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2,330,800
Elevated Aortic Pulse Wave Velocity Relates to Longitudinal Gray and White Matter Changes.
To determine whether baseline aortic stiffness, measured by aortic pulse wave velocity (PWV), relates to longitudinal cerebral gray or white matter changes among older adults. Baseline cardiac magnetic resonance imaging will be used to assess aortic PWV while brain magnetic resonance imaging will be used to assess gray matter and white matter hyperintensity (WMH) volumes at baseline, 18 months, 3 years, 5 years, and 7 years. Approach and Results: Aortic PWV (m/s) was quantified from cardiac magnetic resonance. Multimodal 3T brain magnetic resonance imaging included T</i>1</i></sub>-weighted imaging for quantifying gray matter volumes and T</i>2</i></sub>-weighted fluid-attenuated inversion recovery imaging for quantifying WMHs. Mixed-effects regression models related baseline aortic PWV to longitudinal gray matter volumes (total, frontal, parietal, temporal, occipital, hippocampal, and inferior lateral ventricle) and WMH volumes (total, frontal, parietal, temporal, and occipital) adjusting for age, sex, race/ethnicity, education, cognitive diagnosis, Framingham stroke risk profile, APOE</i> (apolipoprotein E)-&#x3b5;4 carrier status, and intracranial volume. Two hundred seventy-eight participants (73&#xb1;7 years, 58% male, 87% self-identified as non-Hispanic White, 159 with normal cognition, and 119 with mild cognitive impairment) from the Vanderbilt Memory &amp; Aging Project (n=335) were followed on average for 4.9&#xb1;1.6 years with PWV measurements occurring from September 2012 to November 2014 and longitudinal brain magnetic resonance imaging measurements occurring from September 2012 to June 2021. Higher baseline aortic PWV was related to greater decrease in hippocampal (&#x3b2;=-3.6 [mm3</sup>/y]/[m/s]; [95% CI, -7.2 to -0.02] P</i>=0.049) and occipital lobe (&#x3b2;=-34.2 [mm3</sup>/y]/[m/s]; [95% CI, -67.8 to -0.55] P</i>=0.046) gray matter volume over time. Higher baseline aortic PWV was related to greater increase in WMH volume over time in the temporal lobe (&#x3b2;=17.0 [mm3</sup>/y]/[m/s]; [95% CI, 7.2-26.9] P</i>&lt;0.001). All associations may be driven by outliers.</AbstractText>In older adults, higher baseline aortic PWV related to greater decrease in gray matter volume and greater increase in WMHs over time. Because of unmet cerebral metabolic demands and microvascular remodeling, arterial stiffening may preferentially affect certain highly active brain regions like the temporal lobes. These same regions are affected early in the course of Alzheimer disease.</AbstractText>
2,330,801
Enhanced 3D visualization for planning biventricular repair of double outlet right ventricle: a pilot study on the advantages of virtual reality.
We aim to determine any additional benefit of virtual reality (VR) experience if compared to conventional cross-sectional imaging and standard three-dimensional (3D) modelling when deciding on surgical strategy in patients with complex double outlet right ventricle (DORV).</AbstractText>We retrospectively selected 10 consecutive patients with DORV and complex interventricular communications, who underwent biventricular repair. An arterial switch operation (ASO) was part of the repair in three of those. Computed tomography (CT) or cardiac magnetic resonance imaging images were used to reconstruct patient-specific 3D anatomies, which were then presented using different visualization modalities: 3D pdf, 3D printed models, and VR models. Two experienced paediatric cardiac surgeons, blinded to repair performed, reviewed each case evaluating the suitability of repair following assessment of each visualization modalities. In addition, they had to identify those who had ASO as part of the procedure. Answers of the two surgeons were compared to the actual operations performed. There was no mortality during the follow-up (mean&#x2009;=&#x2009;2.5&#x2009;years). Two patients required reoperations. After review of CT/cardiac magnetic resonance images, the evaluators identified the surgical strategy in accordance with the actual surgical plan in 75% of the cases. When using 3D pdf this reached only 70%. Accordance improved to 85% after revision of 3D printed models and to 95% after VR. Use of 3D printed models and VR facilitated the identification of patients who required ASO.</AbstractText>Virtual reality can enhance understanding of suitability for biventricular repair in patients with complex DORV if compared to cross-sectional images and other 3D modelling techniques.</AbstractText>&#xa9; The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.</CopyrightInformation>
2,330,802
Chemorefractory extranodal nasal-type natural-killer/T-cell lymphoma with great response to pembrolizumab in a young patient: a case report.
