Febrile infection-related epilepsy syndrome (FIRES) characteristically affects previously healthy children, who experience a sudden and explosive onset of super-refractory status epilepticus preceded by febrile infection and accompanied by fulminant neurogenic inflammation. FIRES, however, can affect individuals of all ages and is a subcategory of new-onset refractory status epilepticus. This definition of FIRES excludes febrile status epilepticus in infants. FIRES is a rare type of epileptic encephalopathy with rapidly progressive onset of seizures and a devastating prognosis, as drug-resistant epilepsy often follows without a latency period. Although the exact pathogenesis of FIRES has not been elucidated, a functional deficiency in the endogenous interleukin-1 receptor antagonist has been implicated in a genetic predisposition to FIRES. Dysregulation of the interleukin-1 β –interleukin-1 receptor 1 (IL-1 β –IL-1R1) signaling pathway appears to be involved in the pathogenesis of FIRES. In this review, the authors summarize the definition of FIRES, IL-1 β –IL-1R1 signaling, the nucleotide-binding oligomerization domain the NLRP3 inflammasome, and IL-1 targeted therapy for FIRES.
{"title":"Interleukin-1 in Febrile Infection-Related Epilepsy Syndrome","authors":"J. Kang, S. Koh","doi":"10.26815/acn.2023.00031","DOIUrl":"https://doi.org/10.26815/acn.2023.00031","url":null,"abstract":"Febrile infection-related epilepsy syndrome (FIRES) characteristically affects previously healthy children, who experience a sudden and explosive onset of super-refractory status epilepticus preceded by febrile infection and accompanied by fulminant neurogenic inflammation. FIRES, however, can affect individuals of all ages and is a subcategory of new-onset refractory status epilepticus. This definition of FIRES excludes febrile status epilepticus in infants. FIRES is a rare type of epileptic encephalopathy with rapidly progressive onset of seizures and a devastating prognosis, as drug-resistant epilepsy often follows without a latency period. Although the exact pathogenesis of FIRES has not been elucidated, a functional deficiency in the endogenous interleukin-1 receptor antagonist has been implicated in a genetic predisposition to FIRES. Dysregulation of the interleukin-1 β –interleukin-1 receptor 1 (IL-1 β –IL-1R1) signaling pathway appears to be involved in the pathogenesis of FIRES. In this review, the authors summarize the definition of FIRES, IL-1 β –IL-1R1 signaling, the nucleotide-binding oligomerization domain the NLRP3 inflammasome, and IL-1 targeted therapy for FIRES.","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47860433","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Clinical Efficacy of Near-Infrared Reflectance Imaging and Optical Coherence Tomography in Identifying Ocular Manifestations of Neurofibromatosis Type 1 in Korean Children","authors":"Ji Min Park, W. H. Oh, Jin Choi","doi":"10.26815/acn.2022.00444","DOIUrl":"https://doi.org/10.26815/acn.2022.00444","url":null,"abstract":"","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48851836","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Min-Ji Suh, H. Chung, Jungeun Song, Song Li Ahn, Minji Chae, Seong Woo Kim
{"title":"Effectiveness of a Training Program for Parents of Toddlers with or at Risk of Autism Spectrum Disorder","authors":"Min-Ji Suh, H. Chung, Jungeun Song, Song Li Ahn, Minji Chae, Seong Woo Kim","doi":"10.26815/acn.2022.00381","DOIUrl":"https://doi.org/10.26815/acn.2022.00381","url":null,"abstract":"","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46696579","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C. Morais, M. Silva, T. Mota, R. Rocha, A. Ribeiro
Purpose: Status epilepticus (SE) is a life-threatening neurological emergency, frequently diagnosed in pediatric patients. We aimed to characterize our pediatric cases of SE in an 11-year period according to the 2015 International League Against Epilepsy report. Methods: Clinical electronic records were retrospectively reviewed. All pediatric SE cases admitted from January 2010 to December 2020 were included, excluding neonates. SE was considered refractory if it persisted despite the administration of two appropriate antiseizure medications at acceptable doses. Results: We included 102 episodes, 55 (53.9%) in boys. The median age was 2.5 years (interquartile range, 1.3 to 5.0). Most episodes were classified as SE with prominent motor features (92.2%), and the most frequent etiological classification was acute symptomatic cause (84.3%). A benzodiazepine was used as the first-line antiseizure medication in 99 (97%) cases, of which diazepam was preferred (93%). The preferred second-line medication was phenytoin (65.7%). Midazolam was the most frequently responsible for termination of SE when given in a continuous infusion (47%). Episodes of SE were classified as refractory in 81 (79.4%) cases. Episodes of >60 minutes were more frequent in patients diagnosed with epilepsy ( P =0.036), focal motor SE ( P <0.001), and non-convulsive SE ( P =0.037). The in-hospital mortality rate was 2.9%. Conclusion: Most of our findings are in accord with the current literature. Epilepsy, non-convulsive SE, and focal motor SE were associated with prolonged duration (>60 minutes), which rein-forces the significance of the underlying neurological disease and semiological standardization in pediatric SE.
