Bases neurobiológicas de la epilepsia neonatal y sus comorbilidades / Neurobiological basis of neonatal epilepsy and its co morbilities

Resumen La aparición de convulsiones es frecuente durante el periodo neonatal debido a las características de inma durez funcional del cerebro es este periodo. La aparición de estas convulsiones puede llevar a un diagnóstico de epilepsia neonatal, que suele estar asociado a alteracio nes estructurales del cerebro durante el neurodesarrollo. Aproximadamente el 50% de las personas con epilepsia activa padecen al menos un trastorno médico comórbi do, y esto hace que cambie la evolución de la epilepsia. La presencia de trastornos neurológicos que preceden a la aparición de la epilepsia indica que alteraciones es tructurales y/o funcionales del cerebro subyacentes pue den ser causa de la predisposición a padecer epilepsia y de los procesos comórbidos de manera independiente. En esta revisión describimos los procesos cerebrales estructurales y funcionales que subyacen a la aparición de epilepsia neonatal y sus comorbilidades.

Abstract The occurrence of seizures is frequent during the neonatal period due to the functional immaturity of the brain.The presence of these seizures may lead to a diagnosis of neonatal epilepsy, which is usually as sociated with structural alterations of the brain during neurodevelopment. Approximately 50% of people with active epilepsy have at least one comorbid medical di sorder, and the existence of a comorbid process changes the course of the epilepsy. The presence of neurologic disorders preceding the onset of epilepsy indicates that underlying neurobiological alterations may indepen dently cause the predisposition to epilepsy and comor bid processes. In this review we describe the structural and functional brain processes underlying the onset of neonatal epilepsy and its comorbidities.

Eclampsia atípica tardía: compromiso inusual con proteinuria, pero sin hipertensión / Late atypical eclampsia: unusual involvement with proteinuria, but without hypertension

RESUMEN Paciente primigesta de 27 años, sin antecedentes médicos de importancia y con un apropiado control prenatal, quien recibió atención por parto vaginal a las 39 semanas de gestación con anestesia epidural. Una hora después del parto, presentó cefalea holocraneana a predominio derecho, tratada con antiinflamatorios y relajantes muscular por indicación del servicio de neurología. Horas después de su alta, al tercer día posparto, presentó convulsiones tónico-clónicas bilaterales. Por un examen de orina con proteinuria (+) en tira reactiva y elevaciones discretas de la presión arterial, se solicitó un conteo de proteínas en 24 horas, con valores en 1094,5 mg (valor normal: 0-140). La resonancia magnética con contraste solicitada durante su admisión fue normal, recibiendo tratamiento con fenitoína y sulfato de magnesio durante su hospitalización. Fue dada de alta al quinto día, con controles posteriores por consultorio externo, sin cefalea, proteinuria y/o hipertensión.

ABSTRACT A 27-year-old primigravida patient without a relevant medical history and appropriate prenatal control received attention for vaginal delivery at 39 weeks of gestation. One -hour later, she experienced holocranial headache with right predominance, treated with anti-inflammatories and muscle relaxants by the indication of a neurologist. Hours after her discharge, on the third day post-partum, she developed bilateral tonic-clonic seizures.. Following a urine test in the emergency room with proteinuria (+) in a dipstick, we tested 24-hour protein count in 1094 mg (normal values 0-140). Magnetic resonance with contrast at admission was normal. She received Phenytoin and Magnesium Sulfate during her hospitalization. The evolution was favorable, and he was discharged at five days with ambulatory controls in the medical office without headache, proteinuria, and/or hypertension.

Risk factors of secondary epileptic seizures in children with febrile seizures and construction of nomogram prediction model / 中国医师进修杂志

Objective:To analyze the risk factors of secondary epileptic seizures in children with febrile seizures and to construct a nomogram prediction model.Methods:A total of 235 children with febrile seizures who were admitted to Enshi State Hospital for Nationalities from August 2018 to September 2021 were selected. According to whether the children had secondary epileptic seizures during the 6-month follow-up, the children were divided into the seizure group (62 cases) and no-seizure group (173 cases). The best cut-off value of each factor were obtained by the receiver operating characteristic (ROC). Multivariate Cox regression analysis was used to analyze the independent risk factors of secondary seizures in children with febrile seizures. The R software "rms" package was constructed to predict secondary seizures in children with febrile seizures. High-risk nomogram models, calibration curves was used for internal validation of nomogram models, and decision curves to assess the predictive power of nomogram models.Results:The age of the patients in the seizure group was lower than that in the no-seizure group: (14.45 ± 1.54) months vs. (21.47 ± 2.18) months; and the proportion of family history of epilepsy, the proportion of perinatal (abnormal), the proportion of seizure type (comprehensive), the proportion of electroencephalogram (EEG) (abnormal), the number of seizures, the duration of seizure, the tumor necrosis factor-alpha (TNF-α) level in the seizure group were higher than those in the no-seizure group: 56.45%(35/62) vs. 35.84%(62/173), 59.68% (37/62) vs. 15.61%(27/173), 70.97%(44/62) vs. 36.99% (64/173), 74.19% (46/62) vs. 20.81% (36/173), (5.45 ± 2.32) times vs. (2.04 ± 1.02) times, (18.89 ± 4.29) min vs. (12.62 ± 2.34) min, (25.65 ± 5.32) ng/L vs.(18.21 ± 2.29) ng/L, there were statistical differences ( P<0.05). The area under the curve (ACU) of age, number of convulsions, duration of convulsion, and TNF-α were 0.906, 0.913, 0.899, and 0.890, respectively; the best cut-off values were 3 years, 4 times, 15 min, 21 ng/L; age (≤3 years), family history of epilepsy (yes), type of seizures (generalized), perinatal period (abnormal), number of seizures (≥4 times), duration of seizures (≥15 min) were febrile seizures independent risk factors for secondary epileptic seizures in children ( P<0.05), the C-index of this nomogram prediction model was 0.744 (0.567-0.932); the decision curve showed that when the risk threshold was greater than 0.11, the clinical net benefit provided by this prediction model. The benefits were all higher than individual independent risk factors and provided a significant additional net clinical benefit in predicting a high risk of seizures secondary to febrile seizures in children with febrile seizures. Conclusions:This study constructed a nomogram model of the risk of secondary seizures in children with febrile seizures based on age, family history of epilepsy, type of seizures, perinatal period, number of seizures, and duration of seizures. Important strategic guidance.

