Tabla de la normalidad de las pruebas psicométricas para el diagnóstico de encefalopatía hepática subclínica en la población Dominicana / Score of normality psychometric tests for the diagnosis of subclinical hepatic encephalopathy in the Dominican Population

Introducción: La encefalopatía hepática mínima (EHM), es una enfermedad definida por la existencia de varias alteraciones neurofisiológicas, indetectables a la exploración neurológica y el examen clínico. Dentro de las estrategias diagnosticas para la EHM se contemplan las pruebas psicométricas (PHE), pero para su aplicación es indispensable la estandarización previamente en la población de estudio. Objetivo: El estudio se propuso determinar la tabla de la normalidad de las PHE para diagnosticar la encefalopatía hepática subclínica en una muestra de la población dominicana. Método: Se realizó un estudio descriptivo, prospectivo y transversal en un hospital de referencia nacional. Se analizaron 134 personas clasificados por grupos de edades (18-70 años de edad) y años de escolaridad. Se diseñó una tabla de 5x5. Se estudió la influencia de la edad, sexo, uso de espejuelo y de los años de escolarización en el rendimiento de cada uno de las PHE, para lo cual se utilizaron las siguientes pruebas estadísticas: análisis de varianza (ANOVA), prueba t de Student y regresión lineal. Resultado: La escolaridad y la edad fueron variables determinantes en el desempeño de las 5 pruebas psicométricas. Pero, la correlación univariable de la edad con el desempeño de la prueba TMS no hubo diferencias intra e inter grupos estadísticamente significativas (p>0.171). Conclusión: se confecciono la fórmula de predicción de resultados de los test psicométricos. Ninguno sobrepasó el punto de corte de la puntuación que oscila entre los -4 y los +2 puntos.

Introduction: Minimal hepatic encephalopathy (MHE) is a disease defined by the existence of several neurophysiological alterations, undetectable by neurological examination and clinical examination. Among the diagnostic strategies for EHM, psychometric tests (PHE) are contemplated, but for their application, prior standardization in the study population is essential. Objective: The study will need to determine the normality table of PHE to detect subclinical hepatic encephalopathy in a sample of the Dominican population. Method: A descriptive, prospective and cross-sectional study was carried out in a national reference hospital. 134 people classified by age groups (18-70 years of age) and years of schooling were analyzed. A 5x5 board is recommended. The influence of age, sex, use of glasses and years of schooling on the performance of each one of the PHEs was studied, for which the following statistical tests were used: analysis of variance (ANOVA), Student's t test and linear regression. Result: Schooling and age were determining variables in the performance of the 5 psychometric tests. But, the univariate coincidence of age with the performance of the TMS test, there were no statistically significant intra and inter group differences (p>0.171). Conclusion: the formula for predicting the results of the psychometric tests was made. None exceeded the cut-off point of the score that oscillates between -4 and +2 points.

Effect of Qiwei Baizhusan on Cognitive Dysfunction in Rats with Diabetic Encephalopathy Based on PI3K/Akt/GSK-3β Signaling Pathway / 中国实验方剂学杂志

ObjectiveTo observe the therapeutic effect of Qiwei Baizhusan（QWBZS） on diabetic encephalopathy（DE） rat model， and to explore the possible mechanism of QWBZS in the treatment of DE based on phosphatidylinositol 3-kinase（PI3K）/protein kinase B（Akt）/glycogen synthase kinase-3β（GSK-3β） signaling pathway. MethodForty-eight SPF male Wistar rats were randomly divided into blank group（8 rats） and high-fat diet group（40 rats）. After 12 weeks of feeding， rats in the high-fat diet group were intraperitoneally injected with 35 mg·kg-1 of 1% streptozotocin（STZ） for 2 consecutive days to construct a DE model， and rats in the blank group were injected with the same amount of sodium citrate buffer. After successful modeling， according to blood glucose and body weight， model rats were randomly divided into model group， low， medium and high dose groups of QWBZS（3.15， 6.3， 12.6 g·kg-1）， combined western medicine group（metformin+rosiglitazone， 0.21 g·kg-1）， with 6 rats in each group. The administration group was given the corresponding dose of drug by gavage， and the blank group and the model group were given an equal volume of 0.9% sodium chloride solution by gavage， 1 time/day for 6 weeks. Morris water maze was used to detect the spatial memory ability of DE rats. Fasting insulin （FINS） level was detected by enzyme-linked immunosorbent assay（ELISA） and insulin resistance index（HOMA-IR） was calculated. Hematoxylin-eosin（HE） staining was used to observe the morphological changes of hippocampus in rats， ELISA was used to detect the indexes of oxidative stress in hippocampal tissues， real-time fluorescence quantitative polymerase chain reaction（Real-time PCR） was used to detect mRNA expression levels of PI3K， Akt， nuclear transcription factor-κB（NF-κB）， tumor necrosis factor-α（TNF-α） and interleukin-1β（IL-1β） in hippocampus， and Western blot was used to detect the protein expression of PI3K， Akt， phosphorylated（p）-Akt， GSK-3β and p-GSK-3β in hippocampus of rats. ResultCompared with the blank group， FINS and HOMA-IR values of the model group were significantly increased（P<0.01）， the path of finding the original position of the platform was significantly increased， and the escape latency was significantly prolonged（P<0.01）， the morphology of neuronal cells in hippocampal tissues was disrupted， the levels of reactive oxygen species（ROS） and malondialdehyde（MDA） in hippocampus of rats were increased， and the activity of superoxide dismutase（SOD） was decreased（P<0.05， P<0.01）， mRNA expression levels of PI3K and Akt were decreased（P<0.01）， mRNA expression levels of NF-κB， TNF-α and IL-1β were increased（P<0.05， P<0.01）， the protein expression levels of PI3K， p-Akt and p-GSK-3β were significantly decreased， and the protein expression of GSK-3β was significantly increased（P<0.01）. Compared with the model group， the FINS and HOMA-IR values of the medium dose group of QWBZS and the combined western medicine group were significantly decreased（P<0.01）， the path of finding the original position of the platform and the escape latency were significantly shortened（P<0.01）， the hippocampal tissue structure of rats was gradually recovered， and the morphological damage of nerve cells was significantly improved， the contents of ROS and MDA in hippocampus of rats decreased and the level of SOD increased（P<0.01）， the mRNA expression levels of PI3K and Akt were increased（P<0.01）， and the mRNA expression levels of NF-κB， TNF-α and IL-1β were decreased （P<0.05， P<0.01）， the protein expression levels of PI3K， p-Akt and p-GSK-3β were significantly increased（P<0.01）， and the expression of GSK-3β was significantly decreased（P<0.01）. ConclusionQWBZS can alleviate insulin resistance in DE rats， it may repair hippocampal neuronal damage and improve learning and cognitive ability of DE rats by activating PI3K/Akt/GSK-3β signaling pathway.

