Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy that shares many features and characteristics of other treatment-resistant childhood epilepsies. Accurate and early diagnosis is essential to both prognosis and overall patient management. However, accurate diagnosis of LGS can be clinically challenging. This article summarizes key characteristics of LGS and areas of overlap with other childhood epilepsies. Drawing upon input from a committee of established LGS experts convened in June 2012 in Chicago, Illinois, the authors highlight key diagnostic tests for making the differential diagnosis and propose a diagnostic scheme for people with suspected LGS.
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http://dx.doi.org/10.1111/epi.12567 | DOI Listing |
Can J Hosp Pharm
January 2025
BMSc, MD, FRCPC, is with the Department of Critical Care Medicine, Alberta Health Services, and the Departments of Critical Care Medicine and Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Calgary, Alberta.
Epilepsy Res
January 2025
Jane and John Justin Institute for Mind Health, Cook Children's Medical Center, Ft Worth, TX, USA.
Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy characterized by multiple drug-resistant seizure types, cognitive impairment, and distinctive electroencephalographic patterns. Neuromodulation techniques, including vagus nerve stimulation (VNS), deep brain stimulation (DBS), and responsive neurostimulation (RNS), have emerged as important treatment options for patients with LGS who do not respond adequately to antiseizure medications. This review, developed with input from the Pediatric Epilepsy Research Consortium (PERC) LGS Special Interest Group, provides practical guidance for clinicians on the use of these neuromodulation approaches in patients with LGS.
View Article and Find Full Text PDFJ Health Econ Outcomes Res
December 2024
Jazz Pharmaceuticals UK Ltd., London, UK.
Expert Rev Neurother
January 2025
Department of Paediatrics, Cambridge University Hospitals, Cambridge, UK.
Introduction: The seizures in Lennox-Gastaut syndrome are typically resistant to treatment. Seven antiseizure medications (ASMs) in the US (six in the UK/EU) are licensed for the treatment of seizures in LGS: lamotrigine, topiramate, rufinamide, clobazam, felbamate (not licensed in the UK/EU), cannabidiol and fenfluramine. Other options include neurostimulation, corpus callosotomy and dietary therapies, principally the ketogenic diet and its variants.
View Article and Find Full Text PDFSeizure
December 2024
Division of Neurology, Saitama Children's Medical Center, 1-2, Shintoshin, Chuo-ku, Saitama, Japan. Electronic address:
Purpose: Infantile epileptic spasms syndrome (IESS) often has a severe neurodevelopmental prognosis. However, few studies have examined the aspect of elementary school enrollment. This study evaluated elementary school enrollment after adrenocorticotropic hormone (ACTH) therapy in patients with IESS.
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