Association entre l'anémie ferriprive et les convulsions fébriles chez les enfants âgés de 6 mois à 60 mois à Yaoundé / Iron Deficiency Anemia and Febrile Seizures in Children Aged 6 to 60 Months in Yaounde

Introduction.Les convulsions fébriles (CF) sont l'une des affections neurologiques les plus courantes de l'enfance. Plusieurs théories, telles que l'anémie ferriprive, ont été proposées comme pathogénie de cette affection. L'objectif de cette étude était de déterminer l'association entre l'anémie ferriprive et les convulsions fébriles chez les enfants âgés de 6 mois à 60 mois.Méthodologie. Il s'agissait d'une étude cas-témoins menée de Janvier à Mai 2021 chez 43 enfants ayant des CF (cas) et 43 enfants fébriles sans convulsions (témoins) admis dans deux hôpitauxà Yaoundé. Les deux groupes étaient appariés en fonction de l'âge et du sexe. Chez tous, le taux d'hémoglobine, le volume globulaire moyen(VGM), la teneur corpusculaire moyenne en hémoglobine (CCMH) et la ferritine plasmatique ont été déterminés.Résultats.L'âge moyen était de 23 mois. Les antécédents familiaux de convulsions fébriles(CF)étaient retrouvés dans 27,9 % des cas. Les CF simples étaient les plus fréquentes (69,8%) avec une prédominance des crises tonicocloniques généralisées (74,4%). Les infections ORL et le paludisme étaient les causes de la fièvre dans 72,1% des cas. Les enfants avec convulsions fébriles avaient trois fois plus de risque d'avoir une anémie comparée aux témoins (P), par contre les valeurs moyennes , TCMH n'étaient pas significativement différents dans les deux groupes. Les deux groupes présentaient une carence martiale mais sans différence significative. Conclusion.Les enfants avec CF présentaient plus d'anémie, mais la carence en fer bien que présente dans les deux groupes l'était sans différence significative

ntroduction.Febrile convulsions (FC) are one of the most common neurological disorders in childhood. Several theories, such as iron deficiency anemia, have been proposed as the pathogenesis of this condition. The aim of this study was to determine the association between iron deficiency anemia and febrile convulsions in children aged 6 to 60 months. Methodology.This was a case-control study conducted from January to May 2021 involving 43 children with FC (cases) and 43 febrile children without convulsions (controls) admitted to two hospitals in Yaoundé. The two groups were matched for age and sex. Hemoglobin levels, mean corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), and plasma ferritin levels were determined in all participants. Results.The average age was 23 months. Family history of febrile convulsions (FC) was found in 27.9% of cases. Simple FC were the most common (69.8%) with a predominance of generalized tonic-clonic seizures (74.4%). ENT infections and malaria were the causes of fever in 72.1% of cases. Children with febrile convulsions were three times more likely to have anemia compared to controls (P= 0.028), however, the average values of MCV and MCHC were not significantly different in the two groups. Both groups had iron deficiency but without significant differences. Conclusion.Children with FC had more anemia, but iron deficiency

Neutrophil-lymphocyte ratio, red cell distribution width and mean platelet volume as practical markers in febrile seizure classification / Proporção entre linfócitos e neutrófilos, amplitude de distribuição de hemácias e volume médio de plaquetas como marcadores práticos na classificação de convulsões febris

ABSTRACT Objective: To examine the neutrophil-lymphocyte ratio, red cell distribution width and mean platelet volume in patients with febrile seizure and to determine their role in febrile seizure classification. Methods: This was a retrospective hospital-based study conducted among patients aged 5 to 72 months admitted with febrile seizure. Children who had febrile seizures due to upper respiratory tract infection were included in the study. The children were divided into two groups: simple febrile seizures and complex febrile seizures. Patients with a history of febrile status epilepticus, previous convulsions, use of antiepileptic or other chronic drugs, foci of infection other than the upper respiratory tract infection, abnormal biochemical parameters, and chronic mental or physical disease were excluded from the study. Clinical and laboratory findings of the patients were obtained from digital medical records. Results: The records of 112 febrile seizure patients were reviewed, and 89 were grouped as simple and 23 as complex febrile seizures. Although there was no statistically significant difference between the two groups in terms of the mean red cell distribution width values (p=0.703), neutrophil-lymphocyte ratio and mean platelet volume were significantly higher in patients with complex febrile seizures (p=0.034, p=0.037; respectively). Conclusions: This study showed that neutrophil-lymphocyte ratio and mean platelet volume could be practical and inexpensive clinical markers for febrile seizure classification. A similar result could not be reached for red cell distribution width in this study. These findings should be supported by multicenter studies with large samples.

RESUMO Objetivo: Examinar a relação linfócitos-neutrófilos, amplitude de distribuição de hemácias e volume médio de plaquetas em pacientes com convulsão febril, e determinar seu papel na classificação de convulsão febril. Métodos: Este foi um estudo retrospectivo de base hospitalar realizado com pacientes de 5 a 72 meses admitidos com convulsão febril. Crianças que tiveram convulsões febris em razão de infecção do trato respiratório superior foram incluídas no estudo. As crianças foram divididas em dois grupos: convulsões febris simples e complexas. Pacientes com história de Status epiléptico febril, convulsões prévias, uso de drogas antiepilépticas ou outras drogas crônicas, com focos de infecção que não a do trato respiratório superior, parâmetros bioquímicos anormais e doenças crônicas mentais ou físicas foram excluídos do estudo. Os achados clínicos e laboratoriais dos pacientes foram obtidos a partir dos prontuários médicos digitais. Resultados: Registros de 112 pacientes com convulsão febril foram revisados: 89 com convulsões febris simples e 23 com complexas. Embora não tenha havido diferença estatisticamente significativa entre os dois grupos em termos de valor médio de amplitude de distribuição de hemácias (p=0,703), a relação linfócitos-neutrófilos e o volume médio de plaquetas foram significativamente mais elevados em pacientes com convulsões febris simples (p=0,034, p=0,037; respectivamente). Conclusões: Este estudo mostrou que a relação linfócitos-neutrófilos e o volume médio de plaquetas podem ser marcadores clínicos práticos e de baixo custo para a classificação de convulsão febril. Um resultado semelhante não pôde ser alcançado para a amplitude de distribuição de hemácias neste estudo. Esses achados devem ser apoiados por estudos multicêntricos com grandes amostras.