Manganese Associated with Non-Wilsonian Hepatolenticular Degeneration as a Rare Cause of Encephalopathy: Case Report

Aim: To describe the clinical picture, diagnosis, and treatment of a patient with encephalopathy as a manifestation of manganese-induced non-Wilsonian hepatolenticular degeneration (NWHD) in a high-complexity care center in a Latin American country. Case description: A 55-year-old male patient from the United States with a history of liver disease associated with alcohol consumption was admitted to the emergency department due to diarrhea, hematemesis, and psychomotor agitation. During his stay, his state of consciousness deteriorated, requiring orotracheal intubation. In his diagnostic study, cerebrospinal fluid tests were negative for infectious etiologies; the endoscopic examinations showed no marks of portal hypertension bleeding, while ammonium and tests for metabolic causes were normal. However, areas of hyperintensity in the basal ganglia were documented on brain MRI, with normal ceruloplasmin serum and urine copper levels, which ruled out Wilson's disease and determined the diagnosis of manganese-induced NWHD. Conclusion: NWHD is a rare cause of chronic encephalopathy with clinical manifestations of extrapyramidal symptoms secondary to basal ganglia dysfunction due to severe liver disease. Its diagnosis becomes a challenge, given that manganese deposits produce it, and no biomarkers can establish the level of exposure to this metal. Brain MRI is indispensable in reflecting these deposits in the basal ganglia.

Objetivo: Describir la presentación clínica, el diagnóstico y el tratamiento de un paciente con encefalopatía como manifestación de degeneración hepatolenticular no wilsoniana producida por manganeso, en un centro de alta complejidad de un país latinoamericano. Descripción del caso: Paciente masculino de 55 años, procedente de Estados Unidos, con antecedente de enfermedad hepática asociada con consumo de alcohol, quien ingresó al servicio de urgencias por un cuadro de diarrea, hematemesis y agitación psicomotora. Durante la estancia presentó deterioro en el estado de consciencia, por lo que requirió intubación orotraqueal. En su estudio diagnóstico, las pruebas de líquido cefalorraquídeo fueron negativas para etiologías infecciosas, en los estudios endoscópicos no tenía estigmas de sangrado portal hipertensivo y el amonio y los estudios para causas metabólicas fueron normales. Sin embargo, se documentaron áreas de hiperintensidad en los ganglios de la base en la resonancia magnética cerebral, con niveles de ceruloplasmina sérica y cobre urinario normales, lo que descartó enfermedad de Wilson y definió el diagnóstico de degeneración hepatolenticular no wilsoniana por depósitos de manganeso. Conclusión: La degeneración hepatolenticular no wilsoniana es una causa infrecuente de encefalopatía crónica con manifestaciones clínicas de extrapiramidalismo, secundaria a disfunción de los ganglios de la base por enfermedad hepática grave. Su diagnóstico se convierte en un reto, dado que se produce por depósitos de manganeso y no existen biomarcadores que puedan establecer el nivel de exposición a este metal. La resonancia magnética cerebral juega, por tanto, un papel indispensable al reflejar esos depósitos en los ganglios de la base.

Diagnóstico por imágenes de un síndrome de Wernicke Korsakoff con estigmas de Marchiafava-Bignami / maging diagnosis of Wernicke - Korsakoff syndrome with Marchiafava-Bignami stigmat

Las enfermedades de Marchiafava-Bignami y de Wernicke Korsakoff, se consideran complicaciones neuropsiquiátricas causadas por el consumo crónico de bebidas alcohólicas. Son encefalopatías poco frecuentes caracterizadas por una desmielinización y necrosis del cuerpo calloso, con la subsiguiente atrofia por daño en las partes bajas del cerebro (tálamo e hipotálamo). Se presenta un paciente masculino de 29 años, con antecedentes de alcoholismo, el cual acude a consulta de Oftalmología por presentar disminución de la visión del ojo derecho durante un año. Se le realizaron, tomografía simple y resonancia magnética con contraste endovenoso de cráneo, donde se observaron hallazgos radiológicos compatibles con el síndrome de Wernicke Korsakoff (ocasiona afectación de la memoria y el aprendizaje) con estigmas de Marchiafava-Bignami (enfermedad poco conocida). Es necesario el dominio de la epistemología de estas enfermedades, porque, a pesar del mal pronóstico en su forma aguda, se reportan casos con buena evolución, si se le realiza un diagnóstico y tratamiento oportunos.

