Background: Pediatric epileptic encephalopathies comprises a group of severe childhood epilepsy syndromes characterized by refractory seizures associated with progressive cerebral dysfunction, manifesting as cognitive or learning difficulties and behavioural or sensory impairments. Objectives: To describe the clinical and electroencephagraphic profile of children seen with epileptic encephalopathy in Sokoto, Northwestern Nigeria. Methodology: This is a descriptive report of cases of epileptic encephalopathy seen in a Pediatric Neurology Clinic, over a 5-year period (Jan. 2015 to Dec. 2020). Results: Twenty-one cases that satisfied the definition criteria for childhood epileptic encephalopathy where identified over the study period, out of a total number 413 children diagnosed with epilepsy over the period, giving a hospital prevalence of 5.1%. Three of the cases were excluded due to incomplete data. Four (4) different syndromes of EE were recorded, namely: Epileptic spasms/West syndrome, Lennox-Gastaut syndrome, Doose syndrome and Landau-Kleffner syndrome. Epileptic spasm was the most prevalent type recorded in 7 (38.9%) cases, followed by Lennox-Gastaut syndrome in 6 (33.3%), Landau-Kleffner syndrome in 3 (16.7%) and Doose Syndrome in 2 (11.1%) in that order. The mean age at diagnosis was 17.00 ± 6.50 months, with a male to female ratio of 1.6:1. There was a significant association between age of seizure onset and the type of seizure syndrome. Conclusion: Pediatric epileptic encephalopathies are prevalent in our society, with epileptic spasms/West syndrome and Lennox-Gastaut syndromes leading the list. Perinatal asphyxia was the main risk factor identified among the cases. epileptiform abnormalities and other nonspecific EEG findings.
{"title":"Childhood Epileptic Encephalopathy in Sokoto, Northwestern Nigeria","authors":"Ahmad Mm, Ahmed H","doi":"10.36959/595/430","DOIUrl":"https://doi.org/10.36959/595/430","url":null,"abstract":"Background: Pediatric epileptic encephalopathies comprises a group of severe childhood epilepsy syndromes characterized by refractory seizures associated with progressive cerebral dysfunction, manifesting as cognitive or learning difficulties and behavioural or sensory impairments. Objectives: To describe the clinical and electroencephagraphic profile of children seen with epileptic encephalopathy in Sokoto, Northwestern Nigeria. Methodology: This is a descriptive report of cases of epileptic encephalopathy seen in a Pediatric Neurology Clinic, over a 5-year period (Jan. 2015 to Dec. 2020). Results: Twenty-one cases that satisfied the definition criteria for childhood epileptic encephalopathy where identified over the study period, out of a total number 413 children diagnosed with epilepsy over the period, giving a hospital prevalence of 5.1%. Three of the cases were excluded due to incomplete data. Four (4) different syndromes of EE were recorded, namely: Epileptic spasms/West syndrome, Lennox-Gastaut syndrome, Doose syndrome and Landau-Kleffner syndrome. Epileptic spasm was the most prevalent type recorded in 7 (38.9%) cases, followed by Lennox-Gastaut syndrome in 6 (33.3%), Landau-Kleffner syndrome in 3 (16.7%) and Doose Syndrome in 2 (11.1%) in that order. The mean age at diagnosis was 17.00 ± 6.50 months, with a male to female ratio of 1.6:1. There was a significant association between age of seizure onset and the type of seizure syndrome. Conclusion: Pediatric epileptic encephalopathies are prevalent in our society, with epileptic spasms/West syndrome and Lennox-Gastaut syndromes leading the list. Perinatal asphyxia was the main risk factor identified among the cases. epileptiform abnormalities and other nonspecific EEG findings.","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"22 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123021777","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}
Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome associated with certain underlying conditions or medications, with a spectrum of neurologic findings and characteristic neuroimaging. We describe a case of PRES in a 3-year-old girl positive for SARS-CoV-2 with no other underlying risk factors for the development of this syndrome. We review and suggest plausible mechanisms of neurologic involvement in patients positive for SARS-CoV-2.
{"title":"Posterior Reversible Encephalopathy Syndrome in a Pediatric Patient with SARS-CoV-2 Infection","authors":"Mathew Anita, Sultan Richard, Ghersin Zelda J","doi":"10.36959/595/433","DOIUrl":"https://doi.org/10.36959/595/433","url":null,"abstract":"Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome associated with certain underlying conditions or medications, with a spectrum of neurologic findings and characteristic neuroimaging. We describe a case of PRES in a 3-year-old girl positive for SARS-CoV-2 with no other underlying risk factors for the development of this syndrome. We review and suggest plausible mechanisms of neurologic involvement in patients positive for SARS-CoV-2.","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"122 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115608032","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}
Intellectual disability and developmental delay are is common presentations encountered in pediatric clinical practice. Etiology of these illnesses is diverse. Subtle clinical markers may act as pointers to underlying genetic etiology in some of these conditions. Due to the increased availability of genetic tests. There is a robust role of genetic counseling in many of these conditions. However, investigations need to be judiciously advised to balance the anticipated gains with the expenses, more so in resource-limited settings.
