ABSTRACT
Resumen El FIRES (síndrome epiléptico relacionado con una infección febril) es un síndrome epiléptico poco frecuente que ocurre a cualquier edad en pacientes previamente sanos. Clínicamente presenta episodios febriles en un periodo de 24 horas a dos semanas previas al inicio del estatus epiléptico. Se consideran dos estadios de la enfermedad: estadio agudo, donde existe refractariedad al tratamiento de las convulsiones durante varias semanas, seguido por un estadio crónico en el cual la epilepsia es intratable, acompañado de diferentes grados de deterioro cognitivo. El estadio agudo se caracteriza por un estudio de resonancia magnética (RM) cerebral normal en la mayoría de los casos. Sin embargo el lóbulo temporal, así como las estructuras temporo-mesiales e hipocampo, pueden verse afectados. En el estadio crónico de la enfermedad, la atrofia difusa y/o esclerosis temporo-mesial unilateral o bilateral puede ser el hallazgo principal; no obstante también es posible una evolución sin lesiones secuelares. Comprender estas anomalías en la RM permite respaldar el diagnóstico de FIRES en el contexto de un paciente con epilepsia refractaria, ya que es un diagnóstico de exclusión.
Abstract FIRES (febrile infection-related epilepsy syndrome) is a rare epileptic syndrome occurring at any age in previously healthy patients. Clinically it presents with febrile episodes within 24 hours to two weeks prior to the onset of status epilepticus. Two phases or stages of the disease are considered: in the acute phase there is refractoriness in the treatment of seizures for several weeks, followed by a chronic phase in which epilepsy is intractable, accompanied by varying degrees of cognitive impairment. The acute phase is characterized by a normal brain magnetic resonance imaging (MRI) study in most cases. The temporal lobe including the temporo-mesial structures and hippocampus may be affected. In the chronic phase of the disease diffuse atrophy and/or uni- or bilateral temporo-mesial sclerosis may be found, a course without sequelae lesions is also possible. It is necessary to understand these abnormalities on MRI to support the diagnosis of FIRES as it is a diagnosis of exclusion.
ABSTRACT
Resumen Introducción: El estado epiléptico refractario (EER) constituye una emergencia médica grave, donde la crisis no cede a pesar del tratamiento farmacológico convencional. Se describe como estado epiléptico superrefractario (EESR) la continua presencia de episodios con una duración de 24 horas o más. Este reporte de caso detalla la complejidad en el manejo y explora un enfoque multidisciplinario. Presentación del caso: Paciente masculino de 32 años con antecedente de epilepsia focal secundaria a traumatismo craneoencefálico en la niñez, quien ingresó en contexto de EESR, el cual recibió coma barbitúrico, plasmaféresis y dieta cetogénica. En los exámenes, la resonancia magnética reveló una lesión en la región frontotemporal insular derecha; en la tomografía por emisión de positrones se observaron zonas de hipermetabolismo y en el videoelectroencefalograma una continua actividad epileptiforme. Se optó por la cirugía paliativa, logrando la resolución exitosa del EESR y una clasificación Engels IA a los 14 meses. Discusión: El EESR es un evento neurológico crítico con pronóstico reservado y opciones terapéuticas desafiantes. Se describen opciones terapéuticas desde anticonvulsivantes, inmunoterapia y cirugía, donde el abordaje quirúrgico emerge como una opción eficaz, especialmente en casos con lesiones estructurales. La identificación temprana y la terapia adecuada son vitales para prevenir complicaciones. Conclusiones: El EESR representa un desafío crítico con alta carga de morbimortalidad, sin embargo, la cirugía de epilepsia muestra promisorios resultados en el contexto de causa cerebral estructural, responsable de la actividad epileptiforme. Se destaca la importancia de la identificación temprana y el manejo quirúrgico paliativo como opción viable, mejorando la calidad de vida de los pacientes.