Boswellia serrata intoxication manifesting with syndrome of inappropriate antidiuretic hormone secretion, hyponatremia, seizure, and rhabdomyolysis / Intoxicação por Boswellia serrata manifestando-se com síndrome da secreção inapropriada de hormônio antidiurético, hiponatremia, convulsão e rabdomiólise

ABSTRACT Boswellia serrata is an herbal extract from the Boswellia serrata tree that has anti-inflammatory and analgesic properties and alleviates pain caused by rheumatoid arthritis, gout, osteoarthritis, and sciatica. Syndrome of inappropriate antidiuretic hormone secretion accompanied by hyponatremia, seizures, and rhabdomyolysis as a manifestation of Boswellia serrata intoxication has not been reported previously. A 38-year-old female suffered clinically isolated syndrome and has since been regularly taking B. serrata capsules (200mg/d) to strengthen her immune system. She experienced hypersensitivity to light, ocular pain, nausea, dizziness, and lower limb weakness four days after receiving her first BNT162b2 vaccine dose, and she increased the dosage of B. serrata to five capsules (1000mg/d) one week after vaccination. After taking B. serrata at a dosage of 1000mg/d for 3 weeks, she was admitted to the intensive care unit because of a first, unprovoked generalized tonic-clonic seizure. The patient's workup revealed syndrome of inappropriate antidiuretic hormone secretion, which resolved completely upon treatment and discontinuation of B. serrata. In summary, B. serrata potentially causes syndrome of inappropriate antidiuretic hormone secretion when it is taken at high doses. Patients should not self-medicate.

RESUMO Boswellia serrata é um extrato herbal da árvore Boswellia serrata que possui propriedades anti-inflamatórias e analgésicas e alivia a dor ciática e causada por artrite reumatoide, gota, osteoartrite. Não há relato na literatura de síndrome da secreção inapropriada do hormônio antidiurético, acompanhada por hiponatremia, convulsões e rabdomiólise, como manifestação de intoxicação por Boswellia serrata. Uma mulher de 38 anos diagnosticada com síndrome clinicamente isolada tomava regularmente cápsulas de B. serrata (200mg/dia) para fortalecer seu sistema imunológico. Ela desenvolveu hipersensibilidade à luz, dor ocular, náusea, tontura e fraqueza nos membros inferiores 4 dias após tomar a primeira dose da vacina BNT162b2 e aumentou a dose de B. serrata para 1.000mg/dia 1 semana após a vacinação. Após tomar B. serrata na dose de 1.000mg/dia por 3 semanas, ela foi internada na unidade de terapia intensiva devido à convulsão tônico-clônica generalizada não provocada. A investigação diagnóstica revelou síndrome da secreção inapropriada de hormônio antidiurético, que se resolveu completamente após tratamento e interrupção do uso de B. serrata. Em resumo, é possível que B. serrata cause síndrome da secreção inapropriada do hormônio antidiurético quando tomada em doses elevadas. Os pacientes não devem se automedicar.

Neurocisticercosis infantil. A propósito de un caso / Infantile neurocysticercosis. About a case

La neurocisticercosis es una de las infecciones parasitarias más frecuentes, originada por la larva de la Taenia solium al invadir el sistema nervioso central. Esta patología es endémica de algunos países de África, y está relacionada con hábitos dietéticos, costumbres, higiene y factores socioeconómicos, con mayor incidencia en pacientes escolares y adolescentes, aunque puede presentarse a cualquier edad. Su clínica es variable, unida a criterios epidemiológicos que hacen indispensable al médico de asistencia realizar examen clínico y estudios imagenológicos. Se presenta el caso clínico de una niña de 10 años de edad procedente de Kuito-Bie, Angola, que acude a consulta de neurología, con pérdida de la visión de aproximadamente seis meses de evolución, disminución de la fuerza muscular, episodios de alucinaciones, lenguaje incoherente y convulsiones tónico-clónicas generalizadas de siete días de evolución. Se solicita, de urgencia, una tomografía axial computarizada de cráneo, donde se confirma neurocisticercosis activa, con epilepsia sintomática en el curso de la misma. Los hallazgos clínicos e imagenológicos permiten un diagnóstico oportuno y tratamiento eficaz, lo que determina la evolución clínica de la neurocisticercosis en la infancia, según el estadio clínico y la respuesta inmunológica del huésped(AU)

Neurocysticercosis is one of the most frequent parasitic infections caused by the larva of Taenia solium when invading the central nervous system. This pathology is endemic to some African countries, and is related to dietary habits, customs, hygiene and socioeconomic factors, with a higher incidence in school patients and adolescents, although it can occur at any age. Its clinical symptoms are variable, linked to epidemiological criteria that make it essential for the attending physician to perform a clinical examination and imaging studies It is presented the clinical case of a 10-year-old girl from Kuito-Bie, Angola, who attended the neurology clinic with vision loss of approximately 6 months of evolution, decreased muscle strength, episodes of hallucinations, incoherent speech, and generalized tonic-clonic seizures of 7 days of evolution. An emergency computed axial tomography of the skull was requested, where active neurocysticercosis is confirmed, with symptomatic epilepsy in its course. Clinical and imaging findings allow timely diagnosis and effective treatment, which determines the clinical evolution of neurocysticercosis in childhood, depending on the clinical stage and the host's immune response(AU)

