Pub Date : 2022-01-01DOI: 10.1177/2329048X221140783
Sarah Mingels, Marita Granitzer
Background: Although headache is common in pediatrics, data for the Flemish population are missing. We explored headache-prevalence, and its association with communication-technology (CT) and physical activity (PA) in Flemish children and adolescents. Methods: A cross-sectional exploratory school-based questionnaire study was designed. Flemish boys and girls (5-18 years) completed a symptom-questionnaire. Primary outcomes: sociodemographic background, headache-prevalence, headache-characteristics, CT-use and PA characteristics (self-report). Secondary outcomes: associations between headache-characteristics, age, gender, and CT-use and PA-characteristics. Results: Four hundred twenty-four questionnaires were analysed: 5-7-years: n = 58; 8-11-years: n = 84; 12-15-years: n = 137; 16-18-years: n = 145. Fifty-five percent suffered from headache. Prevalence increased with age. More 16-18-year girls versus boys had headache. CT-use was the main headache-provocateur. Headache prevalence was significantly higher in a frequently physical active population. Conclusion: Our results suggest presence of headache in Flemish children and adolescents. PA-level associates with headache prevalence. However, children and adolescents with headache did not report more CT-use compared to controls.
{"title":"Cross-Sectional Study of Headache in Flemish Children and Adolescents.","authors":"Sarah Mingels, Marita Granitzer","doi":"10.1177/2329048X221140783","DOIUrl":"https://doi.org/10.1177/2329048X221140783","url":null,"abstract":"<p><p><b>Background:</b> Although headache is common in pediatrics, data for the Flemish population are missing. We explored headache-prevalence, and its association with communication-technology (CT) and physical activity (PA) in Flemish children and adolescents. <b>Methods:</b> A cross-sectional exploratory school-based questionnaire study was designed. Flemish boys and girls (5-18 years) completed a symptom-questionnaire. <i>Primary outcomes:</i> sociodemographic background, headache-prevalence, headache-characteristics, CT-use and PA characteristics (self-report). <i>Secondary outcomes</i>: associations between headache-characteristics, age, gender, and CT-use and PA-characteristics. <b>Results:</b> Four hundred twenty-four questionnaires were analysed: 5-7-years: n = 58; 8-11-years: n = 84; 12-15-years: n = 137; 16-18-years: n = 145. Fifty-five percent suffered from headache. Prevalence increased with age. More 16-18-year girls versus boys had headache. CT-use was the main headache-provocateur. Headache prevalence was significantly higher in a frequently physical active population. <b>Conclusion:</b> Our results suggest presence of headache in Flemish children and adolescents. PA-level associates with headache prevalence. However, children and adolescents with headache did not report more CT-use compared to controls.</p>","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":"9 ","pages":"2329048X221140783"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/94/38/10.1177_2329048X221140783.PMC9720830.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10372037","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221083761
A. C. Ng, J. Kassiri, H. Goez, F. Morneau-Jacob, J. Mailo
We describe a unique clinical presentation of a child after the acute phase of herpes simplex virus 1 (HSV1) encephalitis. A 17-month-old boy first presented with HSV1 encephalitis and was promptly treated with antiviral medication. Seven months later, he was re-admitted for startle seizures. Magnetic Resonance Imaging of the brain showed diffuse confluent leukoencephalopathy. This constellation of symptoms has not been previously reported in HSV1 encephalitis. In conclusion, we showed that brain injury due to HSV1 encephalitis can be associated with the development of startle seizures and diffuse white matter injury in the post-acute phase.
{"title":"Startle Seizures and Diffuse Leukoencephalopathy After Resolution of Herpes Simplex Virus 1 Encephalitis in a Child","authors":"A. C. Ng, J. Kassiri, H. Goez, F. Morneau-Jacob, J. Mailo","doi":"10.1177/2329048X221083761","DOIUrl":"https://doi.org/10.1177/2329048X221083761","url":null,"abstract":"We describe a unique clinical presentation of a child after the acute phase of herpes simplex virus 1 (HSV1) encephalitis. A 17-month-old boy first presented with HSV1 encephalitis and was promptly treated with antiviral medication. Seven months later, he was re-admitted for startle seizures. Magnetic Resonance Imaging of the brain showed diffuse confluent leukoencephalopathy. This constellation of symptoms has not been previously reported in HSV1 encephalitis. In conclusion, we showed that brain injury due to HSV1 encephalitis can be associated with the development of startle seizures and diffuse white matter injury in the post-acute phase.","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46558046","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}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221082753
R. Pearson, C. Sheridan, Kaylee Kang, A. Brown, Michael Baham, R. Asarnow, C. Giza, M. Choe
Background: Orthostatic tachycardia (OT) affects some patients after concussion/mild traumatic brain injury (mTBI). In this study, we sought to identify the factors associated with increased risk for OT in patients with mTBI. Methods: We conducted a retrospective review of 268 patients (8-25 years) with mTBI/concussion to determine the prevalence of OT, defined as orthostatic heart rate change ≥40 bpm for those ≤19 years of age and ≥30 bpm on active standing test for those >19 years of age. Results: Among the study population, 7% (n = 19) exhibited post-concussive OT. The only significant difference between OT and non-OT groups was that history of prior concussion was more prevalent in the OT group. Conclusion: A substantial subset (7%) of concussion clinic patients exhibit OT. While POTS literature describes female and adolescent predominance, post-concussive OT had similar prevalence across age and gender groups in this study, suggesting that it may be distinct from POTS.