Abstract Introduction: Refractory Status Epilepticus (RSE) is a serious medical emergency where the seizure does not subside despite conventional pharmacological treatment. The continuous presence of episodes lasting 24 hours or more is described as super-refractory status epilepticus (SRSE). This case report details the complex in teraphy management and explores a multidisciplinary approach. Case presentation: A 32-year-old male with a history of focal epilepsy secondary to head trauma in childhood. Enters emergency with a RSE episode. Barbiturate coma, plasmapheresis and ketogenic diet were administered. The Magnetic Resonance Imaging revealed a lesion in the right fronto-temporo-insular cortex, areas of hypermetabolism on Positron Emission Tomography and continuous epileptiform activity on video-electroencephalogram. Palliative surgery was chosen, achieving successful resolution of the SRSE and Engel Scale IA classification at 14 months of follow up. Discussion: The SRSE is a critical neurological event with a guarded prognosis and complex therapeutic options. Therapeutic options are described from anticonvulsants, immunotherapy and surgery. The surgical approach emerges as an effective option, especially in cases with structural injuries. Early identification and appropriate therapy are vital to prevent complications. Conclusions: SRSE represents a critical challenge with a high burden of morbidity and mortality. However, epilepsy surgery shows promising results in the context of the structural brain cause responsible for epileptiform activity. The importance of early identification and palliative surgical management as a viable option is highlighted, improving the quality of life of patients.
ABSTRACT
Background: Status epilepticus (SE) is a medical emergency, and its neurological outcome is a concern to every pediatrician in developing countries. The incidence of convulsive SE in children is approximately 10�/100,000 per year, with the highest incidence in children less than one year of age. Approximately 30% of patients presenting with status epilepticus are having their first seizure. The objective of the study is to evaluate the clinical and etiological pattern of SE and its outcome in children admitted to PICU.Methods: This was a prospective study conducted among 50 patients aged 1 month to 12 years presenting with status epilepticus. The study was conducted over a period of one year from April 2022 to April 2023.Results: A majority of the patients were in the age group of 1-5 years (64%) and higher incidence of male children was observed (74%). Generalized tonic clonic seizure (GTCS) was predominant in 76% of the children and seizure duration was 10-20 minutes in 44% of the children. The most common etiologies were Meningoencephalitis (44%) and febrile seizures (20%). Nearly 70% of the children showed complete recovery with no neurological symptoms and 5 (10%) children died.Conclusions: Status epilepticus is a life-threatening emergency and timely management is essential to prevent morbidity and mortality. CNS infection is a major etiology and majority of the children showed good response with AED treatment.
ABSTRACT
Objective To investigate the predictive value of serum Nesfatin-1 and 25-hydroxyvitamin D3[25(OH)D3]levels for the short-term prognosis of status epilepticus(SE)in children.Methods A total of 104 children with SE admitted to the hospital from March 2020 to March 2023 were enrolled in the study,and the clinical data of the children were collected.According to the Glasgow outcome Scale(GOS)score at dis-charge,the children were divided into a good prognosis group(equal to 5 points)and a poor prognosis group(＜5 points).Univariate analysis and multivariate Logistic regression were used to analyze whether serum Nesfatin-1 and 25(OH)D3 levels were risk factors for poor short-term prognosis in children with SE.The re-ceiver operating characteristic(ROC)curve was drawn to analyze the predictive value of serum Nesfatin-1 and 25(OH)D3 levels for the short-term poor prognosis in children with SE.Results At discharge,85 children[81.73％(85/104)]with a GOS score of 5 were included in the good prognosis group,and 19 children[8.27％(19/104)]with a GOS score of＜5 were included in the poor prognosis group.There was no significant differ-ence in gender,age,previous history of epilepsy,and seizure types between the two groups(P＞0.05).There were significant differences in the duration of SE,the time from medication to seizure cessation,electroenceph-alogram(EEG)results,head CT results,and serum Nesfatin-1 and 25(OH)D3 levels between the two groups(P＜0.05).Multivariate Logistic regression analysis showed that SE duration＞60 min,abnormal head CT results,serum Nesfatin-1 and 25(OH)D3 levels were independent risk factors for the short-term poor progno-sis of children with SE(OR=1.945,2.343,1.731,1.505;P＜0.05).The area under the ROC curve of serum Nesfatin-1 and 25(OH)D3 levels combined to predict poor short-term prognosis of children with SE was 0.840(95％CI:0.732-0.949),which was better than that of serum Nesfatin-1 and 25(OH)D3 levels alone[0.607(95％CI:0.453-0.761),0.742(95％CI:0.604-0.880)],respectively.Conclusion Serum Nesfatin-1 and 25(OH)D3 levels are risk factors for poor prognosis in children with SE,and the combination of them has high predictive value for poor prognosis in children with SE.
