Search

Abstract Background The Brazilian Policy for Comprehensive Care for People with Rare Diseases was implemented in 2014; however, national epidemiological data on rare diseases (RDs) are scarce and mainly focused on specific disorders. To address this gap, University Hospitals, Reference Services for Neonatal Screening, and Reference Services for Rare Diseases, all of which are public health institutions, established the Brazilian Rare Diseases Network (RARAS) in 2020. The objective of this study was to perform a comprehensive nationwide epidemiological investigation of individuals with RDs in Brazil. This retrospective survey collected data from patients receiving care in 34 healthcare facilities affiliated with RARAS in 2018 and 2019. Results The survey included 12,530 participants with a median age of 15.0 years, with women representing 50.5% of the cohort. Classification according to skin color demonstrated that 5044 (47.4%) participants were admixed. Most had a confirmed diagnosis (63.2%), with a predominance of phenylketonuria (PKU), cystic fibrosis (CF), and acromegaly. Common clinical manifestations included global developmental delay and seizures. The average duration of the diagnostic odyssey was 5.4 years (± 7.9 years). Among the confirmed diagnoses, 52.2% were etiological (biochemical: 42.5%; molecular: 30.9%), while 47.8% were clinical. Prenatal diagnoses accounted for 1.2%. Familial recurrence and consanguinity rates were 21.6% and 6.4%, respectively. Mainstay treatments included drug therapy (55.0%) and rehabilitation (15.6%). The Public Health System funded most diagnoses (84.2%) and treatments (86.7%). Hospitalizations were reported in 44.5% of cases, and the mortality rate was 1.5%, primarily due to motor neuron disease and CF. Conclusion This study marks a pioneering national-level data collection effort for rare diseases in Brazil, offering novel insights to advance the understanding, management, and resource allocation for RDs. It unveils an average diagnostic odyssey of 5.4 years and a higher prevalence of PKU and CF, possibly associated with the specialized services network, which included newborn screening services.

ABSTRACT Objective Determining the etiology of hyperprolactinemia is fundamental for selecting the most appropriate treatment strategy. The aim of this study was to evaluate the usefulness and accuracy of prolactin levels in predicting the etiology of nonphysiological hyperprolactinemia. Subjects and methods In this retrospective study, we reviewed medical records of patients with nonphysiological hyperprolactinemia seen at two neuroendocrine reference centers located in Recife, Brazil, from January 2000 to December 2019. Results The study included 770 patients aged 12-73 years (65% female). The three most frequent etiologies of hyperprolactinemia were prolactinomas (n = 263; 34.2%), drug-induced hyperprolactinemia (n = 160; 20.8%), and macroprolactinemia (n = 120; 15.6%). The highest mean prolactin levels were observed in cases of prolactinomas and idiopathic hyperprolactinemia. Most patients with hyperprolactinemia due to other etiologies had prolactin levels < 100 ng/mL, but these levels were also found in 16.5% of patients with microproplactinomas and in 20% of those with idiopathic hyperprolactinemia. Likewise, prolactin levels largely overlapped among patients with microprolactinomas, macroprolactinemia, and drug-induced hyperprolactinemia. Notably, prolactin levels > 250 ng/mL enabled a clear distinction between the etiologies of macroprolactinoma and nonfunctioning pituitary adenoma. Moreover, prolactin levels > 500 ng/mL were highly suggestive of macroprolactinomas, although they were also found in very few patients ( 250 ng/mL allowed a clear distinction between macroprolactinomas and nonfunctioning pituitary adenomas. Furthermore, prolactin levels > 500 ng/mL were almost exclusively found in patients with prolactinomas.

BackgroundSchizophrenia is a disorder associated with neurocognitive deficits that adversely affect daily functioning and impose an economic burden. Cognitive rehabilitation interventions, particularly during the early phases of illness, have been shown to improve cognition, functionality, and quality of life. The Feuerstein Instrumental Enrichment (FIE) program, based on the Mediated Learning Experience and the Structural Cognitive Modifiability theory, has been applied in various disorders, but its applicability in schizophrenia has not yet been clarified. ObjectiveThis study aims to investigate the effects of the FIE program on the functionality of patients with first-episode schizophrenia. MethodsIn total, 17 patients will be recruited for an open-label intervention consisting of twice-weekly sessions for 10 weeks. The primary outcome measure will be changes in the Goal Achievement Scale score. Maze task performance from the Measurement and Treatment Research to Improve Cognition in Schizophrenia (MATRICS) battery will serve as a secondary outcome measure. At the same time, changes in Positive and Negative Syndrome Scale scores and other MATRICS domains will be analyzed as exploratory outcomes. Assessments will be administered before and after the intervention, with a follow-up period of 6 months. ResultsThis trial was preregistered in The Brazilian Registry of Clinical Trials (RBR-4gzhy4s). By February 2024, 11 participants were enrolled in the training. Recruitment is expected to be completed by May 2024. Data analysis will be conducted between May and September 2024. The results are expected to be published in January 2025. ConclusionsThis study may establish a protocol for the FIE program that uses mediation techniques for individuals in the early stages of schizophrenia. The results will add to the knowledge about strategies to promote cognitive skills and functional impairment in daily life. International Registered Report Identifier (IRRID)DERR1-10.2196/57031

Leptomeningeal carcinomatosis (LC) is a rare but serious complication when cancer cells infiltrate the meninges. It is most commonly associated with breast cancer, but only 5% of breast cancer patients develop it. Leptomeningeal carcinomatosis typically presents with headaches, mainly due to hydrocephalus, and the diagnosis involves a cytological analysis of cerebrospinal fluid (CSF) and/or magnetic resonance imaging (MRI) scans. The treatment of LC consists of a combination of intra-CSF chemotherapy, systemic therapy, radiation therapy, and/or supportive care, including CSF drainage. In the case herein reported, a technique known as ventriculovesical shunting was performed on a female patient with LC and breast cancer who had hydrocephalus due to this condition. This procedure is not as common as ventriculoperitoneal shunts, which can lead, in this case, to serious complications such as peritoneal carcinomatosis.

Objective To describe the microsurgical anatomy and the physiology of the endolymphatic sac (ES) that a neurosurgeon should know.

Os objetivos principais deste artigo são a avaliação da concordância entre as informações de peso e estatura medidas e referidas, bem como a identificação das principais características de 150 gestantes que souberam ou não referir seu peso anterior, peso final e estatura. Os resultados evidenciam que as informações referidas para o peso anterior aproximam-se dos valores medidos (coeficiente de correlação de Pearson (0,96) e intraclasse (0,92); p < 0,05), o mesmo ocorrendo em relação à variável peso final (coeficiente de correlação de Pearson (0,96) e intraclasse (0,95); p < 0,05). Contudo, a análise de regressão linear é capaz de detectar que essa relação é problemática para a variável estatura (beta0 = 45,5 e beta1 = 0,71; p < 0,05) e, portanto, as informações referidas para esta devem ser utilizadas com cautela quando os valores medidos não estiverem disponíveis. Quanto à caracterização do grupo de gestantes que não informaram as três medidas (n = 141), elas são as de menor nível de instrução (63,9%), as que não fizeram ou menos freqüentaram o pré-natal (64,3%) e as que deram entrada no pré-natal mais tardiamente - já no segundo ou terceiro trimestre (59,3%).