Extranodal, natural-killer/T-cell lymphoma of nasal type is a rare but aggressive disease usually presenting as progressive necrotic lesions in the nasal cavity that responds poorly to chemotherapy. In this paper, we report a relapsing, chemorefractory case of extranodal natural-killer/T-cell lymphoma responding to checkpoint inhibitor immunotherapy with pembrolizumab.</AbstractText>A 32-year-old Hispanic woman with a history of recurrent sinusitis and preseptal abscess presented with a hoarse voice, swelling around the right eye, and diplopia. Laryngoscopy showed infiltrating tissue extending to bilateral laryngeal ventricles and false vocal cords. On immunohistochemical examination of laryngeal biopsy, the neoplastic cells showed positivity for CD3 (cytoplasmic), CD7, CD56, granzyme B, CD30, and Epstein-Barr virus-encoded ribonucleic acid (RNA). Extranodal natural-killer/T-cell lymphoma, nasal type, was confirmed. In the absence of distant organ involvement, the decision was to perform chemotherapy with etoposide, ifosfamide, mesna, cisplatin, and dexamethasone (VIPD protocol) followed by concurrent chemoradiation with weekly doses of cisplatin and two cycles of VIPD as adjuvant treatment. However, 1&#xa0;month after completion of the treatment; disease recurrence was demonstrated. The patient was scheduled to receive salvage chemotherapy with steroid, methotrexate, ifosfamide, L- asparaginase, and etoposide (SMILE) protocol and CD30-targeting monoclonal antibodies. However, the mass was chemorefractory without response to either L-asparaginase-based salvage chemotherapy in combination with high-dose methotrexate or brentuximab vedotin. However, this case of chemorefractory extranodal natural-killer/T-cell lymphoma, nasal type, responded well to the novel drug pembrolizumab, which was able to control the disease.</AbstractText>Checkpoint inhibitors are potential treatment option in selected chemorefractory extranodal natural-killer/T-cell lymphoma, nasal type, cases.</AbstractText>&#xa9; 2021. The Author(s).</CopyrightInformation>
2,330,803
MVSGAN: Spatial-Aware Multi-View CMR Fusion for Accurate 3D Left Ventricular Myocardium Segmentation.
The accurate 3D left ventricular (LV) myocardium segmentation in short-axis (SAX) view of cardiac magnetic resonance (CMR) is challenged by the sparse spatial structure of CMR. The strategy of multi-view CMR fusion can provide fine-grained spatial structure for accurate segmentation. However, the large information misalignment and lack of dense 3D CMR as fusion target in multi-view CMR fusion, and the different spatial resolution between the fusion result and the ground truth in segmentation limit the strategy. In this study, we propose a multi-view spatial-aware adversarial network (MVSGAN). It studies the perception of fine-grained cardiac structure for accurate segmentation by the spatialaware multi-view CMR fusion. It consists of three modules: (1) A residual adversarial fusion (RAF) module takes inter-slices deep correlation and anatomical prior to refine the spatial structures by residual supplement and adversarial optimization. (2) A structural perception-aggregation (SPA) module establishes the spatial correlation between the dense cardiac model and sparse label for accurate CMR LV myocardium segmentation. (3) A joint training strategy utilizes the dense SAX volume as explicit and implicit goals to jointly optimize the framework. The experiments are applied on a public dataset and a clinical dataset to evaluate the performance of MVSGAN. The average Dice and Jaccard score of LV myocardium segmentation obtained by MVSGAN are highest among seven existing state-of-the-art methods, which are up to 0.92 and 0.75. It is concluded that the spatial-aware multi-view CMR fusion can provide meaningful spatial correlation for accurate LV myocardium segmentation.
2,330,804
P75 neurotrophin receptor controls subventricular zone neural stem cell migration after stroke.
Stroke is the leading cause of adult disability. Endogenous neural stem/progenitor cells (NSPCs) originating from the subventricular zone (SVZ) contribute to the brain repair process. However, molecular mechanisms underlying CNS disease-induced SVZ NSPC-redirected migration to the lesion area are poorly understood. Here, we show that genetic depletion of the p75 neurotrophin receptor (p75<sup>NTR-/-</sup>) in mice reduced SVZ NSPC migration towards the lesion area after cortical injury and that p75<sup>NTR-/-</sup> NSPCs failed to migrate upon BDNF stimulation in vitro. Cortical injury rapidly increased p75<sup>NTR</sup> abundance in SVZ NSPCs via bone morphogenetic protein (BMP) receptor signaling. SVZ-derived p75<sup>NTR-/-</sup> NSPCs revealed an altered cytoskeletal network- and small GTPase family-related gene and protein expression. In accordance, BMP-treated non-migrating p75<sup>NTR-/-</sup> NSPCs revealed an altered morphology and &#x3b1;-tubulin expression compared to BMP-treated migrating wild-type NSPCs. We propose that BMP-induced p75<sup>NTR</sup> abundance in NSPCs is a regulator of SVZ NSPC migration to the lesion area via regulation of the cytoskeleton following cortical injury.
