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Edema Encefálico , COVID-19 , Estado Epiléptico , Humanos , Estado Epiléptico/etiología , Estado Epiléptico/diagnóstico , Estado Epiléptico/complicaciones , COVID-19/complicaciones , Edema Encefálico/etiología , Masculino , SARS-CoV-2 , Anciano , Tomografía Computarizada por Rayos X , FemeninoRESUMEN
OBJECTIVE: To investigate the short-term benefits and adverse effects of ketamine in the treatment of pediatric and adolescent super-refractory status epilepticus (SRSE), with a focus on the inflammatory etiology. METHODS: This retrospective observational cohort study included a consecutive series of 18 pediatric to adolescent patients with SRSE admitted between 2008 and 2023 and treated with ketamine. Seizure frequency per hour before and after ketamine administration and response rate were calculated. Neurological decline, catecholamine administration, and adverse effects were also assessed. The patients were divided into inflammatory and non-inflammatory etiology groups. RESULTS: The median age at SRSE onset was 1 year 5 months (range: 11 days-24 years), and 78% of the patients were male individuals. The median duration of treatment was 7.5 days (interquartile range: 2.8-15.5 days). Fifteen (83%) patients achieved >50% seizure reduction. The median seizure frequency before and after ketamine treatment was 5.9 and 0.9, respectively, showing a significant reduction in seizure frequency (p < 0.0001). Ten patients had inflammatory etiologies including bacterial meningitis (n = 2), viral encephalitis (n = 3), and febrile infection related epilepsy syndrome (n = 5). The inflammatory etiology group required a longer treatment duration (p = 0.0453) and showed lower seizure reduction (p = 0.0264), lower response rate (p = 0.0044), and higher neurological decline (p = 0.0003) than the non-inflammatory etiology group. Three (17%) patients experienced transient adverse events requiring intervention within 24 h of initiating ketamine administration. CONCLUSIONS: Ketamine administration was associated with fewer serious adverse events and a reduced seizure frequency. Additionally, inflammatory conditions may weaken the efficacy of ketamine in patients with SRSE.
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Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Ketamina , Enfermedades Neuromusculares , Estado Epiléptico , Humanos , Niño , Masculino , Adolescente , Recién Nacido , Femenino , Ketamina/efectos adversos , Estudios Retrospectivos , Anticonvulsivantes/uso terapéutico , Estado Epiléptico/complicaciones , Convulsiones/complicaciones , Enfermedades Neuromusculares/complicacionesRESUMEN
OBJECTIVE: To analyze the risk factors affecting psychiatric behavior and study the psychobehavioral conditions of children with epilepsy. METHOD: We randomly selected and enrolled 294 children with epilepsy who visited and were hospitalized in the pediatric clinic of Hebei General Hospital between January 2017 and January 2022, as the study participants. We comprehensively assessed their cognitive functions using the Gesell development schedule or Wechsler Intelligence Scales. The participants were divided into the study group (n = 123) with cognitive impairment and the control group (n = 171) with normal cognitive functions, for analysis. RESULTS: There were statistically significant differences between the two groups in disease course, frequency of epilepsy, status epilepticus, and the number of antiseizure medications (ASMs) used (P < 0.05), while there were no statistically significant differences in age, gender, age of onset, form of onset, interictal epileptiform discharge, history of febrile convulsion, and the time from onset to initial visit (P > 0.05). Based on multivariate logistic regression analysis, the course of disease, frequency of onset, status epilepticus and number of ASMs used were identified as high-risk factors for cognitive impairment in children with epilepsy. Similarly, early onset, long course of disease, known etiology, and combination of multiple drugs have a negative impact on behavioral problems, school education, and social adaptability. CONCLUSION: The course of disease, the frequency of onset, status epilepticus, and the number of ASMs used are high-risk factors for cognitive impairment in children with epilepsy, which can be prevented and controlled early. When selecting ASMs, their advantages and disadvantages should be weighed. Moreover, the availability of alternative treatment options must be considered. With the help of genomic technology, the causes of epilepsy should be identified as early as possible, and precision medicine and gene therapy for children with epilepsy should be actively developed.
