Complex Febrile Seizures Research Articles

Exploring the Age-Old Question: What Is the Predictive Value of EEG for Future Epilepsy in Children With Complex Febrile Seizures?

Children with complex febrile seizures (CFS) have increased risk for the development of epilepsy, but varying prognostic value has been ascribed to abnormal post-CFS electroencephalograms (EEGs). We conducted a retrospective cohort study of 621 children with post-CFS EEGs and identified an association between CFS and midline-vertex discharges, which were present in 52% of the 56 EEGs with interictal epileptiform discharges. Among patients who completed at least 1 year of follow-up, 24.7% subsequently developed epilepsy. Most patients had normal EEGs but 20% had interictal epileptiform discharges. Midline-vertex discharges were seen at a similar rate in children who did not develop epilepsy (55%) and those who developed epilepsy (45%). The development of epilepsy was not associated with any interictal epileptiform discharge localization. Logistic regression modeling identified 4 predictors of future epilepsy: >3 febrile seizures in 24 hours, interictal epileptiform discharges during post-CFS EEG, family history of afebrile seizures, and age of CFS onset ≥ 3 years.

Clinical, radiological and electrophysiological predictors for drug-resistant epilepsy

BackgroundEpilepsy is the third chronic brain illness worldwide. About a third of the epileptic patients will be drug resistant. Early identification of these patients is critical for appropriate treatment selection and prevention of the devastating consequences of recurrent seizures. The objective of this study aims to detect clinical, electrophysiological, and radiological predictors for drug-resistant epilepsy patients.ResultsOne hundred fifty-five patients were included in this study, divided into a well-controlled epilepsy group (103 patients) and a drug-resistant group (52 patients). Both groups were compared regarding clinical, electrophysiological, and neuro-radiological data. Younger age at onset, history of delayed milestones, history of perinatal insult (especially hypoxia), mental retardation, neurological deficits, depression, status epilepticus (SE), complex febrile seizures, focal seizure to bilateral tonic–clonic convulsion as well as multiple seizures and high seizure frequency (daily) at onset, poor response to first anti-seizure drug (ASD), structural and metabolic etiology, abnormal brain imaging, and slow background and multifocal epileptiform discharges in EEG were significant risk factors for the development of drug-resistant epilepsy.ConclusionMRI abnormalities are the most significant predictor for drug-resistant epilepsy. Drug-resistant epilepsy is associated with clinical, electrophysiological, and radiological risk factors that can be used to diagnose drug-resistant patients early and choose the best treatment option and time.

Evaluation and assessment of clinical and demographic profile, aetiology and outcome of status epilepticus in paediatric patients

Background: Status epilepticus (SE) is one of the most frequent neurological emergencies that require prompt diagnosis and treatment. If not managed promptly, it may result in significant neurological morbidity and mortality. Methods: It is prospective observational study. Study for clinical and demographic profile, aetiology, outcome in patient of status epilepticus. Results: Occurrence of SE is in indoor paediatric patients was 2.05%. Male and female M: F ratio is 1.3:1. Fever was common associated symptoms in SE. The commonest type of seizure was generalised tonic-clonic seizure (GTCS). Infective etiology was most common amongst patients with SE. secondary epilepsy with CP was second most common cause followed by complex febrile seizure and primary epilepsy. In infants, metabolic etiology was the most common cause for SE. Majority of seizures were controlled only by benzodiazepines. Requirement of midazolam infusion was only in few patients and requirement of anesthetic agent was rare. Mechanical ventilation was frequently required in patients with refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE). Mortality was almost same in all age groups with SE. Mortality was directly proportional to duration of the SE and was highest in patient having >30 minutes of duration of seizure. Conclusions: SE was more commonly associated with male gender and age <5 years. By improving hygiene, nutritional and vaccination status, overcrowding and education, we can reduce occurrence of SE, by reducing infections. Mortality can be reduced by immediate control of seizure and improving intensive care facilities.

Comparison of Serum Selenium, Homocysteine, Zinc, and Vitamin D Levels in Febrile Children with and without Febrile Seizures: A Prospective Single-Center Study.

