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1.
Acta Medica Philippina ; : 83-87, 2024.
Artigo em Inglês | WPRIM | ID: wpr-1012804

RESUMO

@#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.


Assuntos
Síndrome de Sotos , Carbamazepina
2.
Medicina (B.Aires) ; 83(supl.4): 82-88, oct. 2023. graf
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1521207

RESUMO

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.

3.
Acta neurol. colomb ; 39(3)sept. 2023.
Artigo em Espanhol | LILACS | ID: biblio-1533504

RESUMO

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.


Assuntos
Estado Epiléptico , Hipóxia Encefálica , Córtex Cerebral , Neuroimagem
4.
Medicina (B.Aires) ; 83(2): 202-211, jun. 2023. graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1448622

RESUMO

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.

5.
Arch. argent. pediatr ; 121(2): e202202696, abr. 2023. tab, graf
Artigo em Inglês, Espanhol | LILACS, BINACIS | ID: biblio-1418352

RESUMO

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.


Assuntos
Humanos , Criança , Estado Epiléptico/diagnóstico , Estado Epiléptico/tratamento farmacológico , Argentina , Estudos Transversais , Diazepam/uso terapêutico , Hospitais Pediátricos , Anticonvulsivantes/uso terapêutico
6.
Chinese Journal of Applied Clinical Pediatrics ; (24): 575-579, 2023.
Artigo em Chinês | WPRIM | ID: wpr-990082

RESUMO

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.

7.
Neuroscience Bulletin ; (6): 1683-1702, 2023.
Artigo em Inglês | WPRIM | ID: wpr-1010651

RESUMO

Parvalbumin interneurons belong to the major types of GABAergic interneurons. Although the distribution and pathological alterations of parvalbumin interneuron somata have been widely studied, the distribution and vulnerability of the neurites and fibers extending from parvalbumin interneurons have not been detailly interrogated. Through the Cre recombinase-reporter system, we visualized parvalbumin-positive fibers and thoroughly investigated their spatial distribution in the mouse brain. We found that parvalbumin fibers are widely distributed in the brain with specific morphological characteristics in different regions, among which the cortex and thalamus exhibited the most intense parvalbumin signals. In regions such as the striatum and optic tract, even long-range thick parvalbumin projections were detected. Furthermore, in mouse models of temporal lobe epilepsy and Parkinson's disease, parvalbumin fibers suffered both massive and subtle morphological alterations. Our study provides an overview of parvalbumin fibers in the brain and emphasizes the potential pathological implications of parvalbumin fiber alterations.


Assuntos
Camundongos , Animais , Epilepsia do Lobo Temporal/patologia , Parvalbuminas/metabolismo , Doença de Parkinson/patologia , Neurônios/metabolismo , Interneurônios/fisiologia , Modelos Animais de Doenças , Encéfalo/patologia
8.
Braz. j. biol ; 83: 1-10, 2023. ilus, graf, tab
Artigo em Inglês | LILACS, VETINDEX | ID: biblio-1469019

RESUMO

Only few studies have focus on animals that received Pilocarpine (Pilo) and did not develop behavioral status epilepticus (SE) and, whether they may become epileptic in the model's chronic phase. Previews works observed mossy fiber sprouting in the hippocampus of Non-SE (NSE) rats, while others observed spontaneous and recurrent seizures (SRS) 6 - 8 months after animals received Pilo. It is known that neuronal excitability is influenced by female hormones, as well as, the occurrence of SE in castrated and non-castrated female rats. However, it is not known whether females that received Pilo and did not show SE, may have SRS. The aim of this work was to investigate whether castrated and non-castrated female rats that did not show behavioral SE after Pilo, will develop SRS in the following one-year. For that, animals received 360 mg/kg of Pilo and were video monitored for 12 months. SE females from castrated and non-castrated groups became epileptic since the first month after drug injection. Epileptic behaviors were identified watching video monitoring recordings in the fast speed. Castrated and Non castrated NSE animals showed behaviors resembling seizures described by Racine Scale stages 1 - 3. Motor alterations showed by NSE groups could be observed only when recordings were analyzed in slow speed. In addition, behavioral manifestations as, rhythmic head movements, sudden head movements, whole body movements and immobility were also observed in both, SE and NSE groups. We concluded that NSE female rats may have become epileptic. Adding to it, slow speed analysis of motor alterations was essential for the observation of NSE findings, which suggests that possibly many motor alterations have been underestimated in epilepsy experimental research.