{"title":"Post-Concussive Orthostatic Tachycardia is Distinct from Postural Orthostatic Tachycardia Syndrome (POTS) in Children and Adolescents","authors":"R. Pearson, C. Sheridan, Kaylee Kang, A. Brown, Michael Baham, R. Asarnow, C. Giza, M. Choe","doi":"10.1177/2329048X221082753","DOIUrl":"https://doi.org/10.1177/2329048X221082753","url":null,"abstract":"Background: Orthostatic tachycardia (OT) affects some patients after concussion/mild traumatic brain injury (mTBI). In this study, we sought to identify the factors associated with increased risk for OT in patients with mTBI. Methods: We conducted a retrospective review of 268 patients (8-25 years) with mTBI/concussion to determine the prevalence of OT, defined as orthostatic heart rate change ≥40 bpm for those ≤19 years of age and ≥30 bpm on active standing test for those >19 years of age. Results: Among the study population, 7% (n = 19) exhibited post-concussive OT. The only significant difference between OT and non-OT groups was that history of prior concussion was more prevalent in the OT group. Conclusion: A substantial subset (7%) of concussion clinic patients exhibit OT. While POTS literature describes female and adolescent predominance, post-concussive OT had similar prevalence across age and gender groups in this study, suggesting that it may be distinct from POTS.","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":"9 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41414375","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}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221114970
M. Schmaedick, Devin Midura, Claire Gerall, D. Garey, W. Middlesworth, J. Bain
Objective: Extracorporeal membrane oxygenation (ECMO) is a lifesaving measure for patients in cardiac or respiratory failure. Extracorporeal cardiopulmonary resuscitation (ECPR) is emergent ECMO cannulation during cardiac arrest. All ECMO patients are at high risk for neurologic complications, but the degree of risk of ECPR relative to ECMO without CPR in progress (non-ECPR ECMO) is not well documented in infants. The goal of the present study is to compare neurologic complication rates between infants who underwent ECPR and those who underwent non-ECPR ECMO. Methods: We performed a retrospective chart review on all patients admitted between 2009 and 2020 to the neonatal intensive care unit (NICU) in our quaternary children's hospital. We separated patients by ECPR vs. non-ECPR ECMO cannulation. We compared rates of death and used neuroimaging and video electroencephalogram (vEEG) to determine incidence of stroke, intracranial hemorrhage, and seizure. Chi-square and Fisher's exact tests were used to compare these categorical variables among groups.Results: A total of 181 infants were cannulated onto ECMO. Of these, 40 received ECPR, 56 received non-ECPR ECMO for a cardiac indication, and 85 received non-ECPR ECMO for a respiratory indication. After excluding patients currently admitted (n=1, ECPR), 180 patients were subjected to analysis. ECPR patients were less likely to survive to hospital discharge than patients who underwent non-ECPR ECMO for respiratory indications, and less likely to survive without any neurologic complication compared with infants who underwent non-ECPR ECMO for cardiac or respiratory indications. Interpretation: Significantly fewer ECPR patients survived without experiencing a neurologic complication, compared with non-ECPR ECMO patients.