Primary intraosseous hemangiomas are rare, benign, vascular malformations that account for ∼1% of all primary bone neoplasias. A 59-year-old female patient with unknown comorbidities had a history of headache, visual impairment and dizziness that led to the diagnosis of a clivus tumor. Two resections were attempted through transcranial and transnasal transsphenoidal approach in the last two years in another hospital. The initial MRI scan showed an expansive lesion with T2 hyperintense signal and diffuse, heterogenic contrast enhancement. Clival chordoma was the main diagnostic hypothesis done. A CT scan was performed to evaluate the extent of clival invasion, the sinus anatomy, and the clival destruction - all simulating clival chordoma. The interdisciplinary tumor board decided to proceed with endoscopic endonasal tumor resection. There were no postoperative complications and the histopathological analysis revealed a primary intraosseous haemangioma. Skull base intraosseous hemangiomas are rare entities, with a limited number of case reports found after literature reviews, especially in the clival region. The clinical pattern and imaging characteristics can vary widely according to the tumor extension and development, simulating some other common tumors found at this topography. We present a case report of a clival intraosseous hemangioma presenting as an isolated abducens paresis with a positive outcome after intranasal endoscopic resection after two years of follow-up.

Objective The present study aims to assess and compare the prognostic value of these two scales for predicting mortality.

Abstract Objectives To perform a systematic review of the literature on the anatomy of the medial meniscotibial ligaments (MTLs), and to present the most accepted findings, as well as the evolution of the anatomical knowledge on this structure. Materials and Methods An electronic search was conducted in the MEDLINE/PubMed, Google Scholar, EMBASE and Cochrane library databases with no date restrictions. The following index terms were used in the search: anatomy AND meniscotibial AND ligament AND medial. The review was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. We included anatomical studies of the knee were included, such as cadaver dissections, histological and/or biological investigations, and/or imaging of the medial MTL anatomy. Results Eight articles that met the inclusion criteria were selected. The first article was published in 1984 and the last, in 2020. The total sample in the 8 articles was of 96 patients. Most studies are purely descriptive in terms of the macroscopic morphological and microscopic histological findings. Two studies evaluated the biomechanical aspects of the MTL, and one, the anatomical correlation with the magnetic resonance imaging examination. Conclusion The main function of the medial MTL, a ligament that originates in the tibia and is inserted in the lower meniscus, is to stabilize and maintain the meniscus in its position on the tibial plateau. However, there is a limited amount of information regarding medial MTLs, primarily in terms of anatomy, especially vascularization and innervation.

Abstract Objectives To describe a series of cases of tibial fractures surgically treated using the posterior approach as described by Carlson, focusing on evaluating its functional results and complication rate. Methods Eleven patients with tibial plateau fractures, who underwent surgical treatment using the Carlson approach from July to December 2019, were followed-up. The minimum follow-up period was defined as 6 months. The American Knee Society Score (AKSS), American Knee Society Score/Function (AKSS/Function) and the Lysholm score were used to check treatment results at 6 months after the fracture. The patients underwent standard anteroposterior and lateral radiographs to assess fracture healing, and clinical healing was determined by the absence of pain during full weight-bearing. Results The mean follow-up period was 12 months (9-16 months). The primary mechanism of trauma was motorcycle accident, and the most prevalent side of fracture was the right side. Eight participants were male. The mean age of the patients was 28 years. All fractures healed, and none of the patients presented complications. The AKSS was excellent in 11 patients, with a mean AKSS/Function of 99.1±3, and Lysholm scores with a median of 95.0±5.6. Conclusions The Carlson approach for posterior fractures of the tibial plateau can be considered safe, presenting a low complication rate and satisfactory functional results.

Abstract Liver transplantation has come a long way and is now regarded as the gold standard treatment for end-stage liver failure. The great majority of livers utilized in transplantation come from brain-dead donors. A broad inflammatory response characterizes BD, resulting in multiorgan damage. This process is primarily mediated by cytokines, which increase the immunogenicity of the graft. In male Lewis rats, we evaluated the immune response in a BD liver donor and compared it to that of a control group. We studied two groups: Control and BD (rats subjected to BD by increasing intracranial pressure). After the induction of BD, there was an intense rise in blood pressure followed by a fall. There were no significant differences observed between the groups. Blood tissue and hepatic tissue analyzes showed an increase in plasma concentrations of liver enzymes (AST, ALT, LDH and ALP), in addition to pro-inflammatory cytokines and macrophages in liver tissue in animals submitted to BD. The current study found that BD is a multifaceted process that elicits both a systemic immune response and a local inflammatory response in liver tissue. Our findings strongly suggested that the immunogenicity of plasma and liver increased with time following BD.

Introduction Pathological processes in the arterial wall that result in vessel dilation are the cause of intracranial aneurysms (IAs), and the risk factors for their formation and progression are not well established. Ferritin is associated with inflammation and angiogenesis; it has protective antioxidative activity, and controls cell differentiation. Vitamin B12 is related to neurological and hematological disorders; it can be used as differential diagnosis tool, and acts in the control of homocysteinemia, a predictor of worse prognosis. The present article aims to assess the correlation between serum ferritin and B12 levels and the patient's functional outcome.

Background Aneurysmal subarachnoid hemorrhages (aSAHs) account for 5% of all strokes, an appalling number when it comes to the second most common cause of death worldwide. The basis of the treatment is clinical support and either endovascular or surgical intervention. The purpose of the present study is to analyze if the time from the onset of the thunderclap headache until treatment intervention is related to the degree of disability after 6 months.

Objective To elucidate all the aspects that neurosurgeons should know about the Ménière disease (MD). Methods Review of guidelines, books, and studies from 1933 to 2021, from basic to translational research, using human and animal endolymphatic sac (ES) tissue or cells, as well as reviews, case reports, and papers about surgical experience. This article is divided into three parts. In this last part, we review the MD. Results The MD is one of the most common pathologies in the ES. It was first described by Prosper Ménière in 1861 with its clinical triad: dizziness, tinnitus, and hearing loss. A lot of theories relating ES to the MD have been proposed. Some of them postulate that it is caused by a narrowing and shortening in the endolymphatic duct, and others relate it to severe inflammation on the ES. Mostly due to the lack of understanding of this pathology, the diagnosis is mainly clinical, despite histopathology being helpful to confirm the diagnosis. The treatment of the MD can be done in 3 different ways: pharmacological, nonpharmacological, and surgical. Conclusion The MD is one of the most common pathologies in the inner ear and has been largely studied over the years. The latest diagnosis guidelines must help in the classification and give better basis for diagnosis and treatment, which, despite not being curative yet, has improved over the years. Pharmacological treatment based on the possible etiologies, allied with proper diet and routine exercise, is showing promising results.

