Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101243
Pedro Weisleder , John R. Mytinger , Howard P. Goodkin
William Gordon Lennox (1884–1960) was a pivotal figure in twentieth-century neurology whose pioneering research on electroencephalography, seizure classification, and the biology of epilepsy helped establish the foundations of modern neurology. His work transformed epilepsy from a stigmatized condition into one amenable to scientific study and compassionate clinical care. However, Lennox’s legacy is complicated by his endorsement of eugenic principles and his writings on disability, mental illness, and race. Those opinions are now recognized as deeply problematic. Examining Lennox’s career illuminates the moral tensions within early neurological science and offers a lens through which to trace the medical profession’s evolution from a eugenic framework toward contemporary commitments to human rights and patient autonomy.
威廉·戈登·伦诺克斯（William Gordon Lennox, 1884-1960）是20世纪神经学的关键人物，他在脑电图、癫痫分类和癫痫生物学方面的开创性研究帮助建立了现代神经学的基础。他的工作使癫痫从一种被污名化的疾病转变为一种可以接受科学研究和富有同情心的临床护理的疾病。然而，伦诺克斯对优生学原则的支持，以及他关于残疾、精神疾病和种族的著作，使他的遗产变得复杂起来。这些观点现在被认为是有严重问题的。考察伦诺克斯的职业生涯阐明了早期神经科学中的道德紧张，并提供了一个镜头，通过它可以追溯医学职业从优生框架到当代对人权和患者自主权的承诺的演变。
{"title":"“William G. Lennox: A legacy of compassion and controversy”","authors":"Pedro Weisleder , John R. Mytinger , Howard P. Goodkin","doi":"10.1016/j.spen.2025.101243","DOIUrl":"10.1016/j.spen.2025.101243","url":null,"abstract":"<div><div>William Gordon Lennox (1884–1960) was a pivotal figure in twentieth-century neurology whose pioneering research on electroencephalography, seizure classification, and the biology of epilepsy helped establish the foundations of modern neurology. His work transformed epilepsy from a stigmatized condition into one amenable to scientific study and compassionate clinical care. However, Lennox’s legacy is complicated by his endorsement of eugenic principles and his writings on disability, mental illness, and race. Those opinions are now recognized as deeply problematic. Examining Lennox’s career illuminates the moral tensions within early neurological science and offers a lens through which to trace the medical profession’s evolution from a eugenic framework toward contemporary commitments to human rights and patient autonomy.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101243"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697739","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101240
Yoshimi Hisamoto
Late-Onset Lennox-Gastaut Syndrome, is arbitrarily defined as Lennox-Gastaut Syndrome with onset after age 8 years, as opposed to its typical age of onset between 18 months and 8 years. Little is known about this late onset form of Lennox-Gastaut Syndrome. In this article, available information about this rare population of patients is reviewed, based on English language literature, to elucidate our current knowledge about the characteristic clinical presentation, known etiologies, comorbidities and long-term outcomes, as well as to identify future needs in research.
The clinical characteristics of the Late-Onset Lennox Gastaut Syndrome described in the literature included relatively high number of patients with normal development and cognition at onset, low numbers of patients with preceding infantile spasms, and possibly a better cognitive outcome than its typical early onset form. Limitations rested on the variable age criterion used in different studies, limited data on imaging and genetics in many early studies, and only small numbers of patients included in each study.
A study with a large number of patients with Lennox-Gastaut Syndrome, based on a unified diagnostic criterion, with detailed information on demographics, including evaluation with modern imaging modalities and genetic testing, and with long-term follow-up data is warranted to further delineate its accurate clinical characteristics.