Marchiafava-Bignami and Wernicke-Korsakoff diseases are considered neuropsychiatric complications caused by the chronic consumption of alcoholic beverages. They are rare encephalopathies characterized by demyelination and necrosis of the corpus callosum, with subsequent atrophy due to damage in the lower parts of the brain (thalamus and hypothalamus). We present a 29-year-old male patient with a history of alcoholism who went to the Ophthalmology consultation due to decreased vision in his right eye for a year. Simple tomography and magnetic resonance imaging with intravenous contrast of the skull were performed, observing radiological findings of Wernicke -Korsakoff syndrome (affect memory and learning) with Marchiafava-Bignami stigmata (little-known disease). Mastery of the epistemology of these diseases is necessary, because, despite the poor prognosis in its acute form, cases with good evolution are reported, if an opportune diagnosis and treatment is made.

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.

SCN1A Gene Mutations in Indian Children With Epilepsy: Single Center Experience

Objective: To study prevalence of SCN1A gene mutations in complex seizure disorders. Methods: Retrospective laboratory based study on samples sent for molecular diagnosis in complex seizure disorders. Exome sequencing was performed. Phenotype- genotype correlation was done for patients showing variants in SCN1A gene. Results: 364 samples were evaluated; of which, 54% were of children below 5 years of age. SCN1A mutations were seen in 50 samples of patients with complex seizure disorders; 44 variants were identified. Types of seizure disorders commonly associated were Dravet syndrome and genetic epilepsy with febrile seizures. Conclusions: SCN1A mutations are common in complex seizure disorders, especially Dravet syndrome. Early identification of SCN1A gene in etiology is important for selection of correct antiepileptic and counselling.

Encefalitis aguda por dengue durante el embarazo

El dengue es un problema de salud pública. La mayoría de los pacientes desarrollan signos clínicos que van desde enfermedad leve hasta síndrome hemorrágico. Las manifestaciones neurológicas inusuales son raras y cada vez existen más pruebas de neurotropismo. La encefalitis por dengue es el resultado del trastorno multisistémico que ocurre en la infección grave y durante el embarazo puede ser difícil de diagnosticar. Además, es importante considerarla como diagnóstico diferencial en pacientes en zonas endémicas en pacientes con enfermedad febril aguda y síntomas neurológicos. El manejo de la encefalitis por dengue durante el embarazo es un desafío y es necesario realizar todas las pruebas posibles para decidir el manejo óptimo y preciso para evitar complicaciones maternas. Se presenta un caso de encefalitis aguda por dengue durante el embarazo.

Dengue is a public health problem. Most patients develop clinical signs ranging from mild illness to hemorrhagic syndrome. Unusual neurological manifestations are rare and there is increasing evidence of neurotropism by the virus. Dengue encephalitis is the result of the multisystem disorder that occurs in severe infection and during pregnancy can be difficult to diagnose. In addition, it is important to consider it as a differential diagnosis in patients in endemic areas in patients with acute febrile illness and neurological symptoms. The management of dengue encephalitis during pregnancy is a challenge and it is necessary to perform all possible tests to decide the optimal and accurate management to avoid maternal complications. A case of acute dengue encephalitis during pregnancy is presented.

Anti-inflammatory strategies for hepatic encephalopathy: preclinical studies / Estratégias anti-inflamatórias para encefalopatia hepática: estudos pré-clínicos

Abstract Hepatic encephalopathy (HE) is a potentially reversible neuropsychiatric syndrome. Often, HE causes cognitive and motor dysfunctions due to an acute or chronic insufficiency of the liver or a shunting between the hepatic portal vein and systemic vasculature. Liver damage induces peripheral changes, such as in the metabolism and peripheral inflammatory responses that trigger exacerbated neuroinflammation. In experimental models, anti-inflammatory strategies have demonstrated neuroprotective effects, leading to a reduction in HE-related cognitive and motor impairments. In this scenario, a growing body of evidence has shown that peripheral and central nervous system inflammation are promising preclinical targets. In this review, we performed an overview of FDA-approved drugs and natural compounds which are used in the treatment of other neurological and nonneurological diseases that have played a neuroprotective role in experimental HE, at least in part, through anti-inflammatory mechanisms. Despite the exciting results from animal models, the available data should be critically interpreted, highlighting the importance of translating the findings for clinical essays.

Resumo A encefalopatia hepática (EH) é uma síndrome neuropsiquiátrica potencialmente reversível. Muitas vezes a EH causa disfunções cognitivas e motoras devido à insuficiência do fígado ou por um desvio entre a veia porta hepática e a vasculatura sistêmica. O dano no fígado provoca alterações periféricas, como no metabolismo e nas respostas inflamatórias periféricas, que desencadeiam uma neuroinflamação exacerbada. Em modelos experimentais, estratégias anti-inflamatórias têm demonstrado efeitos neuroprotetores, levando a uma redução dos prejuízos cognitivos e motores relacionados à EH. Neste cenário, evidências crescentes têm mostrado a inflamação periférica e no sistema nervoso central como um promissor alvo pré-clínico. Nesta revisão, abordamos uma visão geral de drogas e compostos naturais aprovados pelo FDA para o uso no tratamento de outras doenças neurológicas e não neurológicas, que tiveram papel neuroprotetor na EH experimental, pelo menos em parte, através de mecanismos anti-inflamatórios. Apesar dos resultados empolgantes em modelos animais, os dados avaliados devem ser criticamente interpretados, destacando a importância da tradução dos achados para ensaios clínicos.

