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El estudio de los tumores del sistema nervioso central (SNC) resulta ser un tema de gran consideración y su conocimiento reviste una alta importancia en la práctica médica. Las clasificaciones de las neoplasias del SNC comenzaron a mediados del siglo XIX hasta que en 1979 la Organización Mundial de la Salud (OMS) publicó la primera edición de una sistemática útil con el objetivo de establecer un lenguaje común para todas las especialidades médicas. Al día de hoy, 5 ediciones actualizaron la taxonomía neoplásica. La quinta edición del año 2021 consolida el cambio de paradigma dado por los avances moleculares, si bien todavía la transición se encuentra en proceso entre la caracterización morfológica y la biológica molecular. En este artículo, se analizan las nuevas modificaciones incorporadas en las diferentes familias tumorales más frecuentes en pediatría haciendo hincapié en aquella información de utilidad para el médico pediatra en su práctica diaria y la consulta multidisciplinaria.
The study of central nervous system (CNS) tumors is a subject of great interest and such knowledge is of great importance in medical practice. The classifications of CNS neoplasms began in the mid-19 th century, until the World Health Organization (WHO) published, in 1979, the first edition of a useful systematic review for the purpose of establishing a common language for all medical specialties. To date, 5 updated editions of neoplastic taxonomy have been published.The fifth edition, from 2021, consolidates the paradigm shift brought about by molecular advances, although the transition between morphological and molecular biological characterization is still in progress. In this article, the new modifications introduced in the different most frequent families of tumors in pediatrics are analyzed, emphasizing useful information for pediatricians in their daily practice and multidisciplinary consultations.
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Humanos , Criança , Neoplasias do Sistema Nervoso Central/classificação , Neoplasias do Sistema Nervoso Central/diagnóstico , Organização Mundial da SaúdeRESUMO
El absceso cerebral es una infección localizada, una colección de material purulento en el parénquima cerebral, que puede surgir como complicación de otra infección o por traumatismo o cirugía. La infección por Nocardia spp. es poco común en pacientes inmunocompetentes, considerándose un agente oportunista. Se describe el caso de un paciente de 51 años, previamente sano, que consultó por cuadro insidioso de meses de evolución de cefalea holocraneana, además de convulsiones tónico-clónicas generalizadas, con picos febriles persistentes, en quien se diagnosticaron abscesos múltiples en el sistema nervioso central a un germen infrecuente.
Brain abscess is a localized infection, a collection of purulent material in the brain parenchyma, which can arise as a complication of another infection or from trauma or surgery. Infection by Nocardia spp. is uncommon in patients without immunocompromised, being considered an opportunistic agent. We present a case of a 51-year-old patient, previously healthy, who consulted for an insidious picture of holocranial headache of months of evolution, in addition to generalized tonic-clonic seizures, with persistent feverish peaks, in whom multiple abscesses were diagnosed in the central nervous system to an infrequent germ, such as Nocardia spp.
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Introduction: Multiple sclerosis significantly affects the quality of life of those suffering from this specific condition. Objective: To assess the quality of life of people with multiple sclerosis and analyse the correlation between the disease and its associated effects and different sociodemographic, clinical, and functional variables. Materials and Methods: An observational, cross-sectional, descriptive-correlational and quantitative study conducted using a non-probabilistic convenience sample composed of 70 patients suffering from multiple sclerosis registered with the Multiple Sclerosis Association of the Central Region of Portugal. The data collection protocol included sociodemographic and clinical questions, the Family Apgar Scale, and the Barthel Index. Descriptive and inferential statistics were used to process the data. Data collection took place between April and July 2021. Results: The majority of participants reported a moderate overall quality of life (M=51,78 ± 24,09). Higher scores were observed in the social relationships and environmental health domains, while lower scores were recorded for the physical domain. Better quality of life was found to be positively associated with being under 45 years old, having higher educational qualifications, living in functional families, and experiencing greater functional independence in activities of daily living. Discussion: The variables with the strongest association were those capable of influencing the physical and social domains. Those variables explained 59.00% and 53.00% of the variability. Conclusions: These results indicate that people with multiple sclerosis have a compromised quality of life, highlighting the need for new strategies focusing on early diagnosis and effective preventive interventions meant to improve quality of life across all its domains.
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Pacientes , Qualidade de Vida , Doenças Desmielinizantes , Estimulantes do Sistema Nervoso Central , Esclerose MúltiplaRESUMO
Se presenta el caso clínico de un paciente de 37 años de edad con el antecedente de haber recibido radioterapia por una lesión tumoral en la región frontal derecha, el cual acudió a consulta en el Instituto de Neurología y Neurocirugía de Cuba por presentar cefalea intensa y hemiparesia izquierda. Luego de realizados los exámenes necesarios, se estableció el diagnóstico clínico-imagenológico de lesión por radionecrosis en el hemisferio contralateral, que fue corroborado en el estudio anatomopatológico una vez que se extirpó el tumor; seguidamente, se indicó inmunoterapia. La evolución del paciente fue satisfactoria, pues se logró el control de la enfermedad y la resolución de los síntomas.