Febrile seizure is a complication that makes physicians and families uneasy when detected in children with a high fevers. This study aimed to compare children with febrile seizures and children without seizures in blood selenium, zinc, homocysteine, vitamin D, vitamin B12, and magnesium levels. The study group included sixty-one children between the ages of 1-5 who came to the pediatric emergency department with febrile seizure. The control group had 61 children with fever without seizure, who were compatible with the study group in age, sex, and elapsed time since the onset of fever. Blood samples were taken from the patients during their admission. Selenium, zinc, vitamin D, homocysteine, vitamin B12, and magnesium levels were measured, and the data of the two groups were compared. Additionally, patients in the study group had two subgroups, simple and complex febrile seizures, and their parameters were compared. Selenium, zinc, vitamin D, and vitamin B12 levels were significantly lower in the study group than in the control group (p < 0.001), and there was no significant difference in homocysteine (p = 0.990) and magnesium levels (p = 0.787) between the two groups. Moreover, no significant difference was found between those with simple and complex febrile seizures in selenium, vitamin D, homocysteine, vitamin B12, and magnesium levels. Elevated levels of selenium, zinc, vitamin D, and vitamin B12 in the blood of children with fevers help to prevent febrile seizures.

Association between high-mobility group box 1 levels and febrile seizures in children: a systematic review and meta-analysis

The relationship between High-mobility group box 1 (HMGB1) and febrile seizures (FS) in children remains unclear. This study aimed to apply meta-analysis to reveal the correlation between HMGB1 levels and FS in children. Databases including PubMed, EMBASE, Web of science, Cochrane library, CNKI, SinoMed and WanFangData were searched for relevant studies. Pooled standard mean deviation and 95% confidence interval were calculated as effect size since the random-effects model was used when I2 > 50%. Meanwhile, between-study heterogeneity was determined by performing subgroup and sensitivity analyses. A total of 9 studies were finally included. Meta-analysis showed that the children with FS had significantly higher HMGB1 levels compared with healthy children and children with fever but no seizures (P<0.05). Additionally, subgroup analysis showed that the HMGB1 level in children with complex FS was higher than those with simple FS (P<0.05), and children with duration >15 min were higher than those with duration ≤15min (P<0.05). There were no statistical differences between children with or without a family history of FS (P>0.05). Finally, children with FS who converted to epilepsy exhibited higher HMGB1 levels than those who did not convert to epilepsy (P<0.05). The level of HMGB1 may be implicated in the prolongation, recurrence and development of FS in children. Thus, it was necessary to evaluate the precise concentrations of HMGB1 in FS patients and to further determine the various activities of HMGB1 during FS by well-designed, large-scale, and case-controlled trials.

Comparison of Clinical Characteristics of Children Infected With Coronavirus Disease 2019 Between Omicron Variant BA.5 and BA.1/BA.2 in Japan.

The emergence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) variants has dramatically altered the clinical profile of pediatric coronavirus disease 2019 (COVID-19). In Japan, we experienced a pandemic of omicron subvariant BA.1/BA.2 from January through June 2022. However, after the emergence of BA.5 in early July 2022, the number of children hospitalized with COVID-19 increased dramatically in Japan. We collected data on monthly numbers of cases and clinical characteristics of hospitalized children with COVID-19 in 13 hospitals, the total number of pediatric COVID-19 cases, and COVID-19 vaccination rates in Niigata, Japan, for the period from January 2020 through August 2022. We compared clinical presentation during the periods of BA.1/BA.2 predominance (January-June 2022) and BA.5 predominance (July-August 2022) and estimated vaccine effectiveness (VE) against hospitalization during the BA.5-predominant period. Between January 1, 2020, and August 31, 2022, 49,387 children (19,085 children/100,000 population) were newly diagnosed as having COVID-19, and 393 were hospitalized for COVID-19. Hospitalization for febrile seizure, especially complex seizure, was significantly higher during BA.5 predominance than during BA.1/BA.2 predominance (27.9% vs. 7.0%, P < 0.01). VE against hospitalization during BA.5 predominance was estimated to be 75% (95% confidence interval, 48%-88%, P < 0.01). The emergence of BA.5 significantly affected children in Japan; the number with complex febrile seizure who required hospitalization was higher than during BA.1/BA.2 predominance. The COVID-19 vaccination rate in children must be increased to prevent hospitalization for COVID-19 and to prepare for current and future variant outbreaks.

