Nutritional management of individuals with Huntington’s disease: nutritional guidelines

The delivery of good nutritional care is a fundamental element of the management of individuals with Huntington’s disease and all patients with Huntington’s disease will, at some time, need dietary intervention because of the sequela of the disease; yet there are no European nutritional guidelines. The European Huntington’s Disease Network Standards of Care Dietitians Group has brought together expert dietitians from across Europe to produce nutritional guidelines to improve the nutritional management of individuals with Huntington’s disease. The guidelines were developed to promote optimal nutritional screening, assessment and management of individuals throughout all stages of the disease, with the aim of improving the standard of nutritional care delivered. Literature was systematically searched in an attempt to ensure that the recommendations are based on sound evidence and where evidence is lacking, specific guidance is based on consensus expert dietetic opinion. The provision of nutritional care varies widely between countries. Implementation of these nutritional guidelines across Europe should improve the quality of nutritional care delivered to individuals with Huntington’s disease

Avaliação de tumores gliais por ressonância magnética do encéfalo e correlação com estudo histopatológico

Os gliomas são os tumores primários mais comuns do sistema nervoso central (SNC), e sua graduação correta é importante para planejamento terapêutico e prognóstico. O estudo histopatológico e, mais recentemente, quando disponível, a associação com análise molecular, são as técnicas padrão ouro para graduação dos tumores do SNC segundo os critérios da World Health Organization (WHO 2021). Um método não invasivo que permita a definição do grau dos gliomas é altamente desejável, uma vez que a biópsia pode ter limitações. O presente estudo teve como objetivo determinar a correlação dos achados de imagem convencionais e de difusão obtidos por ressonância magnética (RM) e baseados nos critérios Visually AcceSAble Rembrandt Images (VASARI) com o grau histopatológico dos tumores gliais. Foi realizada uma análise pré-cirúrgica das imagens de RM de 178 pacientes com gliomas e confirmação histopatológica (Alto-grau, n=140; Baixo-grau, n =38) por dois neurorradiologistas que avaliaram tamanho tumoral, localização e características morfológicas baseados em critérios padronizados do VASARI. Na análise univariada, mais da metade das características avaliadas mostraram uma significativa associação com o grau tumoral. Hemorragia, restrição a difusão, invasão pial, realce pelo meio de contraste e tumor sem realce, cruzando a linha média, foram as características com a associação mais significativa (p<0,0005). Na análise multivariável por regressão logística, a presença de realce (Odds Ratio [OR] = 3,1; p = 0,07) e realce (OR = 3,1; p = 0,02) mantiveram uma importante associação com tumores de alto grau. Na análise de regressão logística do tipo stepwise, hemorragia intratumoral, presença de realce pelo meio de contraste e multicentricidade mantiveram uma importante associação com tumores de alto grau. A técnica de perfusão por sensibilidade dinâmica ao meio de contraste no subgrupo de pacientes com estas sequências disponíveis mostrou uma associação importante com tumores de alto grau (OR = 18,8; p < 0,0001). Na análise de subgrupo de pacientes com pesquisa da mutação IDH, a ausência de realce pelo meio de contraste e ausência de restrição ao estudo da difusão foram associados à presença da mutação. Os dados de RM baseados no VASARI, especialmente hemorragia intratumoral, presença de realce pelo meio de contraste e multicentricidade apresentam uma importante correlação com o grau dos gliomas. A RM é especialmente útil nos casos em que a biópsia está contraindicada ou quando existe uma dissociação entre o grau tumoral sugerido pela biópsia e pela imagem.Gliomas are one of the most common tumors in the central nervous system and the correct grading is important for therapeutic planning and prognosis. The histopathological study, and more recently, when available, the association of molecular analysis, are the gold standard techniques for grading intracranial glial tumors. A non-invasive method that allows the definition of glioma grade is highly desirable, as biopsy may have limitations. This study aimed to determine the correlation of conventional and diffusion imaging findings on magnetic resonance imaging (MRI) of the brain, based on Visually AcceSAble Rembrandt Images (VASARI) criteria, with the histopathological grading of gliomas. Presurgical magnetic resonance images of 178 patients with brain gliomas and pathological confirmation (High-grade, n = 140; Low-grade, n = 38) were rated by 2 neuroradiologists for tumor size, location, and tumor morphology by using a standardized imaging feature set VASARI. In the univariate analysis, more than half of the evaluated characteristics showed a significant association with the tumor grade. Hemorrhage, diffusion restriction, pial invasion, enhancement, and tumor without enhancement crossing midline were the characteristics with the most significant association (p < 0,0005). In a multivariable regression model, the presence of enhancement (OR = 3,1; p = 0,07) and hemorrhage (OR = 3,1; p = 0,02) maintained a significant association with high-grade tumors. In stepwise logistic regression, intratumor hemorrhage, presence of contrast enhancement, and multicentricity maintained an important association with high-grade glial tumors. The dynamic susceptibility contrast (DSC) perfusion technique in the subgroup with these sequences available showed an association with high-grade tumors (OR = 18,8; p < 0,0001). The absence of contrast enhancement and the absence of diffusion restriction were associated with the presence of an IDH mutation. Our data illustrate that magnetic resonance features of VASARI, especially intratumor hemorrhage, presence of contrast enhancement, and multicentricity provided an important correlation with glioma grading. MRI is useful in cases where the biopsy procedure is contraindicated or can be helpful to determine the glioma grade when there is a disassociation between the biopsy and imaging results