The case report of a 37-year-old patient with history of having received radiotherapy due to a tumor lesion in the right frontal region is presented, who attended to the Institute of Neurology and Neurosurgery in Cuba because of intense headache and left hemiparesis. After carrying out the necessary examinations, the clinical-imaging diagnosis of a radionecrosis lesion in the contralateral hemisphere was established, which was corroborated in the pathological examination once the tumor was removed; then, immunotherapy was indicated. The patient had a favorable clinical course because the control of the disease was achieved as well as the resolution of symptoms.
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RESUMEN Las vasculitis de las arterias o vasculitis cerebelosas en el lupus eritematoso sistémico (LES) son extremadamente infrecuentes y podrían estar inmersas o formar parte de los casos de LES neuropsiquiátrico (LESNP), stroke cerebeloso en el LES y grupo de vasculitis del sistema nervioso central (SNC ) por LES. La adición del manejo quirúrgico a esta entidad, mediante descompresión suboccipital, ha sido reportada en muy pocas publicaciones. Se presenta el caso de una paciente mujer de 18 años con LES, LESNP, stroke cerebeloso, con alta sospecha de vasculitis inflamatoria de las arterias cerebelosas a quien, a pesar del manejo médico, se le brindó descompresión suboccipital. La vasculitis inflamatoria de las arterias cerebelosas en el LES es una causa infrecuente de stroke cerebeloso de elevada mortalidad. Existe limitada evidencia científica en la realización de la descompresión suboccipital de esta entidad.
ABSTRACT Arterial vasculitis or cerebellar vasculitis in systemic lupus erythematosus (SLE) are extremely infrequent, and these might be included within cases of neuropsychiatric SLE (NPSLE), cerebellar stroke in SLE, and within the group of central nervous system vasculitis due to SLE. The addition of surgical management to this condition has been reported in few publications. We present the case of a 18- year old female patient with SLE, NPSLE, and cerebellar stroke, highly suspicious of inflammatory vasculitis affecting cerebellar arteries, in whom, in spite of medical management, suboccipital decompression was performed. Inflammatory vasculitis of cerebellar arteries in SLE is an infrequent cause of cerebellar stroke, and it has elevated mortality rates. There is limited scientific evidence with respect to suboccipital decompression for the management of this condition.
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Abstract Extracellular vesicles (EVs) are small vesicles released by cells that facilitate cell signaling. They are categorized based on their biogenesis and size. In the context of the central nervous system (CNS), EVs have been extensively studied for their role in both normal physiological functions and diseases like Alzheimer's disease (AD). AD is a neurodegenerative disorder characterized by cognitive decline and neuronal death. EVs have emerged as potential biomarkers for AD due to their involvement in disease progression. Specifically, EVs derived from neurons, astrocytes, and neuron precursor cells exhibit changes in quantity and composition in AD. Neuron-derived EVs have been found to contain key proteins associated with AD pathology, such as amyloid beta (Aß) and tau. Increased levels of Aß in neuron-derived EVs isolated from the plasma have been observed in individuals with AD and mild cognitive impairment, suggesting their potential as early biomarkers. However, the analysis of tau in neuron-derived EVs is still inconclusive. In addition to Aß and tau, neuron-derived EVs also carry other proteins linked to AD, including synaptic proteins. These findings indicate that EVs could serve as biomarkers for AD, particularly for early diagnosis and disease monitoring. However, further research is required to validate their use and explore potential therapeutic applications. To summarize, EVs are small vesicles involved in cell signaling within the CNS. They hold promise as biomarkers for AD, potentially enabling early diagnosis and monitoring of disease progression. Ongoing research aims to refine their use as biomarkers and uncover additional therapeutic applications.
Resumo As vesículas extracelulares (VEs) são pequenas estruturas liberadas pelas células que agem na sinalização celular. No sistema nervoso central (SNC), as VEs são estudadas em relação à doença de Alzheimer (DA), um distúrbio neurodegenerativo que cursa com declínio cognitivo e morte neuronal. As VEs podem ser biomarcadores potenciais para a DA devido ao seu papel na progressão da doença. As VEs derivadas de neurônios, astrócitos e células precursoras apresentam alterações na DA, contendo proteínas associadas à patologia da DA, como beta-amiloide (Aß) e tau. Níveis elevados de Aß foram observados nas VEs de neurônios de indivíduos com DA, sugerindo seu potencial como biomarcadores precoces. A análise de tau nas VEs de neurônios ainda é inconclusiva. Além disso, as VEs neurais carregam outras proteínas relacionadas à DA, incluindo proteínas sinápticas. As VEs podem ser promissoras como biomarcadores para o diagnóstico precoce e monitoramento da DA, porém mais pesquisas são necessárias para validar seu uso e explorar aplicações terapêuticas. Em resumo, as VEs são vesículas envolvidas na sinalização celular no SNC, com potencial como biomarcadores para a DA.
