Recurrent tethered cord: outcome and follow-up of 20 de-thetering for symptomatic spina bifida: choort study.

OBJECTIVE: The objective of this study was to evaluate the prevalence of tethered cord among patients in the postoperative period of open and occult spina bifida. To identify warning signs for its early diagnosis, as well as outcomes after the new surgical approach. METHODS: Retrospective study of patients followed at the Pediatric Neurosurgery Department of the Federal University of São Paulo with spinal dysraphism. Signs and symptoms indicating reoperation were collected, and postoperative results were classified as improved, unchanged, or worsened. RESULTS: 222 medical records of patients diagnosed with spinal dysraphism were evaluated. Symptomatic Tethered Cord Syndrome (STCS) was identified in 30 patients (13.51%), with clinical manifestations related to orthopedic deformities (66.7%), neurological deficits (56.7%), urological dysfunction (50%), and intestinal dysfunction (40%). 20 cases underwent surgery for tethered cord release. The mean age at the time of surgery was 7.7 ± 4.9 years, with 13 female patients (65%). In the postoperative evaluation, improvement in low back pain (90.9%), urological pattern, and urinary tract infection episodes (45.4%) were particularly noteworthy. 3 patients (33.3%) with constipation showed improvement, and one worsened (11.1%). Improvement in ambulation was seen in two cases (16.7%). Low back pain was the first symptom to improve after surgery, with an average time of 1.3 months, followed by changes in the urological pattern at 15.6 months. Improvement in constipation was observed in the first month in 2 cases (66.7%), positive changes in ambulation were observed around 7 months after surgery, and only one case showed improvement in clubfoot correction. CONCLUSIONS: The prevalence of tethered cord recurrence after primary correction surgery for open or occult neural tube closure defects was similar to that found in the literature. The results were encouraging, with good postoperative evolution of patients, especially in the improvement of low back pain and urological symptoms.

The long-term effect on functional outcome of endoscopic brainwashing for intraventricular hemorrhage compared to external ventricular drainage alone: A retrospective single-center cohort study.

Background: Intraventricular hemorrhage (IVH) is a complex condition with both mechanical and chemical effects, resulting in mortality rates of 50-80%. Recent reports advocate for neuroendoscopic treatment, particularly endoscopic brainwashing (EBW), but long-term functional outcomes remain insufficiently explored. This study aims to outline the step-by-step procedure of EBW as applied in our institution, providing results and comparing them with those of external ventricular drainage (EVD) alone. Methods: We performed a retrospective analysis of adult patients with IVH who underwent EBW and patients submitted to EVD alone at our institution. All medical records were reviewed to describe clinical and radiological characteristics. Results: Although both groups had similar baseline factors, EBW patients exhibited a larger hemoventricle (median Graeb score 25 vs. 23 in EVD, P = 0.03) and a higher prevalence of chronic kidney disease and diabetes. Short-term mortality was lower in EBW (52% and 60% at 1 and 6 months) compared to EVD (80% for both), though not statistically significant (P = 0.06). At one month, 16% of EBW patients achieved a good outcome (Modified Rankin scale < 3) versus none in the EVD group (P = 0.1). In the long term, favorable outcomes were observed in 32% of EBW patients and 11% of EVD patients (P = 0.03), with no significant difference in shunt dependency. Conclusion: Comparing EBW and EVD, patients submitted to the former treatment have the highest modified Graeb scores and, at a long-term follow-up, have better outcomes, demonstrated by the improvement of the patients in the follow-up.

Reduction in the cerebrospinal fluid protein level after bevacizumab treatment in patients with optic pathway low-grade gliomas.

Optic pathway gliomas (OPG) can cause elevated cerebrospinal fluid (CSF) protein concentrations. We report on two patients with suprasellar low-grade gliomas and high CSF protein levels (590 and 551 mg/dl) that precluded shunt implantation. After two and three doses of bevacizumab, respectively, the levels dropped dramatically to 191 and 178 mg/dl, respectively. Bevacizumab treatment was associated with a decrease in CSF protein level, allowing successful shunt placement. Our results are consistent with the pharmacological mechanism of bevacizumab, which decreases protein leakage from blood vessels to the ventricles.

Adhesive Leaf Created by a Corona Discharge.

Here, we report a new concept of both the adhesive manner and material, named "adhesive leaf (AL)," based on the leaf of the plant Heteropanax fragrans. The treatment of the corona discharge on the leaf surface can cause the nano-/microdestruction of the leaf epidermis, resulting in an outward release of sap. The glucose-containing sap provided the AL with a unique ability to stick to various substrates such as steel, polypropylene, and glass. Moreover, we reveal that the AL adhesion strength depends on the AL size, as well as the corona-discharge intensity. Conventional adhesives, such as glue and bond, lose their adhesive property and leave dirty residues upon the removal of the attached material. Unlike the conventional methods, the AL is advantageous as it can be repeatedly attached and detached thoroughly until the sap liquid is exhausted; its adhesive ability is maintained for at least three weeks at room temperature. Our findings shed light on a new concept of a biodegradable adhesive material that is created by a simple surface treatment.