2,330,805
Oxygen Uptake Efficiency Slope Predicts Adverse Outcome Following Atrial Switch Procedure.
Prior to the 1990s, d-TGA was palliated with the atrial switch procedure resulting in a systemic right ventricle associated with significant long-term morbidity and mortality. Determining the optimal timing of heart transplantation (HT) in these patients has been difficult. While cardiopulmonary exercise testing (CPET) is commonly used to try and risk stratify these patients, traditional exercise parameters have lacked the sensitivity and specificity to assess long-term risk. We sought to assess changes in exercise parameters over time in order to determine if any CPET parameter or combination of parameters could reliably identify risk for adverse outcome in this patient group. A retrospective review of serial CPET for 40 patients over 17&#xa0;years was completed. Patients with adverse event within 6&#xa0;months prior to CPET were noted. CPET parameters were compared and linear mixed model regression with repeated measures was performed on serial tests for longitudinal assessment. The linear mixed model regression identified OUES indexed to BSA to be the most sensitive parameter in identifying patients at risk of adverse event and became a stronger predictor of adverse event when combined with peak heart rate. CPET is useful in identifying patients with atrial switch at increased risk of adverse outcome. Indexed OUES and peak heart rate are better prognostic indicators than VO<sub>2</sub> and VE/VCO<sub>2</sub>.
2,330,806
Construction of a nomogram to reveal the prognostic benefit of spontaneous intracranial hemorrhage among Chinese adults: a population-based study.
We aimed to build a nomogram, based on patients with spontaneous intracerebral hemorrhage (SICH), to predict the probability of mortality and morbidity at 7&#xa0;days and 90&#xa0;days, respectively.</AbstractText>We performed a retrospective study, with patients at less than 6&#xa0;h from ictus admitted to the department of neurosurgery in a single institute, from January 2011 to December 2018. A total of 1036 patients with SICH were included, 486 patients (46.9%) were 47-66&#xa0;years old at diagnosis, and 711 patients (68.6%) were male. The least absolute shrinkage and section operator method was performed to identify the key adverse factors predicting the outcomes in patients with SICH, and multivariate logistic regression analysis was built on these variables, and then the results were visualized by a nomogram. The discrimination of the prognostic models was measured and compared by means of Harrell's concordance index (C-index), calibration curve, area under the curve (AUC), and decision curve analysis (DCA).</AbstractText>Multivariate logistic regression analysis revealed that factors affecting 7-day mortality, including the following: age, therapy, Glasgow Coma Scale (GCS) admission, location, ventricle involved, hematoma volume, white blood cell (WBC), uric acid (UA), and L-lactic dehydrogenase (LDH); and factors affecting 90-day mortality, including temperature, therapy, GCS admission, ventricle involved, WBC, international normalized ratio, UA, LDH, and systolic blood pressure. The C-index for the 7-day mortality and 90-day mortality prediction nomogram was 0.9239 (95% CI&#x2009;=&#x2009;0.9061-0.9416) and 0.9241 (95% CI&#x2009;=&#x2009;0.9064-0.9418), respectively. The AUC of 7-day mortality was 92.4, as is true of 90-day mortality. The calibration curve and DCA indicated that nomograms in our study had a good prediction ability. For 90-day morbidity, age, marital status, and GCS at 7-day remained statistically significant in multivariate analysis. The C-index for the prediction nomogram was 0.6898 (95% CI&#x2009;=&#x2009;0.6511-0.7285), and the calibration curve, AUC as well as DCA curve indicated that the nomogram for the prediction of good outcome demonstrated good agreement in this cohort.</AbstractText>Nomograms in this study revealed many novel prognostic demographic and laboratory factors, and the individualized quantitative risk estimation by this model would be more practical for treatment management and patient counseling.</AbstractText>&#xa9; 2021. Fondazione Societ&#xe0; Italiana di Neurologia.</CopyrightInformation>
2,330,807
An assessment of the transport mechanism and intraneuronal stability of L-carnosine.