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Abstract The majority of central nervous system diseases show high signal intensity on T2-weighted magnetic resonance imaging. Diseases of the central nervous system with low signal intensity are less common, which makes it a finding that helps narrow the differential diagnosis. This was a retrospective analysis of brain and spine magnetic resonance imaging examinations in which that finding was helpful in the diagnostic investigation. We selected the cases of patients examined between 2015 and 2022. All diagnoses were confirmed on the basis of the clinical-radiological correlation or the histopathological findings. We obtained images of 14 patients with the following central nervous system diseases: arteriovenous malformation; cavernous malformation; metastasis from lymphoma; medulloblastoma; embryonal tumor; metastasis from melanoma; Rathke's cleft cyst; Erdheim-Chester disease; aspergillosis; paracoccidioidomycosis; tuberculosis; syphilis; immunoglobulin G4-related disease; and metastasis from a pulmonary neuroendocrine tumor. We described lesions of different etiologies in which the T2-weighted imaging profile helped narrow the differential diagnosis and facilitated the definitive diagnosis.
Resumo A grande maioria das doenças do sistema nervoso central apresenta alto sinal em ponderações T2 na ressonância magnética. As alterações com baixo sinal são menos comuns, de forma que essa característica permite estreitar o diagnóstico diferencial. Analisamos, retrospectivamente, pacientes com imagens de ressonância magnética de crânio e/ou coluna em que este achado foi útil na investigação diagnóstica. Os pacientes foram selecionados no período entre 2015 e 2022 e todos tiveram seus diagnósticos confirmados por estudo clinicorradiológico ou por estudo histopatológico. Obtivemos imagens de 14 pacientes com as seguintes afecções: malformação arteriovenosa, cavernoma, metástase de linfoma, meduloblastoma, tumor embrionário, metástase de melanoma, cisto da bolsa de Rathke, doença de Erdheim-Chester, aspergilose, paracoccidioidomicose, tuberculose, sífilis, doença relacionada à IgG4 e metástase de tumor neuroendócrino de pulmão. Descrevemos lesões de diversas origens etiológicas que, a partir de suas características nas imagens ponderadas em T2, foi possível reduzir o quadro de diagnósticos diferenciais e chegar mais facilmente à hipótese final.
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Abstract Background In 2012, the Neurocritical Care Society launched a compilation of protocols regarding the core issues that should be addressed within the first hours of neurological emergencies - the Emergency neurological life support (ENLS). Objective We aim to evaluate this repercussion through a bibliometric analysis. Methods We searched Scopus on October 2022 for articles mentioning ENLS. The following variables were obtained: number of citations; number of citations per year; number of publications per year; year of publication; research type; research subtype; country of corresponding author and its income category and world region; journal of publication and its 5-year impact factor (IF); and section where ENLS appeared. Results After applying eligibility criteria, we retrieved 421 articles, published from 2012 to 2022. The mean number of citations per article was 17.46 (95% Confidence Interval (CI) = 8.20-26.72), while the mean number of citations per year per article was 4.05 (95% CI = 2.50-5.61). The mean destiny journal 5-year IF was 5.141 (95% CI = 4.189-6.093). The majority of articles were secondary research (57.48%; n= 242/421) of which most were narrative reviews (71.90%; n= 174/242). High-Income countries were the most prominent (80.05%; n= 337/421 articles). There were no papers from low-income countries. There were no trials or systematic reviews from middle-income countries. Conclusion Although still low, the number of publications mentioning ENLS is increasing. Articles were mainly published in journals of intensive care medicine, neurology, neurosurgery, and emergency medicine. Most articles were published by authors from high-income countries. The majority of papers were secondary research, with narrative review as the most frequent subtype.
Resumo Antecedentes Em 2012, a Neurocritical Care Society lançou uma compilação de protocolos sobre as questões centrais que devem ser abordadas nas primeiras horas de emergências neurológicas - Emergency neurological life support (ENLS). Objetivo Avaliar a repercussão do ENLS por meio de uma análise bibliométrica. Métodos A base de dados Scopus foi utilizada em outubro de 2022 para a busca por artigos mencionando o ENLS. As seguintes variáveis foram obtidas: número de citações; número de citações por ano; número de publicações por ano; ano de publicação; tipo de pesquisa; país do autor correspondente e sua categoria de renda; revista de publicação e seu fator de impacto de 5 anos (IF); e seção onde o ENLS apareceu. Resultados Os 421 artigos incluídos foram publicados de 2012 a 2022. A média de citações por artigo foi de 17.46 (intervalo de confiança (IC) 95% = 8.20-26.72), enquanto a de citações por ano por artigo foi de 4.05 (IC95% = 2.50-5.61). O IF médio por revista foi de 5.14 (IC95% = 4.19-6.09). A maioria dos artigos era de pesquisa secundária (57.48%; n= 242/421), dos quais a maioria eram revisões narrativas (71.90%; n= 174/242). Os países de alta renda foram os mais prolíficos (80.05%; n= 337/421 artigos). Não houve publicações de países de baixa ou média renda. Conclusão Embora ainda baixo, o número de publicações mencionando o ENLS vem aumentando recentemente. A maioria dos artigos foram publicados em revistas de medicina intensiva, neurologia, neurocirurgia e medicina de emergência. Artigos de pesquisa secundária foram os mais comuns, com revisões narrativas sendo o subtipo mais frequente.
