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المؤلفون: A. Pillai, J. V. Pattisapu, A. G. Diwan, K. Radhakrishnan, S. Gopinath
المصدر: Эпилепсия и пароксизмальные состояния, Vol 13, Iss 3, Pp 249-253 (2021)
مصطلحات موضوعية: medicine.medical_specialty, business.industry, Audiology, medicine.disease, Corpus callosum, lennox–gastaut syndrome, Intracranial Electroencephalography, corpus callosum, epileptic encephalopathy, nervous system, Neurology, Generalization (learning), callosotomy, medicine, Ictal, Neurology. Diseases of the nervous system, Neurology (clinical), RC346-429, business, Lennox–Gastaut syndrome
الوصف: Lennox–Gastaut syndrome (LGS) is an epileptic encephalopathy characterized by delayed mental development and intractable multiple seizure types, predominantly tonic. Drop attacks are the commonest and the most disabling type of seizures. Resective surgery is often not possible in LGS as the electroencephalogram (EEG) abnormalities are usually multifocal and generalized, and magnetic resonance image is often either normal or multilesional. We report a case of LGS with bilateral parieto-occipital gliosis where EEG before and after callosotomy demonstrated synchronized bilateral interictal epileptiform discharges and ictal discharges becoming desynchronized and running down. This phenomenon emphasizes the role of the corpus callosum in secondary bilateral synchrony.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5b97497a07990756df4e1a1976a6a802
https://doi.org/10.17749/2077-8333/epi.par.con.2021.086 -
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المصدر: Seizure. 91:159-166
مصطلحات موضوعية: Pediatrics, medicine.medical_specialty, Prevalence, Rufinamide, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Health care, medicine, Humans, Medical prescription, Retrospective Studies, Lennox Gastaut Syndrome, business.industry, Mortality rate, General Medicine, Patient Acceptance of Health Care, medicine.disease, Neurology, Neurology (clinical), business, 030217 neurology & neurosurgery, Resource utilization, Cohort study, medicine.drug, Lennox–Gastaut syndrome
الوصف: Purpose To retrospectively investigate the prevalence, demography, antiseizure medication (ASM) usage, healthcare resource utilization (HCRU), and mortality of patients with Lennox-Gastaut syndrome (LGS) in primary and secondary care in the UK. Methods Patients with confirmed LGS were anonymously identified from the UK Clinical Practice Research Datalink (CPRD) GOLD database (01/01/1987–31/1b0/2018) using the LGS Read Code (F250500). Probable LGS was identified using the International Classification of Diseases-10/Read Code for epilepsy (Hospital Episode Statistics [HES]/CPRD) plus rufinamide prescription. Period prevalence was calculated based on patients enrolled in CPRD GOLD and alive in 2017. CPRD data were linked to HES to calculate HCRU, and to the Office for National Statistics mortality registry. Results Period prevalence of LGS was 0.578/10,000 (n = 180), with 74 and 106 patients identified with confirmed (0.289/10,000) and probable LGS (0.420/10,000). Mean (max) ASM usage was ~1 (3) per year. In confirmed LGS, valproate (72%), lamotrigine (69%), and clobazam (66%) were the most commonly prescribed ASMs. HCRU (per patient-year) was similar in confirmed and probable LGS and mostly consisted of primary care general practitioner consultations (4–6), outpatient visits (5–10), inpatient admissions (1–4), and A&E visits (1). During the follow-up period, 18 patients died with crude mortality rates of 6.12 (confirmed LGS) and 4.17 (probable LGS) deaths per 1000 person-years. Conclusion Prevalence of LGS appears low in the UK. The similarly high HCRU and mortality rates in confirmed and probable LGS support the validity and specificity of the probable LGS algorithm and high burden of LGS.