Objective This article is divided into three parts. In the second part of this review, the authors focus on describing the endolymphatic sac tumor and presenting illustrative cases. Methods A review of previous studies, from 1957 to 2021, from basic and translational research using human and animal endolymphatic sac (ES) tissue or cells, as well as other reviews on this theme. Results The ES is an inner ear structure, which is responsible for the homeostatic regulation, as well as endolymphatic fluid volume control, immune response etc. One of the possible alterations of the ES is the ELST, a low-grade malign neoplasm that originates from the epithelium of the endolymphatic duct and sac. The clinical presentation of the ELST includes hearing loss, tinnitus, headache, and vertigo. The diagnosis in the early stages is rare, given that this group of symptoms is very similar to other, more common, diseases such as the Meniere syndrome. Its diagnosis is made by computed tomography (CT), magnetic resonance imaging (MRI), immunohistochemistry, and confirmed by histopathology. However, none of these examinations are part of the pathological guidelines for ELST. The treatment for ELST in the early stages has a high rate of success. Conclusion The ELST is a very difficult diagnosis due to its presentation. Furthermore, the interactions between ELST and the Von Hippel-Lindau disease usually result in a more aggressive condition. Despite the difficulty of the diagnosis, doing it early increases the chances of successful treatment.

Objetivo: Identificar aspectos sociodemográficos e clínicos da infecção por COVID-19 em pacientes da lista de espera para transplante renal. Material e métodos: Estudo descritivo, observacional, transversal, realizado com 46 pacientes com COVID-19 (93,8% dos pacientes com teste positivo), acompanhados no ambulatório de transplante renal de um hospital universitário em Fortaleza – CE . A coleta foi realizada em dezembro de 2020 por prontuários e entrevista semiestruturada e análise pelo programa Statistical Package for the Social Sciences (SPSS). Resultados: A maioria apresentou hipertensão (72%) e diabetes (26%). A transmissão mais frequente foi a nosocomial (63%). O modo de diagnóstico predominante foi o RT-PCR (46%). Os sintomas foram: febre (65%), anosmia (64%), digeusia (57%), adinamia (50%), tosse seca (46%), inapetência (43%), dispneia (39%) e mialgia (39%). O monitoramento mais frequente foi o domiciliar (63%), seguido do internamento em enfermaria (30%) e internamento em UTI (7%). O tempo médio de internação foi de 9,11 dias. A maior idade se mostrou significativamente associada ao aumento de dias de internamento. Quanto ao desfecho, 93% tiveram cura e 7% foram a óbito. Conclusão: Os resultados demonstraram semelhança do perfil sociodemográfico e clínico em comparação com os dados da população em diálise. Contudo, a mortalidade encontrada foi maior que a demonstrada na população geral.

Abstract Crime is a major public problem in low- and middle-income countries (LMICs) and its preventive measures could have great social impact. The extent to which multiple modifiable risk factors among children and families influence juvenile criminal conviction in an LMIC remains unexplored; however, it is necessary to identify prevention targets. This study examined the association between 22 modifiable individual and family exposures assessed in childhood (5–14 years, n = 2511) and criminal conviction at a 7-year follow-up (13–21 years, n = 1905, 76% retention rate) in a cohort of young people in Brazil. Population attributable risk fraction (PARF) was computed for significant risk factors. Criminal convictions were reported for 81 (4.3%) youths. Although most children living in poverty did not present criminal conviction (89%), poverty at baseline was the only modifiable risk factor significantly associated with crime (OR 4.14, 99.8% CI 1.38–12.46) with a PARF of 22.5% (95% CI 5.9–36.1%). It suggests that preventing children’s exposure to poverty would reduce nearly a quarter of subsequent criminal convictions. These findings highlight the importance of poverty in criminal conviction, as it includes several deprivations and suggest that poverty eradication interventions during childhood may be crucial for reducing crime among Brazilian youth.

Objective: We report the results of a panel of Brazilian experts and provide recommendations for the management of these patients. Material and Methods: The panel convened composed by 28 local opinion leaders, addressed 59 multiple-choice questions taking into account the published scientific literature and their own clinical experience. The level of agreement among panel members was qualified as (1) consensus, when at least 75% of the voting panel members; (2) majority vote (50%-74.9%); or (3) less than majority vote. Results: There was at least majority vote for eight of 10 questions on staging and follow-up; for 14 of 23 questions on the treatment of early-stage disease; for 12 of 14 questions related to the treatment of locally-advanced disease; and for seven of the 12 questions related to the treatment of recurrent/metastatic disease. Conclusion: The current recommendations may help practitioners from Brazil and other countries to improve the care they provide to patients.

The characterization and cytotoxicity of the essential oil from Conyza bonariensis (L.) aerial parts (CBEO) were previously conducted. The major compound was (Z)-2-lachnophyllum ester (EZ), and CBEO exhibited significant ROS-dependent cytotoxicity in the melanoma cell line SK-MEL-28. Herein, we employed the Molegro Virtual Docker v.6.0.1 software to investigate the interactions between the EZ and Mitogen-Activated Protein Kinases (MAPKs), the Nuclear Factor kappa B (NF-κB), and the Protein Kinase B (PKB/AKT). Additionally, in vitro assays were performed in SK-MEL-28 cells to assess the effect of CBEO on the cell cycle, apoptosis, and these signaling pathways by flow cytometry and the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) assay using MAPKs inhibitors. CBEO induced a significant increase in the sub-G1 peak, as well as biochemical and morphological changes characteristic of apoptosis. The in-silico results indicated that EZ interacts with Extracellular Signal-Regulated Kinase 1 (ERK1), c-Jun N-terminal Kinase 1 (JNK1), p38α MAPK, NF-κB, and PKB/AKT. Moreover, CBEO modulated the ERK1/2, JNK, p38 MAPK, NF-κB, and PKB/AKT activities in SK-MEL-28 cells. Furthermore, CBEO’s cytotoxicity against SK-MEL-28 cells was significantly altered in the presence of MAPKs inhibitors. These findings support the in vitro antimelanoma effect of CBEO through apoptosis induction, and the modulation of ERK, JNK, p38 MAPK, NF-κB, and PKB/AKT activities.