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Trastornos del Conocimiento , Epilepsia , Estado Epiléptico , Niño , Humanos , Cognición , Trastornos del Conocimiento/epidemiología , Comorbilidad , Epilepsia/tratamiento farmacológico , Epilepsia/epidemiología , Epilepsia/psicología , Estado Epiléptico/complicaciones , Masculino , FemeninoRESUMEN
Status gelasticus is a rare form of status epilepticus characterized by prolonged and/or clustered gelastic seizures. The review encompasses an analysis of cases reported in the literature, focusing on causes, clinical-electroencephalographic features, and therapeutic interventions. The study reveals the challenges in defining and understanding status gelasticus due to its diverse etiologies and limited reported cases. The association with hypothalamic hamartomas and other brain abnormalities underscores the importance of thorough evaluations. The review also discusses new treatments, including medications and less invasive surgeries. While progress has been made, the study points out challenges in diagnosing and managing this complex condition, highlighting the importance of ongoing research.
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Encefalopatías , Epilepsias Parciales , Hamartoma , Enfermedades Hipotalámicas , Estado Epiléptico , Humanos , Epilepsias Parciales/diagnóstico , Enfermedades Hipotalámicas/complicaciones , Encefalopatías/complicaciones , Encéfalo , Estado Epiléptico/complicaciones , Hamartoma/complicaciones , Imagen por Resonancia MagnéticaRESUMEN
Epilepsy in Sturge-Weber syndrome (SWS) is common, but drug-refractory epilepsy (DRE) in SWS has rarely been studied in children. We investigated the characteristics of epilepsy and risk factors for DRE in children with SWS. A retrospective study was conducted to analyze the clinical characteristics of children with SWS with epilepsy in our hospital from January 2013 to October 2022. Univariate and multivariate logistic analyses were performed to investigate the factors influencing DRE in children with SWS. A total of 35 SWS children with epilepsy were included (51% male; mean age of presentation 3.6 ± 0.5 years), 71% of children with SWS had their first seizure within the first year of life, and the most common type of seizure was focal seizure (77%). Eleven (31%) patients developed DRE. The median age of onset for the first seizure was 1.0 years and all these cases were of SWS type I. Multivariate logistic analysis revealed that stroke-like episodes and seizure clusters were risk factors for DRE in SWS children. A poor neurological function group was observed in twenty-five children with SWS. Status epilepticus was a risk factor that affected the neurological function of SWS children with epilepsy. Conclusion: The study explored the epileptic features of children with SWS. The results revealed that stroke-like episodes and seizure clusters are risk factors for DRE in children with SWS. The occurrence of status epilepticus impacts the neurological function of SWS children with epilepsy. Thus, long-term follow-up is necessary to monitor outcomes. What is Known: ⢠Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder, over 75% of children with SWS experience seizures, and 30-57% develop drug-refractory epilepsy (DRE), which leads to a poor outcome. ⢠Drug-refractory epilepsy in SWS has been rarely studied in children, and the risk factors associated with DRE are unclear. What is New: ⢠Clinical features of SWS children with drug-refractory epilepsy. ⢠In SWS, stroke-like episodes and seizure clusters are risk factors of DRE, the occurrence of status epilepticus impacts the neurological function.
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Epilepsia Refractaria , Epilepsia , Estado Epiléptico , Accidente Cerebrovascular , Síndrome de Sturge-Weber , Niño , Humanos , Masculino , Preescolar , Lactante , Femenino , Epilepsia Refractaria/etiología , Epilepsia Refractaria/complicaciones , Estudios Retrospectivos , Síndrome de Sturge-Weber/complicaciones , Síndrome de Sturge-Weber/epidemiología , Convulsiones/etiología , Epilepsia/etiología , Epilepsia/complicaciones , Accidente Cerebrovascular/complicaciones , Estado Epiléptico/complicacionesRESUMEN
RATIONALE: Rhabdomyolysis is a serious complication of status epilepticus (SE) caused by muscle cell damage and can lead to a life-threatening acute kidney injury (AKI). PATIENT CONCERNS: A 35-year-old man with a history of seizures treated with 3 different antiepileptic drugs (carbamazepine, lamotrigine, and levetiracetam) presented with SE. The patient received 5 doses of diazepam to control the SE in another hospital and was transferred to our emergency due to AKI. DIAGNOSES: Laboratory tests corresponded with rhabdomyolysis-induced AKI and disseminated intravascular coagulation. Thereafter, the decrease in renal excretion of both drugs (diazepam and carbamazepine) caused acute liver injury and neurotoxicity. The carbamazepine concentration was 16.39 mcg/mL, which considered in toxic level, despite using the usual dose. INTERVENTIONS: The patient was treated with hydration and sodium bicarbonate, however; severe AKI mandated a hemodialysis session. OUTCOMES: The diuresis started to increase, kidney and liver functions improved, and altered mental status reversed. LESSONS: This case alerts physicians to consider the synergistic drug side effects and interactions, especially when patients present with impaired liver or kidney functions. The reduction in metabolism or excretion of drugs can cause an increase in serum concentrations and induce toxicity, even when the drug intake at the usual dose.