{"title":"Late onset Lennox-Gastaut syndrome","authors":"Yoshimi Hisamoto","doi":"10.1016/j.spen.2025.101240","DOIUrl":"10.1016/j.spen.2025.101240","url":null,"abstract":"<div><div>Late-Onset Lennox-Gastaut Syndrome, is arbitrarily defined as Lennox-Gastaut Syndrome with onset after age 8 years, as opposed to its typical age of onset between 18 months and 8 years. Little is known about this late onset form of Lennox-Gastaut Syndrome. In this article, available information about this rare population of patients is reviewed, based on English language literature, to elucidate our current knowledge about the characteristic clinical presentation, known etiologies, comorbidities and long-term outcomes, as well as to identify future needs in research.</div><div>The clinical characteristics of the Late-Onset Lennox Gastaut Syndrome described in the literature included relatively high number of patients with normal development and cognition at onset, low numbers of patients with preceding infantile spasms, and possibly a better cognitive outcome than its typical early onset form. Limitations rested on the variable age criterion used in different studies, limited data on imaging and genetics in many early studies, and only small numbers of patients included in each study.</div><div>A study with a large number of patients with Lennox-Gastaut Syndrome, based on a unified diagnostic criterion, with detailed information on demographics, including evaluation with modern imaging modalities and genetic testing, and with long-term follow-up data is warranted to further delineate its accurate clinical characteristics.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101240"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697740","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101238
Alejandra Vasquez, Anthony L. Fine
Lennox-Gastaut Syndrome (LGS) is a severe developmental and epileptic encephalopathy of childhood characterized by drug-refractory epilepsy. Ketogenic diet (KD) therapies are non-pharmacologic adjuvant treatments that should be considered early in the course of the disease given challenges with pharmacoresistance and polytherapy. KD therapies are diets with high-fat and low-carbohydrates that induce ketotic metabolism with the goal of seizure control. KDs include classic ketogenic diet, medium-chain triglyceride diet (MCT), modified Atkins diet (MAD), and low glycemic index treatment (LGIT). Available evidence supports the efficacy of KD therapies in LGS with ≥50 % seizure reduction and seizure freedom in approximately 40-50 % of patients. KD therapies are considered safe and tolerable with close monitoring in LGS; however, side effects reported in the literature include diarrhea, constipation, emesis, and less frequently, osteopenia and nephrolithiasis. This review aims to provide a comprehensive overview of current dietary therapies with a particular focus on the efficacy and safety of these interventions in LGS.
{"title":"Dietary therapies in Lennox-Gastaut syndrome","authors":"Alejandra Vasquez, Anthony L. Fine","doi":"10.1016/j.spen.2025.101238","DOIUrl":"10.1016/j.spen.2025.101238","url":null,"abstract":"<div><div>Lennox-Gastaut Syndrome (LGS) is a severe developmental and epileptic encephalopathy of childhood characterized by drug-refractory epilepsy. Ketogenic diet (KD) therapies are non-pharmacologic adjuvant treatments that should be considered early in the course of the disease given challenges with pharmacoresistance and polytherapy. KD therapies are diets with high-fat and low-carbohydrates that induce ketotic metabolism with the goal of seizure control. KDs include classic ketogenic diet, medium-chain triglyceride diet (MCT), modified Atkins diet (MAD), and low glycemic index treatment (LGIT). Available evidence supports the efficacy of KD therapies in LGS with ≥50 % seizure reduction and seizure freedom in approximately 40-50 % of patients. KD therapies are considered safe and tolerable with close monitoring in LGS; however, side effects reported in the literature include diarrhea, constipation, emesis, and less frequently, osteopenia and nephrolithiasis. This review aims to provide a comprehensive overview of current dietary therapies with a particular focus on the efficacy and safety of these interventions in LGS.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101238"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697775","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101237
Lisa B.E. Shields , Chima O Oluigbo , Cemal Karakas
Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy with a childhood onset which is marked by multiple seizure types, cognitive impairment, and a characteristic abnormal electroencephalogram pattern. LGS is often resistant to antiseizure medications, making management of this condition challenging. Additional treatment options include a ketogenic diet or surgical intervention such as a corpus callosotomy in patients with predominant atonic seizure types. Neuromodulation therapies may also be valuable in LGS by utilizing electrical stimulation to regulate brain activity and decrease the frequency of seizures. The three main types of neuromodulation consist of responsive neurostimulation, deep brain stimulation, and vagus nerve stimulation. This focused review describes the advantages and disadvantages of the three neuromodulation techniques, important factors to consider with neuromodulation when treating patients with LGS, and future directions of these therapies.
{"title":"Neuromodulation in Lennox-Gastaut Syndrome: Emerging therapies and future directions","authors":"Lisa B.E. Shields , Chima O Oluigbo , Cemal Karakas","doi":"10.1016/j.spen.2025.101237","DOIUrl":"10.1016/j.spen.2025.101237","url":null,"abstract":"<div><div>Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy with a childhood onset which is marked by multiple seizure types, cognitive impairment, and a characteristic abnormal electroencephalogram pattern. LGS is often resistant to antiseizure medications, making management of this condition challenging. Additional treatment options include a ketogenic diet or surgical intervention such as a corpus callosotomy in patients with predominant atonic seizure types. Neuromodulation therapies may also be valuable in LGS by utilizing electrical stimulation to regulate brain activity and decrease the frequency of seizures. The three main types of neuromodulation consist of responsive neurostimulation, deep brain stimulation, and vagus nerve stimulation. This focused review describes the advantages and disadvantages of the three neuromodulation techniques, important factors to consider with neuromodulation when treating patients with LGS, and future directions of these therapies.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101237"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697776","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101235
Christopher W. Hagen, Cynthia G. Keator
Epilepsy surgery remains a viable consideration for the treatment of developmental epileptic encephalopathies (DEEs), specifically Lennox Gastaut Syndrome (LGS). Previously, Keator (2021) reviewed the current literature on epilepsy surgery for LGS including resective and neuromodulating techniques. There continues to be a hesitance to pursuing surgical evaluation in DEEs. Herein, this review continues to explore the recent literature in regard to non-neuromodulating techniques focusing on resective (curative and palliative) and corpus callosotomies in patients with LGS. Specifically, the last five years of the current literature are reviewed in non-neuromodulatory surgical approaches for LGS. In the current literature, there is also special attention focusing on the secondary network involved in LGS as well as the future directions in novel surgical techniques and the identification of potential biomarkers for LGS.
