The electronic database searches initially identified 868 articles, of which 595 were screened after the removal of duplicates. Furthermore, Murray reported in 1993 that parents had to deal with a lot of uncertainty around diagnosis, etiology, seizure activity, treatment and prognosis, which caused feelings of guilt and stress in some parents [60]. Wijnen B, Armstrong N, Ramaekers B, et al. Burden-of-illness and cost-driving factors in Dravet syndrome patients and carers: a prospective, multicenter study from Germany. However, the strength of the study is challenged because it represents reports rather than scientific analysis of the clinical outcomes such as in a randomized controlled trial. (17%) achieved late-life terminal seizure-freedom of at least two years before death, which occurred at a median age of 68 years with a . Other areas of heterogeneity across the studies included the size of the populations, the ages of the patients and the length of the follow-up period. The recognition of a specific disease entity is vital for every aspect of patient care, from diagnosis, management and treatment to advancements in health outcomes through research and health policy (Fig 1).The definition of Lennox-Gastaut syndrome (LGS) has been evolving since Lennox and Davis (1950) [] and Gastaut et al (1960) [] described the electroclinical features of a . [37], LGS is seen in approximately 4% of children with epilepsy, and is more common in males than in females. Data were extracted for patients with LGS, LGS subpopulations (patients with and without seizures and with and without rescue medication) and control populations used by the authors (e.g age matched non-LGS patients). [18], Mutations in the IQSEC2 gene have been associated with this syndrome. Pnd27 economic burden of probable LennoxGastaut syndrome, probable Dravet syndrome, and other refractory epilepsies for united states commercial health plans. A prescription for at least one selected ASM. Expert opinion on the management of LennoxGastaut syndrome: treatment algorithms and practical considerations. Gallop et al. Glauser T, Kluger G, Sachdeo R, et al. The cause of the idiopathic subtype is unknown. Epilepsy surgery program in a resource-limited setting in Vietnam: a multicentered collaborative model. Learn about clinical trials currently looking for people with Lennox-Gastaut syndrome at Clinicaltrials.gov. The appearance of multiple types of seizures in early childhood, particularly a combination of tonic, atonic and atypical absence seizures, is a key diagnostic indicator of Lennox-Gastaut syndrome. Despite the challenges of defining LGS, there have been significant clinical improvements over the years [5, 1316]. Seizures begin in early childhood, usually before the age of 4 years. Rosso M, Chu D, Santoro JD. Children and adolescents with epilepsy, especially those with Lennox-Gastaut syndrome or infantile spasms, have an increased risk of death. Studies showed that the HRQoL of patients and caregivers was adversely affected, although only a few studies were identified. Nabbout R, Dirani M, Teng T, et al. In addition, 3 studies reported on the proportion of LGS among epilepsy patients, varying from 4 to 8.4% during childhood [43, 44, 46] [42, 45]. Mortality is also high (standardized mortality ratio [SMR] = 14) . [citation needed], Nonconvulsive status epilepticus occurs in about 50% of patients. Rescue therapies, a different set of medications, may be given to help stop or shorten clusters of seizures when they occur. Common seizure types include: There may be periods of frequent seizures mixed with relatively seizure-free periods. [62] used an alternative method of measuring patient HRQoL in a quantitative manner whereby patients and/or caregivers of patients with LGS, DS, or other epilepsies were asked to score the patient HRQoL based on hypothetical vignettes of patients with LGS (or DS) according to how many seizures and seizure-free days the hypothetical patient experienced. About 10% to 30% of children with LGS have a prior history of earlier onset epilepsy syndromes, such as West syndrome or Ohtahara syndrome. No limits were applied (e.g regarding publication dates or language), except for conference abstracts that were limited to the previous 3 years. LennoxGastaut Syndrome (LGS): development of conceptual