Poucos são os estudos com foco em animais que receberam Pilocarpina (Pilo) e não desenvolveram status epilepticus (SE) comportamental e, se os mesmos se tornarão epilépticos na fase crônica do modelo. Autores observaram o brotamento das fibras musgosas no hipocampo de ratos Não-SE (NSE), enquanto outros observaram crises espontâneas e recorrentes (CER) 6 - 8 meses após receberam a droga. A excitabilidade neuronal é influenciada pelos hormônios femininos e, da mesma forma, a ocorrência de SE em ratas castradas e não-castradas. Entretanto, não é sabido se as fêmeas que não apresentam SE terão CER. O objetivo deste trabalho foi investigar se fêmeas castradas e não castradas que não tiveram SE comportamental após a injeção de Pilo desenvolverão CER dentro de um ano. Para isto, os animais receberam 360 mg/kg de Pilo e foram videomonitorados por 12 meses. As fêmeas SE castradas e não-castradas se tornaram epilépticas desde o primeiro mês pós Pilo. O comportamento epiléptico foi identificado assistindo as gravações na velocidade rápida. As fêmeas NSE castradas e não-castradas apresentaram comportamentos similares aos estágios 1 - 3 da Escala de Racine. As alterações motoras nestes grupos (NSE) foram observadas apenas quando as videomonitoração foi analisada na velocidade lenta. Além destas, manifestações comportamentais como movimentos rítmicos da cabeça, movimentos súbitos da cabeça, movimentos de todo o corpo e imobilidade também foram observadas em ambos grupos, SE e NSE. Concluímos que as fêmeas NE podem ter se tornado epilépticas. Adicionado a isto, a análise das alterações motoras na velocidade lenta foi essencial para a observação dos achados das fêmeas NSE, o que sugere que possivelmente muitas alterações motoras têm sido subestimados na pesquisa em epilepsia experimental.


Assuntos
Feminino , Animais , Ratos , Epilepsia/induzido quimicamente , Epilepsia/veterinária , Modelos Animais , Pilocarpina/administração & dosagem , Pilocarpina/efeitos adversos , Pilocarpina/farmacologia
9.
Braz. j. biol ; 832023.
Artigo em Inglês | LILACS-Express | LILACS, VETINDEX | ID: biblio-1469238

RESUMO

Abstract Only few studies have focus on animals that received Pilocarpine (Pilo) and did not develop behavioral status epilepticus (SE) and, whether they may become epileptic in the models chronic phase. Previews works observed mossy fiber sprouting in the hippocampus of Non-SE (NSE) rats, while others observed spontaneous and recurrent seizures (SRS) 6 - 8 months after animals received Pilo. It is known that neuronal excitability is influenced by female hormones, as well as, the occurrence of SE in castrated and non-castrated female rats. However, it is not known whether females that received Pilo and did not show SE, may have SRS. The aim of this work was to investigate whether castrated and non-castrated female rats that did not show behavioral SE after Pilo, will develop SRS in the following one-year. For that, animals received 360 mg/kg of Pilo and were video monitored for 12 months. SE females from castrated and non-castrated groups became epileptic since the first month after drug injection. Epileptic behaviors were identified watching video monitoring recordings in the fast speed. Castrated and Non-castrated NSE animals showed behaviors resembling seizures described by Racine Scale stages 1 - 3. Motor alterations showed by NSE groups could be observed only when recordings were analyzed in slow speed. In addition, behavioral manifestations as, rhythmic head movements, sudden head movements, whole body movements and immobility were also observed in both, SE and NSE groups. We concluded that NSE female rats may have become epileptic. Adding to it, slow speed analysis of motor alterations was essential for the observation of NSE findings, which suggests that possibly many motor alterations have been underestimated in epilepsy experimental research.