ABSTRACT
Objective To investigate the etiology and outcome of elderly patients with new-onset status epilepticus(NOSE).Methods According to the electronic medical record,the keywords"Epilepsy"and"Status epilepticus"were searched for elderly patients with status epilepticus admitted to Sichuan Provincial People's Hospital between January 2018 and June 2023.Elderly patients with NOSE were strictly screened according to inclusion and exclusion criteria,and etiology were analyzed according to medical history and ancillary examinations,and factors related to prognosis of epilepsy were analyzed by Logistic regression.Results There were 63 elderly NOSE patients,including 38 males and 25 females,with an average age of(72.71±7.45)years old.Cerebrovascular disease(21%)was determined as the major cause of NOSE in elderly patients,followed by central nervous system infection(17%).Logistic regression analysis of prognostic factors showed that co-infection(OR = 11.67,95%CI:1.391-97.850,P =0.024)and renal insufficiency(OR =18.90,95%CI:3.522-101.43,P = 0.001)were associated with poor patient prognosis.Conclusions Cerebrovascular disease is the main known cause of NOSE in elderly patients.Prevention of infection and improvement of renal function may improve prognosis.
ABSTRACT
Mitochondrial and peroxisome fission deficiency-related encephalopathy caused by DNM1L gene mutation is a rare and fatal epileptic encephalopathy, with clinical phenotype and genetic heterogeneity. The acute stage is drug-resistant epilepsy with poor prognosis and serious neurological sequelae. A case of genetically confirmed encephalopathy related to mitochondrial and peroxisome fission defects is reported, the clinical data, treatment process are summarized, and the previous literature is reviewed to improve the understanding of the rare disease.
ABSTRACT
New onset refractory status epilepticus (NORSE) and its subcategory, febrile infection-related epilepsy syndrome (FIRES) are rare and devastating conditions, initial symptoms of which are often refractory status epilepticus. NORSE/FIRES is characterized by high mortality and disability, but unfortunately, due to the unclear pathogenesis, the diagnosis and treatment of NORSE/FIRES are very challenging. This editorial aims to summarize the key points and recent developments in the etiology, pathophysiology, diagnosis and management of these challenging conditions.
ABSTRACT
@#Electrical status epilepticus during sleep (ESES) is an electrographic pattern associated with specific genetic disorders, brain malformations, and use of some antiseizure medications. This case report aims to present the management of ESES in Sotos syndrome (SoS) on carbamazepine. A nine-year-old Filipino male with clinical features suggestive of overgrowth syndrome presented with febrile seizure at one year old. Cranial imaging showed cavum septum pellucidum, corpus callosal dysgenesis, and ventriculomegaly. He was on carbamazepine monotherapy starting at three years old. A near continuous diffuse spike–wave discharges in slow wave sleep was recorded at nine years old hence shifted to valproic acid. Follow-up study showed focal epileptiform discharges during sleep with disappearance of ESES. Next generation sequencing tested positive for rare nonsense mutation of nuclear receptor binding set-domain protein 1 confirming the diagnosis of SoS. Advanced molecular genetics contributed to determination of ESES etiologies. To date, this is the first documented case of SoS developing ESES. Whether an inherent genetic predisposition or drug-induced, we recommend the avoidance of carbamazepine and use of valproic acid as first-line therapy.