Ketogenic diet in pharmacoresistant epilepsies: a clinical nutritional assessment / Dieta cetogênica na epilepsia fármaco-resistente: avaliação clínica-nutricional

Abstract Background Epilepsies are among the most prevalent chronic neurological diseases, usually beginning in childhood. About 30% of children with epilepsies develop seizures that are difficult to control with medication. Recurrent epileptic seizures hinder diet intake, impairing the nutritional status. Although non-pharmacological interventions (e.g., ketogenic diet therapy) can improve epileptic seizure frequency, few studies analyzed their impact on the nutritional status of children and adolescents with epilepsies. Objective The aim was to evaluate the effects of a ketogenic diet on the nutritional status and clinical course of patients with pharmacoresistant epilepsies. Methods This cross-sectional study included patients under 18 years of age followed up at the Ketogenic Diet Ambulatory Clinic of the Instituto de Medicina Integral Prof. Fernando Figueira between December 2015 and December 2021. Socioeconomic, clinical, nutritional, and laboratory data were collected from medical records at different time points during the ketogenic diet. Results The sample comprised 49 patients aged between 5 months and 17 years (median = 4.4 years), mostly male (62.1%), and from Recife and the metropolitan region (51%). Underweight patients (BMI-for-age) improved their nutritional status in six months. However, patients who were normal weight and overweight maintained their nutritional status. Dyslipidemia was a common and short-term adverse effect. Moreover, the treatment decreased epileptic seizure frequency and antiseizure medication intake. Conclusion The ketogenic diet prevented malnutrition from worsening and reduced epileptic seizures and antiseizure medication intake.

Resumo Antecedentes A epilepsia, uma das doenças neurológicas crônicas mais prevalentes, tem geralmente início na infância. Cerca de 30% das crianças com epilepsia desenvolvem crises de difícil controle medicamentoso. As crises epilépticas recorrentes dificultam a ingestão alimentar, prejudicando o estado nutricional. Intervenções não farmacológicas, como a terapia com dieta cetogênica, podem melhorar a frequência das crises epilépticas, mas existem poucos estudos sobre a repercussão no estado nutricional da criança/adolescente. Objetivo Avaliar o efeito da terapia cetogênica sobre o estado nutricional e a evolução clínica da epilepsia fármaco-resistente. Métodos Estudo tipo corte transversal envolvendo menores de 18 anos acompanhados no Ambulatório de Dieta Cetogênica do Instituto de Medicina Integral Prof. Fernando Figueira entre dezembro de 2015 e dezembro de 2021. Dados socioeconômicos, clínicos, nutricionais e laboratoriais foram coletados nos prontuários dos pacientes em vários momentos da terapia cetogênica. Resultados A amostra foi composta por 49 pacientes com idades entre cinco meses e 17 anos (mediana = 4,4 anos), a maioria do sexo masculino (62,1%) e procedentes de Recife e região metropolitana (51%). Pacientes com baixo peso (de acordo com o IMC para idade) melhoraram seu estado nutricional em seis meses. No entanto, os pacientes com peso adequado e com sobrepeso mantiveram seu estado nutricional. A dislipidemia foi um efeito adverso frequente e de curta duração. Além disso, o tratamento reduziu a frequência de crises epilépticas e a dose de fármacos anticrises. Conclusão A dieta cetogênica preveniu o agravamento da desnutrição e reduziu as crises epilépticas e a dosagem de fármacos anticrises.

Autoimmune encephalitis in a resource-limited public health setting: a case series analysis / Encefalite autoimune em um hospital universitário público: análise de uma série de casos

Abstract Background Autoimmune encephalitis (AE) consists of a group of acquired diseases that affect the central nervous system. A myriad of phenotypes may be present at the onset. Due to the heterogeneity of clinical presentations, it is difficult to achieve uniformity for the diagnostic and therapeutic processes and follow-up strategies. Objective To describe a series of patients diagnosed with AE in a resource-limited public hospital in southern Brazil and to analyze therapeutics and outcomes. Methods We retrospectively reviewed the electronic medical records of patients diagnosed with AE at the Hospital de Clínicas de Porto Alegre from 2014 to 2022. Data collected included clinical presentation, neuroimaging, cerebrospinal fluid testings, electroencephalogram, autoantibodies, treatments, outcomes, follow-up time, degree of neurological impairment, and mortality. Results Data from 17 patients were retrieved. Eleven cases were classified as definite AE and 6 as possible AE. Autoantibodies were identified in 9 patients. Timing for diagnosis was impacted by the high costs associated with autoantibody testing. Most patients became functionally dependent (82.4%) and most survivors remained with autoimmune-associated epilepsy (75%). Five patients died during hospitalization, and one after a 26-month of follow-up. Conclusion In this resource-limited hospital, patients with AE had a worse clinical outcome than that previously described in the literature. Development of epilepsy during follow-up and mortality were greater, whilst functional outcome was inferior. Autoantibody testing was initially denied in most patients, which impacted the definitive diagnosis and the use of second-line therapies.

Resumo Antecedentes A encefalite autoimune (EA) consiste em um grupo de doenças adquiridas que afetam o sistema nervoso central. Objetivo Descrever uma série de pacientes diagnosticados com EA em um contexto de atenção terciária à saúde com recursos limitados e analisar a terapêutica e os resultados. Métodos Revisamos retrospectivamente os prontuários eletrônicos de pacientes diagnosticados com EA no Hospital de Clínicas de Porto Alegre de 2014 a 2022. Os dados coletados incluíram apresentação clínica, neuroimagem, exames de líquido cefalorraquidiano, eletroencefalograma, autoanticorpos, tratamentos, resultados, tempo de acompanhamento, grau de comprometimento neurológico e mortalidade. Resultados Dados de 17 pacientes foram coletados. Onze casos foram classificados como EA definitivo e seis como EA possível. Autoanticorpos foram identificados em nove pacientes. O tempo para o diagnóstico foi afetado pelos altos custos associados ao teste de autoanticorpos. A maioria dos pacientes tornou-se funcionalmente dependente (82,4%), e a maioria dos sobreviventes permaneceu com epilepsia autoimune associada (75%). Cinco pacientes faleceram durante a internação, e um após 26 meses de seguimento. Conclusão No hospital em questão, os pacientes com EA tiveram um desfecho clínico pior do que o previamente descrito na literatura. O desenvolvimento de epilepsia durante o acompanhamento e a mortalidade foram maiores, enquanto o desfecho funcional foi inferior. Os testes de autoanticorpos foram inicialmente negados para a maioria dos pacientes, o que impactou o diagnóstico definitivo e o uso de terapias de segunda linha.