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Introducción: La infiltración del sistema nervioso central por células malignas constituye una complicación grave de algunas neoplasias hematológicas, principalmente leucemias agudas y linfomas agresivos. Objetivo: Resumir la base científica y la significación clínica de los métodos de estudio del líquido cefalorraquídeo para el diagnóstico y el seguimiento de la infiltración neuromeníngea en pacientes con neoplasias hematológicas. Métodos: Se buscó información durante abril de 2021 en las bases de datos PubMed, ScienceDirect y SciELO. Se seleccionaron las publicaciones en base a su tipología, actualidad, alcance y las limitaciones de los estudios. Conclusiones: El estudio citomorfológico del líquido cefalorraquídeo se considera el método estándar para el diagnóstico y el seguimiento de la infiltración neuromeníngea. La citometría de flujo resulta más sensible para la detección de infiltración oculta que la citología convencional; pero aún existen reservas sobre su significación clínica. Se investiga también la sensibilidad de otros estudios moleculares como el uso de la reacción en cadena de la polimerasa y la detección de biomarcadores(AU)
Introduction: Infiltration of the central nervous system by malignant cells constitutes a serious complication of some hematological malignancies, mainly acute leukemias and aggressive lymphomas. Objective: To summarize the scientific basis and clinical significance of cerebrospinal fluid study methods for the diagnosis and follow-up of neuromeningeal infiltration in patients with hematologic malignancies. Methods: Information was searched during April 2021 in PubMed, ScienceDirect and SciELO databases. Publications were selected based on their typology, timeliness, scope, and study limitations. Conclusions: The cytomorphological study of cerebrospinal fluid is considered the standard method for the diagnosis and follow-up of neuromeningeal infiltration. Flow cytometry is more sensitive for the detection of occult infiltration than conventional cytology, but there are still reservations about its clinical significance. The sensitivity of other molecular studies such as the use of PCR and biomarker detection is also investigated(AU)
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Humanos , Neoplasias Hematológicas/líquido cefalorraquidiano , Biomarcadores , Sistema Nervoso Central , Reação em Cadeia da Polimerase , Citometria de FluxoRESUMO
ABSTRACT BACKGROUND AND OBJECTIVES: The presence of central sensitization (CS) in people with chronic neck pain requires clarification, as does the possible disparities between genders. Therefore, this study aimed to evaluate the presence of CS in chronic neck pain according to gender. METHODS: Cross-sectional study with the participation of 260 adults (18-59 years old) with chronic neck pain, carried out between September 2022 and September 2023. Instruments were applied to collect sociodemographic profile, pain characteristics, cervical functional disability and presence of SC. The software SPSS 23.0 was used for comparative analyzes. RESULTS: Regarding disparities between genders, there were no differences in the intensity of pain and cervical functional disability (p=0.134 and p=0.277, respectively). However, SC was higher in women (43.5 ± 14.0; p=0.003). In females, CS was related to "pain interference" in the aspects of walking (r=0.311; p=0.001), relationships with people (r=0.309; p=0.001), appreciation of life (r=0.321; p= 0.001) and with cervical functional disability (r=0.570; p=0.001). In males, CS was related to "pain interference" in aspects such as general activity (r=0.311; p=0.008), mood (r=0.376; p=0.001), walking (r=0.313; p= 0.007), relationships with people (r=0.477; p=0.001), sleep (r=0.321; p=0.006), appreciation of life (r=0.427 p=0.001) and functional disability (r=0.667; p<0.001). CONCLUSION: Women with chronic neck pain showed a greater presence of CS, while CS in men was related to a variety of aspects that negatively affect life. Given this, differences between genders could be considered in the management of patients with chronic neck pain.