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Lesión Renal Aguda , Enfermedad Hepática Inducida por Sustancias y Drogas , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Rabdomiólisis , Estado Epiléptico , Masculino , Humanos , Adulto , Diazepam/uso terapéutico , Anticonvulsivantes/efectos adversos , Estado Epiléptico/inducido químicamente , Estado Epiléptico/tratamiento farmacológico , Estado Epiléptico/complicaciones , Carbamazepina/uso terapéutico , Rabdomiólisis/complicaciones , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/complicaciones , Lesión Renal Aguda/etiología , Enfermedad Hepática Inducida por Sustancias y Drogas/complicacionesRESUMEN
OBJECTIVE: Patients with focal drug resistant epilepsy are excellent candidates for epilepsy surgery. Status epilepticus (SE) and seizure clusters (SC), described in a subset of patients, have both been associated with extended epileptogenic cerebral networks within one or both hemispheres. In this retrospective study, we were interested to determine if a history of SE or SC is associated with a worse surgical outcome. METHODS: Data of 244 patients operated between 2000 to 2018 were reviewed, with a follow-up of at least 2 years. Patients with a previous history of SE or SC were compared to operated patients without these conditions (control group, CG). RESULTS: We identified 27 (11%) and 38 (15.5%) patients with history of SE or SC, respectively. No difference in post-operative outcome was found for SE and SC patients. Compared to the control group, patients with a history of SE were diagnosed and operated significantly at earlier age(p = 0.01), and after a shorter duration of the disease (p = 0.027), but with a similar age of onset. SIGNIFICANCE: A history of SE or SC was not associated with a worse post-operative prognosis. Earlier referral of SE patients for surgery suggests a heightened awareness regarding serious complications of recurrent SE by the referring neurologist or neuropediatrician. While the danger of SE is evident, policies to underline the impact for SC or very frequent seizures might be an efficient approach to accelerate patient referral also for this patient group.
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Epilepsia Generalizada , Epilepsia , Estado Epiléptico , Humanos , Estudios Retrospectivos , Epilepsia/complicaciones , Estado Epiléptico/complicaciones , Convulsiones/complicaciones , Pronóstico , Epilepsia Generalizada/complicaciones , Resultado del TratamientoRESUMEN
OBJECTIVE: Status epilepticus (SE) may lead to long-term consequences. This study evaluated the risk and predictors of seizure occurrence after SE, with a focus on SE due to acute symptomatic etiologies. METHODS: Prospectively collected data about adults surviving a first non-hypoxic SE were reviewed. The outcome was the occurrence of unprovoked seizures during the follow-up. Kaplan-Meier survival curve analysis and log-rank test were used to analyze the time to seizure occurrence and determine the statistical significance between etiological groups. Three subcategories within acute etiology were considered according to the presence of the following: (1) structural lesion (acute-primary); (2) brain involvement during systemic disorders (acute-secondary); and (3) drug or alcohol intoxication/withdrawal (acute-toxic). Cox proportional hazards model was adopted to estimate hazard ratios (HRs) with the 95% confidence intervals (CIs). RESULTS: Two hundreds fifty-seven individuals were included. Fifty-four subjects (21.0%) developed seizures after a median of 9.9 (interquartile range 4.3-21.7) months after SE. The estimated 1-, 2-, and 5-year rates of seizure occurrence