L-Carnosine (&#x3b2;-alanyl-L-histidine) is a well-known antioxidant and neuroprotector in various models on animals and cell cultures. However, while there is a plethora of data demonstrating its efficiency as a neuroprotector, there is a distinct lack of data regarding the mechanism of its take up by neurons. According to literature, cultures of rat astrocytes, SKPT cells and rat choroid plexus epithelial cells take up carnosine via the H<sup>+</sup>-coupled PEPT2 membrane transporter. We've assessed the effectiveness and mechanism of carnosine transport, and its stability in primary rat cortical culture neurons. We demonstrated that neurons take up carnosine via active transport with Km&#x2009;=&#x2009;119&#xa0;&#x3bc;M and a maximum velocity of 0.289&#xa0;nmol/mg (prot)/min. Passive transport speed constituted 0.21&#x2219;10<sup>-4</sup>&#xa0;nmol/mg (prot)/min (with 119&#xa0;&#x3bc;M concentration in the medium)-significantly less than active transport speed. However, carnosine concentrations over 12.5&#xa0;mM led to passive transport speed becoming greater than active transport speed. Using PEPT2 inhibitor zofenopril, we demonstrated that PEPT2-dependent transport is one of the main modes of carnosine take up by neurons. Our experiments demonstrated that incubation with carnosine does not affect PEPT2 amount present in culture. At the same time, after removing carnosine from the medium, its elimination speed by culture cells reached 0.035&#xa0;nmol/mg (prot)/min, which led to a decrease in carnosine quantity to control levels in culture within 1&#xa0;h. Thus, carnosine is taken up by neurons with an effectiveness comparable to that of other PEPT2 substrates, but its elimination rate suggests that for effective use as a neuroprotector it's necessary to either maintain a high concentration in brain tissue, or increase the effectiveness of glial cell synthesis of endogenous carnosine and its shuttling into neurons, or use more stable chemical modifications of carnosine.
2,330,808
Endocardial left ventricular pacing.<Pagination><StartPage>526</StartPage><EndPage>532</EndPage><MedlinePgn>526-532</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1007/s00059-021-05074-7</ELocationID><Abstract><AbstractText>Cardiac resynchronization therapy (CRT) is an effective treatment for dyssynchronous heart failure; however, 30-50% of patients fail to improve after implant. Endocardial left ventricular (LV) pacing is an alternative therapy for patients who do not respond to conventional CRT or in whom placement of a&#xa0;lead via the coronary sinus is not possible. It enables pacing at a&#xa0;wide variety of sites, without restrictions due to coronary sinus anatomy, and there is evidence of superior electrical resynchronization and hemodynamic response compared with conventional epicardial CRT. In this article, we discuss the potential advantages and disadvantages of endocardial LV pacing compared with conventional CRT, review the evidence for the delivery of endocardial LV pacing using both lead-based and leadless systems, and explore possible future directions of this novel technology.</AbstractText><CopyrightInformation>&#xa9; 2021. The Author(s).</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Elliott</LastName><ForeName>Mark K</ForeName><Initials>MK</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK. mark.elliott@kcl.ac.uk.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, London, UK. mark.elliott@kcl.ac.uk.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Mehta</LastName><ForeName>Vishal S</ForeName><Initials>VS</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Sidhu</LastName><ForeName>Baldeep Singh</ForeName><Initials>BS</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Niederer</LastName><ForeName>Steven</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Rinaldi</LastName><ForeName>Christopher A</ForeName><Initials>CA</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, London, UK.</Affiliation></AffiliationInfo></Author></AuthorList><Language>eng</Language><GrantList CompleteYN="Y"><Grant><Acronym>WT_</Acronym><Agency>Wellcome Trust</Agency><Country>United Kingdom</Country></Grant><Grant><GrantID>R01 HL152256</GrantID><Acronym>HL</Acronym><Agency>NHLBI NIH HHS</Agency><Country>United States</Country></Grant></GrantList><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D016454">Review</PublicationType></PublicationTypeList><VernacularTitle>Endokardiale linksventrikul&#xe4;re Stimulation.