BackgroundCommunicable diseases represent a huge economic burden for healthcare systems and for society. Sexually transmitted infections (STIs) are a concerning issue, especially in developing and underdeveloped countries, in which environmental factors and other determinants of health play a role in contributing to its fast spread. In light of this situation, machine learning techniques have been explored to assess the incidence of syphilis and contribute to the epidemiological surveillance in this scenario.ObjectiveThe main goal of this work is to evaluate the performance of different machine learning models on predicting undesirable outcomes of congenital syphilis in order to assist resources allocation and optimize the healthcare actions, especially in a constrained health environment.MethodWe use clinical and sociodemographic data from pregnant women that were assisted by a social program in Pernambuco, Brazil, named Mãe Coruja Pernambucana Program (PMCP). Based on a rigorous methodology, we propose six experiments using three feature selection techniques to select the most relevant attributes, pre-process and clean the data, apply hyperparameter optimization to tune the machine learning models, and train and test models to have a fair evaluation and discussion.ResultsThe AdaBoost-BODS-Expert model, an Adaptive Boosting (AdaBoost) model that used attributes selected by health experts, presented the best results in terms of evaluation metrics and acceptance by health experts from PMCP. By using this model, the results are more reliable and allows adoption on a daily usage to classify possible outcomes of congenital syphilis using clinical and sociodemographic data.

Introduction: The objective of our study is to present the first Brazilian irreversible electroporation experience in the treatment of solid cancer. Material and Methods: A retrospective study with the first ten patients who underwent percutaneous or surgical IRE to treat solid cancer between March 2021 and May 2021. Preoperative data collection included patient demographics along with previous oncologic treatments. Operative and post-operative assessment involved number of probes, number of pulses, initial and final current and 30-days complications. Results: Primary tumor was locally advanced pancreatic cancer (70%), colorectal hepatic metastasis (20%), and cholangiocarcinoma (10%). All patients had previously undergone chemotherapy with stable disease in 80% and partial response in 20% before IRE. Eighty percent of the procedures was performed surgically and 20% percutaneously CT-guided. The mean procedure time of IRE was 38 minutes. Adverse events occurred in 4 patients (40%), all being grade I-II complications.

In this second part, the authors review and suggest a methodology for studies in skull base surgery and training in microsurgical laboratory, based on their experiences and reflections. Not only are the foundations for the acquisition of microsurgical skills presented, but also what is needed to be an effective skullbase surgeon with good results. The present article reflects in particular the philosophy of professor Evandro de Oliveira and also serves to present to the neurosurgical community a new state-of-the-art laboratory for hands-on courses in Brazil, at the Faculdade Evangélica Mackenzie do Paraná.

The learning curve reflects surgeons' experience in managing several patients with the same disease. In skull base surgery, the professional's place on the curve could be related to the number of times the same procedure was performed. Where does curve begin? What amount of training is necessary prior to its application in surgical settings? What were the results of the first few skull base tumor surgeries performed by a surgeon who goes on to produce excellent results, and how is reflected in the start of their learning curve? The only way for neurosurgeons to improve their results from the start is with prior training in the microsurgery laboratory. This learning technique is essential to maximize the chance of success of a neurosurgical procedures, minimizing the morbidity rate to which patients are subjected by less experienced neurosurgeons. This article is divided in two parts, and its purpose is to show how training in the microsurgical laboratory fits into the construction of knowledge about skull base surgery, based on authors' experience and reflections. This first part discusses the technical, psychological, and philosophical aspects of medical knowledge, primarily addressing those training in skull base surgery, the principles of some selected philosophical currents, and their influence on the development of current medical knowledge.

Introduction Endoscopic spine surgery enables the minimally invasive treatment of pathologies affecting the spinal cord and roots. Herein we describe an unprecedented technique Ultrasound-Guided in-plane interlaminar lumbar endoscopic approach with a smartphone and portable light source.

Abstract Background Pneumomediastinum is a rare complication of COVID-19 pneumonia, which may or may not be associated with invasive ventilatory support. Therefore, the report and findings associated with its evolution can be of great contribution in the management of this unknown disease. Case presentation Here, we present a series of four patients with severe pneumomediastinum requiring intensive care unit. These patients developed pneumomediastinum before or during orotracheal intubation (OTI) or without OTI. The four patients were three men and one woman with a mean age of 60.5 years (48–74 years). No patients had a known history of lung disease or traumatic events, except for one patient who had a history of smoking, but who was without parenchymal disease. All intubations were performed without complications. No cases of pneumomediastinum occurred after tracheostomy, and none of the patients had tomographic or bronchoscopic evidence of tracheal injury. Although the pneumomediastinum observed in our cases was apparently not related to a violation of the aerodigestive track, this complication was associated with a worse prognosis. Conclusion Pneumomediastinum is a rare complication of COVID-19 pneumonia, and the most likely etiopathogenesis is severe pulmonary involvement, which may or may not be associated with invasive ventilatory support. Future studies with a greater number of cases should elucidate the relationship of pneumomediastinum to a probable prognostic factor.

Introduction With the COVID-19 pandemic, hospitals in low-income countries were faced with a triple challenge. First, a large number of patients required hospitalisation because of the infection’s more severe symptoms. Second, there was a lack of systematic and broad testing policies for early identification of cases. Third, there were weaknesses in the integration of information systems, which led to the need to search for available information from the hospital information systems. Accordingly, it is also important to state that relevant aspects of COVID-19’s natural history had not yet been fully clarified. The aim of this research protocol is to present the strategies of a Brazilian network of hospitals to perform systematised data collection on COVID-19 through the WHO platform.Methods and analysis This is a multicentre project among Brazilian hospitals to provide data on COVID-19 through the WHO global platform, which integrates patient care information from different countries. From October 2020 to March 2021, a committee worked on defining a flowchart for this platform, specifying the variables of interest, data extraction standardisation and analysis.Ethics and dissemination This protocol was approved by the Research Ethics Committee (CEP) of the Research Coordinating Center of Brazil (CEP of the Hospital Nossa Senhora da Conceicao), on 29 January 2021, under approval No. 4.515.519 and by the National Research Ethics Commission (CONEP), on 5 February 2021, under approval No. 4.526.456. The project results will be explained in WHO reports and published in international peer-reviewed journals, and summaries will be provided to the funders of the study.