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المؤلفون: Prateek Kumar Panda, Indar Kumar Sharawat, Lesa Dawman, Pragnya Panda
المصدر: Seizure. 91:296-307
مصطلحات موضوعية: medicine.medical_specialty, Lennox Gastaut Syndrome, business.industry, General Medicine, Rufinamide, Triazoles, Drug Resistant Epilepsy, Placebo, medicine.disease, Epilepsy, Neurology, Seizures, Sample size determination, Meta-analysis, Internal medicine, medicine, Humans, Anticonvulsants, Neurology (clinical), business, Adverse effect, Randomized Controlled Trials as Topic, medicine.drug, Lennox–Gastaut syndrome
الوصف: Rufinamide is an antiseizure medication that acts through sodium channels and is found to be efficacious in patients with Lennox Gastaut syndrome (LGS). However, no systematic review has been conducted in LGS patients to provide an estimate of the efficacy and safety of rufinamide.Different electronic databases were searched for articles describing the use of rufinamide in patients with LGS. For determining primary efficacy outcomes as compared to placebo, we included only studies comparing the efficacy of rufinamide with placebo in LGS patients. We performed an additional analysis to include other uncontrolled studies with a minimum sample size of 20 to provide a more comprehensive estimate of efficacy.A total of ten studies included 557 patients. Out of them, five studies were placebo-controlled, enrolling a total of 265 patients in the rufinamide group and 203 patients in the placebo group. The average percentage reduction in total seizure frequency per 28 days during the double-blind phase was 29.3% in the rufinamide group compared with 8.3% in the placebo group (difference between the two groups was 20.9%, 95%CI-14.4%-27.3%, p0.00001). Even for individual seizure types like tonic-clonic seizures, atypical absence seizures, atonic seizures, focal seizures, and myoclonic seizures, rufinamide was more efficacious than placebo(p0.00001). The number of patients with at least one treatment-emergent adverse effects was significantly higher in rufinamide treated patients (60.2%vs50.7%, p=0.02, RR-1.24(1.03,1.51).Rufinamide is efficacious as adjunctive therapy in patients with LGS in terms of reduction in total seizure frequency and has mild adverse reaction.
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المؤلفون: Elizabeth A. Thiele, Anup D. Patel, Orrin Devinsky, Daniel Checketts, Arie Weinstock, Wendy G. Mitchell, Antonio Gil-Nagel, Richard F.M. Chin, Michael Scott Perry, Maria Mazurkiewicz-Bełdzińska, Eduardo Dunayevich, Boudewijn Gunning, Jonathan J. Halford
المصدر: Epilepsia. 62:2228-2239
مصطلحات موضوعية: medicine.medical_specialty, Epilepsies, Myoclonic, law.invention, Randomized controlled trial, Seizures, law, Internal medicine, Convulsion, medicine, Cannabidiol, Humans, media_common.cataloged_instance, European union, media_common, Valproic Acid, Lennox Gastaut Syndrome, Maintenance dose, business.industry, medicine.disease, Neurology, Tolerability, Concomitant, Anticonvulsants, Neurology (clinical), medicine.symptom, business, medicine.drug, Lennox–Gastaut syndrome
الوصف: Objective Lennox-Gastaut syndrome (LGS) is an epileptic encephalopathy that is often treatment resistant. Efficacy and safety of add-on cannabidiol (CBD) to treat seizures associated with LGS was demonstrated in two randomized controlled trials (RCTs). Patients who completed the RCTs were invited to enroll in this long-term open-label extension (OLE) trial, GWPCARE5 (NCT02224573). We present the final analysis of safety and efficacy outcomes from GWPCARE5. Methods Patients received plant-derived highly purified CBD (Epidiolex in the United States; Epidyolex in the European Union; 100 mg/ml oral solution), titrated to a target maintenance dose of 20 mg/kg/day over 2 weeks. Based on response and tolerability, CBD could then be reduced or increased up to 30 mg/kg/day. Results Of 368 patients with LGS who completed the RCTs, 366 (99.5%) enrolled in this OLE. Median and mean treatment duration were 1090 and 826 days (range = 3-1421), respectively, with a mean modal dose of 24 mg/kg/day. Adverse events (AEs) occurred in 96% of patients, serious AEs in 42%, and AE-related discontinuations in 12%. Common AEs were convulsion (39%), diarrhea (38%), pyrexia (34%), and somnolence (29%). Fifty-five (15%) patients experienced liver transaminase elevations more than three times the upper limit of normal; 40 (73%) were taking concomitant valproic acid. Median percent reductions from baseline ranged 48%-71% for drop seizures and 48%-68% for total seizures through 156 weeks. Across all 12-week visit windows, 87% or more of patients/caregivers reported improvement in the patient's overall condition on the Subject/Caregiver Global Impression of Change scale. Significance Long-term add-on CBD treatment had a similar safety profile as in the original RCTs. Sustained reductions in drop and total seizure frequency were observed for up to 156 weeks, demonstrating long-term benefits of CBD treatment for patients with LGS.