</VernacularTitle><ArticleDate DateType="Electronic"><Year>2021</Year><Month>10</Month><Day>25</Day></ArticleDate></Article><MedlineJournalInfo><Country>Germany</Country><MedlineTA>Herz</MedlineTA><NlmUniqueID>7801231</NlmUniqueID><ISSNLinking>0340-9937</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D058406" MajorTopicYN="Y">Cardiac Resynchronization Therapy</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D058409" MajorTopicYN="N">Cardiac Resynchronization Therapy Devices</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D004699" MajorTopicYN="N">Endocardium</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006333" MajorTopicYN="Y">Heart Failure</DescriptorName><QualifierName UI="Q000628" MajorTopicYN="N">therapy</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006352" MajorTopicYN="N">Heart Ventricles</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D016896" MajorTopicYN="N">Treatment Outcome</DescriptorName></MeshHeading></MeshHeadingList><OtherAbstract Type="Publisher" Language="ger"><AbstractText>Die kardiale Resynchronisationstherapie (CRT) stellt eine effektive Behandlung der dyssynchronen Herzinsuffizienz dar, jedoch kommt es bei 30&#x2013;50&#x202f;% der Patienten nach Implantation nicht zu einer signifikanten klinischen Verbesserung. Die endokardiale linksventrikul&#xe4;re (LV-)Stimulation bietet eine alternative Therapie f&#xfc;r Patienten, deren Erkrankung sich unter konventioneller CRT nicht bessert oder bei denen die Implantation einer Elektrode &#xfc;ber den Koronarsinus nicht m&#xf6;glich ist. Sie erm&#xf6;glicht eine Stimulation von einer Vielzahl linksventrikul&#xe4;rer Positionen aus ohne die Limitationen der Koronarsinusanatomie, und es bestehen Belege f&#xfc;r eine &#xfc;berlegene elektrische Resynchronisation und H&#xe4;modynamik durch endokardiale verglichen mit konventioneller epikardialer CRT. In dieser &#xdc;bersicht diskutieren die Autoren die m&#xf6;glichen Vor- und Nachteile einer endokardialen LV-Stimulation im Vergleich zur konventionellen CRT, fassen die Evidenz f&#xfc;r die endokardiale LV-Stimulation sowohl f&#xfc;r elektrodenbasierte wie auch f&#xfc;r kabellose Systeme zusammen und untersuchen m&#xf6;gliche zuk&#xfc;nftige Entwicklungen dieser neuen Technologie.</AbstractText><CopyrightInformation>&#xa9; 2021. The Author(s).</CopyrightInformation></OtherAbstract><KeywordList Owner="NOTNLM"><Keyword MajorTopicYN="N">Cardiac resynchronization therapy</Keyword><Keyword MajorTopicYN="N">Conduction system pacing</Keyword><Keyword MajorTopicYN="N">Heart failure</Keyword><Keyword MajorTopicYN="N">Hemodynamics</Keyword><Keyword MajorTopicYN="N">Leadless pacing</Keyword></KeywordList><CoiStatement>M.K.&#xa0;Elliott, V.S.&#xa0;Mehta, B.S.&#xa0;Sidhu, S.&#xa0;Niederer, and C.A.&#xa0;Rinaldi declare that they have no competing interests.</CoiStatement></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="accepted"><Year>2021</Year><Month>9</Month><Day>27</Day></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2021</Year><Month>10</Month><Day>26</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2021</Year><Month>12</Month><Day>15</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2021</Year><Month>10</Month><Day>25</Day><Hour>12</Hour><Minute>25</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">34694427</ArticleId><ArticleId IdType="pmc">PMC8543431</ArticleId><ArticleId IdType="doi">10.1007/s00059-021-05074-7</ArticleId><ArticleId IdType="pii">10.1007/s00059-021-05074-7</ArticleId></ArticleIdList><ReferenceList><Reference><Citation>Al-Majed NS, McAlister FA, Bakal JA, Ezekowitz JA. 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Feasibility of intraprocedural integration of cardiac CT to guide left ventricular lead implantation for CRT upgrades. J Cardiovasc Electrophysiol. 2021;32:802&#x2013;812. doi: 10.1111/jce.14896.</Citation><ArticleIdList><ArticleId IdType="doi">10.1111/jce.14896</ArticleId><ArticleId IdType="pmc">PMC8647921</ArticleId><ArticleId IdType="pubmed">33484216</ArticleId></ArticleIdList></Reference><Reference><Citation>Sohal M, Hamid S, Perego G, et al. A multicenter prospective randomized controlled trial of cardiac resynchronization therapy guided by invasive dP/dt. Heart Rhythm O2. 