Subject(s)
Sotos Syndrome , CarbamazepineABSTRACT
Objective:To validate and compare the value of the Status Epilepticus in Pediatric Severity Score (STEPSS) versus PEDSS in assessing the short-term prognosis of children with status epilepticus (SE).Methods:Clinical data of 152 children with SE hospitalized at the First Affiliated Hospital of Zhengzhou University from January 2020 to December 2022 were retrospectively analyzed.According to the STEPSS and PEDSS scores, children with SE were scored and their prognosis was predicted.Receiver operating characteristic (ROC) curves of the 2 scales in assessing the short-term prognosis of SE in children were plotted, and the area under the curve (AUC), optimal cut-off, sensitivity and specificity were calculated, thus validating and comparing the value of the STEPSS versus PEDSS in assessing the short-term prognosis of children with SE.Results:Of the 152 children with SE, 90 were male and 62 were female, with the age of (5.8±3.9) years (1 month to 15 years). There were 112 cases with good prognosis and 40 cases with poor prognosis, involving 13 deaths.The AUC of STEPSS and PEDSS scores in predicting the death in children with SE were 0.908(95% CI: 0.848-0.967) and 0.887(95% CI: 0.831-0.942), respectively, both with the optimal cut-off value of 4.The sensitivity of STEPSS and PEDSS scores in predicting the death in children with SE were 0.740 and 0.846, respectively, and the specificity were 0.745 and 0.835, respectively.There was no significant difference in predicting the death in children with SE between the 2 scales ( P>0.05). In predicting adverse outcomes, the AUC of the STEPSS and PEDSS scores were 0.869(95% CI: 0.800-0.937) and 0.926(95% CI: 0.873-0.979), respectively, both with the optimal cut-off value of 3.The sensitivity of STEPSS and PEDSS scores in predicting adverse outcomes in children with SE were 0.827 and 0.900, respectively, and the specificity were 0.732 and 0.866, respectively.There was significant difference in predicting the adverse outcomes in children with SE between the 2 scales ( P<0.05). Conclusions:Compared with the STEPSS, the PEDSS has a higher application in predicting the short-term treatment outcome of children with SE, which can be used as a routine method to assess the prognosis of children with SE.
ABSTRACT
Objective To explore EEG characteristics and the therapeutic effect in children with electrical status epilepticus during slow sleep(ESES).Methods The eligible ESES cases in our center from 2014 to 2020 were included.The age at diagnosis of ESES,the duration of ESES,spike wave index(SWI)during wakefulness and the distribution of spike wave during the period of ESES,age at seizure onset,the clinical syndromes and the outcomes after treatment were analyzed.The ESES cases were divided into 4 groups according to the distribution of spike wave:focal ESES,unilateral ESES,bilateral asymmetric ESES,multiple foci ESES.The SWI during the awake stage were divided into 3 groups based on the different rates:≤20%,21%～49%,≥50%.The therapeutic outcomes were classified into three groups:satisfactory response,seizure control and ineffective.Results 50 cases were included,with 32 males and 18 females.The average onset age of ESES was 6 years and 7 months,and the average duration of ESES was 28 months.A significant correlation between the distribution of ESES and the thera-peutic effects was found,bilateral asymmetric ESES had a good therapeutic effects,while multiple foci ESES showed a poor therapeutic effects.The duration of ESES was significantly correlated with therapeutic effects,and the efficacy was worse when the duration was longer than 1 year.A significant relationship between the SWI during wakefulness of ESES and the therapeutic effects was detected,the patient with SWI≤20%during wakefulness had a good therapeutic effect.There was a negative correlation between the onset age of ESES and the duration of ESES and SWI index during wakefulness.There was a positive correlation between the duration of ESES and SWI index during wakefulness.Conclusion Our results suggest that onset age,distribution,duration and SWI during wake-fulness of ESES were correlated with therapeutic outcomes,The patient with SWI≤20%during wakefulness had a good therapeutic effect and have unfavorable outcomes with ESES last more than 1 year.The earlier onset of ESES,the longer duration of ESES and higher SWI during wakefulness will be showed..
ABSTRACT
Resumen El estado epiléptico (SE) es la emergencia neurológica más frecuente en neuropediatría. Es el resultado de la falla de los mecanismos responsables en terminar una crisis epiléptica o del inicio, que conduce a una crisis epiléptica prolongada. La incidencia estimada entre 3-42 casos por cada 100.000 personas por año. Tiene una distribución bimodal afectando a los extremos de la vida; niños y ancianos, las estimaciones de mortali dad son variables en función de la edad y la etiología, en los niños la mortalidad podría ser más baja que en adultos pero alcanza una alta morbilidad hasta un 66%. La definición ha cambiado en el transcurso de los años con el fin de especificar inicio de tratamiento y comple mentar con los datos científicos se ha establecido un tiempo t1 y un t2. El tiempo (t1) es el momento cuando el tratamiento debe comenzar, que varía dependiendo de la semiología, a los 5 minutos para una crisis con vulsiva tónico clónica generalizada y a los 10 minutos para una crisis focal. El segundo tiempo (t2) se refiere al daño neuronal. El tratamiento rápido y eficaz disminuye los riesgos de complicaciones cardíacas y respiratorias, ingreso a unidades de cuidados intensivos y muerte.