O presente artigo objetiva analisar a importância do patrimônio arquitetônico para a compreensão da evolução histórico-geográfica dos lugares e dos grupos sociais que os habitam. O trabalho também apresenta como objetivos específicos: entender a função do conjunto patrimonial histórico arquitetônico para a construção da memória social sobre a cidade de Fortaleza; discutir a relevância das formas de proteção e salvaguarda do patrimônio histórico arquitetônico; apresentar o Palacete do Ceará, relevante patrimônio histórico arquitetônico e sua relação com a memória e a identidade socioespacial fortalezense e entender a visão da sociedade local, quanto aos patrimônios arquitetônicos. O percurso metodológico qualitativo, baseia-se em uma revisão de literatura especializada e a realização de questionário online via google formulário. Os resultados da pesquisa evidenciam que o patrimônio promove e fortalece a identidade de um povo assim como o senso de pertencimento ao lugar habitado. Percebe-se também, que a sociedade fortalezense reconhece a importância da preservação patrimonial e demonstra interesse em conhecer a história desses patrimônios.

This review presents the main cell characteristics altered after in vitro incubation of the parasite with commercial drugs used to treat the disease caused by Giardia intestinalis. This important intestinal parasite primarily causes diarrhea in children. Metronidazole and albendazole are the primary compounds used in therapy against Giardia intestinalis. However, they provoke significant side effects, and some strains have developed resistance to metronidazole. Benzimidazole carbamates, such as albendazole and mebendazole, have shown the best activity against Giardia. Despite their in vitro efficacy, clinical treatment with benzimidazoles has yielded conflicting results, demonstrating lower cure rates. Recently, nitazoxanide has been suggested as an alternative to these drugs. Therefore, to enhance the quality of chemotherapy against this parasite, it is important to invest in developing other compounds that can interfere with key steps of metabolic pathways or cell structures and organelles. For example, Giardia exhibits a unique cell structure called the ventral disc, which is crucial for host adhesion and pathogenicity. Thus, drugs that can disrupt the adhesion process hold promise for future therapy against Giardia. Additionally, this review discusses new drugs and strategies that can be employed, as well as suggestions for developing novel drugs to control the infection caused by this parasite.

ABSTRACT Objective: The COVID-19 pandemic has profoundly disrupted health care worldwide. We aimed to evaluate the impact of the first COVID-19 wave on the treatment of our patients with acromegaly. Subjects and methods: A standard questionnaire was systematically applied to all patients and included questions on general health status, whether all laboratory workup had been done, common signs and symptoms of acromegaly, treatment adherence, and previous COVID-19 symptoms and diagnosis. Results: We attempted to contact 136 patients with acromegaly at regular follow-up at our institution and contacted 101 of them successfully. In all, 37% of the patients reported symptoms of acromegaly, which was more common among women. A total of 27 patients were lost to follow-up (including 19 who interrupted treatment during the pandemic) mainly for fear of becoming infected by the SARS-CoV-2. Of these, 24 resumed follow-up after our contact. Conclusions: The current COVID-19 pandemic has strongly impacted the follow-up of patients with acromegaly. Telemedicine can be an important tool to maintain regular treatment in the current or future pandemics.

Introduction The purpose of this study was to define the anatomical relationships of the pterygopalatine fossa (PPF) and its operative implications in skull base surgical approaches. Methods Ten cadaveric heads were dissected at the Dianne and M Gazi Yasargil Educational Center MicrosurgicaLaboratory, in Little Rock, AK, USA. The PPF was exposed through an extended dissection with mandible and pterygoid plate removal. Results The PPF has the shape of an inverted cone. Its boundaries are the pterygomaxillary fissure; the maxilla, anteriorly; the medial plate of the pterygoid process, and greater wing of the sphenoid process, posteriorly; the palatine bone, medially; and the body of the sphenoid process, superiorly. Its contents are the maxillary division of the trigeminal nerve and its branches; the pterygopalatine ganglion; the pterygopalatine portion of the maxillary artery (MA) and its branches; and the venous network. Differential diagnosis of PPF masses includes perineural tumoral extension along the maxillary nerve, schwannomas, neurofibromas, angiofibromas, hemangiomas, and ectopic salivary gland tissue. Transmaxillary and transpalatal approaches require extensive resection of bony structures and are narrow in the deeper part of the approach, impairing the surgical vision and maneuverability. Endoscopic surgery solves this problem, bringing the light source to the center of the surgical field, allowing proper visualization of the surgical field, extreme close-ups, and different view angles. Conclusion We provide detailed information on the fossa's boundaries, intercommunications with adjacent structures, anatomy of the maxillary artery, and its variations. It is discussed in the context of clinical affections and surgical approaches of this specific region, including pterygomaxillary disjunction and skull base tumors.

Chikungunya (CHIK) is a re-emerging viral infection endemic in tropical and subtropical areas. While the typical clinical presentation is an acute febrile syndrome, long-term articular complications and even death can occur. This review characterizes the global epidemiological and economic burden of chikungunya. The search included studies published from 2007 to 2022 in MEDLINE, Embase, LILACS, and SciELO for a thorough evaluation of the literature. Rayyan software was used for data analysis, and data were summarized descriptively and reported following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Seventy-six publications were included. Chikungunya is widely distributed in the tropics, including Africa, Asia, South America, and Oceania/the Pacific Islands, and co-circulates with other simultaneous arboviruses such as DENV, ZIKV, and YFV. Chikungunya infection can lead to chronic articular manifestations with a significant impact on the quality of life in the long term. In addition, it generates absenteeism and economic and social losses and can cause fatal infections in vulnerable populations, mainly in high-risk patients with co-morbidities and at the extremes of age. Reported costs associated with CHIKV diseases are substantial and vary by region, age group, and public/private delivery of healthcare services. The chikungunya disease burden includes chronicity, severe infections, increased hospitalization risks, and associated mortality. The disease can impact the economy in several spheres, significantly affecting the health system and national economies. Understanding and measuring the full impact of this re-emerging disease is essential.

Schwannomas são tumores que se originam das células de Schawnn. Normalmente se localizam na cabeça, pescoço e membros, mas podem ocorrer em qualquer parte do corpo onde existam nervos mielinizados. O acometimento renal é raro, com poucos casos descritos na literatura. Seu diagnóstico pré-operatório é difícil, visto que se baseiam apenas numa apresentação clínica inespecífica e em características radiológicas limitadas. Embora alguns achados na Ressonância Nuclear Magnética (RNM) possam levantar a suspeição do diagnóstico, esses tumores são frequentemente confundidos com Carcinoma de Células Renais, tendo a ressecção cirúrgica como tratamento padrão. Comumente requerem um diagnóstico patológico. E apesar da maior parte dos casos exibir comportamento benigno, a degeneração maligna pode ocorrer. Nós trazemos o relato de um caso de tumor no hilo renal direito, submetido a ressecção cirúrgica, cujo diagnóstico de Schwannoma renal foi confirmado histologicamente.

The diagnosis of endometriosis can be made with some security when they exhibit typical aspects, using the various imaging methods. Ultrasonography is usually a cheap and accessible method that has high sensitivity and specificity in the diagnosis of endometriotic lesions. This article aims to describe the main findings in ultrasound of pelvic endometriosis with a focus on extra‑uterine involvement.