Cardiac sodium channel, its mutations and their spectrum of arrhythmia phenotypes

The mechanisms of cellular excitability and propagation of electrical signals in the cardiac muscle are very important functionally and pathologically. The heart is constituted by three types of muscle: atrial, ventricular, and specialized excitatory and conducting fibers. From a physiological and pathophysiological point of view, the conformational states of the sodium channel during heart function constitute a significant aspect for the diagnosis and treatment of heart diseases. Functional states of the sodium channel (closed, open, and inactivated) and their structure help to understand the cardiac regulation processes. There are areas in the cardiac muscle with anatomical and functional differentiation that present automatism, thus subjecting the rest of the fibers to their own rhythm. The rate of these (pacemaker) areas could be altered by modifications in ions, temperature and especially, the autonomic system. Excitability is a property of the myocardium to react when stimulated. Another electrical property is conductivity, which is characterized by a conduction and activation process, where the action potential, by the all-or-nothing law, travels throughout the heart. Heart relaxation also stands out as an active process, dependent on the energetic output and on specific ion and enzymatic actions, with the role of sodium channel being outstanding in the functional process. In the gene mutation aspects that encode the rapid sodium channel (SCN5A gene), this channel is responsible for several phenotypes, such as Brugada syndrome, idiopathic ventricular fibrillation, dilated cardiomyopathy, early repolarization syndrome, familial atrial fibrillation, variant 3 of long QT syndrome, multifocal ectopic ventricular contractions originating in Purkinje arborizations, progressive cardiac conduction defect (Lenègre disease), sudden infant death syndrome, sick sinus syndrome, sudden unexplained nocturnal death syndrome, among other sodium channel alterations with clinical overlapping. Finally, it seems appropriate to consider the "sodium channel syndrome" (mutations in the gene of the alpha subunit of the sodium channel, SCN5A gene) as a single clinical entity that may manifest in a wide range of phenotypes, to thus have a better insight on these cardiac syndromes and potential outcomes for their clinical treatment.

Os mecanismos da excitabilidade celular e de propagação dos sinais elétricos no músculo cardíaco são de grande importância funcional e patológica. O coração é composto por três tipos de músculo: atrial, ventricular e das fibras especializadas excitatórias e condutoras. Do ponto de vista fisiológico e fisiopatológico os estados conformacionais do canal de sódio constitui-se um importante aspecto para o diagnóstico e tratamento de doenças cardíacas. A descrição dos estados funcionais do canal de sódio (fechado, aberto e inativado) e sua estrutura ajudam a compreensão dos processos de regulação cardíaca. Há áreas no músculo cardíaco com diferenciação anatômica e funcional que possuem automatismo submetendo as demais fibras ao seu próprio ritmo. A frequência dessas áreas (marca-passo) pode ser alterada por modificações iônicas, pela temperatura e, especialmente, do sistema autonômico. Já a excitabilidade é a propriedade que tem o miocárdio de reagir quando estimulado. A outra propriedade elétrica é a condutibilidade, que se caracteriza por um processo de condução e ativação, no qual o potencial de ação, pela ei do tudo ou nada, percorre todo o coração. Destaca-se que o relaxamento do coração também é um processo ativo, dependente de gasto energético e de ações iônicas e enzimáticas específicas, destacando o papel dos canais de sódio no processo funcional. Nos aspectos das mutações no gene que codifica o canal rápido de sódio (gene SCN5A), este é responsável por vários fenótipos, tais como a síndrome de Brugada; a fibrilação ventricular idiopática, a miocardiopatia dilatada; a síndrome de repolarização precoce; a fibrilação atrial familiar; a síndrome do QT longo variante 3; as contrações ventriculares ectópicas multifocais originadas nas arborizações de Purkinje; o distúrbio progressivo de condução intraventricular cardíaco (doença de Lenègre); a síndrome da morte súbita do recém-nascido; a síndrome do nódulo sinusal doente; a síndrome da morte súbita noturna inesperada, entre outras alterações do canal de sódio com sobreposições clínicas, as chamadas "overpping". Por fim, parece ser apropriado considerar a "síndrome do canal de sódio" (mutações no gene da subunidade alfa do canal de sódio, gene SCN5A) como uma entidade clínica única que pode manifestar-se com um amplo espectro de fenótipos e assim, prover um melhor entendimento destas síndromes cardíacas e potencial desfecho para seu tratamento clínico.

CoGA: An R Package to Identify Differentially Co-Expressed Gene Sets by Analyzing the Graph Spectra.

Gene set analysis aims to identify predefined sets of functionally related genes that are differentially expressed between two conditions. Although gene set analysis has been very successful, by incorporating biological knowledge about the gene sets and enhancing statistical power over gene-by-gene analyses, it does not take into account the correlation (association) structure among the genes. In this work, we present CoGA (Co-expression Graph Analyzer), an R package for the identification of groups of differentially associated genes between two phenotypes. The analysis is based on concepts of Information Theory applied to the spectral distributions of the gene co-expression graphs, such as the spectral entropy to measure the randomness of a graph structure and the Jensen-Shannon divergence to discriminate classes of graphs. The package also includes common measures to compare gene co-expression networks in terms of their structural properties, such as centrality, degree distribution, shortest path length, and clustering coefficient. Besides the structural analyses, CoGA also includes graphical interfaces for visual inspection of the networks, ranking of genes according to their "importance" in the network, and the standard differential expression analysis. We show by both simulation experiments and analyses of real data that the statistical tests performed by CoGA indeed control the rate of false positives and is able to identify differentially co-expressed genes that other methods failed.