Abstract Status epilepticus (SE) is the most frequent neuro logical emergency in neuropediatrics. It is the result of the failure of the mechanisms responsible for terminat ing an epileptic seizure or its onset, which leads to a prolonged epileptic seizure. The estimated incidence between 3-42 cases per 100,000 people per year. It has a bimodal distribution, affecting children and the elderly at the extremes of life. Mortality estimates are variable depending on age and etiology. Mortality in children could be lower than in adults, but it reaches a high morbidity of up to 66%. The definition has changed over the years in order to specify the start of treatment and to complement it with the scientific data, a time t1 and a t2 have been established. The time (t1) is the moment when treatment should begin, which varies depending on the semiology, at 5 minutes for a generalized tonic-clonic seizure and at 10 minutes for a focal seizure. The second time (t2) refers to neuronal damage. Prompt and effective treatment decreases the risks of cardiac and respiratory complications, admission to intensive care units, and death.
ABSTRACT
Introducción: la necrosis laminar cortical es un término radiológico que describe la presencia de lesiones hiperdensas de localización cerebral, las cuales siguen una distribución giriforme y se observan con mayor sensibilidad en los estudios de resonancia magnética cerebral (RM). Esta condición patológica, que afecta a la corteza del cerebro, suele ser secundaria a una depleción de sus fuentes energéticas como consecuencia de hipoxia cerebral, alteraciones metabólicas, hipoglicemia, falla renal o hepática, intoxicaciones o infecciones. Presentación del caso: se reporta el caso de un hombre de 23 años, con antecedente de consumo crónico de alcohol, quien ingresó al servicio de urgencias de nuestra institución con un estado epiléptico. El estudio de resonancia magnética cerebral demostró la presencia de una necrosis laminar cortical con posterior déficit neurocognitivo y funcional. Conclusión: si se consideran las secuelas neurológicas potenciales asociadas a un estado epiléptico, relacionadas con necrosis laminar cortical cerebral, es necesario hacer un diagnóstico etiológico precoz, así como una atención terapéutica temprana a los pacientes.
Introduction: Cortical laminar necrosis (CLN) is radiologically defined as high-intensity cortical lesions on T1-weighted MRI images that follow a gyral distribution in the brain. Histopathologically, this pathological condition is characterized by necrosis of the cortex involving neurons, glial cells, and blood vessels. It is usually triggered by hypoxia, metabolic alterations, drugs, intoxications, or infections. Case description: We report the case of a 23-year-old man with a history of chronic alcohol abuse who was admitted to our institution with status epilepticus. The brain magnetic resonance imaging performed on this patient showed cortical laminar necrosis associated with subsequent neurocognitive deficits. Conclusion: Due to the potential neurological sequelae secondary to status epilepticus in relation to cortical laminar necrosis as permanent brain damage, it is necessary to provide early diagnosis and treatment for these patients.
Subject(s)
Status Epilepticus , Hypoxia, Brain , Cerebral Cortex , NeuroimagingABSTRACT
Abstract Background: Status epilepticus (SE) is a neurological emergency. Non-convulsive status epilepticus (NCSE) can only be diagnosed by electroencephalogram (EEG) because the motor clinical symptoms are usually subtle or absent, with high mortality. The best treatment is still unknown. Objectives: Our aim was to assess anticonvulsive and anesthetic drugs in NCSE and their correlation with Epidemiology-based Mortality Score in Status Epilepticus (EMSE), Status Epilepticus Severity Score (STESS) and mortality. Methods: Retrospective, observational, descriptive, cross-sectional study. Ninety patients in intensive care unit over 18 years-old (57 females [63.3%] and 33 males [36.6%], mean age 63.5 years [SD ± 19]) with NCSE, at the Buenos Aires British Hospital. Data was collected between January 2018 and June 2021. An adjusted mul tivariate statistical analysis was performed. Ninety-five (95%) CI, p<0.05 as statistically significant. EMSE and STESS were used in this study. Results: Total mortality rate was 37.8% (34/90), and in patients ≥ 65 years-old (54/90) it was 40.7% (22/54). Patients with 0-2 STESS (11/90) were discharged, while those with STESS ≥ 3 (79/90) had a 43% death rate (34/79). Patients with EMSE < 34 (27/90) had 7.4% (2/27) death rate, while those with EMSE ≥ 34 (63/90) had 50.8% (32/63). No significant differences were found in survival with regard to the number of antiepileptic drugs administered. Pa tients treated with anesthetics presented a 2.6-fold death risk increase (95% CI 1.001-6.83). Discussion: It could be assumed that mortality rate increases 2.6-fold when patients are treated with anes thetic drugs, regardless of the number of antiepileptic drugs previously administered.