according to acute SE etiologies were 19.4%, 23.4%, and 30.1%, respectively, for acute-primary central nervous system (CNS) pathology; 2.2%, 2.2%, and 8.7%, respectively, for acute-secondary CNS pathology; and 0%, 9.1%, and 9.1%, respectively, for acute-toxic causes. Five-year rates of seizure occurrence for non-acute SE causes were 33.9% for remote, 65.7% for progressive, and 25.9% for unknown etiologies. In multivariate Cox regression model, progressive etiology (adjusted HR [adjHR] 2.27, 95% CI 1.12-4.58), SE with prominent motor phenomena evolving in non-convulsive SE (adjHR 3.17, 95% CI 1.38-7.25), and non-convulsive SE (adjHR 2.38, 95% CI 1.16-4.90) were independently associated with higher hazards of unprovoked seizures. Older people (adjHR .98, 95% CI .96-.99) and people with SE due to acute-secondary CNS pathology (adjHR .18, 95% CI .04-.82) were at decreased risk of seizure occurrence. SIGNIFICANCE: SE carries a risk of subsequent seizures. Both the underlying cause and epileptogenic effects of SE are likely to contribute.
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Alcoholismo , Estado Epiléptico , Adulto , Humanos , Anciano , Anticonvulsivantes/uso terapéutico , Convulsiones/epidemiología , Convulsiones/etiología , Convulsiones/tratamiento farmacológico , Estado Epiléptico/etiología , Estado Epiléptico/complicaciones , Modelos de Riesgos Proporcionales , Estimación de Kaplan-MeierRESUMEN
PURPOSE: The prevalence of epilepsy in patients with multiple sclerosis (MS) is three to six times the prevalence in the general population. Mechanisms resulting in increased seizure risk are not fully understood. Our objective is to characterize patients with MS and epilepsy regarding timing of diagnoses, MS and seizure (SZ) type, EEG findings suggesting cortical dysfunction, frequency of status epilepticus (SE), and seizure freedom. METHODS: This was a single center retrospective study. Cases were obtained via DataDirect via the University of Michigan electronic medical record from January 1, 2006 through October, 12, 2016. The University of Michigan Health System is a large academic institute with a tertiary referral center and an Autoimmunity Center of Excellence. Patients were included if chart listed one or more of the top 62 epilepsy, and one or more of the top 2 MS, most frequently entered ICD9 and ICD10 codes. Patients with alternative epilepsy etiology were excluded. 74 of 361 patients were included. We collected information regarding demographics, MS and SZ type, age at diagnosis, imaging, EEG, seizure freedom, medications, and SE. RESULTS: We found a high percentage of patients with SE. Most patients with imaging had multiple lesions at seizure onset. 27/54 of patients with EEG data showed electrographic evidence of cortical dysfunction. 6/8 of EEGs in PPMS showed features consistent with cortical dysfunction, followed by 9/17 in SPMS and 11/23 in RRMS. 7/8 of patients with PPMS showed EEG evidence of temporal lobe dysfunction. CONCLUSION: Time of seizure onset relative to MS diagnosis varied with MS type suggesting distinct pathophysiology. EEG results correspond with reports of increased cortical damage and temporal dysfunction in PPMS, but are unique as a functional modality (EEG) as indicator of gray matter dysfunction. EEG findings differed in RRMS and progressive MS suggesting possibility of supportive diagnostic marker. Our data suggests higher risk of SE in progressive MS and diminished rate of seizure freedom for MS patients with SE. We conclude that early treatment with antiseizure medication would be beneficial for MS patients with SE and with progressive MS forms and SZ, in agreement with previous studies.
