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Favorable response to ketogenic diet therapy in a patient with -related epilepsy.
Epilepsy Behav Rep
March 2025
Section of Pediatric Neurology, Department of Pediatrics, The University of Chicago, Chicago, IL, United States.
Dynein Cytoplasmic 1 Heavy chain 1 (-related disorders are a spectrum of conditions including neurodevelopmental disorders, congenital brain malformations, and neuromuscular diseases. These clinical features may co-occur, with four main disease entities including epilepsy with developmental epileptic encephalopathy such as infantile epileptic spasms syndrome (IESS) and Lennox-Gastaut syndrome (LGS), axonal Charcot-Marie-Tooth disease type 2O, spinal muscular atrophy with lower extremity-predominance (SMALED), and congenital cortical malformations. Epilepsy associated with this disorder often becomes drug-resistant and requires multiple medications and, in some cases, non-pharmacological treatments.
View Article and Find Full Text PDFFirst case report of effective and safe application of cannabidiol to treat concurrent ALG3-CDG and Lennox-Gastaut Syndrome.
Neurol Sci
January 2025
Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine, 211 Eonju-ro, Gangnam-gu, Seoul, 135-720, Korea.
This study presents the first reported case of a Korean patient with Alpha-1,3-Mannosyltransferase-Congenital Disorder of Glycosylation (ALG3-CDG), characterized by a novel maternally inherited missense mutation and a previously reported paternally inherited nonsense mutation. The patient exhibited typical ALG3-CDG manifestations, including developmental delays, epilepsy, and multisystem involvement, alongside a diagnosis of Lennox-Gastaut Syndrome (LGS). Cannabidiol therapy, combined with dietary management, led to seizure freedom for over 13 months, significant EEG improvement, and enhanced developmental outcomes.
View Article and Find Full Text PDFManagement of Status Epilepticus and Infections in a Patient with Lennox Gastaut Syndrome.
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.
Neuromodulation Strategies in Lennox-Gastaut Syndrome: Practical Clinical Guidance from the Pediatric Epilepsy Research Consortium.
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 PDFCost-Utility Analysis of Add-on Cannabidiol vs Usual Care Alone for the Treatment of Seizures in Patients With Treatment-Resistant Lennox-Gastaut Syndrome or Dravet Syndrome in the Netherlands.
J Health Econ Outcomes Res
December 2024
Jazz Pharmaceuticals UK Ltd., London, UK.
Article Synopsis
- Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS) are severe epilepsy types often seen in young children, and a CBD medication (Epidyolex®) has been reimbursed in the Netherlands since December 2022 to help treat their seizures.
- A cost-effectiveness analysis using a Markov model showed that using CBD along with usual care for LGS resulted in additional costs of €28,338 but provided increased quality-adjusted life-years (QALYs) of 1.318, making it cost-effective below the €80,000 threshold.
- For DS patients, CBD plus usual care showed a cost saving of €23,642 while increasing QAL
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