Among children and adults with the Lennox-Gastaut syndrome, the addition of cannabidiol at a dose of 10 mg or 20 mg per kilogram per day to a conventional antiepileptic regimen resulted in greater reductions in the frequency of drop seizures than placebo. Adverse events with cannabidiol included elevated liver aminotransferase concentrations. (Funded by GW Pharmaceuticals; GWPCARE3 ClinicalTrials.gov number, NCT02224560 .).

SummaryKetogenic dietary therapies (KDTs) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDTs were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, pre‐KDT counseling and evaluation, diet choice and attributes, implementation, supplementation, follow‐up, side events, and KDT discontinuation. It has been helpful in outlining a state‐of‐the‐art protocol, standardizing KDT for multicenter clinical trials, and identifying areas of controversy and uncertainty for future research. Now one decade later, the organizers and authors of this guideline present a revised version with additional authors, in order to include recent research, especially regarding other dietary treatments, clarifying indications for use, side effects during initiation and ongoing use, value of supplements, and methods of KDT discontinuation. In addition, authors completed a survey of their institution's practices, which was compared to responses from the original consensus survey, to show trends in management over the last 10 years.

SUMMARYThe ketogenic diet (KD) is an established, effective nonpharmacologic treatment for intractable childhood epilepsy. The KD is provided differently throughout the world, with occasionally significant variations in its administration. There exists a need for more standardized protocols and management recommendations for clinical and research use. In December 2006, The Charlie Foundation commissioned a panel comprised of 26 pediatric epileptologists and dietitians from nine countries with particular expertise using the KD. This group was created in order to create a consensus statement regarding the clinical management of the KD. Subsequently endorsed by the Practice Committee of the Child Neurology Society, this resultant manuscript addresses issues such as patient selection, pre-KD counseling and evaluation, specific dietary therapy selection, implementation, supplementation, follow-up management, adverse event monitoring, and eventual KD discontinuation. This paper highlights recommendations based on best evidence, including areas of agreement and controversy, unanswered questions, and future research.

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The goal of this paper is to describe epilepsy syndromes that begin in childhood (age 2-12 years). Additional syndromes that have a variable age at onset, including in childhood, are described in the paper on epilepsy syndromes with onset at a variable age. 1 The childhood onset syndromes can be broadly divided into three main groups: (1) self-limited focal epilepsies (SeLFEs); (2) generalized epilepsy syndromes, which are thought to have a genetic basis; and (3) developmental and/or epileptic encephalopathies (DEEs), which often have both focal and generalized seizures, including Lennox-Gastaut syndrome (LGS), developmental epileptic encephalopathy with spike-and-wave activation in sleep (DEE-SWAS), and epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS), or may have generalized seizures alone, such as epilepsy with myoclonic atonic seizures (EMAtS), or focal/multifocal seizures alone, such as hemiconvulsion-hemiplegia-epilepsy syndrome (HHE) and febrile infection-related epilepsy syndrome (FIRES).Childhood is also the typical age of onset of childhood absence epilepsy (CAE); this syndrome is covered in a separate paper on the idiopathic generalized epilepsy (IGE) syndromes. 2