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Epilepsia , Esclerosis Múltiple , Estado Epiléptico , Humanos , Estudios Retrospectivos , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/epidemiología , Esclerosis Múltiple/diagnóstico , Autoinmunidad , Convulsiones/diagnóstico , Epilepsia/epidemiología , Estado Epiléptico/complicaciones , Electroencefalografía/efectos adversosRESUMEN
To assess the association between clinical and MRI characteristics of arterial ischaemic stroke (AIS) and the 3-year risk of post-stroke epilepsy (PSE) in paediatric patients. Retrospective cohort study. Database from a single tertiary referral centre for paediatric stroke in Chile. Two hundred seven neonates and children (1 day to 18 years) with a first-ever supratentorial AIS diagnosed between January 2003 and December 2019 were evaluated. Diagnosis of PSE and explanatory variables were consecutively recorded from hospital inpatient and annual outpatient records in a predesigned database. Competing risk analysis (competing events: death and loss to follow-up) of multiple Cox proportional hazards regression was performed to estimate adjusted subhazard ratios (SHRs) of PSE. Confidence intervals (95% CI) were calculated using bootstrap resampling (1000 replications). Interaction terms were added to investigate moderating effects. The 3-year incidence rate of PSE was 166.5 per 1000 person-years (neonatal: 150.1; childhood: 173.9). The 3-year cumulative incidence was 33%. Patients with acute symptomatic non-status seizures (SHR = 3.13; 95% CI = 1.43-6.82), status epilepticus (SHR = 5.16; 95% CI = 1.90-13.96), abnormal discharge neurological status (SHR = 2.52; 95% CI = 1.12-5.63), cortical lesions (SHR = 2.93; 95% CI = 1.48-5.81), and multifocal infarcts with stroke size < 5% of supratentorial brain volume (SHR = 3.49; 95% CI = 1.44-8.46) had a higher risk of PSE. CONCLUSION: This study identified specific and reliable acute clinical and imaging predictors of PSE in paediatric patients, helping clinicians identify high-risk patients with potential implications for treatment decisions. WHAT IS KNOWN: ⢠Numerous risk factors have been proposed for post-stroke epilepsy, but there is a lack of studies evaluating these variables while accounting for confounding factors and competing risks over time. WHAT IS NEW: ⢠After adjustment for competing events, acute symptomatic seizures, both non-status and status epilepticus, abnormal mental status or motor neurological examination at hospital discharge, cortical involvement, and multifocal ischaemic lesions in small strokes are all independent predictors of post-stroke epilepsy. ⢠Knowing the predictors of post-stroke epilepsy is essential for clinicians to make well-informed and effective decisions about treatment.
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Isquemia Encefálica , Epilepsia , Accidente Cerebrovascular Isquémico , Estado Epiléptico , Accidente Cerebrovascular , Recién Nacido , Humanos , Niño , Estudios de Cohortes , Incidencia , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/diagnóstico , Accidente Cerebrovascular/epidemiología , Isquemia Encefálica/complicaciones , Isquemia Encefálica/epidemiología , Estudios Retrospectivos , Epilepsia/epidemiología , Epilepsia/etiología , Epilepsia/diagnóstico , Convulsiones/etiología , Accidente Cerebrovascular Isquémico/complicaciones , Estado Epiléptico/complicacionesAsunto(s)
Hemimegalencefalia , Malformaciones del Sistema Nervioso , Estado Epiléptico , Humanos , Hemimegalencefalia/complicaciones , Hemimegalencefalia/diagnóstico por imagen , Hemimegalencefalia/genética , Estado Epiléptico/complicaciones , Estado Epiléptico/genética , Mutación , Malformaciones del Sistema Nervioso/complicaciones , Encéfalo/diagnóstico por imagen , Atrofia , Proteínas Activadoras de GTPasa/genéticaRESUMEN
A 69-year-old woman developed non-convulsive status epilepticus during inpatient investigation for abdominal pain. Initial detailed investigations did not identify the cause of seizures, but a jejunal biopsy and PCR testing in various fluids led to the diagnosis of Whipple's disease with neurological involvement. The seizures were controlled but she subsequently had moderate cognitive impairment. Whipple's disease is an important diagnosis, being treatable with antibiotics. Testing for Whipple's disease is not part of the recommended workup in for status epilepticus, but this case highlights the importance of considering this condition.