Incidence of Febrile Seizures in Children with COVID-19.

The coronavirus disease 2019 (COVID-19) has become a common cause of febrile seizures (FS), especially after the Omicron surge. This study aimed to determine the incidence of COVID-19-associated FS in children. The number of confirmed COVID-19 cases in patients aged below five years residing in the Jeonbuk province from January 2020 to June 2022 was obtained from official data provided by the Ministry of Public Administration and Security. During the same period, data on FS patients with COVID-19 were obtained from all local hospitals capable of FS treatment and were analyzed retrospectively. The number of children under five years of age in Jeonbuk was 62,772, of which 33,457 (53.2%) were diagnosed with COVID-19 during the study period. Of these, 476 patients (1.4%) required hospitalization, and 64 (0.19%, 44 boys; 68.8%: 20 girls; 31.2%) developed FS. All patients with FS presented with symptoms after the Omicron surge. Before the Omicron variant, 23.4% of the patients (89 of 381) required hospitalization; however, no children with COVID-19 were hospitalized for FS. Twenty-five patients (39.1%) had complex FS while one (1.6%) presented with febrile status epilepticus. Forty-two patients (65.6%) experienced first-time FS with an average of 1.5 convulsive events.

712 - Complex febrile seizures – are often not so simple.

712 - Complex febrile seizures – are often not so simple.

Clinical and laboratory characteristics of complex febrile seizures in the acute phase: a case-series study in Japan

BackgroundPatients with complex febrile seizures (CFS) often display abnormal laboratory results, unexpectedly prolonged seizures, and/or altered consciousness after admission. However, no standardized values have been established for the clinical and laboratory characteristics of CFS in the acute phase, making the management of CFS challenging. This study aimed to determine the clinical and laboratory characteristics of children with CFS during the acute phase. In particular, the duration of impaired consciousness and the detailed distribution of blood test values were focused.MethodsWe retrospectively reviewed medical records of a consecutive pediatric cohort aged 6–60 months who were diagnosed with CFS and admitted to Kobe Children’s Hospital between October 2002 and March 2017. During the study period, 486 seizure episodes with confirmed CFS were initially reviewed, with 317 seizure episodes included in the analysis. Detailed clinical and laboratory characteristics were summarized.ResultsAmong 317 seizure episodes (296 children with CFS), 302 required two or fewer anticonvulsants to be terminated. In 296 episodes showing convulsive seizures, median seizure duration was 30.5 min. The median time from onset to consciousness recovery was 175 min. Impaired consciousness lasting > 6, 8, and 12 h was observed in 13.9%, 7.6%, and 1.9% patients with CFS, respectively. Additionally, the distribution of aspartate aminotransferase, lactate dehydrogenase, creatinine, and glucose were clarified with 3, 10, 50, 90, and 97 percentile values.ConclusionThis study detailed the clinical and laboratory findings of acute-phase CFS using the data of the largest 15-year consecutive cohort of children with CFS. These results provide important information for appropriate acute management of CFS.

Pathogenetic and etiologic considerations of febrile seizures

Febrile seizure (FS), which occurs in febrile children without underlying health problems, is the most common type of seizure disorder in children. The suggested pathogenesis of FS derived from several animal and human studies is multifactorial and debatable. Neuronal hyperexcitability, which develops during inflammatory responses that accompany fever, provokes seizures. However, the exact role of each inflammatory mediator (e.g., cytokines) is undefined in terms of the connection between systemic or local inflammation and the central nervous system, and the mechanisms by which cytokines increase neuronal excitability remain unclear. In contrast, the cause of fever in most children with FS is usually mild respiratory virus infection (e.g., rhinovirus, influenza virus, adenovirus, and enterovirus) rather than severe bacterial infections. In temperate regions, the major causative respiratory viruses seem to mirror seasonally prevalent respiratory viruses in the community. Therefore, vigorous efforts to identify the causative pathogen of fever may not be necessary in children with FS. Genetic factors seem to play a role in neuronal hyperexcitability, and some types of genetic variation have been identified in several genes encoding ion channels of neurons that participate in neuronal excitation. Although most children with FS have benign outcomes, some characteristics such as complex FS, febrile status epilepticus, consecutive afebrile seizures, and the presence of neurodevelopmental disabilities may require further genetic and neurologic evaluations.