The Virchow-Robin spaces (VRSs), which are often incidentally observed in modern structural neuroimaging examinations, are small cystic cavities that usually surround the small arteries and arterioles at the level of basal ganglia, the anterior perforated substance and the thalamic-mesencephalic junction. Typically, they have similar physicochemical characteristics to cerebral spinal fluid (CSF) and there is no contrast enhancement on brain CT and MRI images. Its real meaning is unknown, although some contemporary studies have suggested that it might be related to certain traumatic brain injury or several other central nervous system (CNS) disorders, as degenerative diseases. Occasionally, some wide and atypical VRS may be mistaken for primary cystic brain tumors, especially in the context of large and symptomatic lesions, multiple clustered cysts, cortical lesions and if there is adjacent reactive gliosis. The present paper reports four patients who were affected by atypical VRS mimicking brain tumors that required imaging follow-up or even a biopsy to confirm the diagnosis or to indicate the correct approach. Although it is not so unusual, one of them occurred concomitantly and adjacent to a diffuse glioma (co-deleted 1p19q, WHO-GII).

Objetivo: analisar os entraves durante o percurso acadêmico no curso de Enfermagem acerca da utilização das tecnologias educativas. Métodos: pesquisa descritiva com abordagem qualitativa. Os dados foram coletados por meio de entrevistas semiestruturadas com discentes de enfermagem no período de novembro de 2016 a janeiro de 2017, sendo os depoimentos analisados através da análise de discurso. Resultados: os discentes discursaram como entraves o fato dos docentes permanecerem fincados no método tradicional de ensino; a falta de interesse em levar essas tecnologias para o ambiente de ensino, além da formação dos próprios educadores serem focadas em um ensino com metodologias tradicionais. Conclusão: surge a necessidade de discutir sobre o papel da gestão e a formação pedagógica necessária para a efetividade da docência em saúde, que, mesmo com todas as diretrizes norteadoras e reestruturadoras desse ensino, não garantem a formação do profissional com o perfil pretendido.

The present report describes the case of a male 17-year-old patient who progressively developed a hydrocephalus and polyradiculopathy due to involvement of central nervous system (CNS) by a diffuse leptomeningeal glioneuronal tumor (DLGNT). The tumor had partial remission in response to the treatment with radiotherapy plus procarbazine, lomustine, and vincristine (PCV) chemotherapy, and the patient had improvement in function and pain levels. The current knowledge about DLGNT, including its clinical manifestations, imaging findings, histological characteristics, and treatment are revised and discussed in the present paper.

Objective To describe the endoscopic and microsurgical anatomy of the cavernous sinus (CS) with focus on the surgical landmarks in microsurgical anatomy. Materials and methods Ten formalin-fixed central skull base specimens (20 CSs) with silicone-injected carotid arteries were examined through an extended endoscopic transsphenoidal approach. Fifteen formalin-fixed heads were dissected to simulate the surgical position in CS approaches. Results Endoscopic access enables identification of the anterior and posterior surgical corridors. Structures within the CS and on its lateral wall could be visualized and studied, but none of the triangular areas relevant to the transcranial microsurgical anatomy were fully visible through the endoscopic approach. Conclusion The endoscopic approach to the CS is an important surgical technique for the treatment of pathological conditions that affect this region. Correlating endoscopic findings with the conventional (transcranial) microsurgical anatomy is a useful way of applying the established knowledge into a more recent operative technique. Endoscope can provide access to the CS and to the structures it harbors.

The outbreak of COVID-19 has led to there being a worldwide socio-economic crisis, with major impacts on developing countries. Understanding the dynamics of the disease and its driving factors, on a small spatial scale, might support strategies to control infections. This paper explores the impact of the COVID-19 on neighborhoods of Recife, Brazil, for which we examine a set of drivers that combines socio-economic factors and the presence of non-stop services. A three-stage methodology was conducted by conducting a statistical and spatial analysis, including clusters and regression models. COVID-19 data were investigated concerning ten dates between April and July 2020. Hotspots of the most affected regions and their determinant effects were highlighted. We have identified that clusters of confirmed cases were carried from a well-developed neighborhood to socially deprived areas, along with the emergence of hotspots of the case-fatality rate. The influence of age-groups, income, level of education, and the access to essential services on the spread of COVID-19 was also verified. The recognition of variables that influence the spatial spread of the disease becomes vital for pinpointing the most vulnerable areas. Consequently, specific prevention actions can be developed for these places, especially in heterogeneous cities.

Background High levels of homocysteine (Hct) have been associated with great risks of ischemic stroke. However, some controversy still exists. We performed a systematic review and meta-analysis to compare the levels of Hct between patients with ischemic stroke and controls. Methods We performed a systematic literature search for articles reporting Hct levels of patients with occurrence of ischemic stroke. We employed a random-effects inverse-variance weighted meta-analytical approach in order to pool standardized mean differences, with estimation of τ2 through the DerSimonian-Laird method. Results The initial search yielded 1361 studies. After careful analysis of abstracts and full texts, the meta-analysis included data from 38 studies, which involved almost 16 000 stroke events. However, only 13 studies reported means and standard deviations for cases and controls, and therefore were used in the meta-analysis. Those studies presented data from 5002 patients with stroke and 4945 controls. Standardized mean difference was 1.67 (95% CI 1.00–2.25, P < 0.01), indicating that Hct levels were significantly larger in patients with ischemic stroke compared to controls. Between-study heterogeneity was very large (I2 = 99%), particularly because three studies showed significantly large mean differences. Conclusion This meta-analysis shows that patients with ischemic stroke have higher levels of Hct compared to controls. Whether this is a modifiable risk factor remains to be assessed through larger prospective cohorts.

Introduction: Ovarian cancer (OC) is one of the leading causes of women’s cancer deaths worldwide. Recent clinical trials with PARP inhibitors showed promising therapeutic opportunities for OC patients. The assessment of BRCA mutation is well established as relevant in the prevention, early diagnostic, and family counseling for OC, and recently BRCA gene mutation was associated as a prognosis for PARP inhibitors treatment. In this scenario, the assessment of the patient’s mutation is proposed on Brazilian oncology guidelines and should be advised by health professionals that treat OC. Objectives: Inquire Brazilian oncologists about BRCA gene testing requesting time in the clinical practice for OC patients. Material and Methods: From May 2018 to June 2019, approximately 400 Brazilian oncologists received an online survey with questions related to the indication and challenges of BRCA gene testing. The survey was sent in 4 periods (waves); each wave received approximately 100 answers. Results: The compiled information showed that, on average, each oncologist treated 3 to 5 patients with ovarian cancer, they would recommend testing for three patients. Most respondents would indicate, BRCA testing during patients initial diagnostic period (w1=44%, w2=50%, w3=58%, and w4=64%). The sample of choice for testing would be blood/saliva assessing the germline mutational status (w1=35%, w2=43%, w3=46%, and w4=47%). The main reasons for oncologists to refrain from recommending BRCA testing were associated with cost and lack of reimbursement followed by lack of genetic counselors, among other factors. Conclusion: BRCA testing is restricted and not recommended for all ovarian cancer patients from the private health care sector. There is a lack of consensus on testing recommendations and discrepancies between coverage and national guidelines standardizing. There main difficulties associated with refraining testing were related to reimbursement and health plan coverage. Besides, the lack of genetic counseling was also pointed to as a bottleneck on oncologic patients’ multidisciplinary treatment.