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المؤلفون: Kyung-Ok Cho, Ja Un Moon
المصدر: International Neurourology Journal
International Neurourology Journal, Vol 25, Iss Suppl 1, Pp S8-18 (2021)مصطلحات موضوعية: 0301 basic medicine, Intractable epilepsy, medicine.medical_specialty, Ohtahara syndrome, Combination therapy, Lennox-Gastaut syndrome, Urology, Antiepileptic drugs, Review Article, Pediatric Disease, 03 medical and health sciences, 0302 clinical medicine, Dravet syndrome, medicine, In patient, Intensive care medicine, business.industry, Epileptic encephalopathy, medicine.disease, Diseases of the genitourinary system. Urology, 030104 developmental biology, Neurology, RC870-923, Neurology (clinical), business, 030217 neurology & neurosurgery, Lennox–Gastaut syndrome
الوصف: Epileptic encephalopathy (EE) is a devastating pediatric disease that features medically resistant seizures, which can contribute to global developmental delays. Despite technological advancements in genetics, the neurobiological mechanisms of EEs are not fully understood, leaving few therapeutic options for affected patients. In this review, we introduce the most common EEs in pediatrics (i.e., Ohtahara syndrome, Dravet syndrome, and Lennox-Gastaut syndrome) and their molecular mechanisms that cause excitation/inhibition imbalances. We then discuss some of the essential molecules that are frequently dysregulated in EEs. Specifically, we explore voltage-gated ion channels, synaptic transmission-related proteins, and ligand-gated ion channels in association with the pathophysiology of Ohtahara syndrome, Dravet syndrome, and Lennox-Gastaut syndrome. Finally, we review currently available antiepileptic drugs used to treat seizures in patients with EEs. Since these patients often fail to achieve seizure relief even with the combination therapy, further extensive research efforts to explore the involved molecular mechanisms will be required to develop new drugs for patients with intractable epilepsy.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::aebbcad6468f099199c9ee4f503de4ea
https://doi.org/10.5213/inj.2142166.083 -
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المؤلفون: J. Helen Cross, Matthew Wong, Daniel Checketts, Hari Bhathal, Elaine C. Wirrell, Maria Mazurkiewicz-Bełdzińska, Kevan E. VanLandingham, Michael Privitera, Eric D. Marsh, Volker Knappertz, Vicente Villanueva
المصدر: Epilepsia
r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe
instnameمصطلحات موضوعية: Adult, Male, 0301 basic medicine, Time Factors, Adolescent, Placebo, drop seizures, law.invention, Responder rate, Young Adult, 03 medical and health sciences, antiepileptic drug, 0302 clinical medicine, Double-Blind Method, Randomized controlled trial, Seizures, law, treatment‐resistant epilepsy, medicine, Cannabidiol, Humans, antiseizure medication, Treatment effect, Child, Adverse effect, Time to onset, Lennox Gastaut Syndrome, business.industry, Middle Aged, medicine.disease, resistant epilepsy, Treatment Outcome, 030104 developmental biology, Neurology, treatment‐, childhood onset epilepsy, Child, Preschool, Anesthesia, Full‐length Original Research, Anticonvulsants, Female, Neurology (clinical), business, 030217 neurology & neurosurgery, medicine.drug, Lennox–Gastaut syndrome
الوصف: Objective To estimate time to onset of cannabidiol (CBD) treatment effect (seizure reduction and adverse events [AEs]), we conducted post hoc analyses of data from two randomized, placebo-controlled, Phase 3 trials, GWPCARE3 (NCT02224560) and GWPCARE4 (NCT02224690), of patients with Lennox-Gastaut syndrome. Methods Patients received plant-derived pharmaceutical formulation of highly purified CBD (Epidiolex, 100 mg/ml oral solution) at 10 mg/kg/day (CBD10; GWPCARE3) or 20 mg/kg/day (CBD20; both trials) or placebo for 14 weeks. Treatment started at 2.5 mg/kg/day for all groups and reached 10 mg/kg/day on Day 7 and 20 mg/kg/day (CBD20 and matching placebo only) on Day 11. Percentage change from baseline in drop seizure frequency was calculated by cumulative day (i.e., including all previous days). Time to onset and resolution of AEs were evaluated. Results Overall, 235 patients received CBD (CBD10 [GWPCARE3 only], n = 67; CBD20 [pooled GWPCARE3&4], n = 168) and 161 received placebo. Mean (range) age was 15.3 years (2.6-48.0). Patients had previously discontinued a median (range) of six (0-28) antiepileptic drugs (AEDs) and were currently taking a median of three (0-5) AEDs. Differences in drop seizure reduction between placebo and CBD emerged during the titration period and became nominally significant by Day 6 (p = .008) for pooled CBD treatment groups. Separation between placebo and CBD in >= 50% responder rate emerged by Day 6. Onset of the first reported AE occurred during the titration period in 45% of patients (CBD10, 46%; CBD20, 52%; placebo, 38%). In patients with AEs, resolution occurred within 4 weeks of onset in 53% of placebo and 39% of CBD patients and by end of study in 63% of placebo and 61% of CBD patients. Significance Treatment effect (efficacy and AEs) of CBD may occur within 1 week of starting treatment. Although AEs lasted longer for CBD than placebo, most resolved within the 14-week period.