Encefalopatía de Hashimoto. Un diagnóstico por exclusión y revisión de la literatura a partir de un reporte de caso

La encefalopatía de Hashimoto es una entidad poco frecuente, con una amplia gama de manifestaciones neurológicas que incluyen déficits focales, alteraciones cognitivas, crisis convulsivas, trastorno del movimiento e incluso el coma. Con un curso de la enfermedad de subagudo a fluctuante. Afecta más a mujeres que a hombres, con edad de presentación alrededor de los 44 años, aunque se han reportado casos en la edad pediátrica. De etiología poco clara, se desarrolla en el contexto de la presencia de anticuerpos antitiroideos, independientemente de la función tiroidea. La presencia de estos anticuerpos, sumado a la exclusión de otras etiologías y la respuesta al manejo esteroide son claves para su diagnóstico. Presentamos un caso clínico de una mujer de 57 años de edad que evoluciona con psicosis, alteración del lenguaje, deterioro cognitivo, mioclonías y crisis convulsivas de 5 meses de evolución, quien se excluyó otras causas de demencia rápidamente progresiva con presencia de anticuerpos anti tiroglobulina de 83,6 UI/mL (V.R. < 100 UI/mL) normal y anti tiroperoxidasa en 217 UI/mL (V.R. < 100 UI/mL) elevado. Recibió valoración por el Servicio de Endocrinología, donde se detectó hipotiroidismo y se indicó manejo con levotiroxina sin mejoría del cuadro neurológico. Se indicó manejo esteroide con pulsos de metilprednisona a 500 mg/día por 5 días, con mejoría clínica y se concluyó por criterios de exclusión como una encefalopatía de Hashimoto.

Hashimoto encephalopathy is a rare entity, with wide range of neurological manifestations including focal deficits, cognitive alterations, seizures, movement disorders, and even coma, with a subacute to fluctuating disease course. It affects more women than men, it has age of presentation around 44 years, although cases have been reported in the pediatric age. Its etiology is unclear, it develops in the presence of antithyroid antibodies, regardless of thyroid function. The presence of these antibodies, added to the exclusion of other etiologies and the response to steroid management are key to the diagnosis. We report a clinical case of a 57-year-old woman who evolved with psychosis, language impairment, cognitive impairment, myoclonus, and seizures of 5 month-duration. Other causes of rapidly progressive dementia with the presence of normal antithyroglobulin antibodies of 83.6 IU/mL (RV < 100 IU/mL) and elevated antithyroperoxidase 217 IU/mL (RV < 100 IU/mL) were excluded. She was evaluated in the Endocrinology Department that detected hypothyroidism and indicated management with levothyroxine with no improvement in the neurological condition. Steroid management with methylprednisone pulses at 500 mg/day for 5 days was indicated. Clinical improvement was observed and was concluded to be a Hashimoto encephalopathy by exclusion criteria.

Brain glucose metabolism and gray matter volume in retired professional soccer players: a cross-sectional [18F]FDG-PET/MRI study / Metabolismo glicolítico cerebral e volume de substância cinzenta em jogadores de futebol profissionais aposentados: um estudo transversal de [18F]FDG-PET/RM

Abstract Background Professional soccer athletes are exposed to repetitive head impacts and are at risk of developing chronic traumatic encephalopathy. Objective To evaluate regional brain glucose metabolism (rBGM) and gray matter (GM) volume in retired soccer players (RSPs). Methods Male RSPs and age and sex-matched controls prospectively enrolled between 2017 and 2019 underwent neurological and neuropsychological evaluations, brain MRI and [18F]FDG-PET in a 3.0-Tesla PET/MRI scanner. Visual analysis was performed by a blinded neuroradiologist and a blinded nuclear physician. Regional brain glucose metabolism and GM volume were assessed using SPM8 software. Groups were compared using appropriate statistical tests available at SPM8 and R. Results Nineteen RSPs (median [IQR]: 62 [50-64.5] years old) and 20 controls (60 [48-73] years old) were included. Retired soccer players performed worse on mini-mental state examination, digit span, clock drawing, phonemic and semantic verbal fluency tests, and had reduced rBGM in the left temporal pole (pFDR = 0.008) and the anterior left middle temporal gyrus (pFDR = 0.043). Semantic verbal fluency correlated with rBGM in the right hippocampus, left temporal pole, and posterior left middle temporal gyrus (p ≤ 0.042). Cray matter volume reduction was observed in similar anatomic regions but was less extensive and did not survive correction for multiple comparisons (pFDR ≥ 0.085). Individual [18F]FDG-PET visual analysis revealed seven RSPs with overt hypometabolism in the medial and lateral temporal lobes, frontal lobes, and temporoparietal regions. Retired soccer players had a higher prevalence of septum pellucidum abnormalities on MRI. Conclusion Retired soccer players had reduced rBCM and CM volume in the temporal lobes and septum pellucidum abnormalities, findings possibly related to repetitive head impacts.

Resumo Antecedentes Jogadores profissionais de futebol estão expostos a impactos cranianos repetitivos e ao risco de desenvolver encefalopatia traumática crônica. Objetivo Avaliar o metabolismo glicolítico cerebral regional (MCCr) e o volume de substância cinzenta (vSC) em jogadores de futebol aposentados (JFAs). Métodos Jogadores de futebol aposentados masculinos e controles pareados por idade e sexo foram incluídos prospectivamente entre 2017 e 2019. Foram realizadas avaliações neurológica e neuropsicológica, ressonância magnética (RM) e [18F]FDG-PET cerebrais (3.0-Tesla PET/RM). As imagens foram analisadas visualmente por um neurorradiologista e um médico nuclear cegos ao grupo de cada participante. O metabolismo glicolítico cerebral regional e o vSC foram avaliados através do programa SPM8. Os grupos foram comparados através de testes estatísticos apropriados disponíveis em SPM8 e R, de acordo com a distribuição e o tipo dos dados. Resultados Dezenove JFAs (mediana [IIQ]: 62 [50-64.5] anos) e 20 controles (60 [48-73] anos) foram incluídos. Os JFAs tiveram pior desempenho no mini-exame do estado mental e nos testes de dígitos, desenho do relógio, fluência verbal e fluência semântica e apresentaram MCCr significativamente reduzido no polo temporal e no giro temporal médio anterior esquerdos. Fluência semântica (animais) apresentou correlação positiva com MCCr no hipocampo direito, no polo temporal esquerdo e no aspecto posterior do giro temporal médio esquerdo. Menor vSC foi observado nas mesmas regiões, porém este achado não sobreviveu à correção para comparações múltiplas. Análise individual do [18F]FDG-PET cerebral revelou sete JFAs com claro hipometabolismo nas faces medial e lateral dos lobos temporais, nos lobos frontais e nas regiões temporoparietais. Os JFAs apresentaram ainda maior prevalência de anormalidades do septo pelúcido. Conclusão Os JFAs apresentam MCCr e vSC reduzidos nos lobos temporais, além de anormalidades do septo pelúcido, achados possivelmente relacionados a impactos cranianos repetitivos.