models of health-related quality of life (HRQL) for caregivers and children. Childrens Hospital of Philadelphia is a charitable 501(c)(3) nonprofit organization. Lennox-Gastaut syndrome (LGS) is a severe form of epilepsy with seizures that begin in early childhood, usually between the ages of 2 and 5, and continue into adulthood. Increased risk of death among children with LennoxGastaut syndrome and infantile spasms. Publication types Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S. Consider participating in a clinical trial so clinicians and scientists can learn more about Lennox-Gastaut syndrome and related disorders. In addition, there is a lack of studies on all aspects of the burden of illness in low and middle income countries, and more research is needed in these areas, possibly through enhanced collaboration with established research groups in high income countries and the pharmaceutical industry. PS reports personal fees and grants from Angelini Pharma, Eisai, Jazz Pharmaceuticals Biomarin, UCB, Proveca, and Zogenix. An official website of the United States government. [54] reported that, compared to matched controls, annual costs for services were 17 to 20 times higher for patients with possible LGS, and annual costs for drugs were 16 to 38 times higher (Fig3B). The https:// ensures that you are connecting to the Families come to our ENGIN clinic from all over the world. LGS is associated with a substantial burden of illness, with seizure events associated with higher costs and worse HRQoL. Suurmeijer, Epilepsy in Children and Adolescents. Data were extracted into tables in MS Word by one reviewer, which was checked for accuracy by a second independent reviewer. Centre for Reviews and Dissemination. It is characterized by multiple seizure types, abnormal electroencephalographic features, and intellectual disability. [53], 22% (2329 of 10,618) of LGS patients with a medically-treated seizure event across insurance types had an injury. Commission on classification and terminology of the international league against epilepsy. Many of the studies identified are recently published, particularly those on costs and healthcare resources, which may reflect the development of new treatment options, whereby the data can be used to inform payers and health technology assessment agencies. The risk of bias in the studies reporting on prevalence/incidence was low in 3 studies [40, 41, 47] and moderate in 5 studies [39, 4245] (Additional file 2: Table S2). J Clin Neurophysiol . [citation needed], Pseudo-LennoxGastaut syndrome can be distinguished from LGS because pseudo-LGS has different spike-and-wave patterns on EEG. More research is needed, especially in evaluating indirect costs and caregiver burden, where there is a notable lack of studies. [15][16] The Epi4K study consortium (2013) observed de novo mutations in at least 15% of a study cohort of 165 patients with LGS and infantile spasms using whole exome sequencing. Asadi-Pooya AA. Three studies used quantitative methods (one assessed caregiver HRQoL using the SF-36 and the HADS (Hospital Anxiety and Depression Scale), while two studies used a VAS (visual analog scale). [13] Usual onset is between the ages of three and five. The epidemiology studies confirm that LGS is a rare syndrome, whereby the prevalence varied from 4.2 to 60.8 per 100,000 people across studies for probable LGS and 2.9 to 28 per 100,000 people for confirmed/narrow definition of LGS. Timeline to show A the history of the characterization and treatment of LGS B the triad of symptoms characteristic of LGS and the clinical implications of the characterization of the syndrome. Sometimes LGS is observed after infantile epileptic spasm syndrome (formely called West syndrome). 2022;63(6):1398442. A combination of anti-seizure medications to control the different seizure types may help to provide partial relief. There is no cure for the disorder. These etiologies are a more precise guide to prognosis and management than the designation of LGS. In Germany the greatest contributors to healthcare costs were inpatient care (33%) especially hospital stays related to epilepsy, followed by home nursing care (including intensive home nursing) (13%), and medication (10%) (Fig4A, Additional file 2: Table S1) [47]. Autry AR, Trevathan E, Van Naarden Braun K, et al.