Resumo Poucos são os estudos com foco em animais que receberam Pilocarpina (Pilo) e não desenvolveram status epilepticus (SE) comportamental e, se os mesmos se tornarão epilépticos na fase crônica do modelo. Autores observaram o brotamento das fibras musgosas no hipocampo de ratos Não-SE (NSE), enquanto outros observaram crises espontâneas e recorrentes (CER) 6 - 8 meses após receberam a droga. A excitabilidade neuronal é influenciada pelos hormônios femininos e, da mesma forma, a ocorrência de SE em ratas castradas e não-castradas. Entretanto, não é sabido se as fêmeas que não apresentam SE terão CER. O objetivo deste trabalho foi investigar se fêmeas castradas e não castradas que não tiveram SE comportamental após a injeção de Pilo desenvolverão CER dentro de um ano. Para isto, os animais receberam 360 mg/kg de Pilo e foram videomonitorados por 12 meses. As fêmeas SE castradas e não-castradas se tornaram epilépticas desde o primeiro mês pós Pilo. O comportamento epiléptico foi identificado assistindo as gravações na velocidade rápida. As fêmeas NSE castradas e não-castradas apresentaram comportamentos similares aos estágios 1 - 3 da Escala de Racine. As alterações motoras nestes grupos (NSE) foram observadas apenas quando as videomonitoração foi analisada na velocidade lenta. Além destas, manifestações comportamentais como movimentos rítmicos da cabeça, movimentos súbitos da cabeça, movimentos de todo o corpo e imobilidade também foram observadas em ambos grupos, SE e NSE. Concluímos que as fêmeas NE podem ter se tornado epilépticas. Adicionado a isto, a análise das alterações motoras na velocidade lenta foi essencial para a observação dos achados das fêmeas NSE, o que sugere que possivelmente muitas alterações motoras têm sido subestimados na pesquisa em epilepsia experimental.

10.
Artigo em Espanhol | LILACS-Express | LILACS | ID: biblio-1431732

RESUMO

Introducción: Las malformaciones arteriovenosas cerebrales (MAV) son un conjunto anormal de arterias y venas dilatadas del cerebro y caracterizadas por la pérdida de la organización vascular con una derivación arteriovenosa anormal. La probabilidad de ruptura de una MAV es baja, pudiendo causar déficit hasta en el 45% de los pacientes. La epilepsia puede asociarse con MAV siendo raro el estado epiléptico. La evolución a estado epiléptico refractario o estado epiléptico super refractario (EESR) es excepcional. Se presenta paciente con epilepsia asociada a MAV, con embolización y posterior complicación que evoluciona a EESR, donde se logró una resolución favorable con cirugía. Caso clínico: Paciente masculino de 70 años, con MAV temporal derecha no rota y epilepsia, libre de crisis desde el diagnóstico de ambas condiciones. Se realizó embolización logrando exclusión, pero con evolución a EESR. Tras estudio se realiza resección de MAV y resección parcial de zona de inicio de actividad epileptógena parieto-temporal. Además, se realizó una desconexión de región fronto-parietal central donde había evidencia de propagación de la actividad epiléptica. Paciente logra salir de estado crítico, con recuperación de conciencia y normalización de electroencefalogramas posquirúrgicos. A los 2 años evoluciona con epilepsia controlada (Engel IIa). Discusión y conclusiones: El abordaje quirúrgico es una opción en EESR y debe plantearse en un tiempo precoz de evolución (1-2 semanas). La cirugía se realizó para controlar EESR y fue una intervención exitosa. Esta estrategia puede lograr un cambio dramático en el pronóstico. La cirugía resectiva está indicada cuando hay evidencia etiológica en imágenes y focalidad eléctrica consistente en los estudios.


Introduction: Brain Arteriovenous Malformations (AVM) are an abnormal set of dilated arteries and veins within the brain and are characterized by loss of vascular organization with an abnormal arteriovenous shunt. The probability of AVM rupture is low, but it can cause deficits in up to 45% ofpatients. Epilepsy could be associated with AVM, and status epilepticus is rare. The evolution to refractory status epilepticus or super refractory status epilepticus (SRSE) is very rare. The objective is to present a patient with epilepsy associated with non-ruptured AVM, treated with embolization and a subsequent complication that evolves into SRSE, in which a favorable resolution was achieved after surgery Clinical case: 70-year-old male, with a diagnosis of non-ruptured right temporal AVM and epilepsy, who has been seizure free since the diagnosis, is reported. Embolization of the AVM was performed, achieving total exclusion, after the procedure presented seizures that evolved into SRSE. After investigation, an AVM resection and partial resection of the ictal onset zone on the right parieto-temporal region was performed, including a disconnection of the central fronto-parietal region, where there was evidence of propagation of ictal activity. Patient recovered from the critical condition after surgery, and also presented electrographic normalization. After 2 years, the epilepsy is well controlled (Engel IIa). Discussion and conclusions: The surgical approach is an option in SRSE, and it should be proposed early on, within a reasonable time of evolution (1-2 weeks). The active approach in this case, where the surgery was done for SRSE control, was a successful intervention. Especially when the features are consistent -there is etiological evidence in imaging and electrical focality in studies- there can be a dramatic change in the prognosis.