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Estado Epiléptico , Enfermedad de Whipple , Femenino , Humanos , Anciano , Enfermedad de Whipple/complicaciones , Enfermedad de Whipple/diagnóstico , Enfermedad de Whipple/patología , Antibacterianos/uso terapéutico , Estado Epiléptico/complicacionesRESUMEN
INTRODUCTION: Acute kidney injury is a well-known complication of generalized tonic-clonic seizures, most commonly due to rhabdomyolysis. Elevated serum uric acid resulting in uric acid nephropathy is an overlooked cause of acute kidney injury in these patients, with only a few published case reports. CASE REPORT: In the first case, a 23-year-old male was admitted with status epilepticus. His kidney function worsened and he developed anuria. He had a serum uric acid level of 20.7 mg/dL and required multiple sessions of hemodialysis. In the second case, a 32-year-old male was admitted with acute kidney injury after experiencing a breakthrough seizure. He had a serum uric acid level of 20.4 mg/dL and was treated with rasburicase with recovery of renal function. In the third case, a 29-year-old male was admitted with status epilepticus. His renal function deteriorated. His serum uric acid level was 19.5 mg/dL. He required hemodialysis and rasburicase. CONCLUSION: Uric acid nephropathy is a rare complication of generalized tonic-clonic seizures, which is poorly recognized by healthcare providers. We advocate for Nephrology consultation early in a patient's hospitalization to discuss the use of rasburicase to avoid the associated morbidity of renal replacement therapies.
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Lesión Renal Aguda , Estado Epiléptico , Masculino , Humanos , Adulto Joven , Adulto , Ácido Úrico , Convulsiones/tratamiento farmacológico , Convulsiones/complicaciones , Estado Epiléptico/complicaciones , Lesión Renal Aguda/inducido químicamente , Lesión Renal Aguda/complicacionesRESUMEN
Dravet syndrome is currently considered as an developmental and epileptic encephalopathy and, recently, mandatory, alert, and exclusionary criteria have been proposed. Here, we describe three patients with Dravet syndrome with the typical early presentation including febrile and afebrile alternating hemiclonic seizures due to loss-of-function SCN1A variants. Subsequently, they developed episodes of febrile focal status epilepticus (SE) associated with hemiparesis and cerebral hemiatrophy with posterior focal seizures, as a consequence of Dravet syndrome. This sequence of events has been previously published in patients with Dravet syndrome and does not contradict the recent classification by the International League Against Epilepsy (ILAE). The ILAE guidance identifies "Focal neurological findings" as alert criteria and "MRI showing a causal focal lesion" as exclusionary criteria for making an initial diagnosis of Dravet syndrome at presentation. Our three patients would correspond to a severe phenotype, similar to the well-known presentation of generalized atrophy following prolonged status epilepticus. Common genetic findings in cases of diffuse and unilateral brain involvement may help explain these clinical presentations. Further genotype-phenotype studies may provide additional insights into this electroclinical behavior.
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Epilepsias Mioclónicas , Epilepsia , Convulsiones Febriles , Estado Epiléptico , Humanos , Mutación , Canal de Sodio Activado por Voltaje NAV1.1/genética , Epilepsia/diagnóstico , Estado Epiléptico/genética , Estado Epiléptico/complicaciones , Convulsiones Febriles/complicaciones , Atrofia , Paresia/complicacionesRESUMEN
Febrile seizures during early childhood may result in central nervous system developmental disorders. However, the specific mechanisms behind the impact of febrile seizures on the developing brain are not well understood. To address this gap in knowledge, we employed a hyperthermic model of febrile seizures in 10-day-old rats and tracked their development over two months. Our objective was to determine the degree to which the properties of the hippocampal glutamatergic system are modified. We analyzed whether pyramidal glutamatergic neurons in the hippocampus die after febrile seizures. Our findings indicate that there is a reduction in the number of neurons in various regions of the hippocampus in the first two days after seizures. The CA1 field showed the greatest susceptibility, and the reduction in the number of neurons in post-FS rats in this area appeared to be long-lasting. Electrophysiological studies indicate that febrile seizures cause a reduction in glutamatergic transmission, leading to decreased local field potential amplitude. This impairment could be attributable to diminished glutamate release probability as evidenced by decreases in the frequency of miniature excitatory postsynaptic currents and increases in the paired-pulse ratio of synaptic responses. We also found higher threshold current causing hind limb extension in the maximal electroshock seizure threshold test of rats 2 months after febrile seizures compared to the control animals. Our research suggests that febrile seizures can impair glutamatergic transmission, which may protect against future seizures.