Introduction Hemangioblastomas of the pineal region or pituitary stalk are extremely rare. Only two cases of hemangioblastomas involving the pineal region have been reported, and four involving the pituitary stalk. The purpose of the present manuscript is to describe an unusual case of supposed hemangioblastoma found concomitantly in the pineal region and pituitary stalk of a patient diagnosed with Von Hippel-Lindau (VHL) disease. Case Report A 35-year-old female patient with a previous diagnosis of VHL complaining of occipital headaches and balance disturbances for three weeks, who previously had a cerebellar hemangioblastoma resected. The visual characteristics of the tumor suggested a friable vascular lesion with a reddish-brown surface, and an incisional biopsy was performed. The tumor consisted of a dense vascular network surrounded by fibrous stroma abundant in reticulin and composed by both fusiform and dispersed xanthomatous cells; the immunohistochemistry was immunopositive for neuron-specific enolase and immunonegative for epithelial membranous antigen. The patient has been monitored closely for 2 years, and the supratentorial masses have not presented any volume alteration. Conclusion This rare association must be taken into account in patients with VHL disease, or at least be suspected in patients who present a thickening of the pituitary stalk and a pineal-region mass. We believe a biopsy of our asymptomatic patient could have been dangerous due to inherent complications like intraoperative bleeding. We recommend close observation of asymptomatic lesions with MRIs every six months or until the lesions become symptomatic. If the pineal-region tumor does become symptomatic, gross resection via a transcallosal approach would be ideal.

Objetivo: analisar os entraves durante o percurso acadêmico no curso de Enfermagem acerca da utilização das tecnologias educativas. Métodos: pesquisa descritiva com abordagem qualitativa. Os dados foram coletados por meio de entrevistas semiestruturadas com discentes de enfermagem no período de novembro de 2016 a janeiro de 2017, sendo os depoimentos analisados através da análise de discurso. Resultados: os discentes discursaram como entraves o fato dos docentes permanecerem fincados no método tradicional de ensino; a falta de interesse em levar essas tecnologias para o ambiente de ensino, além da formação dos próprios educadores serem focadas em um ensino com metodologias tradicionais. Conclusão: surge a necessidade de discutir sobre o papel da gestão e a formação pedagógica necessária para a efetividade da docência em saúde, que, mesmo com todas as diretrizes norteadoras e reestruturadoras desse ensino, não garantem a formação do profissional com o perfil pretendido.

Stroke is the third most common cause of death worldwide. About 10% to 15% of strokes related to the territory of the carotid artery are associated with its complete occlusion. There is an important subgroup of patients with cerebrovascular occlusive diseases who might benefit from an external-carotid-to-internal-carotid bypass. In the present study, we report a case of a 53-year-old male patient with stenosis of the M2 branch of the middle cerebral artery (MCA), with a history of ∼ 20 episodes of transient ischemic accidents (TIA)s, in whom an anastomosis of the M4 branch of the superficial temporal artery-MCA was performed. The patient was discharged in three days, and in the two years of follow-up, they were no more TIAs. We also conducted a review of the literature on cerebrovascular occlusive disease and extracranial-intracranial bypass surgery. New methods to evaluate cerebral hemodynamics made it possible to classify a new subgroup of patients with symptomatic cerebrovascular disease and documented cerebrovascular compromise in whom the drug therapy fails, who can benefit from the extracranial-intracranial bypass. Our case report illustrates the advantages of revascularization in these selected patients.

Introduction Parkinson’s disease (PD) is the second most common neurodegenerative disorder in Brazil. Physical activity is a complementary intervention in managing inherent declines associated with the disease like strength, balance, gait, and functionality and benefit health-related outcomes. Here, we report the PARK-BAND Study protocol, which aims to investigate potential benefits of power training using elastic devices in participants with PD. Our intervention will be provided in patients with PD using elastic devices like elastic bands and tubes. Therefore, we used the term Park from Parkinson’s disease and band from elastic bands.Methods and analysis This randomised single-blind single-centre two-arm parallel, superiority trial will include 50 participants with PD attending the clinical setting. Those who meet the eligibility criteria and provide consent to participate will be randomised in a 1:1 ratio to either the exercise group, which will receive power training programme or the health education group, which will receive the education programme. Randomisation will be performed by permuted block randomisation with a block size of eight. Both groups will receive a 12-week intervention. The exercise group will have two sessions per week and the health education group will have one session per week. Changes from baseline in bradykinesia, as assessed by the Unified Parkinson's Disease Rating Scale motor examination subscore and physical functional performance, will be the primary outcomes. Secondary outcomes include other neurological, neurophysiological and physical variables, as well as the quality of life, depression, cognition, sleep quality and disturbances, assessed before and after interventions. We hypothesise that the exercise group will have greater improvement in primary and secondary outcomes than the health education group.Ethics and dissemination The study is approved by the Research Ethics Committee of Hospital Universitário Walter Cantidio and all participants will provide their written informed consent (register number 91075318.1.0000.5045).Trial results will be disseminated via peer reviewed journal articles and conference presentations, reports for organisations involved with PD and for participants.Trial registration number Registro Brasileiro de Ensaios Clínicos Registry (RBR-5w2sqt); Pre-results.

O abastecimento público com água de qualidade é uma medida essencial à saúde, uma vez que a água pode atuar como veículo de agentes patogênicos oriundos de fezes humanas e animais. Uma das formas de identificação de tais contaminações se dá a partir do monitoramento da presença de Escherichia coli que é utilizada como padrão microbiológico preconizado pela Portaria 2.914/2011 do Ministério da Saúde, este estabelece como própria para consumo humano a água ausente de Escherichia coli em 100 mL da amostra. Desta maneira o objetivo deste estudo foi avaliar a qualidade microbiológica de água subterrânea proveniente de poços da Região Metropolitana de Fortaleza–CE. Para tanto foram analisadas 44 amostras oriundas das cidades de Fortaleza, Aquiraz, Horizonte, São Gonçalo do Amarante, Maracanaú, Pindoretama, Eusébio e Caucaia–CE. As amostras coletadas foram analisadas por profissionais do laboratório da Superintendência Estadual do Meio Ambiente – SEMACE, que adotaram a Técnica dos Tubos Múltiplos com meio de cultura A-1. Das 44 amostras analisadas 40,9% foram positivas para Escherichia coli, estando assim em desacordo com o padrão estabelecido pela legislação nacional em vigor. O consumo de água fora do padrão de potabilidade põe em risco a saúde da população, desta forma os resultados obtidos alertam para a necessidade de monitoramento das águas subterrâneas da Região Metropolitana de Fortaleza–CE, como medida preventiva contra doenças de veiculação hídrica.

