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Readable research linked to original sources
Browse normalized, publication-ready research with direct links to its evidence and source.
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Readable research linked to original sources
Browse normalized, publication-ready research with direct links to its evidence and source.
9 articles
Newest firstObjective This real-world, retrospective, multicenter study aims to investigate the effectiveness of highly purified cannabidiol (CBD) in a large cohort of patients with epilepsy of genetic etiology due to an identified monogenic cause. Additionally, we examine the potential relationship between specific genetic subgroups and treatment response. Methods This study was conducted across 27 epilepsy centers and inclu…
Open article record in new tab ↗Introduction Effectiveness and tolerability of plant-derived highly purified cannabidiol (CBD) in patients with Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), or tuberous sclerosis complex (TSC)-associated epilepsy in clinical practice in Germany were evaluated. Methods This multicenter, retrospective, chart review study analyzed patients with LGS, DS, or TSC-associated epilepsy receiving ≥ 1 dose of adjunct…
Background Highly purified Cannabidiol (CBD, epidiolex) is approved for treating Lennox-Gastaut syndrome (LGS), Dravet syndrome (DS), and tuberous sclerosis (TSC), with off-label use in drug-resistant epilepsies. The aim of this study is to evaluate efficacy, safety, and long-term retention of CBD in a large pediatric population. Methods We prospectively included all consecutive patients initiating CBD for drug-re…
Introduction Lennox-Gastaut syndrome (LGS) is a particularly severe developmental epileptic encephalopathy (DEE) characterized by multiple types of drug-resistant, incapacitating seizures. Despite aggressive therapy including polypharmacy, surgery, implanted devices, and dietary therapy, the prognosis remains poor, with frequent ongoing seizures and risk of injury and early death. Cenobamate (CNB) is an antiseizur…
Open article record in new tab ↗This article is co-authored by a parent of a 32-year-old male patient with Lennox-Gastaut syndrome (LGS) and his epileptologist. It discusses the parent's experience of having a child with LGS from diagnosis through living day-to-day with the disease and the physician's perspective when treating this devastating epilepsy syndrome. The patient's mother, who is his legal representative, provided written consent for…
Open article record in new tab ↗The SH3 and multiple ankyrin repeat domains (SHANKs) are a family of scaffolding proteins located in excitatory synapses required for their development and function. Molecular defects of SHANK3 are a well-known cause of several neurodevelopmental entities, in particular autism spectrum disorders and epilepsy, whereas relatively little is known about disease associations of SHANK1. Here, we propose a novel de novo…
Open article record in new tab ↗Background and Aim: Data on the clinical pharmacokinetics of cannabidiol (CBD) are scanty. We explored the effect of demographic and clinical variables on plasma concentrations of purified CBD in patients with Dravet (DS) and Lennox-Gastaut syndrome (LGS). Methods: The study design was an open, prospective, multicenter expanded access program (EAP). Venous blood samples were drawn from patients between 8 and 9 am,…
In recent years, cannabis has been gaining increasing interest in both the medical research and clinical fields, with regard to its therapeutic effects in various disorders. One of the major fields of interest is its role as an anticonvulsant for refractory epilepsy, especially in the pediatric population. This paper presents and discusses the current accumulated knowledge regarding artisanal cannabis and Epidiole…
Open article record in new tab ↗The Lennox-Gastaut syndrome is a devastating early-onset epileptic encephalopathy, associated with severe behavioural abnormalities. Its pathophysiology, however, is largely unknown. A de novo mutation (c.G358A, p.D120N) in the human GABA type-A receptor β3 subunit gene (GABRB3) has been identified in a patient with Lennox-Gastaut syndrome. To determine whether the mutation causes Lennox-Gastaut syndrome in vivo i…
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