{"title":"Neurologic Complications of Extracorporeal Cardiopulmonary Resuscitation in Neonates and Infants","authors":"M. Schmaedick, Devin Midura, Claire Gerall, D. Garey, W. Middlesworth, J. Bain","doi":"10.1177/2329048X221114970","DOIUrl":"https://doi.org/10.1177/2329048X221114970","url":null,"abstract":"Objective: Extracorporeal membrane oxygenation (ECMO) is a lifesaving measure for patients in cardiac or respiratory failure. Extracorporeal cardiopulmonary resuscitation (ECPR) is emergent ECMO cannulation during cardiac arrest. All ECMO patients are at high risk for neurologic complications, but the degree of risk of ECPR relative to ECMO without CPR in progress (non-ECPR ECMO) is not well documented in infants. The goal of the present study is to compare neurologic complication rates between infants who underwent ECPR and those who underwent non-ECPR ECMO. Methods: We performed a retrospective chart review on all patients admitted between 2009 and 2020 to the neonatal intensive care unit (NICU) in our quaternary children's hospital. We separated patients by ECPR vs. non-ECPR ECMO cannulation. We compared rates of death and used neuroimaging and video electroencephalogram (vEEG) to determine incidence of stroke, intracranial hemorrhage, and seizure. Chi-square and Fisher's exact tests were used to compare these categorical variables among groups.Results: A total of 181 infants were cannulated onto ECMO. Of these, 40 received ECPR, 56 received non-ECPR ECMO for a cardiac indication, and 85 received non-ECPR ECMO for a respiratory indication. After excluding patients currently admitted (n=1, ECPR), 180 patients were subjected to analysis. ECPR patients were less likely to survive to hospital discharge than patients who underwent non-ECPR ECMO for respiratory indications, and less likely to survive without any neurologic complication compared with infants who underwent non-ECPR ECMO for cardiac or respiratory indications. Interpretation: Significantly fewer ECPR patients survived without experiencing a neurologic complication, compared with non-ECPR ECMO patients.","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46908409","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}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221146982
Mekka R Garcia, Lena Bell, Claire Miller, Devorah Segal
The incidence of childhood central nervous system tumors in infants is about 6 per 100 000 children. Recent studies have showed recurrent fusion of the neurotrophic tyrosine receptor kinase (NTRK) gene in 10% of non-brainstem high grade glioma in very young children suggesting an oncogenic effect of the NTRK fusion genes. In this report, we present a rare, severe case of a full-term neonate who was noted to have widely splayed sutures and a bulging fontanelle at birth who was found to have infant-type hemispheric glioma with NTRK1 fusion with course complicated by seizures refractory to medical treatment. Patient was deemed a poor surgical candidate due to the size of the mass and thus parents opted for comfort care.
{"title":"A Case of Infant-Type Hemispheric Glioma with <i>NTRK1</i> Fusion.","authors":"Mekka R Garcia, Lena Bell, Claire Miller, Devorah Segal","doi":"10.1177/2329048X221146982","DOIUrl":"https://doi.org/10.1177/2329048X221146982","url":null,"abstract":"<p><p>The incidence of childhood central nervous system tumors in infants is about 6 per 100 000 children. Recent studies have showed recurrent fusion of the neurotrophic tyrosine receptor kinase <i>(NTRK)</i> gene in 10% of non-brainstem high grade glioma in very young children suggesting an oncogenic effect of the <i>NTRK</i> fusion genes. In this report, we present a rare, severe case of a full-term neonate who was noted to have widely splayed sutures and a bulging fontanelle at birth who was found to have infant-type hemispheric glioma with <i>NTRK1</i> fusion with course complicated by seizures refractory to medical treatment. Patient was deemed a poor surgical candidate due to the size of the mass and thus parents opted for comfort care.</p>","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":"9 ","pages":"2329048X221146982"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/81/47/10.1177_2329048X221146982.PMC9806371.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10481469","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221093173
Veronica Birca, K. Myers
Background: Distal 7q11.23 deletions are variably associated with epilepsy, intellectual disability and neurobehavioural abnormalities. The relative importance of different genes in this region in contributing to different phenotypes is not clear, though HIP1 and YWHAG are both thought to play important roles. Patients and Methods: We performed thorough phenotyping on members of a family in which multiple members carried a relatively small 0.8 Mb distal 7q11.23 deletion, affecting 17 genes. Results: Two brothers and a half-brother had all inherited the 7q11.23 deletion from their mother. The eldest two both had global developmental impairment and genetic generalized epilepsy, involving absence, myoclonic or myoclonic-atonic seizures. There was no history of seizures in the mother or her youngest son, but both also had developmental impairment. Conclusion: Distal 7q11.23 deletions affecting HIP1 and YWHAG may cause developmental impairment and genetic generalized epilepsy, with considerable intrafamilial phenotypic variability.