11.
Braz. j. biol ; 83: e237412, 2023. tab, graf
Artigo em Inglês | LILACS, VETINDEX | ID: biblio-1355854

RESUMO

Abstract Only few studies have focus on animals that received Pilocarpine (Pilo) and did not develop behavioral status epilepticus (SE) and, whether they may become epileptic in the model's chronic phase. Previews works observed mossy fiber sprouting in the hippocampus of Non-SE (NSE) rats, while others observed spontaneous and recurrent seizures (SRS) 6 - 8 months after animals received Pilo. It is known that neuronal excitability is influenced by female hormones, as well as, the occurrence of SE in castrated and non-castrated female rats. However, it is not known whether females that received Pilo and did not show SE, may have SRS. The aim of this work was to investigate whether castrated and non-castrated female rats that did not show behavioral SE after Pilo, will develop SRS in the following one-year. For that, animals received 360 mg/kg of Pilo and were video monitored for 12 months. SE females from castrated and non-castrated groups became epileptic since the first month after drug injection. Epileptic behaviors were identified watching video monitoring recordings in the fast speed. Castrated and Non-castrated NSE animals showed behaviors resembling seizures described by Racine Scale stages 1 - 3. Motor alterations showed by NSE groups could be observed only when recordings were analyzed in slow speed. In addition, behavioral manifestations as, rhythmic head movements, sudden head movements, whole body movements and immobility were also observed in both, SE and NSE groups. We concluded that NSE female rats may have become epileptic. Adding to it, slow speed analysis of motor alterations was essential for the observation of NSE findings, which suggests that possibly many motor alterations have been underestimated in epilepsy experimental research.


Resumo Poucos são os estudos com foco em animais que receberam Pilocarpina (Pilo) e não desenvolveram status epilepticus (SE) comportamental e, se os mesmos se tornarão epilépticos na fase crônica do modelo. Autores observaram o brotamento das fibras musgosas no hipocampo de ratos Não-SE (NSE), enquanto outros observaram crises espontâneas e recorrentes (CER) 6 - 8 meses após receberam a droga. A excitabilidade neuronal é influenciada pelos hormônios femininos e, da mesma forma, a ocorrência de SE em ratas castradas e não-castradas. Entretanto, não é sabido se as fêmeas que não apresentam SE terão CER. O objetivo deste trabalho foi investigar se fêmeas castradas e não castradas que não tiveram SE comportamental após a injeção de Pilo desenvolverão CER dentro de um ano. Para isto, os animais receberam 360 mg/kg de Pilo e foram videomonitorados por 12 meses. As fêmeas SE castradas e não-castradas se tornaram epilépticas desde o primeiro mês pós Pilo. O comportamento epiléptico foi identificado assistindo as gravações na velocidade rápida. As fêmeas NSE castradas e não-castradas apresentaram comportamentos similares aos estágios 1 - 3 da Escala de Racine. As alterações motoras nestes grupos (NSE) foram observadas apenas quando as videomonitoração foi analisada na velocidade lenta. Além destas, manifestações comportamentais como movimentos rítmicos da cabeça, movimentos súbitos da cabeça, movimentos de todo o corpo e imobilidade também foram observadas em ambos grupos, SE e NSE. Concluímos que as fêmeas NE podem ter se tornado epilépticas. Adicionado a isto, a análise das alterações motoras na velocidade lenta foi essencial para a observação dos achados das fêmeas NSE, o que sugere que possivelmente muitas alterações motoras têm sido subestimados na pesquisa em epilepsia experimental.