The mortality rates of subarachnoid hemorrhage (SAH) average 51%, and survivors frequently experience acute and long-term neurological conditions, including seizures and epilepsy. The incidence of post SAH-related seizures and epilepsy, its risk factors, outcomes and management are controversial. The present paper aims to discuss these aspects, to provide the pros and cons of different management options of this condition. A review on PubMed was performed encompassing the incidence of seizures and epilepsy following SAH, the risk factors for its occurrence, its related outcomes, and treatment. The incidence of seizures and epilepsy following SAH varies widely in the literature (from 6 to 26%). Some possible risk factors were identified, such as middle cerebral artery (MCA) aneurysm, Hunt and Hess grade III, aneurysm clipping, thick subarachnoid clot, intracerebral hemorrhage, rebleeding, ischemic brain infarction, Fisher grade III or IV, acute hydrocephalus, hypertension history and poor World Federation of Neurosurgical Societies (WFNS) grade. Nevertheless, these relations are frequently uncertain. Also, it appears that the outcome of patients who suffered SAH is worsened by seizures. Given these uncertainties, the need for antiepileptic drug (AED) prophylaxis, the choice of the best drug and dose, as well as the duration of the treatment are controversial topics. However, some recommendations based on low quality evidence are reasonable to be adopted. These include considering AED prophylaxis when a seizure occur after SAH, considering routine long-term AED prophylaxis in specific populations, considering electroencephalogram (EEG) monitoring, and avoiding phenytoin prescription. That is, an individualized approach appears to be the best option, since there is no high-quality evidence.

Papillary tumor of the pineal region (PTPR) is a neuroectodermal tumor thought to originate from cells of the subcommissural organ. Its oncologic properties are still under investigation, as well as the most suitable therapeutic measures for this type of neoplasm. We report the case of a 36-year-old woman with a 1-year history of headache and intermittent diplopia. The magnetic resonance imaging (MRI) scan showed a heterogeneously enhancing mass in the pineal region that caused an acute hydrocephalus, and an emergency shunt derivation was necessary. One week later, the patient was submitted to subtotal tumor resection, and remained asymptomatic in the post-operative period. In the follow-up, the patient remained asymptomatic; in the imaging control 3.5 years after the surgical resection, local recurrence was identified, and the patient was submitted to a local radiation protocol. Our literature review showed an early clinical onset due to intracranial hypertension signs. Definitive clinical onset might be reached only through a histopathological examination. Gross total resection followed by radiotherapy is the current standard of care. Local recurrence is often observed, with rare dissemination to the cerebral spinal fluid. The natural history of the PTPR remains unknown, as well as the best treatment strategy. Large case series with longer follow-ups are necessary for further conclusions.

The brain represents ∼ 2% of the adult body mass; conversely, it is responsible for 20% to 25% of the glucose and 20% of the oxygen consumption, receiving 15% of the cardiac output. This substantial metabolic rate is associated with a significant production of biological debris, which is potentially toxic. Therefore, a complex and efficient clearance system is required to prevent the accumulation of byproducts and ensure optimal function. However, until today, there is little knowledge about this topic. The glymphatic system, also known as perivascular pathway, is a recently described glial-dependent network that is responsible for the clearance of metabolites from the central nervous system (CNS), playing a role equivalent to the one played by the lymphatic vessels present in other organs. Studies have demonstrated that the glymphatic pathway has a paramount role in protein homeostasis, and that the malfunction of this system may be related to the development of neurodegenerative disorders such as Alzheimer disease and normal pressure hydrocephalus. They also showed that body posture, exercise and the state of consciousness influence the glymphatic transport. In this context, the understanding of this clearance system could not only clarify the pathophysiology of several diseases, but also contribute to future therapeutic interventions. In the present article, we will evaluate the glymphatic pathway, focusing on the factors that regulate its flow, as well as on its role in CNS physiology and in disease initiation and progression, including dementia, hydrocephalus, glaucoma and traumatic brain injury. Ultimately, this review also aims to encourage further research on novel therapeutic targets.

Langerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system, characterized by the aberrant proliferation of specific dendritic cells. The clinical presentation ranges from a single bone lesion to widespread multiorgan involvement. This disease is usually considered to be a disease of childhood; however, the diagnosis is frequently made in adulthood. The course of the disease is fairly unpredictable and varies from spontaneous resolution to progress into a debilitating form, which compromises the vital functions with occasional fatal consequences. Langerhans cell histiocytosis exhibits a predilection for the hypothalamic-pituitary-axis, with diabetes insipidus being the most common endocrine consequence related to the disease, which may be prior to diagnosis or develop at any time during the course of the disease. The diagnosis of LCH should be based on histologic and immunophenotypic examination of a lesional biopsy, although other testing may be done, depending on the symptoms. There is no established, widely agreed-upon treatment of LCH, in general. The treatment depends upon the individual patient and the extent and areas of involvement. The present article aims to describe the case of a 26-year-old male patient whose symptoms started with a headache and occipital bone lesion that progressed later with diabetes insipidus.

The pilomyxoid astrocytoma (PMA) is a rare glioma recently described as a separate entity, which is generally located on the hypothalamic area. The PMA was previously described as pilocytic astrocytoma (PA) due to similarities shared between them. Recent studies provided a deeper understanding of PMA, setting it as a separate entity, though PMA is still considered by many authors a variant of PA. The PMA is considered to be more aggressive than PA; however, further studies are necessary for a better comprehension of its behavior and, hence, for neurosurgeons and neurologists to get to a consensus about its management. This study presents a 16-year-old female patient who looked for medical assistance complaining of headaches of over 6 months and vomiting for 2 weeks prior to the visit to the doctor. She presented no other symptoms. The physical examination displayed only bilateral papilledema. The magnetic resonance imaging (MRI) scans showed an intraventricular and thalamic lesion composed of solid and cystic material associated with peritumoral edema. The patient underwent ventriculoperitoneal shunt and subtotal resection of the lesion. The histological and immunohistochemical studies showed typical features of PMA. The patient started adjuvant therapy with chemotherapy and radiosurgery. She has been asymptomatic for 9 months and has shown no signs of progression of the disease on the follow-up scans.