{"title":"Antenatal Counselling in Developmental Delay and Intellectual Disability: Case Series","authors":"Bhatt Reema, Puri Ratna Dua, G. Narayan","doi":"10.36959/595/428","DOIUrl":"https://doi.org/10.36959/595/428","url":null,"abstract":"Intellectual disability and developmental delay are is common presentations encountered in pediatric clinical practice. Etiology of these illnesses is diverse. Subtle clinical markers may act as pointers to underlying genetic etiology in some of these conditions. Due to the increased availability of genetic tests. There is a robust role of genetic counseling in many of these conditions. However, investigations need to be judiciously advised to balance the anticipated gains with the expenses, more so in resource-limited settings.","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"51 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"121054905","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}
Declusin Anthony, Bricklin Laura, Tanverdi Melisa, Whitelaw Andrew, Brajcich Michelle, S. John, D. Lauren, Breitnauer Nicholas, Stence Nicholas, M. William, Palat Padmini
Coronavirus disease 2019 (COVID-19) has been associated with a variety of neurologic manifestations, including Guillain-Barre syndrome (GBS), but few pediatric cases have been reported. We would like to present a pediatric case of GBS in association with a recent COVID-19 infection that presented with an isolated clinical facial nerve palsy.
{"title":"Guillain-Barré Syndrome Triggered by COVID-19: A Pediatric Case Report and Literature Review","authors":"Declusin Anthony, Bricklin Laura, Tanverdi Melisa, Whitelaw Andrew, Brajcich Michelle, S. John, D. Lauren, Breitnauer Nicholas, Stence Nicholas, M. William, Palat Padmini","doi":"10.36959/595/427","DOIUrl":"https://doi.org/10.36959/595/427","url":null,"abstract":"Coronavirus disease 2019 (COVID-19) has been associated with a variety of neurologic manifestations, including Guillain-Barre syndrome (GBS), but few pediatric cases have been reported. We would like to present a pediatric case of GBS in association with a recent COVID-19 infection that presented with an isolated clinical facial nerve palsy.","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"21 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125632389","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}
Sardarzada Javid, Dilara Mat Sibel Öz, Öncel İbrahim, Düzova Ali, A. Banu
The posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity caused by impairment of cerebral autoregulation leading to vasogenic edema. Common etiologies include immunosuppressive drugs, autoimmune disorders and hypertension.
{"title":"Posterior Reversible Encephalopathy Syndrome Caused by Autonomic Dysreflexia in an Adolescent after Spinal Cord Injury","authors":"Sardarzada Javid, Dilara Mat Sibel Öz, Öncel İbrahim, Düzova Ali, A. Banu","doi":"10.36959/595/426","DOIUrl":"https://doi.org/10.36959/595/426","url":null,"abstract":"The posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity caused by impairment of cerebral autoregulation leading to vasogenic edema. Common etiologies include immunosuppressive drugs, autoimmune disorders and hypertension.","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"458 ","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-08-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"133880495","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}
An 11-year-old Colombian female on isoniazid for recently diagnosed latent tuberculosis presented to the emergency department after a first-time, generalized tonic-clonic seizure. She exhibited mild bilateral hypometria on finger-to-nose testing, lower limb ataxia on heel-to-shin testing, and dysmetria on rhythmic finger tapping. MRI brain revealed symmetric T2/FLAIR hyper intensities with diffusion restriction and apparent diffusion coefficient correlate in bilateral dentate nuclei (Figure 1A, Figure 1B, Figure 1C).
{"title":"Reversible Signal Abnormalities in Bilateral Dentate Nuclei Secondary to Isoniazid Neurotoxicity","authors":"CaragherS, J. McLaren, D. Balaban","doi":"10.36959/595/424","DOIUrl":"https://doi.org/10.36959/595/424","url":null,"abstract":"An 11-year-old Colombian female on isoniazid for recently diagnosed latent tuberculosis presented to the emergency department after a first-time, generalized tonic-clonic seizure. She exhibited mild bilateral hypometria on finger-to-nose testing, lower limb ataxia on heel-to-shin testing, and dysmetria on rhythmic finger tapping. MRI brain revealed symmetric T2/FLAIR hyper intensities with diffusion restriction and apparent diffusion coefficient correlate in bilateral dentate nuclei (Figure 1A, Figure 1B, Figure 1C).","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"13 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127559118","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}
A 10.5-year-old girl presented with abnormal movements and behavioural problems for three months. initially she had lip-twitching and eye-blinking during wakefulness. After six weeks, she started having shoulder-shrugs, neck-turning, throat-clearing at a frequency of 10-20/hour, exacerbated by stress. In addition, she exhibited temper-tantrums, unprovoked crying and scholastic deterioration.