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المصدر: Journal of Child Neurology. 36:752-759
مصطلحات موضوعية: Male, Pediatrics, medicine.medical_specialty, genetic structures, Lennox Gastaut Syndrome, business.industry, Epileptic encephalopathy, Infant, Electroencephalography, medicine.disease, 03 medical and health sciences, 0302 clinical medicine, Pediatrics, Perinatology and Child Health, Disease Progression, medicine, Etiology, Humans, Female, 030212 general & internal medicine, Neurology (clinical), business, Spasms, Infantile, 030217 neurology & neurosurgery, Retrospective Studies, Lennox–Gastaut syndrome
الوصف: Objective:Children with infantile spasms may develop Lennox-Gastaut syndrome. The diagnostic criteria for Lennox-Gastaut syndrome are vague, and many experts use varying combinations of the following criteria for diagnosis: paroxysmal fast activity on electroencephalography (EEG), slow spike and wave on EEG, developmental delay, multiple seizure types, and nocturnal tonic seizures. Our objective was to determine the prevalence of Lennox-Gastaut syndrome in a high-risk cohort of children with a history of infantile spasms and the characteristics of infantile spasms that were associated with the diagnosis of Lennox-Gastaut syndrome.Methods:Children with infantile spasms who were diagnosed and treated at Children’s Hospital Colorado between 2012 and 2018 were included. Lennox-Gastaut syndrome was defined as having 3 of 5 of the following characteristics: paroxysmal fast activity, slow spike and wave, current developmental delay, multiple seizure types, or tonic seizures. Descriptive statistics were performed using median and interquartile range. Univariable analysis was performed with Pearson chi-square, Fisher exact, or the Kruskal-Wallis test.Results:Ninety-seven children met inclusion criteria, and 36% (35/97) met criteria for Lennox-Gastaut syndrome. Developmental delay and history of seizures prior to the onset of infantile spasms were identified as risk factors for the development of Lennox-Gastaut syndrome ( P = .003) as was poor response to first treatment for spasms ( P = .004). Children with an unknown etiology of infantile spasms were less likely to develop Lennox-Gastaut syndrome ( P = .019). Eighty percent (28/35) of the children who met Lennox-Gastaut syndrome criteria lacked a documented diagnosis.Conclusions:Thirty-six percent of children with infantile spasms met criteria for Lennox-Gastaut syndrome. Risk factors for development of Lennox-Gastaut syndrome were developmental delay and seizures prior to the onset of infantile spasms and poor response to first treatment for infantile spasms. Children with an unknown etiology of infantile spasms were less likely to develop Lennox-Gastaut syndrome. Eighty percent of the children who met our criteria were not given a documented diagnosis of Lennox-Gastaut syndrome, which highlights the fact that many children may not be receiving a diagnosis of Lennox-Gastaut syndrome. We recommend establishing clear guidelines for the diagnosis of Lennox-Gastaut syndrome to ensure that the diagnosis is being made accurately.