Increased risk of death among children with Lennox-Gastaut syndrome and [40] However, in a physician survey conducted December 2004, only 28% of LennoxGastaut and West syndrome patients improved on zonisamide.[41]. International League Against Epilepsy Proposal for revised classification of epilepsies and epileptic syndromes. Chris Cooper developed the search terms, and assistance with the literature screening and medical writing, under the direction of the authors, was provided by Amanda Prowse (Lochside Medical Communications Ltd) and Emma Melchor. Death is often related to accidents. The results of the quality assessments/risk of bias are presented in Additional file 2: Tables S25. [citation needed], Findings that strongly suggest LGS include consistent slow spike-wave (< 3 hertz [Hz]) on awake EEG. FR reports personal fees from Angelini Pharma, Arvelle Therapeutics, Eisai GmbH, GW Pharmaceuticals companies, and UCB and grants from the Detlev-Wrobel-Fonds for Epilepsy Research, the Deutsche Forschungsgemeinschaft, the LOEWE Programme of the State of Hesse, and the European Union. Strzelczyk A, Kalski M, Bast T, et al. Seizures typically continue through childhood and into adulthood, with the type and frequency of the seizures changing over time. We searched MEDLINE, Embase and APA PsychInfo (all via OVID), Cochranes database of systematic reviews (CDSR, Wiley), and Epistemonikos. Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Cannabidiol and fenfluramine are the most recently approved therapies for the treatment of seizures associated with LGS (Fig1). Furthermore, readmission rates were high, with approximately 910% of patients readmitted within the month and 4245% within a year of discharge [56]. Genetic causes likely account for a majority of unexplained LGS. [35] A case series study showed 50% seizure reduction reported in almost half of children with LGS after 1 year of ketogenic diet. We combine cutting-edge clinical care and advanced genetic testing with innovative research to identify the underlying cause of a childs epilepsy and develop an individualized approach to treatment and management. Moher D, Liberati A, Tetzlaff J, et al. Most children have some level of permanent cognitive impairment. This site is protected by reCAPTCHA and the GooglePrivacy Policyand Terms of Serviceapply. [24][25], Felbamate is the treatment of last resort in the event that everything else fails,[26] and was found to be superior to placebo in controlling treatment resistant partial seizures and atonic seizures. Radu X, Damera V, Martin M, et al. Children and adults with Lennox-Gastaut syndrome are at an increased risk of premature mortality, often from sudden unexpected death in epilepsy (SUDEP), injury, seizures, or as a consequence of the underlying brain disorder. These studies have estimated the prevalence of probable LGS to be 13.060.8 per 100,000 people in the USA [41], 39.2 per 100,000 people in Germany [47], and 4.2 per 100,000 people in the UK [40].
Lennox-Gastaut syndrome: MedlinePlus Genetics After the primary screening, a total of 149 articles were retrieved for secondary screening (full-text assessment), and of these, 22 met the eligibility criteria (Fig2). Demonstrating the feasibility of digital health to support pediatric patients in South Africa. Riney K, Bogacz A, Somerville E, et al. The reasons for exclusion at secondary screening were clearly documented according to the PICOS criteria.
Long-term prognosis of patients with Lennox-Gastaut syndrome in recent Disagreements regarding the inclusion and/or exclusion of studies at both primary and secondary screening were generally resolved by discussion between the two reviewers, or if needed by a third reviewer. government site. In the study by Reaven et al. Lennox-Gastaut syndrome is considered an epileptic encephalopathy, a condition in which the epileptic seizures can directly contribute to cognitive and behavioral impairments. In most patients with LGS, the treatment does not end seizure recurrence. Epilepsy Foundation, 3401 Civic Center Blvd. SZ reports research support from Epilepsy Research UK, Scottish Government Digital Health & Care, UCB Pharma, Dravet Syndrome UK, Dravet Syndrome Foundation, Tenovus Foundation and Glasgow Childrens Hospital Charity. LennoxGastaut syndrome: a comprehensive review. The risk of bias in the studies reporting on costs and/or resources was assessed to be low in 6 studies [40, 46, 47, 51, 53, 54] and moderate in 3 studies, which were conference abstracts that provided limited information [52, 55, 56] (Additional file 2: Table S3). How common is this condition? They can help connect patients with new and upcoming treatment options. Cannabidiol in patients with seizures associated with LennoxGastaut syndrome (GWPCARE4): a randomised, double-blind, placebo-controlled phase 3 trial. By country, most of the studies were from the USA (7 out of 9 studies). Lagae L, Brambilla I, Mingorance A, et al. The online version contains supplementary material available at 10.1186/s13023-023-02626-4. For information about participating in clinical research visit, . Knupp KG, Scheffer IE, Ceulemans B, et al. 2022:36(10):1079111. Overall, the studies showed that mortality was substantially higher in LGS patients than in children with epilepsy and controls (Table (Table3)3) [40, 47, 48]. The protocol was registered with Prospero (CRD42022333410). FOIA Lennox-Gastaut syndrome (LGS) is a severe epileptic and developmental encephalopathy that is associated with a high rate of morbidity and mortality.