{"title":"Surgical management of Lennox-Gastaut syndrome: A focused update on resective surgery and corpus callosotomy","authors":"Christopher W. Hagen, Cynthia G. Keator","doi":"10.1016/j.spen.2025.101235","DOIUrl":"10.1016/j.spen.2025.101235","url":null,"abstract":"<div><div>Epilepsy surgery remains a viable consideration for the treatment of developmental epileptic encephalopathies (DEEs), specifically Lennox Gastaut Syndrome (LGS). Previously, Keator (2021) reviewed the current literature on epilepsy surgery for LGS including resective and neuromodulating techniques. There continues to be a hesitance to pursuing surgical evaluation in DEEs. Herein, this review continues to explore the recent literature in regard to non-neuromodulating techniques focusing on resective (curative and palliative) and corpus callosotomies in patients with LGS. Specifically, the last five years of the current literature are reviewed in non-neuromodulatory surgical approaches for LGS. In the current literature, there is also special attention focusing on the secondary network involved in LGS as well as the future directions in novel surgical techniques and the identification of potential biomarkers for LGS.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101235"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101242
Amanda W. Pong
Lennox-Gastaut Syndrome (LGS) is a severe developmental epileptic encephalopathy (DEE) that develops in response to genetic, traumatic, or other factors. It is defined as a triad of drug refractory epilepsy with multiple seizure types including tonic seizures, cognitive impairment, and signature EEG findings. The dire need for more effective, well-tolerated therapies calls for further innovation and development in oral drug therapies, which remain the cornerstone of LGS management. The purpose of this paper is to address three such aspects of treatment evolution for LGS: (1) To review data supporting the repurposing of existing drugs for use in LGS, specifically, perampanel and cenobamate. (2) To present recent (soticlestat), ongoing (carisbamate, bexicaserin, clemizole) and upcoming (opakalim) clinical drug trials for LGS. (3) To discuss potential future challenges in clinical drug trial development for LGS, including decentralized clinical drug trials and issues related to competing studies. Durable and sustained seizure freedom in LGS has been recently described using newer antiseizure medications, with positive impact on behavior, mood, cognition and verbal communication, with potential weaning of concomitant antiseizure medications. With the richness of recent trial development for LGS combined with the nascence of clinical trials for specific genetic epilepsies comes a new era in which treatment options for LGS will continue to expand. Increasing understanding of the underlying genetic and molecular underpinnings of LGS should enable development of unique therapies, with the continued aims of sustained, durable seizure control and additional positive impact on central nervous system outcomes and beyond.