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b7619a23f46e0d1b63700d7d6604d5c7
https://doi.org/10.1177/08830738211000514 -
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المؤلفون: Mauro Silvestrini, Simona Lattanzi, Pasquale Striano, Eugen Trinka, Francesco Brigo, Chiara Rocchi, Sergio Salvemini
المصدر: CNS Drugs
مصطلحات موضوعية: Pediatrics, medicine.medical_specialty, Clobazam, Anticonvulsants, Cannabidiol, Case-Control Studies, Cross-Sectional Studies, Double-Blind Method, Epilepsies, Myoclonic, Epilepsy, Epileptic Syndromes, Humans, Lennox Gastaut Syndrome, Seizures, Epilepsies, law.invention, 03 medical and health sciences, 0302 clinical medicine, Dravet syndrome, Randomized controlled trial, law, Medicine, media_common.cataloged_instance, Pharmacology (medical), European union, media_common, business.industry, medicine.disease, 030227 psychiatry, Clinical trial, Psychiatry and Mental health, Epilepsy syndromes, Systematic Review, Neurology (clinical), Myoclonic, business, 030217 neurology & neurosurgery, medicine.drug, Lennox–Gastaut syndrome
الوصف: Background Cannabidiol (CBD), which is one major constituent of the Cannabis sativa plant, has anti-seizure properties and does not produce euphoric or intrusive side effects. A plant-derived, highly purified CBD formulation with a known and constant composition has been approved by the US Food and Drug Administration for the treatment of seizures associated with Dravet syndrome, Lennox–Gastaut syndrome, and tuberous sclerosis complex. In the European Union, the drug has been authorized by the European Medicines Agency for the treatment of seizures associated with Dravet syndrome and Lennox–Gastaut syndrome, in conjunction with clobazam, and is under regulatory review for the treatment of seizures in patients with tuberous sclerosis complex. Objectives This systematic review aimed to summarize the currently available body of knowledge about the use of this US Food and Drug Administration/European Medicines Agency-approved oral formulation of pharmaceutical-grade CBD in patients with epileptic conditions, especially developmental and epileptic encephalopathies other than Dravet syndrome and Lennox–Gastaut syndrome. Methods The relevant studies were identified through MEDLINE and the US National Institutes of Health Clinical Trials Registry in October 2020. There were no date limitations or language restrictions. The following types of studies were included: clinical trials, cohorts, case-control, cross-sectional, clinical series, and case reports. Participants had to meet the following criteria: any sex, any ethnicity, any age, diagnosis of epilepsy, receiving plant-derived, highly purified (> 98% w/w) CBD in a sesame oil-based oral solution for the treatment of seizures. Data extracted from selected records included efficacy, tolerability, and safety outcomes. Results Five hundred and seventy records were identified by database and trial register searching. Fifty-seven studies were retrieved for detailed assessment, of which 42 were eventually included for the review. The participants of the studies included patients of both pediatric and adult age. Across the trials, purified CBD was administered at dosages up to 50 mg/kg/day. In a randomized double-blind controlled trial in patients with tuberous sclerosis complex, CBD was associated with a significantly greater percent reduction in seizure frequency than placebo over the treatment period. Open-label studies suggested the effectiveness of CBD in the treatment of children and adults presenting with other epilepsy syndromes than those addressed by regulatory trials, including CDKL5 deficiency disorder and Aicardi, Dup15q, and Doose syndromes, SYNGAP1 encephalopathy, and epilepsy with myoclonic absences. The most common adverse events observed during treatment with CBD included somnolence, decreased appetite, diarrhea, and increased serum aminotransferases. Conclusions The currently available data suggest that response to treatment with a highly purified, plant-derived CBD oil-based solution can be seen in patients across a broad range of epilepsy disorders and etiologies. The existing evidence can provide preliminary support for additional research. Supplementary Information The online version contains supplementary material available at 10.1007/s40263-021-00807-y.
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المؤلفون: Cecilie Johannessen Landmark, Heidrun Potschka, Elaine C. Wirrell, Jo M. Wilmshurst, Svein I. Johannessen, Stéphane Auvin, Dorothée G.A. Kasteleijn-Nolst Trenité
المصدر: Epilepsia
مصطلحات موضوعية: 0301 basic medicine, Drug, medicine.medical_specialty, media_common.quotation_subject, Drug repurposing, Epilepsies, Myoclonic, Context (language use), Dravet syndromes, Orphan drug, 03 medical and health sciences, 0302 clinical medicine, Dravet syndrome, Fenfluramine, Cannabidiol, Humans, Medicine, Everolimus, Precision Medicine, Intensive care medicine, media_common, Antiseizure medication, Lennox Gastaut Syndrome, business.industry, Seizure types, Drug Repositioning, Disease Management, medicine.disease, Drug repositioning, Treatment Outcome, 030104 developmental biology, Lennox–Gastaut syndromes, Neurology, Anticonvulsants, Neurology (clinical), business, 030217 neurology & neurosurgery, Lennox–Gastaut syndrome, medicine.drug