RESUMO JUSTIFICATIVA E OBJETIVOS: A presença de sensibilização central (SC) em pessoas com dor cervical crônica carece de esclarecimentos, bem como as possíveis disparidades entre os sexos. Portanto, objetivou-se avaliar a presença de SC na dor cervical crônica de acordo com o sexo. MÉTODOS: Estudo transversal com a participação de 260 adultos (18-59 anos) com dor cervical crônica, realizado entre setembro de 2022 e setembro de 2023. Foram aplicados instrumentos para coletar perfil sociodemográfico, características da dor, incapacidade funcional da cervical e presença de SC. Para análises comparativas foi utilizado o SPSS 23.0. RESULTADOS: Nas disparidades entre os sexos, não houve diferenças na intensidade da dor e incapacidade funcional cervical (p=0,134 e p=0,277, respectivamente). No entanto, a SC foi mais elevada nas mulheres (43,5 ± 14,0; p=0,003). No sexo feminino, a SC apresentou relação com a "interferência da dor" nos aspectos: caminhar (r=0,311; p=0,001), relacionamento interpessoal (r=0,309; p=0,001), apreciação da vida (r=0,321; p=0,001) e com incapacidade funcional da cervical (r=0,570; p=0,001). Já no sexo masculino, a SC teve relação com a "interferência da dor" em aspectos como atividade geral (r=0,311; p=0,008), humor (r=0,376; p=0,001), caminhar (r=0,313; p=0,007), relacionamento interpessoal (r=0,477; p=0,001), sono (r=0,321; p=0,006), apreciação da vida (r=0,427 p=0,001) e incapacidade funcional (r=0,667; p<0,001). CONCLUSÃO: Mulheres com dor cervical crônica mostraram maior presença da SC, enquanto nos homens a SC esteve relacionada a uma variedade de aspectos que afetam negativamente a vida. Diante disso, as diferenças entre os sexos poderiam ser consideradas no gerenciamento de pacientes com dor cervical crônica.
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Abstract Objective The aim of this study was to evaluate the GSH effect on functional and histological recovery after experimental spinal cord injury in rats. Methods Forty Wistar rats were subjected to spinal cord injury through the Multicenter Animal Spinal Cord Injury Study (MASCIS) Impactor system. The rats were sorted and divided into four groups, as follows: Group 1 ‒ Laminectomy and spinal cord injury; Group 2 ‒ Laminectomy, spinal cord injury and Saline Solution (SS) 0.9%; Group 3 ‒ Laminectomy, spinal cord injury, and GSH; and Group 4 ‒ lLaminectomy without spinal cord injury. GSH and SS were administered intraperitoneally. Groups 1 and 4 received no intervention. Results The rats were evaluated for locomotor function recovery at seven different times by the Basso, Beattie, and Bresnahan (BBB) scale on days 2, 7, 14, 21, 28, 35, and 42 after the spinal cord injury. On day 42, the rats were sacrificed to analyze the histological findings of the injured spinal cord. In the group submitted to GSH, our experimental study revealed better functional scores on the BBB scale, horizontal ladder scale, and cranial and caudal axon count. The differences found were statistically significant in BBB scores and axonal count analysis. Conclusion This study demonstrated that using glutathione in experimental spinal trauma can lead to better functional recovery and improved axonal regeneration rate in Wistar rats submitted to experimental spinal cord injury.
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ABSTRACT BACKGROUND: Embolization is a promising treatment strategy for cerebral arteriovenous malformations (AVMs). However, consensus regarding the main complications or long-term outcomes of embolization in AVMs remains lacking. OBJECTIVE: To characterize the most prevalent complications and long-term outcomes in patients with AVM undergoing therapeutic embolization. DESIGN AND SETTING: This systematic review was conducted at the Federal University of Alagoas, Arapiraca, Brazil. METHODS: This systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria. Data were obtained from MEDLINE, PubMed, LILACS, and SciELO databases, which included the epidemiological profile of the population, characteristics of the proposed therapy, complications (hemorrhagic events and neurological deficits), and long-term outcomes (modified Rankin scale pre- and post-treatment, AVM recanalization, complete obliteration, and deaths). RESULTS: Overall, the analysis included 34 articles (2,799 patients). Grade III Spetzler-Martin AVMs were observed in 34.2% of cases. Notably, 39.3% of patients underwent embolization combined with radiosurgery. The most frequently reported long-term complication was hemorrhage, which occurred in 8.7% of patients at a mean follow-up period of 58.6 months. Further, 6.3% of patients exhibited neurological deficits after an average of 34.7 months. Complete obliteration was achieved in 51.4% of the cases after a mean period of 36 months. Recanalization of AVMs was observed in 3.5% of patients. Long-term death occurred in 4.0% of patients. CONCLUSION: Embolization of AVMs is an increasingly safe strategy with low long-term complications and satisfactory outcomes, especially in patients who have undergone combination therapies. SYSTEMATIC REVIEW REGISTRATION: https://www.crd.york.ac.uk/prospero/ Registration number CRD42020204867.
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ABSTRACT Introduction: The diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL) and, despite all the progress in this field, central nervous system infiltration (CNSi) still occurs at an incidence of 2-10%. The objective of the present study was to evaluate the Central Nervous System International Prognostic Index (CNS-IPI) score in daily practice regarding the reproducibility in a heterogeneous cohort apart from a clinical trial. Methods: Primary DLBCL patients were eligible for this study, between January 2007 and January 2017. All patients were treated with rituximab-based chemotherapy, mostly R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone). The CNSi was diagnosed by liquor (positive cytology and/or immunophenotype), computerized tomography, magnetic resonance image and/or fluorodeoxy-glucose-positron emission tomography, requested only in symptomatic patients when the CNSi was clinically suspected. The CNS-IPI was assessed by graphical comparison and calibration. Results: After applying the inclusion/exclusion criteria, 322 patients were available for the analysis. The median follow-up was 60 months and the median age was 58 years. Seven patients experienced CNSi, characterizing an incidence of 2.17% (7/322). Comparing groups of patients with and without CNSi, we observed that the lactate dehydrogenase (LDH), number of extranodal sites, IPI, kidney/adrenal and absence of complete response were statistically different. The CNS-IPI model stratified patients in a three-risk group model as low-, intermediate- and high-risk. In our cohort, using the same stratification, we obtained an equivalent the 2-year rate of CNS relapse of 0.0%, 0.8% and 13.8%, respectively. Conclusion: Our study reinforces the reproducibility of the CNS-IPI, specifically apart from clinical trials, and suggests the CNS-IPI score as a tool to guide therapy.