2021;2:19&#x2013;27. doi: 10.1016/j.hroo.2021.01.005.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.hroo.2021.01.005</ArticleId><ArticleId IdType="pmc">PMC8183864</ArticleId><ArticleId IdType="pubmed">34113901</ArticleId></ArticleIdList></Reference><Reference><Citation>Spragg DD, Dong J, Fetics BJ, et al. 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Cardiac resynchronization therapy (CRT) is an effective treatment for dyssynchronous heart failure; however, 30-50% of patients fail to improve after implant. Endocardial left ventricular (LV) pacing is an alternative therapy for patients who do not respond to conventional CRT or in whom placement of a&#xa0;lead via the coronary sinus is not possible. It enables pacing at a&#xa0;wide variety of sites, without restrictions due to coronary sinus anatomy, and there is evidence of superior electrical resynchronization and hemodynamic response compared with conventional epicardial CRT. In this article, we discuss the potential advantages and disadvantages of endocardial LV pacing compared with conventional CRT, review the evidence for the delivery of endocardial LV pacing using both lead-based and leadless systems, and explore possible future directions of this novel technology.<CopyrightInformation>&#xa9; 2021. The Author(s).</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Elliott</LastName><ForeName>Mark K</ForeName><Initials>MK</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK. mark.elliott@kcl.ac.uk.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, London, UK. mark.elliott@kcl.ac.uk.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Mehta</LastName><ForeName>Vishal S</ForeName><Initials>VS</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Sidhu</LastName><ForeName>Baldeep Singh</ForeName><Initials>BS</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Niederer</LastName><ForeName>Steven</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Rinaldi</LastName><ForeName>Christopher A</ForeName><Initials>CA</Initials><AffiliationInfo><Affiliation>School of Biomedical Engineering and Imaging Sciences, St Thomas' Hospital, King's College London, SE1 7EH, London, UK.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Cardiology, Guy's and St Thomas' NHS Foundation Trust, London, UK.</Affiliation></AffiliationInfo></Author></AuthorList><Language>eng</Language><GrantList CompleteYN="Y"><Grant><Acronym>WT_</Acronym><Agency>Wellcome Trust</Agency><Country>United Kingdom</Country></Grant><Grant><GrantID>R01 HL152256</GrantID><Acronym>HL</Acronym><Agency>NHLBI NIH HHS</Agency><Country>United States</Country></Grant></GrantList><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D016454">Review</PublicationType></PublicationTypeList><VernacularTitle>Endokardiale linksventrikul&#xe4;re Stimulation.</VernacularTitle><ArticleDate DateType="Electronic"><Year>2021</Year><Month>10</Month><Day>25</Day></ArticleDate></Article><MedlineJournalInfo><Country>Germany</Country><MedlineTA>Herz</MedlineTA><NlmUniqueID>7801231</NlmUniqueID><ISSNLinking>0340-9937</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D058406" MajorTopicYN="Y">Cardiac Resynchronization Therapy</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D058409" MajorTopicYN="N">Cardiac Resynchronization Therapy Devices</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D004699" MajorTopicYN="N">Endocardium</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006333" MajorTopicYN="Y">Heart Failure</DescriptorName><QualifierName UI="Q000628" MajorTopicYN="N">therapy</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006352" MajorTopicYN="N">Heart Ventricles</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D016896" MajorTopicYN="N">Treatment Outcome</DescriptorName></MeshHeading></MeshHeadingList><OtherAbstract Type="Publisher" Language="ger">Die kardiale Resynchronisationstherapie (CRT) stellt eine effektive Behandlung der dyssynchronen Herzinsuffizienz dar, jedoch kommt es bei 30&#x2013;50&#x202f;% der Patienten nach Implantation nicht zu einer signifikanten klinischen Verbesserung. Die endokardiale linksventrikul&#xe4;re (LV-)Stimulation bietet eine alternative Therapie f&#xfc;r Patienten, deren