{"title":"Expanding the toolkit: An update on the evolution of new therapies for Lennox-Gastaut Syndrome","authors":"Amanda W. Pong","doi":"10.1016/j.spen.2025.101242","DOIUrl":"10.1016/j.spen.2025.101242","url":null,"abstract":"<div><div>Lennox-Gastaut Syndrome (LGS) is a severe developmental epileptic encephalopathy (DEE) that develops in response to genetic, traumatic, or other factors. It is defined as a triad of drug refractory epilepsy with multiple seizure types including tonic seizures, cognitive impairment, and signature EEG findings. The dire need for more effective, well-tolerated therapies calls for further innovation and development in oral drug therapies, which remain the cornerstone of LGS management. The purpose of this paper is to address three such aspects of treatment evolution for LGS: (1) To review data supporting the repurposing of existing drugs for use in LGS, specifically, perampanel and cenobamate. (2) To present recent (soticlestat), ongoing (carisbamate, bexicaserin, clemizole) and upcoming (opakalim) clinical drug trials for LGS. (3) To discuss potential future challenges in clinical drug trial development for LGS, including decentralized clinical drug trials and issues related to competing studies. Durable and sustained seizure freedom in LGS has been recently described using newer antiseizure medications, with positive impact on behavior, mood, cognition and verbal communication, with potential weaning of concomitant antiseizure medications. With the richness of recent trial development for LGS combined with the nascence of clinical trials for specific genetic epilepsies comes a new era in which treatment options for LGS will continue to expand. Increasing understanding of the underlying genetic and molecular underpinnings of LGS should enable development of unique therapies, with the continued aims of sustained, durable seizure control and additional positive impact on central nervous system outcomes and beyond.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101242"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697774","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101239
Anne Francine Pino , Sunil Naik , Gozde Erdemir
Lennox–Gastaut syndrome (LGS) is a rare, severe developmental and epileptic encephalopathy marked by drug-resistant seizures, abnormal EEG patterns, and a broad spectrum of comorbidities. In this review we provide an overview of the main neurodevelopmental, psychiatric, motor, systemic, and epilepsy-related comorbidities in LGS, and their impact on patients and caregivers. Cognitive impairment and intellectual disability are nearly universal, often accompanied by behavioral disturbances such as hyperactivity, irritability, anxiety, and autism spectrum disorder. Motor impairments, including cerebral palsy, spasticity, and gait difficulties, are common. Epilepsy-related complications, such as recurrent status epilepticus, seizure-related injuries, and sudden unexpected death, add to the disease’s burden. Systemic comorbidities, including sleep and gastrointestinal disturbances, further contribute to long-term care. Collectively, these comorbidities often impact patients and families more than seizures themselves, underscoring the need for comprehensive, multidisciplinary care and targeted interventions. In this review, we highlight comorbidities in LGS across neurodevelopmental, psychiatric, motor, systemic, and epilepsy-related domains, emphasizing their contribution to disease burden, functional impairment, and quality of life.
{"title":"Lennox-Gastaut syndrome: Comorbidities and clinical implications","authors":"Anne Francine Pino , Sunil Naik , Gozde Erdemir","doi":"10.1016/j.spen.2025.101239","DOIUrl":"10.1016/j.spen.2025.101239","url":null,"abstract":"<div><div>Lennox–Gastaut syndrome (LGS) is a rare, severe developmental and epileptic encephalopathy marked by drug-resistant seizures, abnormal EEG patterns, and a broad spectrum of comorbidities. In this review we provide an overview of the main neurodevelopmental, psychiatric, motor, systemic, and epilepsy-related comorbidities in LGS, and their impact on patients and caregivers. Cognitive impairment and intellectual disability are nearly universal, often accompanied by behavioral disturbances such as hyperactivity, irritability, anxiety, and autism spectrum disorder. Motor impairments, including cerebral palsy, spasticity, and gait difficulties, are common. Epilepsy-related complications, such as recurrent status epilepticus, seizure-related injuries, and sudden unexpected death, add to the disease’s burden. Systemic comorbidities, including sleep and gastrointestinal disturbances, further contribute to long-term care. Collectively, these comorbidities often impact patients and families more than seizures themselves, underscoring the need for comprehensive, multidisciplinary care and targeted interventions. In this review, we highlight comorbidities in LGS across neurodevelopmental, psychiatric, motor, systemic, and epilepsy-related domains, emphasizing their contribution to disease burden, functional impairment, and quality of life.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101239"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101241
Gita Gupta , Louise M. O’Brien , Fauziya Hassan , Jonathan Jun , Stephanie M.D. Rau , Arushi Gupta , Raayed Saeed , Renée A. Shellhaas
Background
Sleep disorders in children with Lennox-Gastaut Syndrome (LGS) are reported to be common, consequential, and potentially treatable. To design effective studies of sleep in LGS, feasible methods of sleep assessment in affected children must be determined.
Methods
We prospectively studied the feasibility of the following sleep assessment modalities among families whose child had LGS: 1. polysomnogram, 2. home sleep apnea test (HSAT), 3. biweekly sleep questionnaire, 4. daily sleep tracking device (Apple Watch), and 5. daily sleep logs. Participants were enrolled in a 2-week study with the option to participate for an additional two weeks. Caregivers rated their experience of each modality. The primary outcome was percent completion of each sleep assessment modality.
Results
Among 32 participants (mean age 12.0 ± 3.2 years), 10/10 families who agreed to polysomnography, and 15/16 of families who agreed to HSAT completed these one-time modalities. Completion rates of the biweekly sleep questionnaires were 78 % (baseline), 25 % (two weeks), and 36 % (four weeks). Completion rates of the daily modalities for the combined sample were: 1. Apple Watch: median 100 % (IQR 100 %,100 %) of planned nights by the 16/32 participants who opted in, and 2. daily sleep logs: median 64 % of planned days (IQR 0,95 %). Nearly 90 % of participants remained in the study at the end of two weeks. The Apple Watch was rated as the most convenient and most preferred modality.