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Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Linfoma não Hodgkin , Sistema Nervoso Central , LinfomaRESUMO
Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin's lymphoma that accounts for 4% of newly diagnosed central nervous system (CNS) tumors. Most primary lymphomas of the central nervous system are of the subtype of diffuse large B-cell lymphomas, which have highly aggressive behavior and may involve the brain, leptomeninges, eyes or spinal cord without evidence of systemic disease. Primary CNS lymphomas are very rare in immunocompetent patients, but their rates are increasing. So far, only 11 primary Gasser ganglion lymphomas have been reported, with an incidence of 2.5 cases per 30,000,000 inhabitants. However, B cell lymphomas of the marginal zone of the Gasserian ganglion have been very rarely reported. We report here a clinical presentation characteristic of B cell lymphoma of the marginal zone of the Gasser ganglion in an immunocompetent patient who was treated with surgery and radiotherapy, evolving with improvement of symptoms and without recurrence in 3 months of follow-up.
O linfoma primário do sistema nervoso central (PCNSL, na sigla em inglês) é um subtipo raro de linfoma não-Hodgkin extranodal que representa 4% dos tumores recém-diagnosticados do sistema nervoso central (SNC). A maioria dos linfomas primários do sistema nervoso central é do subtipo dos linfomas difusos de grandes células B, que tem comportamento altamente agressivo e pode envolver cérebro, as leptomeninges, os olhos ou a medula espinhal sem evidências de doença sistêmica. Os linfomas primários do SNC são muito raros em pacientes imunocompetentes, mas suas taxas estão aumentando. Até o momento, apenas 11 linfomas primários do gânglio de Gasser foram relatados, com uma incidência de 2,5 casos por 30.000.000 de habitantes. No entanto, linfomas de células B da zona marginal do gânglio de Gasserian foram muito raramente relatados. Relatamos aqui uma apresentação clínica característica de um linfoma de células B da zona marginal do gânglio de Gasser em um paciente imunocompetente que foi tratado com cirurgia e radioterapia, evoluindo com melhora dos sintomas e sem recorrência em três meses de acompanhamento.
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Body temperature is primarily regulated by the hypothalamus, ensuring proper metabolic function. Envenomation by Phoneutria nigriventer can cause symptoms such as hypothermia, hyperthermia, sweating, and shivering, all related to thermoregulation. This study aims to analyze and identify components of the venom that affect thermoregulation and to evaluate possible mechanisms. Rats were used for thermoregulation analysis, venom fractionation by gel filtration and reverse-phase chromatography (C18), and sequencing by Edman degradation. The venom exhibited hypothermic effects in rats, while its fractions demonstrated both hypothermic (pool II) and hyperthermic (pool III) effects. Further separations of the pools with C18 identified specific peaks responsible for these effects. However, as the peaks were further purified, their effects became less significant. Tests on U87 human glioblastoma cells showed no toxicity. Sequencing of the most active peaks revealed masses similar to those of the Tachykinin and Ctenotoxin families, both known to act on the nervous system. The study concludes that molecules derived from venom can act synergistically or antagonistically. Additionally, toxins that affect thermoregulation are poorly studied and require further characterization. These toxins could potentially serve as sources for the development of new thermoregulatory drugs.
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Here we introduce a first-in-class microRNA-sensitive oncolytic Zika virus (ZIKV) for virotherapy application against central nervous system (CNS) tumors. The described methodology produced two synthetic modified ZIKV strains that are safe in normal cells, including neural stem cells, while preserving brain tropism and oncolytic effects in tumor cells. The microRNA-sensitive ZIKV introduces genetic modifications in two different virus sites: first, in the established 3′UTR region, and secondly, in the ZIKV protein coding sequence, demonstrating for the first time that the miRNA inhibition systems can be functional outside the UTR RNA sites. The total tumor remission in mice bearing human CNS tumors, including metastatic tumor growth, after intraventricular and systemic modified ZIKV administration, confirms the promise of this virotherapy as a novel agent against brain tumors—highly deadly diseases in urgent need of effective advanced therapies.