Assuntos
Animais , Feminino , Ratos , Pilocarpina/toxicidade , Convulsões/induzido quimicamente , Estado Epiléptico/induzido quimicamente , Ratos Wistar , Agonistas Muscarínicos/toxicidade , Modelos Teóricos
12.
Artigo | IMSEAR | ID: sea-216951

RESUMO

Background: Seizure is a common problem evaluated in pediatric emergency departments. Seizure disorders are among the most frequent neurologic problems that occur in childhood. Around 4 to 10% of children experience at least one episode of seizure in the first 16 years of their life. Objectives: To study clinical and etiological profile of children presenting with seizures. Material and Methods: A total of 126 consecutive children aged 1 month to 18 years presenting with seizures defined as per international league against epilepsy classification, participated in this study. A detailed history was taken, and clinical examination was done, along with the investigation for the aetiology of seizures with routine and specific tests, computerized tomography (CT) scan and/or magnetic resonance imaging (MRI) and electroencephalography (EEG) as and when needed. Results: In our present study, we recorded the highest incidence of seizures in the age group of 1year to 5 years. The incidence of seizures in our study was more in males, accounting for 73 cases (57.4%) while females were 53 cases (42.06%). Generalized seizures were the commonest, among them GTCS accounting for 88 cases (69.84). The identified major etiologic factors were febrile convulsions followed by CNS infections. Conclusion: The incidence of convulsions is highest in the age group of 1 month to 5 years and in males. The commonest type of seizure is generalized tonic-clonic (40.6%).The most common cause of convulsion is febrile seizures. Milestones were achieved normally in majority of cases.

13.
Arq. neuropsiquiatr ; 80(5,supl.1): 193-203, May 2022. tab, graf
Artigo em Inglês | LILACS-Express | LILACS | ID: biblio-1393950

RESUMO

Abstract Status epilepticus (SE) is a frequent neurological emergency associated with high morbidity and mortality. According to the new ILAE 2015 definition, SE results either from the failure of the mechanisms responsible for seizure termination or initiation, leading to abnormally prolonged seizures. The definition has different time points for convulsive, focal and absence SE. Time is brain. There are changes in synaptic receptors leading to a more proconvulsant state and increased risk of brain lesion and sequelae with long duration. Management of SE must include three pillars: stop seizures, stabilize patients to avoid secondary lesions and treat underlying causes. Convulsive SE is defined after 5 minutes and is a major emergency. Benzodiazepines are the initial treatment, and should be given fast and an adequate dose. Phenytoin/fosphenytoin, levetiracetam and valproic acid are evidence choices for second line treatment. If SE persists, anesthetic drugs are probably the best option for third line treatment, despite lack of evidence. Midazolam is usually the best initial choice and barbiturates should be considered for refractory cases. Nonconvulsive status epilepticus has a similar initial approach, with benzodiazepines and second line intravenous (IV) agents, but after that, aggressiveness should be balanced considering risk of lesion due to seizures and medical complications caused by aggressive treatment. Usually, the best approach is the use of sequential IV antiepileptic drugs (oral/tube are options if IV options are not available). EEG monitoring is crucial for diagnosis of nonconvulsive SE, after initial control of convulsive SE and treatment control. Institutional protocols are advised to improve care.


RESUMO O estado de mal epiléptico (EME) é uma emergência frequente, com alta morbi-mortalidade. Segundo nova definição da ILAE de 2015, EME acontece pela falha dos mecanismos responsáveis ​​pelo término ou início das crises, sendo anormalmente prolongadas. Pela definição existem diferentes tempos entre EME convulsivo, focal e ausência. Tempo é cérebro. Ocorrem alterações nos receptores sinápticos, levando estado mais pró-convulsivante, com aumento risco de lesão cerebral e sequelas. O manejo do EME deve incluir três pilares: parar a crise, estabilizar o paciente para evitar lesão secundária e tratar a etiologia. EME convulsivo é definido quando duração é maior que 5 minutos e trata-se grande emergência. Os benzodiazepínicos são o tratamento inicial, devem ser administrados rapidamente e na dose adequada. Fenitoína/fosfenitoína, levetiracetam e ácido valpróico são opções com evidência para tratamento de segunda linha. Se EME persistir, uso dos anestésicos é provavelmente a melhor opção como terceira linha tratamento, apesar da falta de evidências adequadas. O midazolam costuma ser a melhor escolha inicial e os barbitúricos devem ser considerados para casos refratários. O EME não convulsivo tem abordagem inicial semelhante, com benzodiazepínicos e agentes segunda linha, mas após, a agressividade deve ser equilibrada considerando risco de lesão pelas crises e complicações pelo tratamento agressivo. A abordagem sugerida é uso de fármacos IV sequenciais (via oral/tubo quando opções IV não disponíveis). A monitorização por EEG é fundamental para o diagnóstico do EME não convulsivo, após controle inicial EME convulsivo e para controle do tratamento. Protocolos institucionais são recomendados.