Beneficio de la cirugía en un caso de crisis epilépticas focales motoras negativas secundarias a displasia cortical parietal / Benefit of surgery in a case of negative motor focal epileptic seizures secondary to parietal cortical dysplasia

Resumen Las crisis atónicas focales son poco reconocidas como fenómenos ictales, pueden corresponder tanto a una epilepsia generalizada como a una epilepsia focal. Las áreas del cerebro implicadas en la gestión de este tipo de crisis son: el área motora negativa y las cortezas motora primaria y somatosensitiva primaria, aunque aún la neurofisiología que las genera no está aclarada. Presentamos el caso de un paciente con crisis atónicas focales farmacorresistentes en miembro superior iz quierdo. Se realizó resonancia de cerebro con diagnóstico de displasia cortical parietal, se monitoreó con video EEG de scalp y luego a video EEG con electrodos profundos. Se definieron el área epileptógena y su relación con áreas elocuentes, se realizó resección quirúrgica de la lesión, logrando el control completo de las crisis.

Abstract Focal atonic seizures are recognized rarely as ictal phenomena, they can correspond to both generalized epilepsy and focal epilepsy. The areas of the brain in volved in the management of this type of seizure are: the negative motor area and the primary motor and primary somatosensory cortices, although the neurophysiology that generates them is still unclear. We present the case of a patient with focal atonic seizures in the left upper limb, refractory to drug treatment. Neuroimaging was performed, a parietal cortical lesion was diagnosed. A scalp Video EEG and then a Stereo EEG was performed, defining the epileptogenic area and its relationship with eloquent areas. Surgical resection of the lesion was performed, achieving complete seizure control.

Crisis no epilépticas psicógenas: Semiología en una cohorte de pacientes de una unidad de epilepsia de referencia nacional en Lima, Perú / Non-epileptic psychogenic seizures: semiology in a patient's cohort from a national reference epilepsy unit in Lima, Peru

RESUMEN Objetivo : Describir la semiología de crisis no epilépticas psicógenas (CNEP) en pacientes diagnosticados mediante videoelectroencefalograma (VEEG) en un centro nacional de epilepsia. Material y métodos : Estudio observacional, descriptivo y retrospectivo que incluyó a pacientes mayores de 14 años, ingresados a monitoreo de telemetría en el Hospital Nacional Edgardo Rebagliati Martins en Lima, Perú, entre el 1 de enero de 2017 y el 31 de diciembre de 2022, y definitivamente diagnosticados con CNEP mediante VEEG. Resultados : La mayoría de los 26 pacientes (20 mujeres y 6 varones) presentó pérdida de postura con caída (92 %), así como una instauración gradual (88 %) y cursaron con algún grado de alteración de conciencia o responsividad durante el evento (73 %). Los signos motores más prevalentes fueron movimientos asincrónicos de tronco y/o extremidades (73 %), fluctuación en la actividad motora (73 %), temblor global o en extremidades (54 %) y cierre palpebral forzado (50 %). Las manifestaciones clínicas no motoras fueron, en su mayoría, síntomas subjetivos (34 %). La mayoría (73 %) presentó CNEP motoras (73 %), en comparación con otras manifestaciones no motoras (12 %) y mixtas (15 %). Conclusiones : Los eventos clínicos descritos fueron de larga duración e instauración gradual y mostraron alteraciones de conciencia/responsividad. Los signos clínicos motores más frecuentes fueron movimientos asincrónicos de tronco y/o extremidades, fluctuación motora, temblor y cierre palpebral forzado. Los hallazgos no motores fueron, en su mayoría, síntomas subjetivos. La mayoría de los pacientes presentaron CNEP motoras.

ABSTRACT Objective : To describe the semiology of Psychogenic Non-Epileptic Seizures (PNES) in patients diagnosed by video-electroencephalogram (VEEG) in a national epilepsy center. Methods : An observational, descriptive, and retrospective study of patients older than 14 years, admitted for telemetry monitoring at the Epilepsy Unit of the National Hospital Edgardo Rebagliati Martins in Lima, Peru, between January 1st, 2017, and December 31, 2022. A definitive diagnosis of PNES was reached through VEEG. Results : Twenty of 26 studied patients were female and 6 male. Most patients experienced loss of posture with falls (92%), gradual onset (88%), some degree of altered consciousness or responsiveness during the event (73%). The most prevalent motor signs included asynchronous movements of the trunk and/or extremities (73%), fluctuation in motor activity (73%), global or limb tremors (54%), and forced eyelid closure (50%). Non-motor signs were predominantly subjective symptoms (34%). Most of the patients presented motor PNES (73%) compared to non-motor (12%) and mixed (15%). Conclusions : The described clinical events had a prolonged duration, gradual onset, and altered levels of consciousness/responsiveness. The most frequent clinical motor signs were asynchronous movements of the trunk and/or extremities, motor fluctuation, tremors, and forced eyelid closure. Non-motor findings were, primarily, subjective symptoms. Most of the patients presented motor PNES.

