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Classification of The Epilepsies
Published in Carl L. Faingold, Gerhard H. Fromm, Drugs for Control of Epilepsy:, 2019
Henri Gastaut, Benjamin G. Zifkin
Many specific progressive disorders may give rise to secondary generalized epilepsy. In their early stages, they can be confused with the nonspecific Lennox-Gastaut syndrome or even with a benign, primary generalized epilepsy. These progressive encephalopathies include the ceroid lipofuscinoses, especially infantile Bielschowsky-Jansky disease; and Lafora disease and subacute sclerosing panencephalitis, found in the older child and adolescent. These are discussed in Appendix II of the 1985 ILAE proposal.21
Occupational Therapy and Epilepsy
Published in Jerry A. Johnson, David A. Ethridge, Developmental Disabilities: A Handbook for Occupational Therapists, 2013
Severe childhood epileptic encephalopathy characterized by a variety of intractable seizure forms including absence and drop attacks, associated with mental retardation, and having an irregular, slow, spike and wave EEG pattern has been described by a large number of seizure combination descriptions. This syndrome is now commonly called Lennox-Gastaut syndrome.
Epilepsy and Sleep Disorders
Published in John W. Scadding, Nicholas A. Losseff, Clinical Neurology, 2011
The Lennox-Gastaut syndrome is characterized by multiple seizure types including tonic and atonic seizures and complex absences. Tonic–clonic convulsions and myoclonic seizures may also occur.
Thalamic neuromodulation in epilepsy: A primer for emerging circuit-based therapies
Published in Expert Review of Neurotherapeutics, 2023
Bryan Zheng, David D. Liu, Brian B Theyel, Hael Abdulrazeq, Anna R. Kimata, Peter M Lauro, Wael F. Asaad
While thalamic involvement in focal epilepsies may be regarded as secondary to cortical pathology, the thalamus may in fact be a central node in primary generalized epilepsies. In contrast to relay or ‘core’ nuclei, primary generalized epilepsies have uniquely implicated the intralaminar nuclei, particularly the CMT. Epileptiform activity in the CMT was observed during generalized seizures in adults and children with Lennox-Gastaut Syndrome (LGS)[130–132]. Intraoperative iEEG recordings from eight Lennox-Gastaut patients found 86% of generalized paroxysmal fast activity events were seen at both the site of cortical onset and the CMT. Furthermore, activity in multiple frequency bands appeared to propagate from cortex to thalamus during periods of ictal activity, implying propagation of seizures through the thalamus[132]. However, a recent case series suggested that the CMT may in fact lead ictogenic activity in cortex in generalized epilepsy and that the nucleus itself has independent ictal discharge[133].
Fenfluramine hydrochloride for the treatment of Dravet syndrome
Published in Expert Opinion on Orphan Drugs, 2020
Blandine Dozières-Puyravel, Stéphane Auvin
The most intriguing question is that fenfluramine may have a special effect in DS compared to any other epilepsy syndrome. It seems that the MoA of fenfluramine is multiple, acting through 5-HT1D, 5-HT2C, and σ1 receptors and through a reduction in dopamine and noradrenaline levels [25]. The effect of fenfluramine on σ1 receptors has been further established by a recent radioligand binding and cellular function study [29]. However, a significant decrease in serotonin levels in the heads of homozygous scn1Lab(-/-) mutants compared to those in wild-type zebrafish was found, suggesting that neurochemical defects might play a crucial role in the pathophysiology of DS. Fenfluramine might then act with particular efficacy in DS due to the special neurobiological state in this syndrome. Recent data in another epilepsy syndrome, Lennox-Gastaut syndrome, found a positive effect of fenfluramine in a smaller extent than in DS [33,34]. It is still insufficient to conclude on the special effect in DS. Lennox-Gastaut syndrome is a heterogeneous syndrome with various underlying etiologies and levels of severity in the electroclinical phenotypes. We need to wait for future trials evaluating fenfluramine in the treatment of homogeneous epilepsy syndrome to conclude on the special effect of fenfluramine in DS.
An evaluation of clobazam tablets and film (AQST-120) for the treatment of Lennox-Gastaut syndrome
Published in Expert Opinion on Pharmacotherapy, 2019
Frank M.C. Besag, Michael J. Vasey
Lennox-Gastaut syndrome (LGS) is an age-specific, chronic epileptic encephalopathy characterized by the triad of 1) multiple seizure types, primarily tonic, atonic and atypical absences 2) abnormal electroencephalogram (EEG) with waking diffuse slow (2–2.5 Hz) spike-wave patterns and paroxysmal fast rhythms during sleep, and 3) cognitive and behavioral impairment and/or regression [1]. Onset is typically before the age of 8 years, most commonly between the ages of 3 and 5 years [1]. Incidence is estimated to be between 1% and 10% of childhood epilepsies [2]. European studies estimate a prevalence of 0.1–0.28 per 1000 population [3] with boys more affected than girls (relative risk 5.31) [4]. Diagnosis is complicated by heterogeneous clinical presentations: not all patients will show the indicative seizure types at onset nor is the characteristic EEG itself pathognomonic [5]. Etiology is also diverse: genetic mutation, developmental abnormality, pre-, peri- or neo-natal brain insult, infection and tumor may all be implicated [5,6]. Approximately one-fifth of cases progress from West syndrome [2], an epileptic syndrome with onset in infancy or early childhood, typically during the first year, characterized by the clinical triad of clustered spasms, hypsarrhythmic EEG, and developmental delay or regression [7]. Cause is undetermined in 25–33% of LGS cases [6]. After seizure onset, developmental impairment or regression is seen in most patients: autistic traits and other behavioral and/or cognitive disabilities may be present in more than 50% of cases [8,9].