14.
Artigo | IMSEAR | ID: sea-222185

RESUMO

Super refractory status epilepticus (SRSE) is a rare but serious neurological emergency. This case report aims to highlight the challenges faced during the diagnosis and management of SRSE in a 36-year-old preeclamptic patient taken for cesarean delivery under spinal anesthesia. The patient developed a seizure episode minutes after administration of intrathecal bupivacaine, necessitating administration of general anesthesia. Postoperatively, SRSE developed which did not respond to multiple anti-epileptics and thiopentone sodium infusion. Although the clinical picture and magnetic resonance imaging findings supported the diagnosis of posterior reversible encephalopathy syndrome, the role of intrathecal bupivacaine in causing SRSE could not be ruled out due to the temporal association of events. The patient had a prolonged and complicated hospital stay and despite a multimodal approach to the treatment, suffered neurological sequelae.

15.
Indian Pediatr ; 2022 Apr; 59(4): 300-306
Artigo | IMSEAR | ID: sea-225322

RESUMO

Justification: Febrile seizures are quite common in children but there are controversies in many aspects of their diagnosis and management. Methods: An expert group consisting of pediatric neurologists and pediatricians was constituted. The modified Delphi method was used to develop consensus on the issues of definitions and investigations. The writing group members reviewed the literature and identified the contentious issues under these subheadings. The questions were framed, pruned, and discussed among the writing group members. The final questions were circulated to all experts during the first round of Delphi consensus. The results of the first round were considered to have arrived at a consensus if more than 75% experts agreed. Contentious issues that reached a 50- 75% agreement was discussed further in online meetings and subsequently voting was done over an online platform to arrive at a consensus. Three rounds of Delphi were conducted to arrive at final statements. Results: The expert group arrived at a consensus on 52 statements. These statements pertain to definitions of febrile seizures, role of blood investigations, urine investigations, neuroimaging, electroencephalography (EEG), cerebrospinal fluid analysis and screening for micronutrient deficiency. In addition, role of rescue medications, intermittent anti-seizure medication and continuous prophylaxis, antipyretic medication and micronutrient supplementation have been covered. Conclusion: This consensus statement addresses various contentious issues pertaining to the diagnosis and management of febrile seizures. Adoption of these statements in office practice will improve and standardize the care of children with this disorder.

16.
J Indian Med Assoc ; 2022 Apr; 120(4): 79-81
Artigo | IMSEAR | ID: sea-216522

RESUMO

Brivaracetam (BRV), a propyl analog of levetiracetam, has been shown to be safe and effective in Indian patients with uncontrolled focal epilepsy. A series of advisory board meetings involving pediatricians, neurologists, and physicians were held across India to evaluate the role of IV BRV in India and formulate a position statement. The panelists opined that the potential role of BRV in the acute management of increased seizure activity, especially status epilepticus, should be explored in the Indian context. Further, there is a dearth of Indian studies on the use of BRV in epilepsy patients aged below 16 years. IV BRV holds great potential to be the therapy of choice in epilepsy management owing to the fast mode of action and lesser risk of adverse effects.

17.
Chinese Journal of Neurology ; (12): 1431-1434, 2022.
Artigo em Chinês | WPRIM | ID: wpr-958049

RESUMO

Status epilepticus is a neurological emergency with unknown pathogenesis, controversial treatment options, and poor prognosis. In recent years, with the development of 18F-flurodeoxyglucose positron emission tomography ( 18F-FDG PET) imaging technology, further studies on status epilepticus have been carried out from the perspective of molecular metabolism. This article reviews the current role of 18F-FDG PET in patients with status epilepticus on etiology, disease activity, electric activity, location of epileptogenic foci, and prognosis evaluation.