Crisis neonatales: evaluacion, diagnostico y tratamiento / Neonatal seizures: evaluation, diagnosis, and treatment

Resumen Las crisis convulsivas tienen una alta incidencia en la etapa neonatal, representando la principal manifes tación de disfunción neurológica. Ciertas condiciones fisiológicas del cerebro neonatal facilitan su aparición. Su diagnóstico puede ser un reto debido a que su semio logía no es tan clara comparado con niños mayores, y además, es necesario la confirmación por medio de EEG continuo o aEEG. Su reconocimiento oportuno es muy importante para un adecuado tratamiento y así evitar un impacto negative en el pronóstico a largo plazo. En la siguiente revisión, recapitulamos la fisiopatología, las causas y la clasificación de las crisis convulsivas neo natales, además de su correcto abordaje y las mejores opciones terapéuticas para su tratamiento dependiendo de la causa.

Abstract Seizures have a high incidence in the neonatal stage, being the main manifestation of neurological dysfunc tion. Certain physiological conditions of the neonatal brain facilitate its appearance. Its diagnosis can be a challenging because its semiology is not as clear as in older children, furthermore, confirmation by either EEG or aEEG is necessary. Its timely recognition is very im portant for adequate treatment and thus avoid a nega tive impact on the long-term outcome. In the following review, we recapitulate the pathophysiology, causes, and classification of neonatal seizures, as well as their correct approach and the best therapeutic options for their treatment depending on the cause.

Encefalopatías epilépticas y del desarrollo. ¿Que hay de nuevo? / Developmental and epileptic encephalopathy. What is new?

Resumen Este artículo no tiene como objetivo el presentar una descripción detallada de cada una de las encefalopatías epilépticas y del desarrollo, sino más bien discutir cam bios recientes en la terminología y criterios diagnósticos de ciertas encefalopatías, en base a una revisión actua lizada de los últimos 10 años. Se analizan cambios importantes en definiciones de síndromes específicos y nuevos tratamientos que han demostrado eficacia en el manejo de crisis convulsivas en estos pacientes. En conclusión: Las nuevas terapias de modulación genética, contribuirán no solo a reducir la carga de crisis epilépticas, sino también a mejorar el pronóstico cognitivo, y por lo tanto la calidad de vida.

Abstract It is not the intend of this article to present a de tailed description of each developmental and epileptic encephalopathy, but to discuss recent changes in the terminology and diagnostic criteria of specific disorders, based on an updated review of the last 10 years. Important changes in the definitions of specific syn dromes and new treatments that have shown efficacy in the management of seizures in these patients are analyzed. In conclusion: New gene modulation therapy will likely improve not only seizure frequency, but also cog nitive outcome and therefore quality of life.

Epilepsias generalizadas idiopáticas / Idiopathic generalized epilepsies

Resumen Las epilepsias generalizadas idiopáticas (EGI) son un grupo de epilepsias generalizadas edad de pendientes, subgrupo de las Epilepsias genéticas generalizadas(EGG), con hallazgos electro-clínicos característicos y herencia poligénica. Las EGI inclu yen las cuatro epilepsias generalizadas clásicas más comunes de las EGG: la epilepsia de ausencias de la infancia (EAI), epilepsia de ausencias juveniles (EAJ), epilepsia mioclónica juvenil (EMJ) y la epilepsia con crisis tónico clónicas generalizadas. Clínicamente caracterizadas por la presencia de una o una com binación de crisis de ausencias, mioclonías, tónica-clónicas omioclónica-tónica-clónicas con patrón elec troencefalográfico de punta onda lenta de 2.5 a 6cps y activación con la hiperventilación y fotoestimula ción, Sobresalen de las EGG por compartir atributos particulares como el buen pronóstico con control frecuente de las crisis, la no evolución a encefalopa tías epilépticas, frecuente superposición clínica entre las tres primeras, pudiendo evolucionar entre ellas; la probabilidad y edad de remisión varía en cada una.Más del 80% se controlan adecuadamente con medicamentos anticrisis de amplio espectro como el ácido valproico y pueden empeorar con bloqueadores de sodio o gabaérgicos. Si bien los pacientes son previamente sanos con neurodesarrollo normal, frecuentemente se asocian con trastornos del ánimo, déficit de atención e hiperactividad (TDAH) y problemas del aprendizaje pero no presentan déficit cognitivo. El reconocimiento de este grupo de EGI es importan te para el uso adecuado del recurso, evitando estudios innecesarios, adecuada orientación del pronóstico y un tratamiento óptimo.

Abstract Idiopathic generalized epilepsies (IGE) is a group of epilepsies age-dependent, a subgroup of EGG genetic generalized epilepsies, with electro-clinical features and polygenic inheritance. Four syndromes comprising the IGEs: childhood absence epilepsy (CAD), juvenile absence epilepsy (JAE), juvenile myoclonic epilepsy (JME), and generalized tonic-clonic seizures epilepsy. Clinically characterized by the presence of one or a combination of absence seizures, myoclonus, tonic-clonic, or myoclonic-tonic-clonic with common electroencephalographic pat terns of 2.5-5.5 Hz generalized spike-wave and activated by hyperventilation or photic stimulation. They generally have a good prognosis for seizure control, not evolve to an epileptic encephalopathy. Frequent clinical overlap between the first three, being able to evolve between them; the probability and age of remission varies in each one. About 80% responding to broad-spectrum anti-seizure drugs such as valproic acid, may worsen with sodium or GABAergic blockers. Development is typically normal; however, they are frequently associated with mood disorders, attention-deficit/hyperactivity disorder (ADHD), and learning dis abilities, but do not have cognitive deficits. The recognition of this group of EGI is important for the adequate use of the resources, avoiding unnecessary studies, adequate orientation of the prognosis and an optimal treatment.