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Hipertermia Inducida , Convulsiones Febriles , Estado Epiléptico , Preescolar , Humanos , Ratas , Animales , Ratas Sprague-Dawley , Hipertermia Inducida/efectos adversos , Hipocampo/fisiología , Región CA1 Hipocampal , Estado Epiléptico/complicaciones , Modelos Animales de EnfermedadRESUMEN
BACKGROUND: Epilepsy affects over 65 million people worldwide and significantly burdens patients, caregivers, and society. Drug-resistant epilepsy occurs in approximately 30% of patients and growing evidence indicates that oxidative stress contributes to the development of such epilepsies. Activation of the Nrf2 pathway, which is involved in cellular defense, offers a potential strategy for reducing oxidative stress and epilepsy treatment. Dimethyl fumarate (DMF), an Nrf2 activator, exhibits antioxidant and anti-inflammatory effects and is used to treat multiple sclerosis. METHODS: The expression of Nrf2 and its related genes in vehicle or DMF treated rats were determined via RT-PCR and Western blot analysis. Neuronal cell death was evaluated by immunohistochemical staining. The effects of DMF in preventing the onset of epilepsy and modifying the disease were investigated in the kainic acid-induced status epilepticus model of temporal lobe epilepsy in rats. The open field, elevated plus maze and T-Maze spontaneous alteration tests were used for behavioral assessments. RESULTS: We demonstrate that administration of DMF following status epilepticus increased Nrf2 activity, attenuated status epilepticus-induced neuronal cell death, and decreased seizure frequency and the total number of seizures compared to vehicle-treated animals. Moreover, DMF treatment reversed epilepsy-induced behavioral deficits in the treated rats. Moreover, DMF treatment even when initiated well after the diagnosis of epilepsy, reduced symptomatic seizures long after the drug was eliminated from the body. CONCLUSIONS: Taken together, these findings suggest that DMF, through the activation of Nrf2, has the potential to serve as a therapeutic target for preventing epileptogenesis and modifying epilepsy.
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Epilepsia , Estado Epiléptico , Humanos , Ratas , Animales , Dimetilfumarato/farmacología , Dimetilfumarato/uso terapéutico , Factor 2 Relacionado con NF-E2/metabolismo , Reposicionamiento de Medicamentos , Epilepsia/tratamiento farmacológico , Epilepsia/prevención & control , Convulsiones/tratamiento farmacológico , Estado Epiléptico/complicaciones , Estado Epiléptico/tratamiento farmacológico , Modelos Animales de EnfermedadRESUMEN
RATIONALE: Phantom absences refer to mild and short-lasting absence seizures, which are usually accompanied by infrequent generalized tonic-clonic seizures and absence status. Generally, phantom absences do not impair the individual neurological functions. Herein, we report the case of a young woman with idiopathic generalized epilepsy, phantom absences, absence status, and generalized tonic-clonic seizures. PATIENT CONCERNS: A 31-year-old woman presented with a 16-year history of paroxysmal convulsions. DIAGNOSES: Electroencephalogram (EEG) showed recurrent universal and synchronized 3~4 Hz spike waves and spike-slow waves in the interictal phase with normal background activity. During the ictal phases, EEG revealed bursts of 3~4 Hz spike waves and spike-slow waves that were universal, synchronized, and symmetrical. Additionally, there was 1 seizure episode induced by a 3-Hz flash in the current case. Based on these findings, a diagnosis of idiopathic generalized epilepsy was made. INTERVENTIONS: The patient was treated with oral sodium valproate, and the epileptic seizures were controlled. OUTCOMES: The frequency of absence seizures was significantly reduced and there were no generalized tonic-clonic seizures. LESSONS: Idiopathic generalized epilepsy with phantom absences, absence status, and generalized tonic-clonic seizures is an extremely rare condition. EEG is the exclusive method for diagnosis. Antiepileptic drugs are effective for controlling epileptic seizures in this disease.