{"title":"Genetic Generalized Epilepsy and Intrafamilial Phenotypic Variability with Distal 7q11.23 Deletion","authors":"Veronica Birca, K. Myers","doi":"10.1177/2329048X221093173","DOIUrl":"https://doi.org/10.1177/2329048X221093173","url":null,"abstract":"Background: Distal 7q11.23 deletions are variably associated with epilepsy, intellectual disability and neurobehavioural abnormalities. The relative importance of different genes in this region in contributing to different phenotypes is not clear, though HIP1 and YWHAG are both thought to play important roles. Patients and Methods: We performed thorough phenotyping on members of a family in which multiple members carried a relatively small 0.8 Mb distal 7q11.23 deletion, affecting 17 genes. Results: Two brothers and a half-brother had all inherited the 7q11.23 deletion from their mother. The eldest two both had global developmental impairment and genetic generalized epilepsy, involving absence, myoclonic or myoclonic-atonic seizures. There was no history of seizures in the mother or her youngest son, but both also had developmental impairment. Conclusion: Distal 7q11.23 deletions affecting HIP1 and YWHAG may cause developmental impairment and genetic generalized epilepsy, with considerable intrafamilial phenotypic variability.","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41985770","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}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221105743
Lindsay Schleifer, S. Vogel, A. Arun, Y. Lum, Courtney E. Lawrence, F. Hui, Lisa R. Sun
Arterial thoracic outlet syndrome is a rare condition characterized by compression of the subclavian artery, often with post-stenotic aneurysm formation. Artery-to-artery embolic strokes related to thoracic outlet syndrome have been reported in the posterior circulation and in the ipsilateral anterior circulation. We present a case in which a thrombus secondary to thoracic outlet syndrome caused a contralateral anterior circulation stroke in an adolescent and postulate mechanisms of this rare occurrence. This case demonstrates that a subclavian thrombus due to thoracic outlet syndrome can take a circuitous path and cause an anterior circulation stroke contralateral to the diseased subclavian artery. In addition, this case illustrates the importance of a high index of suspicion for thoracic outlet syndrome in patients with stroke and associated arm pain or discoloration.
{"title":"Stroke Caused by Arterial Thoracic Outlet Syndrome in an Adolescent","authors":"Lindsay Schleifer, S. Vogel, A. Arun, Y. Lum, Courtney E. Lawrence, F. Hui, Lisa R. Sun","doi":"10.1177/2329048X221105743","DOIUrl":"https://doi.org/10.1177/2329048X221105743","url":null,"abstract":"Arterial thoracic outlet syndrome is a rare condition characterized by compression of the subclavian artery, often with post-stenotic aneurysm formation. Artery-to-artery embolic strokes related to thoracic outlet syndrome have been reported in the posterior circulation and in the ipsilateral anterior circulation. We present a case in which a thrombus secondary to thoracic outlet syndrome caused a contralateral anterior circulation stroke in an adolescent and postulate mechanisms of this rare occurrence. This case demonstrates that a subclavian thrombus due to thoracic outlet syndrome can take a circuitous path and cause an anterior circulation stroke contralateral to the diseased subclavian artery. In addition, this case illustrates the importance of a high index of suspicion for thoracic outlet syndrome in patients with stroke and associated arm pain or discoloration.","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41676421","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}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221079093
Eve Kroenke, Alex Ankar, Nikita Malani Shukla
Background: MOG antibody associated demyelinating disease (MOGAD) is a newly described autoimmune disorder that presents with monophasic or multiphasic demyelination in children. Case: We report a case of MOGAD that was refractory to current treatment algorithms and required rapid escalation of immunotherapy to achieve disease control. Conclusion: This case helps to further expand the phenotype of MOGAD and emphasizes the need to consider MOGAD in patients presenting with focal neurologic deficits, altered mental status, and/or seizures.
{"title":"Refractory MOG-Associated Demyelinating Disease in a Pediatric Patient","authors":"Eve Kroenke, Alex Ankar, Nikita Malani Shukla","doi":"10.1177/2329048X221079093","DOIUrl":"https://doi.org/10.1177/2329048X221079093","url":null,"abstract":"Background: MOG antibody associated demyelinating disease (MOGAD) is a newly described autoimmune disorder that presents with monophasic or multiphasic demyelination in children. Case: We report a case of MOGAD that was refractory to current treatment algorithms and required rapid escalation of immunotherapy to achieve disease control. Conclusion: This case helps to further expand the phenotype of MOGAD and emphasizes the need to consider MOGAD in patients presenting with focal neurologic deficits, altered mental status, and/or seizures.","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42988850","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}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221105960
WajdA Alotaibi, S. Bashir, A. Mir
We report an interesting case of a young girl with LGI1-antibody encephalitis who presented at 7 years old with very frequent seizures and severe neurocognitive decline. She responded very well to high dose corticosteroids and intravenous immunoglobulin (IVIG) initially but relapsed after 7 months. The relapse included frequent faciobrachial dystonic seizures (FBDS) that were successfully treated with rituximab. This case report highlights a few important points about LGI1-antibody encephalitis in children to help clinicians recognize this condition early and start prompt treatment with immunosuppressants. Data is lacking about LGI1-antibody encephalitis in children as it is mostly reported in adults. Our patient presented with frequent drug-resistant seizures including FBDS, along with amnesia, confusion, medial temporal lobe involvement, and hyponatremia similar to the presentation in adults. In contrast, none of the patients in the recent systematic review had FBDS or hyponatremia, making our case unique and suggesting variability in clinical presentation in children similar to adults. To our knowledge, FBDS have never been reported in children and our patient was initially misdiagnosed as having Childhood Epilepsy with Centrotemporal spikes. Since receiving rituximab, our patient is seizure-free for 1 year and 9 months and was successfully weaned of topiramate. She is going to school and has normal attention, concentration, memory, and mood. We propose early consideration of rituximab to accelerate recovery and prevent relapse.