Resumen Introducción: El estado de mal epiléptico (SE) es una emergencia neurológica. El SE no convulsivo (SENC) se diagnostica únicamente por electroencefalograma de bido a la ausencia o sutileza de sintomatología clínica motora, con una mortalidad elevada. No se conoce aún el mejor tratamiento. Objetivos: Evaluar drogas anticonvulsivas y anestési cas en el SENC y su correlación con Epidemiology-based Mortality Score in Status Epilepticus (EMSE), Status Epilep ticus Severity Score (STESS) y el índice de mortalidad. Métodos: Estudio retrospectivo, observacional, de scriptivo, de corte transversal. Noventa pacientes ≥ 18 años (57 mujeres [63.3%] y 33 hombres [36.6%], media de edad 63.5 años [DS ± 19]) con diagnóstico de SENC, en el Hospital Británico. Estudio realizado entre enero 2018 y junio 2021. Análisis estadístico multivariado ajustado. IC 95% p< 0.05 como estadísticamente significativo. Se utilizaron escalas de EMSE y STESS. Resultados: La mortalidad total fue de 37.8% (34/90). Los pacientes ≥ 65 años (54/90) presentaron una mayor tasa de muerte 40.7% (22/54), todos aquellos con STESS de 0-2 (11/90) egresaron, mientras que entre los que presentaron ≥ 3 (79/90) el 43% (34/79) falleció. De los pacientes con EMSE < 34 (27/90) dos fallecieron (7.4%) y de aquellos con EMSE ≥ 34 (63/90) falleció el 50.8% (32/63). No hallamos diferencias significativas entre cantidad de drogas antiepilépticas utiliza das y supervivencia. Pacientes con anestésicos tuvieron un aumento del riesgo de muerte 2.6 veces (IC 95% 1.001-6.83). Discusión: De acuerdo a esto la mortalidad con drogas anestésicas aumenta, independientemente de la cantidad de drogas anticonvulsivas utilizadas previamente.
ABSTRACT
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.
ABSTRACT
Introducción. El estado epiléptico constituye la emergencia neurológica más frecuente. Si bien la mortalidad en niños es baja, su morbilidad puede superar el 20 %. Objetivo. Conocer las pautas de manejo del estado epiléptico referidas por médicos pediatras que atienden esta patología en forma habitual. Población y métodos. Estudio descriptivo, transversal, basado en una encuesta a médicos de tres hospitales pediátricos monovalentes de gestión pública de la Ciudad Autónoma de Buenos Aires. Resultados. Se administraron 292 encuestas (la tasa de respuesta completa alcanzó el 86 %); el 77 % se administró a pediatras y el 16 %, a especialistas en cuidados intensivos. Un 47 % de los participantes refiere indicar la primera benzodiacepina en el tiempo correcto; el 56 % utilizar diazepam intrarrectal en ausencia de un acceso intravenoso; el 95 % elige lorazepam como benzodiacepina inicial en caso de contar con acceso intravenoso; el 58 % refiere iniciar la etapa de fármacos de segunda línea en tiempo adecuado; el 84 % opta por fenitoína como fármaco inicial de segunda línea, un 33 % no cronometra el tiempo durante el tratamiento. La adherencia global a las recomendaciones internacionales fue del 17 %. Conclusiones. Nuestro estudio advierte una baja adherencia referida de los pediatras a las guías internacionales, en particular en las decisiones tiempo-dependientes. También se observó mayor heterogeneidad en las conductas terapéuticas a medida que se avanza en el algoritmo de tratamiento.