Acute cerebellitis caused by herpes simplex virus

Case presentation A 29 year-old woman presented to the emergency with gait imbalance and dysarthria. At admission, neurologic examination revealed normal cognition, ataxia, dysarthria, dysmetria on both sides of the body, bilateral vertical nystagmus and loss of the lateral eye movement. Blood examination was notable for a increase in WBC count and demonstrated erythrocyte sedimentation rate of 18 mm/h. Examination of cerebral spinal fluid (CSF) revealed a protein concentration of 166 mg/ dL, a glucose concentration of 56 mg/dL, and pleocytosis. Serum glucose concentration was 126 mg/dL. The patient had no history of immunosuppression or another comorbidity and anti-HIV test was negative Neurological evaluation included a head computed tomography (CT) scan which revealed normal findings. An MRI of the brain revealed bilateral increased signal intensity in the cerebellum on fluid-attenuated inversion recovery images (FLAIR)/T2, without contrast enhancement, suggesting an inflammatory process confined to the cerebellum (Figure 1 and 2). Furthermore, the cerebellar cortex appeared swollen, a finding consistent with diffuse cerebellitis. There were no alterations in the brainstem. Initially, the possibility of bacterial rhomboencephalitis caused by Listeria monocytoges was considered, since it is the most commom cause of rhomboencephalitis. After a few days with antibiotic therapy (ceftriaxone and ampicillin), polymerase chain reaction (PCR) test of the CSF was positive for Herpes Simplex Virus 1/2 (HSV) Bacterial culture of CSF samples showed no growth, and the results of Gram staining of CSF were negative. Anti-Listeria antibody was also negative and ampicillin discontinued. CSF PCR analysis for other herpesviruses (varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, and human herpesvirus types 6–8) and enteroviruses were also negative. Upon treatment with acyclovir (50 mg/kg/day) during 21 days, symptoms improved. One month later after the first MRI, a significantly reduce of imaging abnormalities was detected (Figure 3)

Infecção do sistema nervoso central : os achados de imagem sugestivos de fungo como causa

Fungal infections of the central nervous system (CNS) are rare. However, because of the increase in the number of immunocompromised individuals, they have been gaining prominence in the differential diagnosis of CNS infections. Imaging techniques are sensitive for detecting and localizing an abnormality, in many cases allowing the origin of a lesion to be categorized as infectious, inflammatory, neoplastic, or vascular. This essay illustrates the magnetic resonance imaging and computed tomography findings of the most common fungal infections of the CNS, based on the experience of the Radiology Department of the Hospital de Clínicas de Porto Alegre, in the city of Porto Alegre, RS, Brazil.As infecções fúngicas do sistema nervoso central (SNC) são raras, entretanto, com o aumento no número de indivíduos imunocomprometidos elas têm ganhado destaque no diagnóstico diferencial de infecções no SNC. As atuais técnicas de imagem são sensíveis para detectar uma anormalidade, localizá-la, e em muitos casos categorizar a lesão como de origem infecciosa e/ou inflamatória, neoplásica ou vascular. Este ensaio ilustra os achados de ressonância magnética e tomografia computadorizada mais comuns nas infecções fúngicas do SNC baseadas na experiência do Serviço de Radiologia do Hospital de Clínicas de Porto Alegre