18.
Journal of Chinese Physician ; (12): 1445-1448, 2022.
Artigo em Chinês | WPRIM | ID: wpr-956319

RESUMO

Objective:To explore the clinical features, diagnosis and treatment of febrile infection-related epilepsy syndrome (FIRES).Methods:The clinical data of 2 cases of FIRES admitted to Changde First People′s Hospital from 2020 to 2022 were retrospectively analyzed, and the clinical characteristics, diagnosis and treatment methods and prognosis of the disease were discussed in combination with relevant literature.Results:Two children with FIRES were all acute onset, with fever as the first symptom, and status epilepticus appeared 3 to 5 days later. The efficacy of various antiepileptic drugs was poor. After ketogenic diet treatment, one child recovered well, and another child still had frequent convulsions under ventilator assisted breathing on the 27th day of admission. His family signed to give up the treatment. At the same time, compared with the domestic and foreign literature, the clinical characteristics of the two children are basically the same as those of the reported cases.Conclusions:FIRES is common in healthy children in the past. After seizures, it rapidly progresses to status epilepticus. MRI of the head and cerebrospinal fluid can have no characteristic changes. The effect of routine antiepileptic drugs is not good. Ketogenic diet can improve the condition of children, but the overall prognosis is not good.

19.
Chinese Journal of Applied Clinical Pediatrics ; (24): 1407-1412, 2022.
Artigo em Chinês | WPRIM | ID: wpr-954761

RESUMO

Objective:To study the protective effects of various doses of Glycyrrhizin on hippocampus of young rats with status epilepticus (SE).Methods:Lithium chloride and pilocarpine were injected intraperitoneally into male Sprague-Dawley (SD) rats (with a postnatal age of 18-21 days), so as to induce SE in rats.The rats were divided into 5 groups according to the random number table method: control group, SE group, SE+ low dose Glycyrrhizin group, SE+ medium dose Glycyrrhizin group and SE+ high dose Glycyrrhizin group.Three different doses of Glycyrrhizin (20 mg/kg, 40 mg/kg and 60 mg/kg) were injected intraperitoneally into the rats.The levels of tumor necrosis factor (TNF)-α, interleukin (IL)-1β and IL-6 in serum of SE rats were determined by enzyme linked immunosorbent assay.Quantitative real-time PCR (qRT-PCR) was used to detect the mRNA expression levels of TNF- α, IL-1β and IL-6 in hippocampus of SE rats.The expression levels of Bax, Bcl-2 and Caspase-3 in hippocampus were detected by Western blot.The damage of neurons was measured by hematoxylin and eosin (HE) staining and Nissl staining.Neurons apoptosis was examined by terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL). The mitochondrial changes were observed under transmission electron microscopy.One-way ANOVA followed by Tukey post-hoc test was used for statistical analysis. Results:Compared to the control group, TNF-α[(369.69±58.07) ng/L vs. (75.46±14.64) ng/L], IL-1β[(242.27±25.23) ng/L vs. (45.29±5.90) ng/L] and IL-6[(288.15±24.60) ng/L vs. (46.59±8.80) ng/L] in the serum of SE rats were significantly up-regulated(all P<0.05). Compared to SE group, low, medium and high doses Glycyrrhizin could effectively reduce the levels of TNF-α[(216.67±8.31) ng/L, (158.81±5.03) ng/L and (113.69±12.54) ng/L vs. (369.69±58.07) ng/L], IL-1β[(131.21±5.50) ng/L, (86.60±7.79) ng/L and (65.06±4.39) ng/L vs. (242.27±25.23) ng/L] and IL-6[(150.24±9.48) ng/L, (101.70±5.85) ng/L and (91.60±2.81) ng/L vs. (288.15±24.60) ng/L] released in serum after SE occurred (all P<0.05). The neuronal damage, loss, apoptosis and mitochondrial damage were found in the hippocampus of SE rats.Glycyrrhizin could ameliorate these symptoms.Compared to the control group, Bax levels(0.57±0.01 vs. 0.14±0.01)and Caspase-3 levels(0.54±0.00 vs. 0.11±0.01)in the hippocampus of SE rats were markedly increased, while Bcl-2 levels(0.27±0.01 vs. 0.57±0.02)were decreased(all P<0.05). Compared to the SE group, low, medium and high doses Glycyrrhizin could effectively reduce the levels of Bax(0.51±0.02, 0.45±0.03 and 0.40±0.02 vs. 0.57±0.01)and Caspase-3(0.47±0.02, 0.42±0.02 and 0.37±0.01 vs. 0.54±0.00), and increase the levels of Bcl-2(0.41±0.02, 0.45±0.02 and 0.51±0.01 vs. 0.27±0.01)(all P<0.05). Conclusions:Glycyrrhizin can effectively protect the hippocampus of young rats with SE.