The Role of Mean Platelet Volume/Platelet Count in Differentiation of Simple and Complex Febrile Seizures

Background and Aim: Our study aimed to find the relationship between MPV (Mean Platelet Volume) levels, a marker of inflammation, and platelet counts between simple and complex febrile seizures. Materials and Methods: In this retrospective comparative study, we analyzed the records of 283 children between the ages of 6 months and 6 years who were diagnosed with simple and complex febrile seizures (BFN/CFN) in the Pediatric Neurology Clinic of XXX University, Faculty of Medicine between January 2021 and December 2022. Results: The mean age and gender characteristics of the patients were similar in both groups. The MPV of the CFN group (69 patients) (6.53±0.39fl) was lower than that of the BFN group (214 patients) (7.81±0.43fl) and this difference was statistically significant (p&lt;0.01). The platelet count of the CFN group (257.42×103±126.01×103) was higher than that of the BFN group (191.23×103±68.29×103) and the difference was statistically significant (p&lt;0.01). Conclusion: There was a significant difference between BFN and CFN groups in terms of both MPV values and platelet counts. This study suggests that detailed and comprehensive studies on MPV and platelet counts may guide cases where clinical findings are suspicious and the distinction between BFN and CFN cannot be clearly differentiated.

Analysis of selected pro-inflammatory cytokines: IL-1β, IL-6, CXCL-8, and TNF-α in children with seizure disorders during acute infection. Is there a specific pro-inflammatory cytokine profile in these patients?

Introduction and objective: This study aimed to analyse the levels of selected pro-inflammatory cytokines in children with seizures during acute infection. Materials and methods: The study was conducted in the Department of Infectious Diseases and Child Neurology of the Poznan University of Medical Sciences from 19 January 2017 to 5 December 2020. Pro-inflammatory cytokines were measured in 64 patients with febrile seizures and 11 patients after an epileptic seizure in generalised epilepsy. The control group comprised 46 patients with delayed development. Serum pro-inflammatory cytokines were determined using the BioLegend’s ELISA MAX™ Deluxe Set. Pathogens were detected by standard diagnostic methods. Total white blood cell count, C-reactive protein and procalcitonin were determined using standard diagnostic methods. Results: Significantly higher levels of all analysed pro-inflammatory cytokines were found in patients with simple and simple plus febrile seizures; interleukin 6, CXCL-8 in those with complex febrile seizures; interleukin 6, CXCL-8, tumour necrosis factor α following epileptic seizure. The intensity of the inflammatory response in simple and simple plus febrile seizure patients corresponded to significantly higher levels of all pro-inflammatory cytokines and inflammatory markers. Pro-inflammatory profiles differed depending on the aetiology of the infection. Significantly higher levels interleukin 6, CXCL-8, tumour necrosis factor α were found in simple and simple plus febrile seizure patients infected with human herpesvirus-6 compared to the control group. Conclusions: In patients with febrile seizures and epileptic seizures, the involvement of interleukin 1β, interleukin 6, CXCL-8, and tumour necrosis factor α was confirmed in the inflammatory process, with a different distribution in the analysed groups. Pro-inflammatory cytokine profiles varied depending on the infectious aetiology.