Conclusion
It may be feasible to conduct longitudinal multi-modal ambulatory assessments of sleep of children with LGS for up to two weeks.
{"title":"Feasibility of sleep assessment modalities in children with Lennox-Gastaut syndrome","authors":"Gita Gupta , Louise M. O’Brien , Fauziya Hassan , Jonathan Jun , Stephanie M.D. Rau , Arushi Gupta , Raayed Saeed , Renée A. Shellhaas","doi":"10.1016/j.spen.2025.101241","DOIUrl":"10.1016/j.spen.2025.101241","url":null,"abstract":"<div><h3>Background</h3><div>Sleep disorders in children with Lennox-Gastaut Syndrome (LGS) are reported to be common, consequential, and potentially treatable. To design effective studies of sleep in LGS, feasible methods of sleep assessment in affected children must be determined.</div></div><div><h3>Methods</h3><div>We prospectively studied the feasibility of the following sleep assessment modalities among families whose child had LGS: 1. polysomnogram, 2. home sleep apnea test (HSAT), 3. biweekly sleep questionnaire, 4. daily sleep tracking device (Apple Watch), and 5. daily sleep logs. Participants were enrolled in a 2-week study with the option to participate for an additional two weeks. Caregivers rated their experience of each modality. The primary outcome was percent completion of each sleep assessment modality.</div></div><div><h3>Results</h3><div>Among 32 participants (mean age 12.0 ± 3.2 years), 10/10 families who agreed to polysomnography, and 15/16 of families who agreed to HSAT completed these one-time modalities. Completion rates of the biweekly sleep questionnaires were 78 % (baseline), 25 % (two weeks), and 36 % (four weeks). Completion rates of the daily modalities for the combined sample were: 1. Apple Watch: median 100 % (IQR 100 %,100 %) of planned nights by the 16/32 participants who opted in, and 2. daily sleep logs: median 64 % of planned days (IQR 0,95 %). Nearly 90 % of participants remained in the study at the end of two weeks. The Apple Watch was rated as the most convenient and most preferred modality.</div></div><div><h3>Conclusion</h3><div>It may be feasible to conduct longitudinal multi-modal ambulatory assessments of sleep of children with LGS for up to two weeks.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101241"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697752","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-12-01DOI: 10.1016/j.spen.2025.101244
Mary Wojnaroski , Megan Votoupal , Sandi Lam , Anup D. Patel
Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy with frequent intractable seizures and non-seizure symptoms, such as cognitive impairment and challenging behaviors. Individuals with LGS and their caregivers often report poor quality of life (QoL). Despite this knowledge, QoL is not routinely evaluated or addressed in individuals with LGS and their families. Reduction in seizure frequency through appropriate antiseizure medications (ASM) and epilepsy surgery are critical for improved QoL; however, given the rarity of seizure freedom in this population, assessment and treatment of non-seizure symptoms is also critical. Validating existing QoL measures for individuals with LGS and their families will support a more comprehensive understanding of the factors that shape their lived experience. Multidisciplinary interventions to address development, behavior, and family functioning, as well as referrals to family support groups and improved care coordination are recommended.
{"title":"Quality of life in individuals with Lennox-Gastaut syndrome and their families","authors":"Mary Wojnaroski , Megan Votoupal , Sandi Lam , Anup D. Patel","doi":"10.1016/j.spen.2025.101244","DOIUrl":"10.1016/j.spen.2025.101244","url":null,"abstract":"<div><div>Lennox-Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy with frequent intractable seizures and non-seizure symptoms, such as cognitive impairment and challenging behaviors. Individuals with LGS and their caregivers often report poor quality of life (QoL). Despite this knowledge, QoL is not routinely evaluated or addressed in individuals with LGS and their families. Reduction in seizure frequency through appropriate antiseizure medications (ASM) and epilepsy surgery are critical for improved QoL; however, given the rarity of seizure freedom in this population, assessment and treatment of non-seizure symptoms is also critical. Validating existing QoL measures for individuals with LGS and their families will support a more comprehensive understanding of the factors that shape their lived experience. Multidisciplinary interventions to address development, behavior, and family functioning, as well as referrals to family support groups and improved care coordination are recommended.</div></div>","PeriodicalId":49284,"journal":{"name":"Seminars in Pediatric Neurology","volume":"56 ","pages":"Article 101244"},"PeriodicalIF":2.1,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145697751","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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