{"title":"Faciobrachial Dystonic Seizures as a Sign of Relapse in a Child with LGI-1 Encephalitis","authors":"WajdA Alotaibi, S. Bashir, A. Mir","doi":"10.1177/2329048X221105960","DOIUrl":"https://doi.org/10.1177/2329048X221105960","url":null,"abstract":"We report an interesting case of a young girl with LGI1-antibody encephalitis who presented at 7 years old with very frequent seizures and severe neurocognitive decline. She responded very well to high dose corticosteroids and intravenous immunoglobulin (IVIG) initially but relapsed after 7 months. The relapse included frequent faciobrachial dystonic seizures (FBDS) that were successfully treated with rituximab. This case report highlights a few important points about LGI1-antibody encephalitis in children to help clinicians recognize this condition early and start prompt treatment with immunosuppressants. Data is lacking about LGI1-antibody encephalitis in children as it is mostly reported in adults. Our patient presented with frequent drug-resistant seizures including FBDS, along with amnesia, confusion, medial temporal lobe involvement, and hyponatremia similar to the presentation in adults. In contrast, none of the patients in the recent systematic review had FBDS or hyponatremia, making our case unique and suggesting variability in clinical presentation in children similar to adults. To our knowledge, FBDS have never been reported in children and our patient was initially misdiagnosed as having Childhood Epilepsy with Centrotemporal spikes. Since receiving rituximab, our patient is seizure-free for 1 year and 9 months and was successfully weaned of topiramate. She is going to school and has normal attention, concentration, memory, and mood. We propose early consideration of rituximab to accelerate recovery and prevent relapse.","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49427675","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}
Pub Date : 2022-01-01DOI: 10.1177/2329048X221143689
Fernando Galan, Douglas R Nordli, Milad Yazdani, Jessica Klein
In current literature, there is uncertainty in the pathophysiology and management of influenza-associated Acute Necrotizing Encephalitis. Because of this and the rarity of the disease, no clear treatment guidelines exist. It is thought that treatment after 24 h of symptom onset or known brainstem involvement are poor predictors of outcome. Here, we present a case that provides support for aggressive management of the inflammatory cascade with combination high-dose steroid, immunoglobulin, and anti-viral therapy with oseltamivir despite initiation after 24 h from symptom onset, brainstem involvement, and a pathogenic RANBP2 gene mutation which mechanistically increases oxidative stress, cytokine effects, and possibly viral invasion into brain tissue and vasculature.
{"title":"A Favorable Treatment Outcome in <i>RANBP2</i> and Influenza Associated Acute Necrotizing Encephalitis.","authors":"Fernando Galan, Douglas R Nordli, Milad Yazdani, Jessica Klein","doi":"10.1177/2329048X221143689","DOIUrl":"https://doi.org/10.1177/2329048X221143689","url":null,"abstract":"<p><p>In current literature, there is uncertainty in the pathophysiology and management of influenza-associated Acute Necrotizing Encephalitis. Because of this and the rarity of the disease, no clear treatment guidelines exist. It is thought that treatment after 24 h of symptom onset or known brainstem involvement are poor predictors of outcome. Here, we present a case that provides support for aggressive management of the inflammatory cascade with combination high-dose steroid, immunoglobulin, and anti-viral therapy with oseltamivir despite initiation after 24 h from symptom onset, brainstem involvement, and a pathogenic <i>RANBP2</i> gene mutation which mechanistically increases oxidative stress, cytokine effects, and possibly viral invasion into brain tissue and vasculature.</p>","PeriodicalId":72572,"journal":{"name":"Child neurology open","volume":"9 ","pages":"2329048X221143689"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/38/e4/10.1177_2329048X221143689.PMC9755544.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10391024","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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