MR Imaging in Eclampsia

Background : The aim of this study is to determine the distribution and nature of Cranial MRI findings in eclamptic patients, and to correlate them with clinical and laboratory data. Materials and Methods : This study was conducted in the Department of Obstetrics and Gynecology in Sri Ramachandra Institute of Higher Education and Research. A total number of 35 Eclamptic patients were included in this study and they were analyzed retrospectively. Laboratory parameters, Blood Pressure and Cranial MRI was performed for all and the same were analyzed statistically. Results : Out of 35 Eclamptic patients, MR Imaging was normal in 6 patients. Among the 29 patients with abnormal MRI, Cortical-subcortical Lesion, appeared iso/hypo-intense in T-1 weighted images and hyper intense in T-2 weighted images. In most of the patients, occipital lobe was involved followed by involvement of other lobes such as Parietal, Frontal, Temporal, Basal Ganglia and Cerebellum. When patients with and without positive MRI findings were compared regarding clinical features such as Headache, Blurred Vision, Nausea and Vomiting, Epigastric Pain, Loss of Consciousness, Reduced Urine Output there was no statistically significant difference between the two groups. Similarly, there was no statistical difference in mean arterial pressures between MRI positive and MRI negative patients (p=0.218) however, it was found that those with MR imaging positive features had a higher Blood Pressure than those with MRI negative findings. Among the laboratory parameters, in the patients with abnormal MRI findings Fibrinogen was found to be significantly low than those with normal MRI findings (p=0.0002).

Frecuencia y características de la encefalopatia hipertensiva secundaria a glomerulonefritis posinfecciosa en pacientes internados en un hospital de referencia nacional

Introducción: La glomerulonefritis aguda pos infecciosa (GNPI) puede cursar con complicaciones como la encefalopatía hipertensiva en 7-11% de los casos. Objetivo : determinar la frecuencia y características de la encefalopatía hipertensiva (EH) secundaria a GNPI en pacientes internados en el Departamento de Pediatría del Hospital Nacional en el periodo enero/2000-diciembre/2018. Materiales y Métodos : Estudio observacional, descriptivo, retrospectivo de pacientes con síndrome nefrítico (SN) con C3 disminuido y normalización a los tres meses, con hipertensión arterial (HTA) severa acompañada de manifestaciones neurológicas (cefalea, náuseas, vómitos, alteración de conciencia, convulsiones), que cedieron al regularizarse la HTA. Se estudiaron las características sociodemográficas (edad, sexo, procedencia, escolaridad de los padres, número de hijos) y clínicas (edema periférico, edema agudo de pulmón, hematuria, y manifestaciones neurológicas). Los datos fueron analizados utilizando estadística descriptiva mediante EPIINFO (CDC, Atlanta), expresando las variables cuantitativas como mediana y rango intercuartílico (RIC) y las cualitativas como frecuencia absoluta y porcentual. Resultados: 27 /160 (16,8%) pacientes, desarrollaron EH. La edad varió entre 3 a 16 años (mediana: 10 años; RIC: 5); el antecedente infeccioso más frecuente fue piodermitis (40,7%), seguido de faringitis aguda (37%). Todos los pacientes presentaron edema periférico y cefalea intensa. La duración de la HTA tuvo una mediana de 5 días (RIC: 4) y los días de internación una mediana de 7 (RIC: 6). Ningún paciente requirió diálisis ni quedó con secuelas, no se registraron óbitos. Conclusión: en pacientes con EH debe considerarse el diagnóstico de GNPI, investigando antecedentes infecciosos y valorando adecuadamente la volemia.

Introduction: Acute post-infectious glomerulonephritis (APGN) can present with complications such as hypertensive encephalopathy in 7-11% of cases. Objective: to determine the frequency and characteristics of hypertensive encephalopathy (HE) secondary to APGN in patients admitted to the Department of Pediatrics of the National Hospital from January/2000 to December/2018. Materials and Methods: This was an observational, descriptive and retrospective study of patients with nephritic syndrome (NS) with decreased C3 and normalization at three months, with severe arterial hypertension (AHT) accompanied by neurological manifestations (headache, nausea, vomiting, altered consciousness, seizures), which subsided when the AHT was controlled. Sociodemographic (age, sex, place of residence, parental education level, number of children in home) and clinical (peripheral edema, acute pulmonary edema, hematuria, and neurological manifestations) characteristics were studied. The data were analyzed using descriptive statistics through EPI INFO (CDC, Atlanta), expressing the quantitative variables as median and interquartile range (IQR) and the qualitative ones as absolute frequency and percentage. Results: 27/160 (16.8%) patients developed HE. Age ranged from 3 to 16 years (median: 10 years; IQR: 5); the most frequent infectious history was pyodermitis (40.7%), followed by acute pharyngitis (37%). All patients presented peripheral edema and severe headache. The duration of AHT had a median of 5 days (IQR: 4) and the days of hospitalization a median of 7 (IQR: 6). No patient required dialysis or was left with sequelae, no deaths were recorded. Conclusion: in patients with HE, the diagnosis of APGN should be considered, a history of infections obtained and adequately assessing fluid status.

