIn patients with Lennox‐Gastaut syndrome (LGS), recurrent epileptic activity is thought to contribute to impaired cognition (epileptic encephalopathy). Using concurrent electroencephalography‐functional magnetic resonance imaging (EEG‐fMRI), we recently showed that epileptiform discharges in LGS recruit large‐scale networks that normally support key cognitive processes. In LGS, given that...
Drop seizures are especially problematic in patients with Lennox‐Gastaut syndrome (LGS) because of their potential for serious injury. In this post hoc analysis of phase 3 OV‐1012 data, a medical review was conducted of seizure‐related injuries based on Medical Dictionary for Regulatory Activities (MedDRA) preferred terms from all adverse event (AE) listings. Patients receiving clobazam experienced...
Distinguishing adult patients with Lennox‐Gastaut syndrome from those with Dravet syndrome is challenging. We have previously reported that patients with Dravet syndrome present a very peculiar motor phenotype. Here we sought to confirm that this association was not linked to the chronic use of antiepileptic drugs or the many lifetime seizures. To this aim, we studied 14 adult patients with Lennox‐Gastaut...
We previously observed that adults with Lennox‐Gastaut syndrome (LGS) show abnormal functional connectivity among cognitive networks, suggesting that this may contribute to impaired cognition. Herein we report network reorganization following seizure remission in a child with LGS who underwent functional magnetic resonance imaging (fMRI) before and after resection of a cortical dysplasia. Concurrent...
Objective
Lennox‐Gastaut syndrome (LGS) is a drug‐resistant, childhood onset electroclinical epilepsy syndrome with multiple seizure types and diagnostic electroencephalogram findings. ZX008 (fenfluramine HCl oral solution) was well tolerated and reduced seizure frequency in Dravet syndrome, prompting this phase 2, open‐label, dose‐finding study of add‐on ZX008 in patients with LGS (NCT02655198)...
Objective
Developmental epileptic encephalopathies (DEEs) are genetically heterogeneous severe childhood‐onset epilepsies with developmental delay or cognitive deficits. In this study, we explored the pathogenic mechanisms of DEE‐associated de novo mutations in the CACNA1A gene.
Methods
We studied the functional impact of four de novo DEE‐associated CACNA1A mutations, including the previously described...
Objective
To evaluate the potential impact of concomitant clobazam (CLB) use on the efficacy of cannabidiol (CBD) treatment in patients with Dravet syndrome and Lennox‐Gastaut syndrome using meta‐analytical techniques.
Methods
We searched for randomized, placebo‐controlled, single‐ or double‐blinded trials. The proportion of patients who achieved ≥50% reduction from baseline in seizure frequency...
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