Role of lactate measurement in predicting recurrence of seizure within 24 hours in simple febrile seizure patients

Pediatric febrile seizure (FS) is classified into two types, simple febrile seizures (SFSs) and complex febrile seizures (CFSs). The prognosis of CFS and SFS is distinct from each other. Even after diagnosis of SFS, patients with recurrence of seizure within 24 hours are defined as CFS. And it is crucial to predict the recurrence of seizure in SFS patients. The purpose of this study was to investigate the role of lactate levels in predicting recurrence of seizure within 24 hours in SFS patients. This retrospective study was conducted on patients who visited the emergency department with FS from January 2017 to February 2020 at a single tertiary university hospital. They were divided into the recurrence group and the SFS group according to the recurrence of seizures. Multivariable analysis was performed to confirm whether lactate levels could be an independent factor in predicting recurrence of seizure within 24 hours in SFS patients. Of the 177 patients, 38 patients were classified into the recurrence group. High lactate levels (odds ratio = 1.45; 95%confidence interval: 1.04–2.03, p = 0.031) were found to be a significant factor in predicting recurrence of seizure within 24 hours in SFS patients. The areas under the ROC curve for lactate was 0.733. In patients with FS, high lactate levels were shown to be a useful and independent factor in predicting recurrence of seizure within 24 hours in SFS patients.

The Impact of Omicron Wave on Pediatric Febrile Seizure.

The coronavirus disease 2019 (COVID-19) omicron (B.1.1.529) variant reduced the risk of severe disease compared with the original strain and other variants, but it appeared to be highly infectious, which resulted in an exponential increase in confirmed cases in South Korea. As the number of confirmed cases increased, so did the number of pediatric patients' hospitalization. This study aims to evaluate the frequency and clinical features of febrile seizure associated with the COVID-19 omicron variant in children. We retrospectively reviewed the medical records of children aged under 18 years with febrile seizure who were tested for COVID-19 from February 2020 to April 2022 at Ajou University Hospital, South Korea. Based on the dominant variants, we divided the period into the pre-omicron (from February 2020 to December 2021) and omicron periods (from January 2022 to April 2022) and compared the clinical characteristics between the two. Also, we compared the clinical characteristics of febrile seizure between COVID-19 positive and negative group during the omicron period. Among the 308 children, 211 patients (9.2 patients/months) and 97 patients (24.3 patients/months) were grouped into pre-omicron and omicron periods, respectively. Compared with the pre-omicron period, patients in the omicron period showed significantly higher mean age (pre-omicron vs. omicron, 22.0 vs. 28.0 months; P = 0.004) and COVID-19 positive results (pre-omicron vs. omicron, 0.5% vs. 62.9%; P < 0.001). As the COVID-19 confirmed cases in the omicron period increased, the number of COVID-19 associated febrile seizure also increased. In the omicron period, 61 children were confirmed to be positive for COVID-19, and COVID-19 positive group showed statistically significant higher mean age (positive vs. negative, 33.0 vs. 23.0 months; P = 0.003) and peak body temperature than the negative group (positive vs. negative, 39.1°C vs. 38.6°C; P = 0.030). Despite the lack of significance, COVID-19 positive group showed longer seizure time, multiple seizure episodes, and higher prevalence of complex febrile seizure. The frequency of COVID-19 associated febrile seizure increased in the omicron periods. In addition, in this period, children with febrile seizure diagnosed with COVID-19 had a higher mean age and higher peak body temperature.

MRI Abnormalities of the Brain After Complex Febrile Seizures in Children.

Background Though simple febrile seizures do not cause significant and lasting neurological deficits, complex febrile seizures (CFS) can result in neurologic sequelae.Because CFS causes cortical focal injuries and other brain lesions, itneeds to be evaluated. Objective The objective of this study was to evaluate the MRI brain changes in CFS, the incidence of seizures in children aged six months to five years, and their severity in relation to MRI findings of the brain. Methods In this observational study, 36 children aged six months to five years, having fever with seizure, and fulfilling the criteria of CFS were enrolled within 48 hours of the episode. Detailed clinical and neurological examinations were performed with MRI scans to find out the probable CNS lesions of CFS. Two radiologists separately evaluated all MRI brains. Results MRI lesions of the brain were found in11 (30.56%) CFS patients. Generalized tonic-clonic seizures (GTCS) (n=31, 86.11%) were the most common type of seizure among CFS cases. Cortical focal hyperintensity (CFH) (42.1%) was the most common MRI presentation. Positive MRI findings were significantly associated with focal convulsions (n=5, 100%) as compared to GTCS cases (n=6, 19%) (p=0.001). Conclusions CFH is the most common abnormality in the MRI brain amongCFS cases. CFS patients with focal seizures or prolonged seizures in 24 hours have higher abnormal neuroimaging findings. MRI should be considered a preferred investigatory tool for detecting CNS pathology in CFS cases. Follow-up studies are needed to determine the long-term outcomes of CNS lesions in children with CFS.