{"title":"Tourette Syndrome Mimic: An Atypical Presentation of Subacute Sclerosing Panencephalitis","authors":"P. Sandeep, G. Narayan","doi":"10.36959/595/423","DOIUrl":"https://doi.org/10.36959/595/423","url":null,"abstract":"A 10.5-year-old girl presented with abnormal movements and behavioural problems for three months. initially she had lip-twitching and eye-blinking during wakefulness. After six weeks, she started having shoulder-shrugs, neck-turning, throat-clearing at a frequency of 10-20/hour, exacerbated by stress. In addition, she exhibited temper-tantrums, unprovoked crying and scholastic deterioration.","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114799750","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}
A 21-year-old male with history of refractory epilepsy presented for long-term video EEG monitoring. His home antiepileptic medications were weaned off over a seven-day period. On hospital day 4, he had a right temporal electro clinical seizure with secondary generalization and a second right temporal electrographic seizure. On hospital day 8, MRI brain identified an ovoid circumscribed lesion in the splenium of the corpus callosum which had not been present on prior scans (Figure 1).
{"title":"Reversible MRI Changes in the Splenium Related to Recent Cessation of Antiepileptic Medications","authors":"J. McLaren, A. Misra, Cjiang-Liang Chu","doi":"10.36959/595/425","DOIUrl":"https://doi.org/10.36959/595/425","url":null,"abstract":"A 21-year-old male with history of refractory epilepsy presented for long-term video EEG monitoring. His home antiepileptic medications were weaned off over a seven-day period. On hospital day 4, he had a right temporal electro clinical seizure with secondary generalization and a second right temporal electrographic seizure. On hospital day 8, MRI brain identified an ovoid circumscribed lesion in the splenium of the corpus callosum which had not been present on prior scans (Figure 1).","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"14 2","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114006955","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}
One hypothesis is that the activation of innate immunity and release of cytokines breaks down the blood brain barrier (BBB) and causes neuroinflammation. Here, I present a case of COVID-19 associated MIS-C (multisystem inflammatory syndrome in children) with neurological complications manifesting as encephalopathy and a cytotoxic lesion of the corpus callosum (CLOCC) on the brain MRI, in association with extremely elevated levels of circulating interleukin-6 (IL-6), and soluble interleukin-2 receptor (sIL2R).
{"title":"Encephalopathy and Cytotoxic Lesion of the Corpus Callosum Associated with Cytokine Storm in COVID-19: A Case Report","authors":"Duyu Nie","doi":"10.36959/595/422","DOIUrl":"https://doi.org/10.36959/595/422","url":null,"abstract":"One hypothesis is that the activation of innate immunity and release of cytokines breaks down the blood brain barrier (BBB) and causes neuroinflammation. Here, I present a case of COVID-19 associated MIS-C (multisystem inflammatory syndrome in children) with neurological complications manifesting as encephalopathy and a cytotoxic lesion of the corpus callosum (CLOCC) on the brain MRI, in association with extremely elevated levels of circulating interleukin-6 (IL-6), and soluble interleukin-2 receptor (sIL2R).","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"30 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-07-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128427328","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}
Growing skull fracture (GSF) is a well-known complication of pediatric cranial trauma but its physiopathology has been, so far, only assumed. When the basic laws of hydraulics are applied to the underlying GSF pathology, a clearer understanding of the process involved in it emerges and accounts for the frequent spontaneous arrest of the skull erosion. Moreover the process causing the commonly associated intra-axial pathology is also better defined.
{"title":"Growing Skull Fracture [GSF], a Hydrodynamic Perspective: An Original Article","authors":"A. Drapkin","doi":"10.36959/595/421","DOIUrl":"https://doi.org/10.36959/595/421","url":null,"abstract":"Growing skull fracture (GSF) is a well-known complication of pediatric cranial trauma but its physiopathology has been, so far, only assumed. When the basic laws of hydraulics are applied to the underlying GSF pathology, a clearer understanding of the process involved in it emerges and accounts for the frequent spontaneous arrest of the skull erosion. Moreover the process causing the commonly associated intra-axial pathology is also better defined.","PeriodicalId":432995,"journal":{"name":"Journal of Pediatric Neurology and Neuroscience","volume":"28 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-06-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"123191807","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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