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Resumen Existen formas de presentación poco frecuentes de sífilis, dentro de las cuales se incluyen la neurosífilis, otosífilis y sífilis ocular. La neurosífilis es la infección del sistema nervioso central por Treponema pallidum. Las manifestaciones clínicas de neurosífilis son variadas e incluyen formas tempranas, tardías y atípicas. Además, la sífilis puede comprometer prácticamente cualquier estructura ocular, en cualquier etapa de la enfermedad, como así también la otosífilis. El diagnóstico de estas en tidades suele ser dificultoso. Sin embargo, resulta impor tante considerarlas como diagnósticos diferenciales, ya que la mayoría de estas manifestaciones son reversibles con tratamiento antibiótico adecuado. Se presenta una serie de casos de pacientes con diagnóstico de neurosí filis, otosífilis y sífilis ocular, que cursaron internación en un hospital de tercer nivel: meningitis sifilítica con compromiso de pares craneales y convulsiones (caso 1), sífilis ocular (caso 2), paresis general (caso 3) y tabes dorsalis (caso 4). La mitad de los pacientes presentó hipoacusia neurosensorial bilateral. El 50% presentó VDRL reactiva en líquido cefalorraquídeo. Todos fueron tratados con penicilina G sódica y en el 50% se optó por el uso de ceftriaxona como modalidad para finalizar el tratamiento en internación domiciliaria. Respecto a la evolución de los pacientes, uno de ellos falleció como consecuencia del cuadro de neurosífilis (caso 1), otro se perdió en el seguimiento (caso 4) mientras que, de los dos restantes, el caso 3 presentó recaída de su enferme dad a los 6 meses del tratamiento y el caso 2 resolvió ad integrum su sintomatología.
Abstract Uncommon forms of syphilis exist, among which neurosyphilis, otosyphilis, and ocular syphilis are included. Neurosyphilis is the infection of the central nervous system caused by Treponema pallidum. The clinical manifestations of neurosyphilis are diverse and include early, late, and atypical forms. Syphilis can affect virtually any ocular structure and can oc cur at any stage of the disease, as well as otosyphilis. The diagnosis of these conditions is often challeng ing. However, it is important to consider them as a differential diagnosis, as most of these clinical mani festations are reversible with appropriate antibiotic treatment. A case series study of patients diagnosed with neurosyphilis, otosyphilis, and ocular syphilis, who were admitted to a tertiary-level hospital, is here presented: syphilitic meningitis with cranial nerve in volvement, and seizures (case 1), ocular syphilis (case 2), general paresis (case 3), and tabes dorsalis (case 4). Half of the patients presented bilateral sensori neural hearing loss; and also half of the patients had reactive VDRL in cerebrospinal fluid. All were treated with aqueous penicillin G, and in two of these cases, ceftriaxone was chosen to complete ambulatory treat ment. One patient had an unfavorable outcome and died (case 1); another was lost in follow-up (case 4); one completely resolved his symptoms (case 2); and another one experienced symptom relapse six months after treatment (case 3).
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Fundamento: los biomarcadores de estrés oxidativo en la enfermedad de Huntington pudieran predecir el curso de la enfermedad y evaluar nuevos tratamientos, pero su naturaleza inespecífica parece impedir la identificación de algún marcador útil. Esclarecer similitudes y diferencias de este fenómeno y su comportamiento con características clínicas puede ser esencial. Objetivo comparar biomarcadores de estrés oxidativo entre pacientes con enfermedad de Huntington y con otros desórdenes neurológicos. Métodos se realizó un estudio analítico, retrospectivo y caso-control (enfermedad de Huntington esclerosis lateral amiotrófica, ataxia espinocerebelosa tipo 2 e ictus isquémico: en etapa aguda y crónica). Se recogieron variables demográficas, clínicas y marcadores de daño oxidativo (malonildialdehído, productos avanzados de oxidación a proteínas) y antioxidantes (superóxido dismutasa: catalasa: glutatión peroxidasa, capacidad antioxidante del plasma). Resultados hubo diferencias significativas del malonildialdehído en la enfermedad de Huntington respecto al control (p=0,02), pero no con el resto de los grupos. La enzima superóxido dismutasa en enfermedad de Huntington fue menor estadísticamente en comparación con esclerosis lateral amiotrófica, aunque para la catalasa fue superior en relación con el resto de los pacientes. El FRAP en enfermedad de Huntington resultó menor significativamente versus esclerosis lateral amiotrófica e ictus isquémico agudo. Los productos avanzados de la oxidación de proteína se correlacionaron directamente con las edades biológicas y de inicio de la enfermedad de Huntington. La actividad motora en la esclerosis lateral amiotrófica y el déficit neurológico en el ictus isquémico agudo se correlacionaron con el malonildialdehído y glutatión peroxidasa respectivamente. Conclusiones la enfermedad de Huntington parece mostrar características específicas en su sistema antioxidante. La oxidación de proteínas pudiera estar relacionada con la acumulación de huntingtina mutada en el tiempo.