Introduction. Status epilepticus is the most common neurological emergency. Although mortality in children is low, morbidity may exceed 20%. Objective. To evaluate the management of status epilepticus by pediatricians who usually treat this condition. Population and methods. Descriptive, cross-sectional study based on a survey administered to physicians from 3 pediatric hospitals in the City of Buenos Aires. Results. A total of 292 surveys were administered (complete response rate as high as 86%); 77% were administered to pediatricians and 16% to intensive care specialists. Forty-seven percent of the participants reported that they administer the first dose of a benzodiazepine within the correct timeframe; 56% use intrarectal diazepam when intravenous access is not available; 95% choose lorazepam as the initial benzodiazepine if an intravenous access is available; 58% initiate the administration of a second-line drug within the correct timeframe; 84% administer phenytoin as the first-choice, second-line drug; and 33% do not measure treatment time. Overall adherence to international recommendations was 17%. Conclusions. Our study highlights poor adherence of pediatricians to international guidelines, particularly in time-dependent decisions. Greater heterogeneity was observed in treatment approaches as the treatment algorithm progressed.
Subject(s)
Humans , Child , Status Epilepticus/diagnosis , Status Epilepticus/drug therapy , Argentina , Cross-Sectional Studies , Diazepam/therapeutic use , Hospitals, Pediatric , Anticonvulsants/therapeutic useABSTRACT
Objective:To evaluate the predictive values of the Status Epilepticus in Pediatric Patients Severity Score (STEPSS) and END-IT score in the short-term prognosis of children with status epilepticus (SE).Methods:It was a retrospective study involving 103 children with SE who were admitted to the Qingdao Women and Children′s Hospital Affiliated to Qingdao University from January 1, 2012 to January 1, 2022.Glasgow Outcome Scale was used to evaluate the prognosis at discharge, and the children were divided into good prognosis group ( n=78) and poor prognosis group ( n=25). Risk factors for poor prognosis of SE in children were analyzed by Logistic regression.Receiver operating characteristic (ROC) curve was used to evaluate the prognostic values of STEPSS and END-IT score in children with SE. Results:Compared with those of the good prognosis group, significantly younger age [16 (9, 58) months vs.56 (21, 84) months, Z=-3.068, P=0.002], higher blood lactic acid levels [3.16 (2.43, 4.01) mmol/L vs.1.67 (1.32, 2.10) mmol/L, Z=-6.085, P<0.001], STEPSS scores [3.0(3.0, 4.0) points vs.1.0(1.0, 2.0) points, Z=-6.956, P<0.001], END-IT scores [3.0(1.5, 4.0) points vs.1.0(0, 1.0) points, Z=-5.502, P<0.001], proportion of developmental delay ( χ2=16.756, P<0.001), abnormal brain magnetic resonance imagine examination ( χ2=5.860, P=0.015), use of ventilator and multiple drugs (all P<0.001), and longer duration of anti-SE therapy time( Z=1.488, P=0.024) were detected in the poor prognosis group. Logistic regression analysis indicated that increased blood lactic acid ( OR=7.975, 95% CI: 2.705-23.518), increased drug types ( OR=14.562, 95% CI: 2.035-104.173), STEPSS scores( OR=8.914, 95% CI: 2.824-28.140) and END-IT scores ( OR=2.209, 95% CI: 1.046-4.667) were risk factors for the poor prognosis of SE in children.The area under the curve (AUC) of STEPSS in predicting the poor prognosis of SE in children was 0.939, with the cut-off value, sensitivity, specificity and Youden index of 2.5 points, 96.0%, 85.9% and 0.82, respectively.AUC of END-IT scores in predicting the poor prognosis of SE in children was 0.853, with the cut-off value, sensitivity, specificity and Youden index of 1.5 points, 76.0%, 75.6% and 0.52, respectively.AUC of STEPSS in predicting the poor prognosis of SE in children was significantly higher than that of END-IT scores ( U=36.91, P<0.05). The predictive value of STEPSS combined with END-IT was higher, and the sensitivity and negative predictive value of parallel test were 100.0%, while the specificity and positive predictive value of series test were 94.9% and 81.8%, respectively. Conclusions:STEPSS and END-IT scores may be used as predictors for the poor prognosis of SE in children.Their combination provides a better prediction.