Aspectos electroclínicos de la epilepsia temporal mesial / Neuropsychological risk in temporal lobe epilepsy

Introducción: La epilepsia del lóbulo temporal mesial se considera la más frecuente de las epilepsias focales, con signos y síntomas característicos que ayudan a definir su diagnóstico. Contenidos: Dentro de su historia natural, las crisis pueden iniciar en los primeros años de vida, usualmente como episodios febriles con un periodo de remisión, para reaparecer en la adolescencia o en el adulto joven. La presentación electroencefalográfica tiene un patrón característico, con aparición de puntas y ondas agudas interictales en la región temporal anterior, por lo general unilaterales, y con actividad ictal generalmente theta en la misma localización. La causa más frecuente es la esclerosis del hipocampo. El tratamiento con medicamentos anticrisis puede controlar la epilepsia, aunque algunos casos pueden evolucionar a la farmacorresistencia, en la cual la cirugía de epilepsia está indicada, y tiene buenos resultados. Conclusiones: Esta revisión se centra en la descripción de las características electroclínicas de la epilepsia temporal mesial, para hacer un diagnóstico temprano e iniciar un tratamiento adecuado, a efectos de lograr un mejor pronóstico y una mejor calidad de vida para los pacientes con epilepsia y sus familiares.

Introduction: Mesial temporal lobe epilepsy is considered the most common of the focal epilepsies, with characteristic signs and symptoms that help define its diagnosis. Contents: In the natural history of the disease, seizures can begin in the first years of life, usually as febrile seizures with a period of remission, to reappear in adolescence or in the young adult. The electroencephalographic presentation has a characteristic pattern with the appearance of interictal sharp waves and spikes in the anterior temporal region, usually unilateral, and with generally theta ictal activity in the same location. The most common cause is hippocampal sclerosis. Treatment with antiseizure medication can control epilepsy. However, in some cases evolution of drug resistance can occur, leading to epilepsy surgery as the most appropriate treatment, based on its good results. Conclusions: This review focuses on the description of the electroclinical characteristics of temporal mesial epilepsy, in order to make an early diagnosis and adequate treatment, thus providing a better prognosis and quality of life for patients with epilepsy and their families.

Dependencia y abstinencia de Zolpidem. Reporte de un caso de convulsiones generalizadas

El zolpidem es un agente hipnótico no benzodiacepínico utilizado con suma frecuencia en el tratamiento del insomnio e indicado para emplearlo en el corto plazo. No está indicado para el tratamiento crónico de los trastornos del sueño, pese a lo cual se evidencia en la práctica clínica que gran cantidad de pacientes lo reciben por arios. Si bien se ha descrito que presenta un mejor perfil de efectos adversos que las benzodiacepinas y que genera menos riesgo de dependencia y abstinencia que estas, existen sendos reportes de casos de dependencia y abstinencia de zolpidem. Se presenta el reporte de un caso de convulsiones tónico-clónicas generalizadas por abstinencia a dosis de 300 mg/día de Zolpidem y se realiza una breve revisión de la literatura.

Zolpidem is a non-benzodiazepine hypnotic agent used most frequently in the treatment of insomnia, indicated for short-term use. It is not indicated for the chronic treatment of sleep disorders, despite which there is evidence in clinical practice that a large number of patients receive it for years. Although it has been described that it presents a better profile of adverse effects than benzodiazepines and that it generates a lower risk of dependence and withdrawal than these, there are significant reports of cases of dependence and withdrawal from zolpidem. A report of a case of generalized tonic-clonic seizures due to with drawal at a dose of 300 mg per day of zolpidem is presented and a brief review of the literature is carried out.

The challenges to control epilepsy in an isolated indigenous tribe in Brazil's Amazon: 15 years of follow-up / Os desafios do controle de epilepsia em indígenas de uma tribo isolada da Amazônia: 15 anos de seguimento

Abstract Background Epilepsy is a common neurological disease that affects people all over the world, but it is rarely described in indigenous peoples. Objective To study the epilepsy characteristics and risk factors for seizure control in people from an isolated indigenous population. Methods This is a retrospective and historical cohort study conducted from 2003 to 2018 (15 years), at a neurology outpatient clinic, of 25 Waiwai tribes' indigenous individuals with epilepsy, inhabitants of an isolated forest reserve in the Amazon. Clinical aspects, background, comorbidities, exams, treatment, and response were studied. Factors that impacted seizure control over 24 months were identified using Kaplan-Meier curves and Cox and Weibull regression models. Results The majority of cases started in childhood, with no difference regarding gender. Focal epilepsies were predominant. Most patients had tonic-clonic seizures. A quarter of them had a family history, and 20% had referred febrile seizures. There was intellectual disability in 20% of patients. Neurological examination and psychomotor development were altered in one third of the participants. The treatment controlled 72% of the patients (monotherapy in 64%). Phenobarbital was the most prescribed anti-seizure medication, followed by carbamazepine and valproate. The most relevant factors that impacted seizure control over time were abnormal neurological exam and family history. Conclusion Family history and abnormal neurological exam were predicted risk factors for refractory epilepsy. Even in an isolated indigenous tribe, the partnership between the indigenous people and the multidisciplinary team ensured treatment adherence. The public healthcare system must guarantee modern anti-seizure medications, mainly for this vulnerable population, which has no other source of treatment.

Resumo Antecedentes A epilepsia é uma doença neurológica que afeta povos do mundo todo, mas raramente é descrita em povos indígenas. Objetivos Estudar as características da epilepsia e os fatores de risco para o controle das crises em pessoas de uma população indígena isolada. Métodos Este é um estudo de coorte retrospectivo e histórico, conduzido de 2003 a 2018 (15 anos) no ambulatório de neurologia, de 25 indígenas Waiwai com epilepsia, habitantes de uma reserva florestal na Amazônia. Aspectos clínicos, antecedentes, comorbidades, exames, tratamento e resposta foram estudados. Identificou-se os fatores que afetaram o controle das crises ao longo de 24 meses usando curvas de Kaplan-Meier e modelos de regressão de Cox e Weibull. Resultados A maioria dos casos teve início na infância, sem diferença quanto ao gênero. Predominavam as epilepsias focais. A maioria dos pacientes apresentava crises tônico-clônicas. Um quarto deles tinha história familiar e 20% referiram convulsões febris. Vinte por cento dos pacientes apresentava deficiência intelectual. Um terço tinha exame neurológico e desenvolvimento psicomotor alterados. O tratamento controlou 72% dos pacientes (monoterapia em 64%). Fenobarbital foi o medicamento mais prescrito, seguido por carbamazepina e valproate, e os fatores que mais impactaram o controle das crises ao longo do tempo foram exame neurológico anormal e história familiar. Conclusão História familiar e exame neurológico anormal foram fatores de risco preditores para epilepsia refratária. Mesmo em uma tribo indígena isolada, a parceria entre os indígenas e a equipe multidisciplinar garantiu a adesão ao tratamento. O sistema público de saúde deve garantir medicamentos modernos anticrise, principalmente para essa população vulnerável, que não tem outra fonte de tratamento.