Erkrankung sich unter konventioneller CRT nicht bessert oder bei denen die Implantation einer Elektrode &#xfc;ber den Koronarsinus nicht m&#xf6;glich ist. Sie erm&#xf6;glicht eine Stimulation von einer Vielzahl linksventrikul&#xe4;rer Positionen aus ohne die Limitationen der Koronarsinusanatomie, und es bestehen Belege f&#xfc;r eine &#xfc;berlegene elektrische Resynchronisation und H&#xe4;modynamik durch endokardiale verglichen mit konventioneller epikardialer CRT. In dieser &#xdc;bersicht diskutieren die Autoren die m&#xf6;glichen Vor- und Nachteile einer endokardialen LV-Stimulation im Vergleich zur konventionellen CRT, fassen die Evidenz f&#xfc;r die endokardiale LV-Stimulation sowohl f&#xfc;r elektrodenbasierte wie auch f&#xfc;r kabellose Systeme zusammen und untersuchen m&#xf6;gliche zuk&#xfc;nftige Entwicklungen dieser neuen Technologie.<CopyrightInformation>&#xa9; 2021. The Author(s).</CopyrightInformation></OtherAbstract><KeywordList Owner="NOTNLM"><Keyword MajorTopicYN="N">Cardiac resynchronization therapy</Keyword><Keyword MajorTopicYN="N">Conduction system pacing</Keyword><Keyword MajorTopicYN="N">Heart failure</Keyword><Keyword MajorTopicYN="N">Hemodynamics</Keyword><Keyword MajorTopicYN="N">Leadless pacing</Keyword></KeywordList><CoiStatement>M.K.&#xa0;Elliott, V.S.&#xa0;Mehta, B.S.&#xa0;Sidhu, S.&#xa0;Niederer, and C.A.&#xa0;Rinaldi declare that they have no competing interests.</CoiStatement></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="accepted"><Year>2021</Year><Month>9</Month><Day>27</Day></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2021</Year><Month>10</Month><Day>26</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2021</Year><Month>12</Month><Day>15</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2021</Year><Month>10</Month><Day>25</Day><Hour>12</Hour><Minute>25</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">34694427</ArticleId><ArticleId IdType="pmc">PMC8543431</ArticleId><ArticleId IdType="doi">10.1007/s00059-021-05074-7</ArticleId><ArticleId IdType="pii">10.1007/s00059-021-05074-7</ArticleId></ArticleIdList><ReferenceList><Reference><Citation>Al-Majed NS, McAlister FA, Bakal JA, Ezekowitz JA. 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Acute hemodynamic effect of left ventricular endocardial pacing in cardiac resynchronization therapy: assessment by pressure-volume loops. Circ Arrhythm Electrophysiol. 2012;5:460&#x2013;467. doi: 10.1161/CIRCEP.111.970277.</Citation><ArticleIdList><ArticleId IdType="doi">10.1161/CIRCEP.111.970277</ArticleId><ArticleId IdType="pubmed">22589286</ArticleId></ArticleIdList></Reference><Reference><Citation>Shetty AK, Sohal M, Chen Z, et al. A comparison of left ventricular endocardial, multisite, and multipolar epicardial cardiac resynchronization: an acute haemodynamic and electroanatomical study. Europace. 2014;16:873&#x2013;879. doi: 10.1093/europace/eut420.</Citation><ArticleIdList><ArticleId IdType="doi">10.1093/europace/eut420</ArticleId><ArticleId IdType="pubmed">24525553</ArticleId></ArticleIdList></Reference><Reference><Citation>Behar JM, Jackson T, Hyde E, et al. Optimized left ventricular endocardial stimulation&#xa0;is superior to optimized epicardial&#xa0;stimulation in ischemic patients with&#xa0;poor response to cardiac resynchronization&#xa0;therapy: a&#xa0;combined magnetic resonance imaging, electroanatomic&#xa0;contact mapping, and he. JACC Clin Electrophysiol. 2016;2:799&#x2013;809. doi: 10.1016/j.jacep.2016.04.006.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.jacep.2016.04.006</ArticleId><ArticleId IdType="pmc">PMC5196018</ArticleId><ArticleId IdType="pubmed">28066827</ArticleId></ArticleIdList></Reference><Reference><Citation>van Deursen C, van Geldorp IE, Rademakers LM, et al. Left ventricular endocardial pacing improves resynchronization therapy in canine left bundle-branch hearts. 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Circ Arrhythm Electrophysiol. 2015;8:1164&#x2013;1172. doi: 10.1161/CIRCEP.115.002677.</Citation><ArticleIdList><ArticleId IdType="doi">10.1161/CIRCEP.115.002677</ArticleId><ArticleId IdType="pubmed">26136400</ArticleId></ArticleIdList></Reference><Reference><Citation>Fish JM, di Diego JM, Nesterenko V, Antzelevitch C. Epicardial activation of left ventricular wall prolongs QT interval and transmural dispersion of repolarization: implications for biventricular pacing. Circulation. 