ABSTRACT
An adult female patient of internal nuclear inclusion disease(NIID)with a major clinical manifestation of decreased intelligence and recurrent disturbance of consciousness was followed up for nearly 4 years.In particular,the evolution of the video-electroencephalogram(VEEG),the auxiliary diagnosis of VEEG,and the prediction value of VEEG for clinical outcome were summarized.We found that(1)the background rhythm in NIID patients evolved with the progression of the disease;(2)electroencephalogram reactivity could predict the outcome of NIID coma;(3)VEEG helped to determine whether the consciousness disturbance caused by NIID was due to non-convulsive status epilepticus,This case provides a new idea to explore the application of VEEG in NIID.
ABSTRACT
Febrile infection-related epilepsy syndrome(FIRES)is a lethal encephalopathy with refractory status epilepticus as the main manifestation,and the selection of effective antiepileptic drugs(AEDs)is the focus and difficulty of treatment.This review summarises the mechanism of action,drug administration and adverse effects of different anti-inflammatory immunotherapies in the treatment of this disease,and finds that early initiation of ketogenic diet(KD)is one of the most effective treatments at present,and further studies are needed to clarify the adverse effects of the drugs and the effects of the combination of different drugs.
ABSTRACT
Objective:To improve the knowledge of clinicians about absence status epilepsy (ASE).Methods:The ictal clinical manifestations, the interictal and ictal phase electroencephalogram (EEG) characteristics of 1 patient with ASE in the Department of Epilepsy, Beijing Tiantan Hospital, Capital Medical University on November 20, 2022 were analyzed, and the pathogenesis, clinical and EEG characteristics, differential diagnosis, treatment experience of ASE were summarized by retrospective analysis combined with previous literature review.Results:The patient was a 38-year female, with onset age of 18 years. The main clinical manifestations were repeated prolonged decline of consciousness with generalized tonic-clonic seizures. The long-range video EEG showed generalized spike-slow wave and slow wave sporadic in the interictal period, and continuous or sub-continuous 2.5-3.0 Hz generalized spike-slow wave discharge in the ictal period, and the laboratory and imaging findings were normal. A total of 17 patients consistent with ASE diagnosis were reported in 7 English literatures, and there were totally 18 cases (including this patient) analyzed in this paper. ASE is a specific subtype of idiopathic generalized epilepsy (IGE). The ratio of male to female was 1∶1. The onset age of 16/18 patients was in youth to middle age, and the average onset age was 25.3 years. Absence status epilepticus was the representative or main seizure type, and 15/18 patients presented with infrequent generalized tonic-clonic seizures, no independent myoclonic seizures were found. The interictal EEG showed generalized or incomplete generalized spike-wave or poly spike-wave discharges around 3.0 Hz under normal background, and may have focal non-localizing discharges. The ictal EEG presented as continuous or sub-continuous rhythmical or non-rhythmical≥2.5 Hz (mainly 2-4 Hz) generalized spike or poly spike-wave discharges. ASE mainly needed to be distinguished from de novo late-onset absence status epilepticus, adult IGE with phantom absences. The first choice of drugs included valproate sodium and lamotrigine. The correct drug selection resulted in better prognosis. Conclusions:ASE is clinically rare. By far, its pathogenesis is not well understood. ASE can easily be misdiagnosed as non-epileptic or focal seizure. For patients with late onset, infrequent generalized tonic-clonic seizures who are clinically consistent with IGE, ASE is necessary to be vigilant when there are long-term changes in consciousness, response ability and behavior. Long-term video EEG monitoring should be perfected in time to avoid misdiagnosis and mistreatment.
ABSTRACT
@#Electrical status epilepticus is a special electroencephalogram phenomenon，which means that the spike and slow waves are almost continuously emitted during the wake-sleep phases. Related concepts are epileptic encephalopathy with electrical status epilepticus during slow wave sleep，electrical status epilepticus in sleep，and subclinical electrographic seizures. The above related concepts are widely used in clinical practice，but there is a lack of unified criteria. There are abuses and misuses of these concepts. Clarifying related concepts is of great significance for scientific research and clinical practice.