Latency of epileptic and psychogenic nonepileptic seizures / Latencia de las crisis epilépticas y psicógenas no epilépticas

Abstract Background Due to their semiological similarities, psychogenic nonepileptic seizures (PNESs) can occasionally hardly be differentiated from epileptic seizures (ESs), and long-term video-electroencephalographic monitoring (VEM) is needed for the differential diagnosis. Objective To investigate the time of the first clinical event and its distribution on the days of VEM in ES and PNES patients. Methods In total, a consecutive series of 48 PNES and 51 ES patients matched for gender and age were retrospectively and consecutively evaluated. The time distribution of the seizures during the day was noted. Seizure latency was determined as the time in hours from the start of the video-electroencephalographic recording to the first clinical event. Results The seizure latency was significantly shorter in PNES patients compared to ES patients (p < 0.001). Seventy-two percent of PNES patients and 49.1% of ES patients had their first seizure in the 24 hours of video-EEG recording (p = 0.023). Recording longer than 48 hours was required for 12.5% of PNES patients and 37.3% of ES patients (p = 0.006). While ESs were almost evenly distributed throughout the day, most PNESs occurred during the evening hours (p = 0.011). Conclusion We observed that the PNESs appeared earlier than the ESs in the VEM and were concentrated during daylight hours. Although not strictly reliable, seizure latency can contribute to the differential diagnosis of ES and PNES.

Resumen Antecedentes Debido a sus similitudes semiológicas, las crisis no epilépticas psicógenas (CNEP) en ocasiones apenas se pueden diferenciar de las crisis epilépticas (CE), y se necesita una monitorización video-electroencefalográfica (EEG) prolongada para el diagnóstico diferencial. Objectivo Investigar el momento del primer evento clínico y su distribución en los días de monitorización video-EEG en pacientes con CE y CNEP. Métodos Se evaluó retrospectivamente a una serie consecutiva de 48 pacientes con CNEP y 51 con ES emparejados por sexo y edad. Se anotó la distribución temporal de las incautaciones durante el día. La latencia de las crisis se determinó como el tiempo en horas desde el inicio de la grabación del video-EEG hasta el primer evento clínico. Resultados La latencia de las crisis fue significativamente menor en los pacientes con CNEP en comparación con los pacientes con CE (p < 0,001). El 72% de los pacientes con CNEP y el 49,1% de los pacientes con CE tuvieron su primera crisis en las 24 horas de registro del video-EEG (p = 0,023). Se requirió un registro de más de 48 horas para el 12,5% de los pacientes con CNEP y el 37,3% de los pacientes con CE (p = 0,006). Mientras que las CE se distribuyeron casi uniformemente a lo largo del día, la mayoría de las CNEP ocurrieron durante las horas después del anochecer (p = 0,011). Conclusión Observamos que las CNEPs aparecieron antes que las CEs en la monitorización video-EEG, y se agruparon durante las horas del día. Aunque no es estrictamente confiable, la latencia de las crisis puede contribuir al diagnóstico diferencial de ES y CNEP.

A Study On Haematological Parameters In Febrile Seizures

Febrile seizures are the most common neurologic disorder in the pediatric age group, affecting 2–5% of children between 6 months and 5 years of age in the United States and Western Europe with a peak incidence between 12 and 18 months. Although febrile seizure is seen in all ethnic groups, it is more frequently seen in the Asian population (5–10% of Indian children and 6–9% of Japanese children). Systemic inflammatory response has been implicated as a contributor to the onset of febrile seizure [1]. It is studied that IL-B, IL-6, TNF-ALPHA can play an important role in generation of febrile seizure. Although they are the useful biomarkers, its availability in day to day practice is very limited. So there is a need for low cost and widely used inflammatory response markers like NLR, MPV, PLT, and RDW as independent predictors of febrile seizure and to compare different Hematological parameters in febrile seizure in patients with an unclear seizure history. The 5 novel indices of inflammatory response: i. Neutrophil Lymphocyte Ratio ii. Mean Platelet Volume iii. Platelet Count Ratio iv. Red cell Distribution Width v. Serum Ferritin The study is carried out for the assessment of other hematological parameters in febrile seizures like HB, RBC, PCV, RDW, Platelets, MPV and PCR. Also to compare the variation of hematological parameters in simple and complex febrile seizures based on the laboratory parameters which are otherwise classified clinically.

A rare case of intracranial dermoid cyst in a young woman � A case report

Intracranial dermoid cysts generally occurring along the midline are rare. They are benign, congenital, slow-growing cystic lesions located inside the skull. They account for<1% of all primary intracranial tumors and are more common in females in the first three decades of life. Many intracranial dermoid cysts are asymptomatic and are found by chance when brain imaging is carried out for other reasons. Clinical presentation usually relates to compression of adjacent structures or spontaneous rupture of the cyst. The signs and symptoms may range from headaches, seizures to cerebral ischemia. On CT imaging these lesions usually appear as well-defined lobulated midline masses with low attenuation and hyperintense on T1-weighted MRI imaging. We hereby report a case of an 18-year-old female presented with history of seizures involving right upper limb which spread to other limbs associated with frothing and tongue bite- 3 episodes since 2 months. She also had cleft lip. On examination CT images showed hypodense lesion in intrahemispheric region in frontal lobe and MRI image with contrast showed hyperintense T2W, hypointense T1W/FLAIR lesion. A diagnosis of an intracranial dermoid cyst in the intrahemispheric region of frontal lobe was made and the patient was advised a surgical excision of the cyst.