Study of the scientific collaboration network in nanotechnology at the Brazilian Agricultural Research Corporation

O estudo da colaboração científica permite observar resultados que podem orientar a gestão da pesquisa em uma organização. Nesse sentido, o objetivo deste estudo é analisar a rede de colaboração científica em nanotecnologia na Empresa Brasileira de Pesquisa Agropecuária e sua evolução, considerando-se ser um tema que vem gerando resultados inovadores para o agronegócio brasileiro. Para tanto, foi empregada a metodologia de Análise de Redes Sociais, com o objetivo de perceber as relações de coautoria e de como analisar a evolução dessa rede de colaboração. Para trabalhar a perspectiva dos dados, foi escolhida a Base de Dados da Pesquisa Agropecuária, de acesso público, organizada e gerenciada pela Embrapa. A análise dos dados baseou-se na produção científica entre 2006 e 2014. Para a análise da evolução da rede, o período foi dividido em três fases: de 2006 a 2008, de 2009 a 2011 e de 2012 a 2014. Os dados, recuperados a partir do ano de 2006, permitiram compor um período significativo de análise de quase dez anos, evidenciando que a evolução da rede aconteceu de forma progressiva, e que a colaboração científica sobre o tema na Embrapa se torna cada vez mais efetiva e dinâmica.The study of scientific collaboration allows us to observe, for instance, results that can guide research management in an organization. In this sense, the objective of this study is to analyze the network for scientific collaboration in nanotechnology at the Brazilian Agricultural Research Corporation, and the evolution thereof, since this is a field which has produced innovative results for Brazilian agribusiness. To this end, the methodology of Social Network Analysis was used in order to identify co-authorship connections and to analyze the evolution of this network. The origin of the data was Agricultural Research Database (Base de Dados da Pesquisa Agropecuária), which is freely accessible to the public. The analysis was based on scientific production from 2006 to 2014. For the analysis of network evolution, this timeframe was divided into three periods, consisting of the periods from 2006 to 2008, 2009 to 2011, and 2012 to 2014. The results showed that the data represents a significant timeframe for analysis, nearly 10 years, providing the perception that, insofar as the evolution of the network is concerned, it evidently occurred in a gradual manner, highlighting the fact that scientific collaboration in nanotechnology at Embrapa becomes increasingly effective and dynamic

The central vein sign in multiple sclerosis : a biomarker evaluated on a 3T MRI scanner

The central vein sign (CVS) is a promising MRI biomarker in multiple sclerosis (MS). CVS has recently been proposed to improve the accuracy and speed of MS diagnosis. Evidence indicates that the presence of CVS in individual lesions can accurately differentiate MS from other diseases that mimic this condition, such as hypertensive microangiopathy, atypical demyelination, and neuromyelitis optica. Most studies have used 7T MRI scanners, which limits their clinical applicability. Recently, it has been demonstrated that the fusion of the FLAIR and SWI sequences, generating FLAIR*, allows CVS visualization even on 3T scanners. Many studies have confirmed that CVS at 3T is a specific imaging finding for MS