Foundation: biomarkers of oxidative stress in Huntington's disease could predict the course of the disease and evaluate new treatments, but their nonspecific nature seems to prevent the identification of any useful marker. Clarifying similarities and differences of this phenomenon and its behavior with clinical characteristics may be essential. Objective: compare biomarkers of oxidative stress between patients with Huntington's disease and other neurological disorders. Methods: an analytical, retrospective and case-control study was carried out (Huntington's disease, amyotrophic lateral sclerosis, spinocerebellar ataxia type 2 and ischemic stroke: acute and chronic stage). Demographic and clinical variables and markers of oxidative damage (malonildialdehyde, advanced protein oxidation products) and antioxidants (superoxide dismutase: catalase: glutathione peroxidase, plasma antioxidant capacity) were collected. Results: there were significant differences in malonyldialdehyde in Huntington's disease compared to the control (p=0.02), but not with the rest of the groups. The enzyme superoxide dismutase in Huntington's disease was statistically lower compared to amyotrophic lateral sclerosis, although for catalase it was higher in relation to the rest of the patients. FRAP in Huntington's disease was significantly lower versus amyotrophic lateral sclerosis and acute ischemic stroke. Advanced products of protein oxidation were directly correlated with the biological and onset ages of Huntington's disease. Motor activity in amyotrophic lateral sclerosis and neurological deficit in acute ischemic stroke were correlated with malonyldialdehyde and glutathione peroxidase, respectively. Conclusions: huntington's disease seems to show specific characteristics in its antioxidant system. Protein oxidation could be related to the accumulation of mutated huntingtin over time.
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La encefalitis autoinmune es un trastorno inmunomediado que compromete distintos territorios del parénquima cerebral, involucrando frecuentemente la materia gris profunda o la corteza, con o sin compromiso de la materia blanca, meninges o médula espinal. Se asocia frecuentemente con enfermedades autoinmunes o paraneoplásicas, y constituye un reto diagnóstico. Reportamos el caso de una mujer de 55 años con antecedente de síndrome de Sjögren que consultó a Emergencias por cefalea y confusión. El líquido cefalorraquídeo (LCR) presentaba leucocitosis con neutrofilia. En la resonancia magnética nuclear (RMN) cerebral se evidenciaron múltiples imágenes de comportamiento restrictivo, de señal hiperintensa en T2 y FLAIR, a predominio córtico-subcortical a nivel occipital bilateral, hemisferio cerebeloso derecho y parietal derecho. Se descartaron infecciones y neoplasias. El panel de anticuerpos para encefalitis autoinmune aquaporina-4 y anti-MOG en LCR fue negativo. Recibió metilprednisolona endovenosa con mejoría progresiva de los síntomas.
Autoimmune encephalitis is an immune-mediated disorder that affects different areas of the brain parenchyma, often involving deep gray matter or the cortex, with or without involvement of white matter, meninges, or spinal cord. It is frequently associated with autoimmune or paraneoplastic diseases and is a diagnostic challenge. We report the case of a 55-year-old woman with history of Sjögren's syndrome who presented to the emergency department with headache and episodes of confusion. Cerebrospinal fluid (CSF) analysis showed leukocytosis with neutrophilia. Brain MRI revealed multiple restricted diffusion lesions with hyperintense signal on T2 and FLAIR sequences, predominantly in the bilateral occipital region, right cerebellar hemisphere, and right parietal region. Infections and neoplasms were ruled out. The panel of antibodies for autoimmune encephalitis, including Aquaporin-4 and anti-MOG in CSF, was negative. She received intravenous methylprednisolone, leading to symptom improvement.
Assuntos
Feminino , Sistema Nervoso CentralRESUMO
La anencefalia es una anomalía gestacional que produce la ausencia de gran parte del cerebro y del cráneo; aunque se desconoce el motivo principal de su aparición, puede estar relacionado con toxinas ambientales, y pobre ingesta de ácido fólico durante el embarazo. Los fetos que padecen de esta malformación, en su mayoría, no llegan a nacer vivos o mueren a las pocas horas. Se presentó una paciente recibida en emergencia en el Centro de Atención Integral Materno Infantil, municipio Ixchiguan, departamento de San Marcos, Guatemala con embarazo a término, según refirió la paciente, sin atención prenatal, dolor en hipogastrio y pérdida de líquido amniótico de un día de evolución. Siendo este un defecto genético multifactorial, para el cual no existe tratamiento, el único recurso para su prevención fue brindar una atención prenatal pormenorizada con énfasis especial en el programa de genética para la detección precoz de anomalías congénitas.
Anencephaly is a gestational anomaly that produces the absence of a large part of the brain and skull; although the main reason for its appearance is unknown, it may be related to environmental toxins and poor intake of folic acid during pregnancy. Most of the fetuses that suffer from this malformation are not born alive or die within a few hours. We present a female patient who was received in the emergency service of the Comprehensive Maternal and Child Care Center in Ixchiguan municipality, San Marcos department, Guatemala, with a full-term pregnancy, as reported by the patient, without prenatal care, hypogastric pain and loss of amniotic fluid of one day of evolution. Since there is no treatment for this multifactorial genetic defect, the only resource for its prevention was to provide detailed prenatal care with special emphasis on the genetics program for the early detection of congenital anomalies.