Lesión cerebral post paro cardiaco: aspectos médicos, éticos y legales / Brain injury after cardiac arrest: Medical, ethical and legal issues disorder

RESUMEN Los sobrevivientes de la reanimación cardiopulmonar posterior a un paro cardiaco pueden tener un amplio rango de desenlaces y van desde recuperación neurológica completa, estado de vigilia sin respuesta, compromiso cognoscitivo diverso o la muerte. La lesión del tejido cerebral se presenta inmediatamente después del paro cardíaco, durante la reanimación y al retornar la circulación espontánea. La severidad y duración de la noxa isquémica determinarán el devenir neurológico. El examen clínico es el punto de partida en el abordaje multimodal del neuropronóstico. Se debe complementar con electroencefalograma, potenciales evocados somatosensoriales, neuroimágenes y biomar-cadores séricos. Entre un 10 a 15% de los pacientes con lesión cerebral posterior al paro cardiaco evolucionan hacia muerte por criterios neurológicos y son potenciales candidatos a la donación de órganos. Un retiro temprano de las terapias de sostenimiento de vida puede malograr la posibilidad de un potencial donante de órganos. Se puede estimar de manera temprana qué pacientes tienen mayor riesgo de evolucionar a muerte por criterios neurológicos. El neurólogo tiene un papel protagónico en el manejo de pacientes con lesión cerebral post paro cardiaco y sus decisiones tienen implicaciones éticas y legales.

ABSTRACT People who survive cardiopulmonary resuscitation (CPR) after cardiac arrest, have a wide range of outcomes including complete neurological recovery, coma, compromised cognitive function and death. Injury of the brain parenchyma starts immediately after a cardiac arrest, during CPR and return of spontaneous circulation. The severity of the ischemic injury will define the neurological outcome. The first step needed to determine a neurological prognosis is the clinical exam, with the help of electroencephalography, somatosensory evoked potentials, neuroimaging, and serum biomarkers. Between 10 and 15% of patients with brain injury after a cardiac arrest, develop brain death and become potential candidates for organ donation. A premature withdrawal of vital support can hamper the possibility of organ donation. The patients with higher risk of developing brain death can be identified early based on neurological criteria. The neurologist has a major role in the approach of patients with brain injury after cardiac arrest and the decision making with legal and ethical consequences.

Evaluación renal y multiorgánica en el paciente con encefalopatía hipóxico-isquémica / Renal and multiorgan evaluation in infants with post-asphyxial hypoxic-ischaemic encephalopathy

Resumen Introducción: La encefalopatía hipóxico-isquémica (EHI) moderada-grave secundaria a asfixia perinatal puede afectar a cualquier órgano, empeorando el pronóstico. Objetivo: Evaluar la afectación renal y multiorgánica de estos pacientes. Material y método: Se incluyó a recién nacidos > 35 semanas con EHI moderada-grave tratados con hipotermia activa entre 2010 y 2020. Se evaluó la creatinina en tres periodos: 48-72 horas de vida, entre el 3.o y 7.o día de vida y del 7.o al 28.o día de vida. Resultados: Se incluyeron 135 pacientes: 112 con EHI moderada y 23 con EHI grave. Al comparar ambos grupos, se obtuvieron diferencias significativas a las 48-72 horas y entre 3.o-7.o día de vida. No hubo diferencias al comparar el método de hipotermia. Los pacientes con EHI grave presentaron mayor afectación hemodinámica, respiratoria y hepática. Conclusiones: Neonatos con EHI grave presentan aumento de los niveles de creatinina sérica y mayor afectación multiorgánica respecto a aquellos con EHI moderada.

Abstract Background: Hypoxic-ischemic encephalopathy (HIE) secondary to perinatal asphyxia can affect any organ, worsening the prognosis. Objective: To describe renal and multiorgan involvement in moderate-severe HIE. Material and method: Newborns > 35 weeks diagnosed with moderate-severe HIE who required active hypothermia between 2010-2020 were included. To assess renal involvement, serum creatinine was measured in three different periods: at 48-72 hours, between the 3rd and the 7th day, and from the 7th to the 28th day. Results: A total of 135 patients were included, 112 (83%) with moderate and 23 (17%) with severe HIE. Significant differences were obtained when comparing median creatinine levels at 48-72 hours and between 3-7 days in both groups. There were no differences in creatinine according to the hypothermia method. Patients with severe HIE presented greater hemodynamic, respiratory, and hepatic involvement. Conclusions: Neonates with severe HIE present increased serum creatinine levels and greater multi-organ involvement than those with moderate HIE.

Wernicke-Korsakoff Syndrome associated with hyperemesis gravidarum and fetal death: Case report and literature review / Síndrome de Wernicke-Korsakoff associado a hiperêmese gravídica e morte fetal: Relato de caso e revisão de literatura

We describe a clinical case of a pregnant patient with hyperemesis gravidarum who progressed to abortion, Wernicke's encephalopathy, and Korsakoff's psychosis, all related to thiamine deficiency. The patient presented symptoms of disorientation, nonspecific limb movements, and fever, initially treated with metronidazole and ceftriaxone for suspected infected abortion. Subsequently, the patient was diagnosed with retained and infected abortion, and thiamine replacement therapy was initiated with an intravenous loading dose of 900 mg/day. During hospitalization, the patient presented with tetraparesis, nystagmus, decreased level of consciousness, anterograde and retrograde amnesia, confabulation, and aphasia. Magnetic resonance imaging showed lesions in the pons, typical of Wernicke's encephalopathy. The patient was empirically treated with acyclovir and ampicillin and showed clinical improvement. The text also provides a brief narrative review of the literature on the topic.