Survival of patients submitted to allogeneic bone marrow transplantation

Introdução: O transplante de células-tronco hematopoiéticas (TCTH) alogênico é um procedimento que oferece um potencial de cura para doenças hematológicas malignas e benignas. O benefício da técnica está especialmente relacionado ao aumento da sobrevida em pacientes com doadores HLA-compatíveis em cujos casos o tratamento quimioterápico mostrouse insuficiente ou ineficaz. Objetivos: Analisar a sobrevida de pacientes que receberam TCTH alogênico aparentado no Serviço de Hematologia Clínica e Transplante de Medula Óssea (SHCTMO) do Hospital de Clínicas de Porto Alegre (HCPA). Métodos: Estudo de coorte prospectiva com análise de sobrevida de pacientes transplantados entre 1994 e 2003. Resultados: Foram analisados 133 pacientes com idade média de 30,8±14,8 anos com um tempo médio de 26,8 meses entre o diagnóstico e o TCTH. Cinco anos após o transplante, 71 pacientes (53,4%) estavam vivos, 22 pacientes tinham leucemia mieloide aguda (LMA), 54, leucemia mieloide crônica (LMC), e seis padeciam de síndrome mielodisplásica (SMD), sendo que, em 5 anos, a sobrevida foi de 52, 50 e 33%, respectivamente. Dos 26 pacientes transplantados por anemia aplásica (AA), 66,7% tinham idade inferior a 20 anos, e 61,5% dos que tinham mais de 20 anos estavam vivos. Conclusão: Embora, no nosso estudo, o tempo médio entre o diagnóstico e o transplante tenha sido superior a 2 anos, e embora nossa análise tenha sido apenas estratificada pelo tipo da doença, independentemente do regime de condicionamento ou da fase da doença no momento do TCTH, nossos resultados são superponíveis aos descritos na literatura mundial.Background: Hematopoietic stem cell transplantation (HSCT) represents a curative alternative for malignant and benign hematological diseases. The benefits of the technique are especially related to an increase in the survival of patients with HLA-compatible hematopoietic stem cell donors when chemotherapy or clinical therapy has resulted ineffective. Objectives: To analyze the survival of patients submitted to allogeneic HSCT at the Hematology and Bone Marrow Transplant Service of Hospital de Clínicas de Porto Alegre. Methods: A prospective cohort of all patients submitted to transplantation between 1994 and 2003 was analyzed for overall survival. Results: A total of 133 patients were submitted to transplantation in the study period, with a mean age of 30.8±14.8 years; mean time elapsed between diagnosis and transplant was 26.8 months. Five years after the procedure, 71 patients (53.4%) were alive, 22 patients had acute and 54 had chronic myeloid leukemia, and six patients presented myelodysplastic syndrome; the 5 year overall survival was 52, 50, and 33%, respectively. Of the 26 patients transplanted for aplastic anemia, 66.7% had 20 or less years of age, and 61.5% of the patients older than 20 years were alive. Conclusion: Although the mean time elapsed between diagnosis and transplantation was over 2 years and although our results were stratified by type of disease only, the findings herein reported are similar to those found in the literature, independently of conditioning regimen or disease stage at the time of transplant

Bioactive properties and phytochemical assessment of Bacupari-anão (Garcinia brasiliensis Mart.) leaves native to Rondônia, Brazil

Leaf fractions of Garcinia brasiliensis were evaluated concerning their antioxidant, antimicrobial, anti inflammatory and cytotoxic properties, and the most active fraction was then fully characterized regarding its phenolic composition using HPLC-DAD-ESI/MSn. The ethyl acetate fraction from partitioning of the methanolic leaf extract revealed a strong antioxidant activity that was comparable to Trolox, the positive control. This fraction was also able to show a significant antimicrobial activity against Gram-positive and Gram-negative bacteria and the fungus Candida albicans. However, the dichloromethane fraction was found to present the highest anti-inflammatory (83 ± 9 µg mL−1) and cytotoxic activities, thus presenting slight toxicity using a non-tumor cell line. Regarding the phenolic profile, the ethyl acetate fraction presented twelve flavonoids, with morelloflavone-7’’-O-glucoside (52.1 ± 0.4 mg g−1) and gardinia biflavonoid 2a glucoside (27.5 ± 0.2 mg g−1) being the major compounds identified. These results indicate that leaves of G. brasiliensis might be a potential source of natural biomolecules for pharmaceutical and medicinal applications.The authors are grateful to the Foundation for Science and Technology (FCT, Portugal) and FEDER under Programme PT2020 for financial support to CIMO (UID/AGR/00690/2013) and L. Barros’s contract; to the FEDER-Interreg España- Portugal programme for financial support through the project 0377_Iberphenol_6_E.info:eu-repo/semantics/publishedVersio