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Epilepsia Tipo Ausencia , Epilepsia Generalizada , Epilepsia Tónico-Clónica , Estado Epiléptico , Femenino , Humanos , Adulto , Epilepsia Generalizada/diagnóstico , Epilepsia Generalizada/tratamiento farmacológico , Convulsiones/diagnóstico , Convulsiones/tratamiento farmacológico , Convulsiones/etiología , Epilepsia Tipo Ausencia/diagnóstico , Epilepsia Tipo Ausencia/tratamiento farmacológico , Epilepsia Tipo Ausencia/complicaciones , Estado Epiléptico/complicaciones , Ácido Valproico/uso terapéutico , Electroencefalografía , Epilepsia Tónico-Clónica/diagnóstico , Epilepsia Tónico-Clónica/tratamiento farmacológicoRESUMEN
BACKGROUND: The risk of developing epilepsy after de novo status epilepticus (SE) is nonnegligible. The individualized management of patients with high risk of subsequent epilepsy could improve long-term quality of life and cognitive impairment. We aimed to ascertain potential biomarkers of subsequent epilepsy and to construct a scoring system possessing predictive value for the diagnosis of post-SE epilepsy during follow-up. METHODS: The study data were obtained from a prospective registry of all SE episodes occurring in patients over 16 years attended in our tertiary center from February 2011 to April 2022. Clinical data, electroencephalography findings, treatment, and long-term clinical data were prospectively recorded. We selected SE patients at risk of developing epilepsy (acute symptomatic and cryptogenic etiologies with no previous history of epilepsy) and analyzed the risk of developing subsequent epilepsy. RESULTS: We included 230 patients. Median age was 65 years ± 16.9 SD and 112/230 (48.7 %) were women. One-hundred ninety-eight patients (86.1 %) had an acute symptomatic SE, whereas 32 patients (13.9 %) presented with a cryptogenic SE. A total of 55 patients (23.9 %) developed an unprovoked remote seizure and were diagnosed with epilepsy. After adjusting for identifiable confounders in a multivariable Cox regression analysis cryptogenic etiology (HR 2.24 [1.13-4.46], p = 0.022), first-line treatment initiation ≥1 h (HR 2.12 [1.03-4.36], p = 0.041], RDA/LPD/GPD EEG patterns (HR 1.88 [1.07-3.32], p = 0.028), and super-refractoriness (HR 2.90 [1.40-5.99], p = 0.004) emerged as independent predictors of post-SE epilepsy. Based on these findings, we constructed the AFTER score (1 point for each item) with a robust capability to predict post-SE epilepsy at 5 years (AUC 74.3 %, 95 %CI 64.3-84.3 %, p < 0.001). CONCLUSIONS: The AFTER score is a robust predictor of the development of epilepsy after new onset SE using clinical and electroencephalographic biomarkers (such as etiology, time to first-line treatment initiation, EEG pattern and super-refractoriness). Prospective studies are warranted to validate the score in other populations.
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Epilepsia , Estado Epiléptico , Humanos , Femenino , Anciano , Masculino , Calidad de Vida , Estudios Retrospectivos , Epilepsia/complicaciones , Epilepsia/diagnóstico , Estado Epiléptico/complicaciones , Estado Epiléptico/diagnóstico , Medición de Riesgo , Electroencefalografía/efectos adversos , BiomarcadoresRESUMEN
Febrile seizures (FSs) are the most common cause of pediatric seizures. They are defined as seizures in children age 6 months to 5 years with a temperature higher than 100.4°F, although they are more common at higher temperatures. A family history of FS is the most common risk factor. FSs are classified into three types (simple, complex, or febrile status epilepticus) based on duration and quality, with simple FSs accounting for many cases. Most FSs persist for less than 10 minutes and are self-limiting. Approximately one-third of patients will have recurrence of FSs. Safe and effective prophylaxis for FS has yet to be identified. Most patients will not have any long-term sequelae, although there is an increased risk of epilepsy, particularly for those with febrile status epilepticus. FSs are associated with caregiver anxiety, "fever phobia," and high health care use, emphasizing the importance of education and reassurance for both the provider and family. [Pediatr Ann. 2023;52(10):e388-e393.].