Association between Iron &amp; Other Trace Elements Deficiency and Febrile Seizure in Children

Introduction: Febrile seizures are the most common type of seizure in children occurring in 2-5 % of them with a peak incidence in 16-18 months. Various factors have been described in the pathophysiology of febrile seizures like susceptibility of the immature brain to temperature, an association between interleukins, circulating toxins, and trace elements deficiency.&#x0D; Aim: To study the association between serum levels of trace elements (Iron, zinc, copper, calcium and magnesium) in children with febrile seizures.&#x0D; Study Design: An observational cross-sectional study.&#x0D; Place and Duration of Study: Department of Pediatrics of PGIMER, Dr RML Hospital, New Delhi between November 2016 and March 2018.&#x0D; Methodology: This study was conducted at a tertiary care centre and enrolled 75 children of which 55 were febrile seizures and 20 patients were controls. The serum specimen was collected with all aseptic techniques, and all due precautions were taken for the blood sample to protect them from sunlight exposure, haemolysis and contamination. Then samples were centrifuged at 3,000 rpm for 5 minutes and were stored at -20 c until the final assay. Serum Iron, Zinc, Copper and Magnesium levels were measured using a fully automated chemistry analyser (AU400). Data was entered in MS Excel spreadsheets and analysed using the statistical package for social science (SPSS) version 21.0. &#x0D; Results: Children with febrile seizures (cases) were found to have significant iron deficiency (higher TIBC, higher UIBC and lower Ferritin) than the controls (P&lt;0.001). There was no significant difference in the serum levels of copper, zinc, magnesium, and ionized calcium in the two groups. There was no difference in serum levels of trace elements in cases with simple or complex febrile seizure.&#x0D; Conclusion: Children with febrile seizures showed significant iron deficiency (higher TIBC, higher UIBC and lower Ferritin) in cases than those with controls. But, there was no significant association between other serum trace elements (zinc, copper, calcium, magnesium) and febrile seizure. However, further large multi-centric trials are required to suggest any association between them.

Effectiveness of prophylactic iron supplementation in the reduction of recurrence of febrile seizures in children: A prospective study with comparison with historical controls.

The primary objective of the study was to compare the number of patients with febrile seizure recurrence within 1 year of presenting to our institute, among patients who received and didn't receive oral iron supplementation. This prospective intervention study with historical controls was conducted to compare the number of patients with febrile seizure recurrence within 1 year, among patients who received and did not receive oral iron supplementation. The intervention group additionally received prophylactic iron supplementation of 20 mg biweekly for 1 year. A total of 53 patients each were enrolled in both the groups, with comparable baseline characteristics. Although there was a trend toward a lower rate of recurrence of febrile seizures in the interventional group, as compared to the control group, it did not reach the point of statistical significance (P = 0.35). Both in the worst-case scenario and best-case scenario, there was a trend toward less risk of recurrence of febrile seizure in the intervention group, but it did not reach the point of statistical significance (P = 0.43 and 0.52). For the original scenario, worst-case scenario, and best-case scenario, the absolute risk reduction was 6.5%, 7%, and 6%, respectively, with corresponding number needed to treat (NNT) being 15, 14, and 16, respectively. The trend for absolute risk reduction was more pronounced in those with complex febrile seizures with an NNT of 6.5, but it still did not reach the point of statistical significance (P = 0.16). Moderate/severe IDA was also found to be an independent risk factor for recurrence of febrile seizure in the intervention group (P = 0.03). Oral serum iron supplementation does not significantly reduce the recurrence rate of febrile seizures in children aged 6-60 months. However, there is a trend toward reduction in the frequency of recurrence of febrile seizures, which is more pronounced in the subset with complex febrile seizures.

Febrile Seizures in Children: A Review.