{"title":"Status Epilepticus in Children: Experience in a Portuguese Tertiary Hospital","authors":"C. Morais, M. Silva, T. Mota, R. Rocha, A. Ribeiro","doi":"10.26815/acn.2022.00465","DOIUrl":"https://doi.org/10.26815/acn.2022.00465","url":null,"abstract":"Purpose: Status epilepticus (SE) is a life-threatening neurological emergency, frequently diagnosed in pediatric patients. We aimed to characterize our pediatric cases of SE in an 11-year period according to the 2015 International League Against Epilepsy report. Methods: Clinical electronic records were retrospectively reviewed. All pediatric SE cases admitted from January 2010 to December 2020 were included, excluding neonates. SE was considered refractory if it persisted despite the administration of two appropriate antiseizure medications at acceptable doses. Results: We included 102 episodes, 55 (53.9%) in boys. The median age was 2.5 years (interquartile range, 1.3 to 5.0). Most episodes were classified as SE with prominent motor features (92.2%), and the most frequent etiological classification was acute symptomatic cause (84.3%). A benzodiazepine was used as the first-line antiseizure medication in 99 (97%) cases, of which diazepam was preferred (93%). The preferred second-line medication was phenytoin (65.7%). Midazolam was the most frequently responsible for termination of SE when given in a continuous infusion (47%). Episodes of SE were classified as refractory in 81 (79.4%) cases. Episodes of >60 minutes were more frequent in patients diagnosed with epilepsy ( P =0.036), focal motor SE ( P <0.001), and non-convulsive SE ( P =0.037). The in-hospital mortality rate was 2.9%. Conclusion: Most of our findings are in accord with the current literature. Epilepsy, non-convulsive SE, and focal motor SE were associated with prolonged duration (>60 minutes), which rein-forces the significance of the underlying neurological disease and semiological standardization in pediatric SE.","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46269187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ahmed Masoud Ali, Ahmad Darwish Mohamed, Mona Ahmed Elbilsha
{"title":"Assessment of Sleep Disorders in Children with Transfusion-Dependent Hemoglobinopathies","authors":"Ahmed Masoud Ali, Ahmad Darwish Mohamed, Mona Ahmed Elbilsha","doi":"10.26815/acn.2023.00045","DOIUrl":"https://doi.org/10.26815/acn.2023.00045","url":null,"abstract":"","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41374928","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Hassan Mottaghi Moghadam shahri, Ehsan Hassan Nejad, M. B. Toosi, A. Malek, Asma Payandeh
{"title":"Clinical Findings of Sydenham Chorea in Pediatric Patients: A Single-Center Retrospective Study","authors":"Hassan Mottaghi Moghadam shahri, Ehsan Hassan Nejad, M. B. Toosi, A. Malek, Asma Payandeh","doi":"10.26815/acn.2023.00038","DOIUrl":"https://doi.org/10.26815/acn.2023.00038","url":null,"abstract":"","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43039437","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yoo Jung Lee, Yoon Hee Jo, Mun-Bum Kim, Young Mi Kim
{"title":"Hypochondroplasia with Acanthosis Nigricans and Moyamoya Disease","authors":"Yoo Jung Lee, Yoon Hee Jo, Mun-Bum Kim, Young Mi Kim","doi":"10.26815/acn.2022.00402","DOIUrl":"https://doi.org/10.26815/acn.2022.00402","url":null,"abstract":"","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41701134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"New Daily Persistent Headache after COVID-19 Vaccination in an Adolescent Patient","authors":"Hyewon Woo, Jon Soo Kim, W. Kim, Joon Kee Lee","doi":"10.26815/acn.2022.00395","DOIUrl":"https://doi.org/10.26815/acn.2022.00395","url":null,"abstract":"","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45431493","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Long-Term Pharmacological and Psychosocial Outcomes of Adolescent-Onset Epilepsy: A Single-Center Experience","authors":"Hyun-Jin Kim, H. Jang, Hyunji Ahn, M. Yum, T. Ko","doi":"10.26815/acn.2022.00451","DOIUrl":"https://doi.org/10.26815/acn.2022.00451","url":null,"abstract":"","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44797356","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Vikas Sharma, K. Rajeshwari, Deepak Kumar, Garima Gupta
{"title":"Clinicoepidemiological Profile and Prognostic Factors in Neurotuberculosis in Children","authors":"Vikas Sharma, K. Rajeshwari, Deepak Kumar, Garima Gupta","doi":"10.26815/acn.2022.00409","DOIUrl":"https://doi.org/10.26815/acn.2022.00409","url":null,"abstract":"","PeriodicalId":33305,"journal":{"name":"Annals of Child Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2023-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48683050","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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