Descrevemos um caso clínico de uma paciente grávida com hiperêmese gravídica que evoluiu para aborto, Encefalopatia de Wernicke e Psicose de Korsakoff, ambas relacionadas à deficiência de tiamina. A paciente apresentou sintomas de desorientação, movimentos inespecíficos dos membros e febre, sendo, inicialmente, tratada com metronidazol e ceftriaxona por suspeita de aborto infectado. Posteriormente, a paciente foi diagnosticada com aborto retido e infectado e iniciou-se a reposição de tiamina com dose endovenosa de ataque de 900 mg/dia. Durante o internamento, a paciente apresentou tetraparesia, nistagmo, rebaixamento do nível de consciência, amnésia anterógrada e retrógrada, confabulação e afasia. A ressonância magnética mostrou lesões na ponte, típicas da Encefalopatia de Wernicke. A paciente foi tratada com aciclovir e ampicilina empiricamente e apresentou melhoras no quadro clínico. O texto também faz uma breve revisão narrativa da literatura sobre o tema.

The role of SUMOylation in the protective mechanisms of therapeutic hypothermia on neonatal hypoxic-ischemic encephalopathy / 中国新生儿科杂志

Objective:To study the role of SUMOylation in the process of therapeutic hypothermia on neural stem cells (NSCs) in neonatal hypoxic-ischemic encephalopathy.Methods:SUMOylation is an essential post-translational modification involving small ubiquitin-like modifiers (SUMOs). Primary-cultured NSCs from mice were assigned into four groups: control group, hypoxia group, hypothermia group and hypoxia+hypothermia group. Western Blot was used to detect the protein levels of SUMO2/3, hypoxia-inducible factor-1α (HIF-1α), peroxisome proliferator-activated receptor γ coactivator factor 1α (PGC-1α) and octamer binding transcription factor 4 (Oct4). The diameters of NSCs were compared. ELISA was used to detect lactate dehydrogenase (LDH) level. Apoptosis was examined using flow cytometry. Immunofluorescence method was used to measure the differentiation of NSCs into neuronal cells.Results:Compared with the control group, the levels of SUMO2/3, HIF-1αand PGC-1α in NSCs of the hypoxia group increased 33%, 126% and 140%, respectively ( P<0.05). Compared with the control group, the levels of SUMO2/3 and PGC-1α in NSCs of the hypothermia group increased 52% and 536%, respectively ( P<0.05). Compared with the hypoxia group, the levels of SUMO2/3, HIF-1α, PGC-1α and Oct4 in the hypoxia+hypothermia group increased 44%, 40%, 230% and 59%, respectively ( P<0.05). The diameters of NSCs in hypoxia group, hypothermia group and hypoxia+hypothermia group were smaller than control group, and hypoxia+hypothermia group smaller than hypoxia group ( P<0.05). No significant differences existed in LDH levels between hypothermia group and control group ( P>0.05). LDH level in hypoxia+hypothermia group were significantly lower than hypoxia group ( P<0.05). No significant differences existed in the cell death rates between hypothermia group and control group ( P>0.05). The cell death rate in hypoxia+hypothermia group was significantly lower than hypoxia group ( P<0.05). Compared with the control group, the expressions of Nestin in both hypoxia group and hypothermia group were increased, but neuron specific enolase (NSE) were decreased ( P<0.05). Compared with hypoxia group and hypothermia group, the level of Nestin in hypoxia+hypothermia group was further increased, while NSE was further decreased ( P<0.05). Conclusions:Therapeutic hypothermia may increase the tolerance of NSCs to hypoxia by enhancing SUMO modification of proteins, providing theoretical basis for the treatment of hypoxic-ischemic encephalopathy with therapeutic hypothermia.

Early enteral feeding guided by intestinal ultrasound during therapeutic hypothermia for hypoxic-ischemic encephalopathy in neonates / 中国新生儿科杂志

Objective:To study the safety and feasibility of early enteral feeding during therapeutic hypothermia guided by intestinal ultrasound in neonates with hypoxic-ischemic encephalopathy (HIE).Methods:From January 2019 to December 2021, neonates with HIE who received therapeutic hypothermia in the neonatology department of our hospital were retrospectively selected. They were assigned into the ultrasound-guided observation group (admitted from May 2020 to December 2021) and the control group (admitted from January 2019 to April 2020). In the ultrasound-guided observation group, intestinal ultrasound was performed during therapeutic hypothermia. Based on clinical manifestations and ultrasound results, a small amount of enteral feeding [20 ml/(kg·d)] was initiated and gradually increased to total enteral feeding after rewarming. In the control group, 5 ml (once every 3 h) of glucose and sodium chloride solution was given during 72 h of therapeutic hypothermia. After rewarming, enteral feeding was started and gradually increased to total enteral feeding without intestinal ultrasound. The time to start enteral feeding, the time to achieve total enteral feeding, the incidences of feeding intolerance, necrotizing enterocolitis (NEC) and late-onset sepsis were compared between the two groups.Results:A total of 17 cases were in the ultrasound-guided observation group and 18 cases in the control group. The median time to start enteral feeding and to achieve total enteral feeding in the ultrasound-guided observation group were earlier than the control group [36.0 (33.5, 39.0) h vs. 77.0 (74.0, 79.3) h, 6.0 (5.5, 6.5) d vs. 8.0 (7.0, 9.0) d, P<0.001]. No significant difference existed in the incidence of feeding intolerance between the two groups. Neither groups had NEC or late-onset sepsis. Conclusions:Early enteral feeding during therapeutic hypothermia in neonates with HIE is safe and feasible. Intestinal ultrasound helps implementing feeding plan and achieving early total enteral feeding.