الوصف: Developmental and epileptic encephalopathies (DEEs) are among the most chal- lenging of all epilepsies to manage, given the exceedingly frequent and often severe seizure types, pharmacoresistance to conventional antiseizure medications, and nu- merous comorbidities. During the past decade, efforts have focused on development of new treatment options for DEEs, with several recently approved in the United States or Europe, including cannabidiol as an orphan drug in Dravet and Lennox– Gastaut syndromes and everolimus as a possible antiepileptogenic and precision drug for tuberous sclerosis complex, with its impact on the mammalian target of rapamycin pathway. Furthermore, fenfluramine, an old drug, was repurposed as a novel therapy in the treatment of Dravet syndrome. The evolution of new insights into pathophysi- ological processes of various DEEs provides possibilities to investigate novel and repurposed drugs and to place them into the context of their role in future manage- ment of these patients. The purpose of this review is to provide an overview of these new medical treatment options for the DEEs and to discuss the clinical implications of these results for improved treatment. C.J.L. has received an educational grant from GW Pharma and a speaker's honorarium from Eisai. H.P. has received research funding, consulting fees, and speaker's honoraria from Zogenix, Eisai, Bial, Roche, and MSD. S.A. has served as a consultant for or received honoraria for lectures from Arvelles, Eisai, GW Pharma, Lundbeck, Nutricia, UCB Pharma, Xenon, and Zogenix. He has been an investigator for clinical trials for Advicenne Pharma, Eisai, UCB Pharma, and Zogenix, and is an associate editor of Epilepsia. J.M.W. is an associate editor of Epilepsia. S.I.J. has no conflicts of interest to disclose. D.K.-N.T. has received research funding and consulting fees from UCB Pharma, GW Pharma, and Otsuka Pharma. E.C.W. has received consulting fees from Biocodex.
وصف الملف: application/pdf
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المؤلفون: Sandi Lam, Vineeth Thirunavu, Joyce Y. Wu, Anne T. Berg, Rebecca Y. Du
المصدر: Epilepsia. 62:888-907
مصطلحات موضوعية: 0301 basic medicine, medicine.medical_specialty, medicine.medical_treatment, 03 medical and health sciences, 0302 clinical medicine, Quality of life, medicine, Humans, Corpus callosotomy, Epilepsy surgery, Prospective Studies, Retrospective Studies, Lennox Gastaut Syndrome, business.industry, Disease Management, Retrospective cohort study, medicine.disease, Confidence interval, Psychosurgery, Surgery, 030104 developmental biology, Neurology, Meta-analysis, Neurology (clinical), business, 030217 neurology & neurosurgery, Vagus nerve stimulation, Lennox–Gastaut syndrome
الوصف: Lennox-Gastaut syndrome (LGS) is a severe form of childhood onset epilepsy in which patients require multiple medications and may be candidates for palliative surgical intervention. In this meta-analysis, we sought to evaluate the impact of palliative vagus nerve stimulation (VNS), corpus callosotomy (CC), and resective surgery (RS) by analyzing their impact on seizure control, antiepileptic drug (AED) usage, quality of life (QOL), behavior, cognition, prognostic factors, and complications. A systematic search of PubMed MEDLINE, Scopus, and Cochrane Database of Systematic Reviews was performed to find articles that met the following criteria: (1) prospective/retrospective study with original data, (2) at least one LGS surgery patient aged less than 18 years, and (3) information on seizure frequency reduction (measured as percentage, Engel class, or qualitative comment). Seizures were analyzed quantitatively in a meta-analysis of proportions and a random-effects model, whereas other outcomes were analyzed qualitatively. Forty studies with 892 LGS patients met the selection criteria, with 19 reporting on CC, 17 on VNS, four on RS, two on RS + CC, one on CC + VNS, and one on deep brain stimulation. CC seizure reduction rate was 74.1% (95% confidence interval [CI] = 64.5%-83.7%), and VNS was 54.6% (95% CI = 42.9%-66.3%), which was significantly different (p < .001). RS seizure reduction was 88.9% (95% CI = 66.1%-99.7%). Many VNS patients reported alertness improvements, and most had no major complications. VNS was most effective for atonic/tonic seizures; higher stimulation settings correlated with better outcomes. CC patients reported moderate cognitive and QOL improvements; disconnection syndrome, transient weakness, and respiratory complications were noted. Greater callosotomy extent correlated with better outcomes. AED usage most often did not change after surgery. RS showed considerable QOL improvements for patients with localized seizure foci. In the reported literature, CC appeared to be more effective than VNS for seizure reduction. VNS may provide a similar or higher level of QOL improvement with lower aggregate risk of complications. Patient selection, anatomy, and seizure type will inform decision-making.
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