Fever-induced seizures are referred to as febrile seizures (FSs). The most prevalent kind of epilepsy and neurological illness in infants and young children is FS. With a high occurrence seen between the ages of 12 and 18 months, they frequently affect children aged six months to five years. FS is a benign condition that seldom results in brain damage. Nevertheless, they cause stress and emotional anguish for the parents, who may believe that the death of their child is going to occur during the seizure. Lately, a more broad-based phrase has been used, fever-associated seizures or epilepsy that includes simple, complicated, and extended FSs. These are the three different kinds of FSs. Febrile status epilepticus is a subgroup of complex FS. The other kinds of FSs are FS plus, Dravet syndrome,hereditary epilepsy with FSplus, and febrile infection-related epilepsy syndrome. The most frequent, brief, and generalizedsimple FSshave a greater likelihood of causing temporal lobe epilepsy than complex FSs. These seizures are linked to the release of inflammatory mediatorslike interleukin (IL)-1, IL-6, and tumor necrosis factor, which are well-known fever inducers.This article details thefactors that contribute to the occurrence of FSs, epidemiology, pathophysiology, evaluation, and management of the child.

Anti–Gamma Aminobutyric Acid B Autoimmune Encephalitis in an Indian Child with Early-Onset Seizures, Neurodegeneration, and Brain Calcification due to NRROS Variation: The First Reported Case Worldwide

AbstractA 1.5-year-old boy presented to us with a history of normal growth and developmental parameters until 6 months of age. However, at 7 months of age, he developed multiple types of seizures consisting initially of complex febrile seizures, followed by afebrile seizures. Multifocal clonic, generalized tonic–clonic, and myoclonic (multifocal and generalized) were the evolving seizure types. He had truncal hypotonia, but his appendicular hypotonia progressed to hypertonia over the next few months and further to decorticate posturing. Brain magnetic resonance imaging (MRI) showed generalized atrophy, predominantly frontotemporal, without any focal signal abnormalities or contrast enhancement. Computed tomography (CT) showed speckled calcification in subcortical white matter. Electroencephalogram showed bilateral frontotemporal epileptiform discharges with secondary generalization. His cerebrospinal fluid had normal cytology and biochemical results but was positive for anti–gamma aminobutyric acid B antibodies. Whole exome sequencing showed likely pathogenic, novel autosomal recessive homozygous variation of NRROS gene on chromosome 3 [c.1487G &gt; A (p.Trp496Ter)], which impairs the functioning of anti-inflammatory cytokine transforming growth factor beta, resulting in a proinflammatory state within the central nervous system and thereby promoting autoimmune encephalitis. Parental Sanger sequencing validated the variation in both his parents. He was treated with both pulse methylprednisolone (30 mg/kg/day for 5 days) and intravenous immunoglobulin (2 g/kg), followed by slowly tapering of oral prednisolone and monthly intravenous immunoglobulin infusion (1 g/kg). There was significant reduction in seizure frequency and disappearance of epileptiform discharges from the electroencephalogram. However, the motor and cognitive improvement did not occur, and he had microcephaly and growth failure at the last follow-up. This is the 11th case report of neurodegeneration associated with NRROS gene variations, but the first report of autoimmune encephalitis being triggered by the variation in a child.

Type III Sturge Weber Syndrome, An Uncommon Cause of Status Epilepticus

Abstract Introduction Sturge Weber syndrome (SWS) is a rare neurocutaneous condition due to the mutation of the GNAQ gen. This condition is characterized by skin, eye, and brain compromise, but the type III only affects the brain, making it a challenging condition to diagnose. Clinical Case A Hispanic 4 year-old female, with a history of complex febrile seizure in her medical records, presented to the emergency room in status epilepticus after 24 hours of upper respiratory symptoms. After a neurological and radiologic evaluation, SWS III was diagnosed, which led to a pharmacological adjustment for achieving control of the seizures, with a great clinical evolution. Discussion The pathophysiology, diagnostics, and proper management of this disease are discussed. Conclusion SWS is a rare neurocutaneous disease, usually diagnosed in patients with pathognomonic features, however it is important to know that type III SWS exists and represents a challenging diagnosis, leading to a time-race for starting proper management, considering that the outcome includes a better life-quality, a higher cognitive result, and reduced morbimortality.