Predictive value of controlled nutritional status score for overt hepatic encephalopathy after transjugular intrahepatic portosystemic stent-shunt of Budd-Chiari syndrome / 中华消化外科杂志

Objective:To investigate the predictive value of controlled nutritional status (CONUT) score for overt hepatic encephalopathy (OHE) after transjugular intrahepatic portosys-temic stent-shunt (TIPSS) in Budd-Chiari syndrome patients.Method:The retrospective case-control study was conducted. The clinicopathological data of 48 Budd-Chiari syndrome patients who underwent TIPSS in the First Affiliated Hospital of Zhengzhou University from August 2014 to March 2021 were collected. There were 26 males and 22 females, aged (46±13)years. Observation indicators: (1) surgical situations and follow-up; (2) analysis of influencing factors of OHE after TIPSS; (3) predic-tion of OHE after TIPSS. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was performed using the t test. Measurement data with skewed distribution were represented by M( Q1, Q3), and comparison between groups was performed using the Mann-Whitney U test. Count data were expressed as absolute numbers or percentages, and comparison between groups was performed using the chi-square test or Fisher exact probability. Multivariate analysis was performed using the Logistic regression model with forward method. The receiver operating characteristic (ROC) curve was drawn and the area under the curve (AUC) was calculated to evaluate the efficacy. Comparison among AUC was performed using the Delong test. Results:(1) Surgical situations and follow-up. All 48 patients underwent TIPSS successfully, and the operation time of the 48 patients was (131±29)minutes. All patients were implanted with 8 mm covered stent. All 48 patients were followed up for 46(25,71)months, and there were 14 cases with OHE and 34 cases without OHE after TIPSS. Of the 14 cases with OHE, 12 cases were evaluated as West-Haven Ⅱ grade and 2 cases were evaluated as West-Haven Ⅲ grade. (2) Analysis of influencing factors of OHE after TIPSS. Results of multivariate analysis showed that history of hepatic encephalo-pathy and CONUT score were independent factors influencing the incidence of OHE of Budd-Chiari syndrome patients who underwent TIPSS ( odds ratio=8.36, 1.74, 95% confidence interval as 1.02?68.75, 1.12?2.69, P<0.05). (3) Prediction of OHE after TIPSS. Results of ROC curve showed that the AUC of the CONUT score, the Child-Pugh score of liver function and the integrated model of end-stage liver disease (iMELD) score in predicting the incidence of OHE after TIPSS was 0.77(95% confidence interval as 0.64?0.91, P<0.05), 0.71(95% confidence interval as 0.56?0.87, P<0.05) and 0.71(95% confidence interval as 0.53?0.88, P<0.05), respectively, and there was no significant difference between the AUC of the CONUT score and the Child-Pugh score of liver function or the iMELD score ( Z=0.84, 0.59, P>0.05). The optimal cutoff value of CONUT score in predicting the incidence of OHE after TIPSS was 7, with the sensitivity, specificity and Yodon index as 78.6%, 61.8% and 0.40, respectively. Conclusion:The CONUT score can be used to predict the incidence of OHE in Budd-Chiari syndrome patients who underwent TIPSS, and the discrimination of CONUT score is equivalent to the Child-Pugh score of liver function and the iMELD score.

Advances on diagnosis of sepsis-associated encephalopathy in children / 中国小儿急救医学

Sepsis-associated encephalopathy(SAE) is a diffuse brain dysfunction caused by sepsis.The main clinical manifestations are abnormal mental state, high mortality and poor prognosis.At present, there is no unified diagnostic standard for SAE.The exclusion diagnosis is mainly based on clinical symptoms and signs, combined with laboratory examination and imaging auxiliary examination.Among them, brain magnetic resonance imaging and quantitative electroencephalography can early detect brain dysfunction and predict the prognosis of children, which play an important role in the early diagnosis and prognosis assessment of SAE.Cerebral oxygen monitoring can dynamically reflect the changes of brain function and can be used for long-term monitoring of children with severe brain function injury.SAE is closely associated with poor prognosis, and mortality will increase with the extention of hospitalization.Therefore, early identification of SAE is of great significance to reduce mortality.

Clinical features of three children with severe COVID-19 encephalopathy and literature review / 中国小儿急救医学

Objective:To analyze the clinical characteristics, diagnosis and treatment of 3 children with severe COVID-19 encephalopathy, aiming to improve the clinicians′ understanding of the disease.Methods:The clinical features, laboratory examinations, imaging data and diagnosis as well as treatment process of 3 cases of severe COVID-19 encephalopathy admitted to the Pediatric Intensive Care Unit of Shengjing Hospital of China Medical University from December 1, 2022 to December 31, 2022 were retrospectively analyzed.Results:Among the 3 patients, 2 were female, age was 2-11 years old, all of them had 2-3 days of medical history.All of them had clinical manifestations of high fever(≥40 ℃), convulsions and consciousness disorders, nucleic acid and antigen tests of SARS-CoV-2 were positive, and mycoplasma pneumonia IgM antibody was positive in 1 case.Within 24 hours after admission, the levels of white blood cells were basically normal, neutrophil fraction was dominant, and procalcitonin was significantly increased.Total T cells and NK cells in the blood of the three patients were significantly decreased, and the levels of blood ammonia, blood glucose and bilirubin were basically normal.During the early stage of the disease, the cell counts of the cerebrospinal fluid was normal in all three patients, the protein level was significantly increased, and there were new symmetrical lesions on head magnetic resonance imaging in 3 patients.After symptomatic treatment and immunotherapy including early use of hormone, human gamma globulin and plasma exchange, all patients were survived, but had different degrees of new dysfunction of the nervous system.Conclusion:Severe COVID-19 encephalopathy can occur in the acute phase of SARS-CoV-2 infection, mostly manifested as high fever, convulsions and severe disturbance of consciousness, combining with multiple organ dysfunction and irreversible nervous system damage.Early supportive treatment, brain protective treatment and immunotherapy are helpful to improve the prognosis of the patients.