20.
Chinese Journal of Applied Clinical Pediatrics ; (24): 1407-1412, 2022.
Artigo em Chinês | WPRIM | ID: wpr-954744

RESUMO

Objective:To study the protective effects of various doses of Glycyrrhizin on hippocampus of young rats with status epilepticus (SE).Methods:Lithium chloride and pilocarpine were injected intraperitoneally into male Sprague-Dawley (SD) rats (with a postnatal age of 18-21 days), so as to induce SE in rats.The rats were divided into 5 groups according to the random number table method: control group, SE group, SE+ low dose Glycyrrhizin group, SE+ medium dose Glycyrrhizin group and SE+ high dose Glycyrrhizin group.Three different doses of Glycyrrhizin (20 mg/kg, 40 mg/kg and 60 mg/kg) were injected intraperitoneally into the rats.The levels of tumor necrosis factor (TNF)-α, interleukin (IL)-1β and IL-6 in serum of SE rats were determined by enzyme linked immunosorbent assay.Quantitative real-time PCR (qRT-PCR) was used to detect the mRNA expression levels of TNF- α, IL-1β and IL-6 in hippocampus of SE rats.The expression levels of Bax, Bcl-2 and Caspase-3 in hippocampus were detected by Western blot.The damage of neurons was measured by hematoxylin and eosin (HE) staining and Nissl staining.Neurons apoptosis was examined by terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling (TUNEL). The mitochondrial changes were observed under transmission electron microscopy.One-way ANOVA followed by Tukey post-hoc test was used for statistical analysis. Results:Compared to the control group, TNF-α[(369.69±58.07) ng/L vs. (75.46±14.64) ng/L], IL-1β[(242.27±25.23) ng/L vs. (45.29±5.90) ng/L] and IL-6[(288.15±24.60) ng/L vs. (46.59±8.80) ng/L] in the serum of SE rats were significantly up-regulated(all P<0.05). Compared to SE group, low, medium and high doses Glycyrrhizin could effectively reduce the levels of TNF-α[(216.67±8.31) ng/L, (158.81±5.03) ng/L and (113.69±12.54) ng/L vs. (369.69±58.07) ng/L], IL-1β[(131.21±5.50) ng/L, (86.60±7.79) ng/L and (65.06±4.39) ng/L vs. (242.27±25.23) ng/L] and IL-6[(150.24±9.48) ng/L, (101.70±5.85) ng/L and (91.60±2.81) ng/L vs. (288.15±24.60) ng/L] released in serum after SE occurred (all P<0.05). The neuronal damage, loss, apoptosis and mitochondrial damage were found in the hippocampus of SE rats.Glycyrrhizin could ameliorate these symptoms.Compared to the control group, Bax levels(0.57±0.01 vs. 0.14±0.01)and Caspase-3 levels(0.54±0.00 vs. 0.11±0.01)in the hippocampus of SE rats were markedly increased, while Bcl-2 levels(0.27±0.01 vs. 0.57±0.02)were decreased(all P<0.05). Compared to the SE group, low, medium and high doses Glycyrrhizin could effectively reduce the levels of Bax(0.51±0.02, 0.45±0.03 and 0.40±0.02 vs. 0.57±0.01)and Caspase-3(0.47±0.02, 0.42±0.02 and 0.37±0.01 vs. 0.54±0.00), and increase the levels of Bcl-2(0.41±0.02, 0.45±0.02 and 0.51±0.01 vs. 0.27±0.01)(all P<0.05). Conclusions:Glycyrrhizin can effectively protect the hippocampus of young rats with SE.

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