2004;109:2136&#x2013;2142. doi: 10.1161/01.CIR.0000127423.75608.A4.</Citation><ArticleIdList><ArticleId IdType="doi">10.1161/01.CIR.0000127423.75608.A4</ArticleId><ArticleId IdType="pubmed">15078801</ArticleId></ArticleIdList></Reference><Reference><Citation>Medina-Ravell VA, Lankipalli RS, Yan GX, et al. Effect of epicardial or biventricular pacing to prolong QT interval and increase transmural dispersion of repolarization: Does resynchronization therapy pose a&#xa0;risk for patients predisposed to long QT or torsade de pointes? Circulation. 2003;107:740&#x2013;746. doi: 10.1161/01.CIR.0000048126.07819.37.</Citation><ArticleIdList><ArticleId IdType="doi">10.1161/01.CIR.0000048126.07819.37</ArticleId><ArticleId IdType="pubmed">12578878</ArticleId></ArticleIdList></Reference><Reference><Citation>Mendonca Costa C, Neic A, Gillette K, et al. Left ventricular endocardial pacing is less arrhythmogenic than conventional epicardial pacing when pacing in proximity to scar. Heart Rhythm. 2020;17:1262&#x2013;1270. doi: 10.1016/j.hrthm.2020.03.021.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.hrthm.2020.03.021</ArticleId><ArticleId IdType="pmc">PMC7397521</ArticleId><ArticleId IdType="pubmed">32272230</ArticleId></ArticleIdList></Reference><Reference><Citation>Mendonca Costa C, Neic A, Kerfoot E, et al. Pacing in proximity to scar during cardiac resynchronization therapy increases local dispersion of repolarization and susceptibility to ventricular arrhythmogenesis. Heart Rhythm. 2019;16:1475&#x2013;1483. doi: 10.1016/j.hrthm.2019.03.027.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.hrthm.2019.03.027</ArticleId><ArticleId IdType="pmc">PMC6774764</ArticleId><ArticleId IdType="pubmed">30930329</ArticleId></ArticleIdList></Reference><Reference><Citation>Roque C, Trevisi N, Silberbauer J, et al. Electrical storm induced by cardiac resynchronization therapy is determined by pacing on epicardial scar and can be successfully managed by catheter ablation. Circ Arrhythm Electrophysiol. 2014;7:1064&#x2013;1069. doi: 10.1161/CIRCEP.114.001796.</Citation><ArticleIdList><ArticleId IdType="doi">10.1161/CIRCEP.114.001796</ArticleId><ArticleId IdType="pubmed">25221332</ArticleId></ArticleIdList></Reference><Reference><Citation>Tankut S, Goldenberg I, Kutyifa V, et al. Cardiac resynchronization therapy and ventricular tachyarrhythmia burden. Heart Rhythm. 2021;18:762&#x2013;769. doi: 10.1016/j.hrthm.2020.12.034.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.hrthm.2020.12.034</ArticleId><ArticleId IdType="pubmed">33440249</ArticleId></ArticleIdList></Reference><Reference><Citation>Yuyun MF, Erqou SA, Peralta AO, et al. Risk of ventricular arrhythmia in cardiac resynchronization therapy responders and super-responders: a systematic review and meta-analysis. Europace. 2021;23(8):1262&#x2013;1274. doi: 10.1093/europace/euaa414.</Citation><ArticleIdList><ArticleId IdType="doi">10.1093/europace/euaa414</ArticleId><ArticleId IdType="pubmed">33496319</ArticleId></ArticleIdList></Reference><Reference><Citation>Morgan JM, Biffi M, Gell&#xe9;r L, et al. ALternate Site Cardiac ResYNChronization (ALSYNC): a prospective and multicentre study of left ventricular endocardial pacing for cardiac resynchronization therapy. Eur Heart J. 2016;37:2118&#x2013;2127. doi: 10.1093/eurheartj/ehv723.</Citation><ArticleIdList><ArticleId IdType="doi">10.1093/eurheartj/ehv723</ArticleId><ArticleId IdType="pubmed">26787437</ArticleId></ArticleIdList></Reference><Reference><Citation>Graham AJ, Providenica R, Honarbakhsh S, et al. Systematic review and meta-analysis of left ventricular endocardial pacing in advanced heart failure: clinically efficacious but at what cost? Pacing Clin Electrophysiol. 2018;41:353&#x2013;361. doi: 10.1111/pace.13275.</Citation><ArticleIdList><ArticleId IdType="doi">10.1111/pace.13275</ArticleId><ArticleId IdType="pubmed">29344950</ArticleId></ArticleIdList></Reference><Reference><Citation>Gamble JHP, Herring N, Ginks M, et al. Endocardial left ventricular pacing for cardiac resynchronization: systematic review and meta-analysis. Europace. 2018;20:73&#x2013;81. doi: 10.1093/europace/euw381.</Citation><ArticleIdList><ArticleId IdType="doi">10.1093/europace/euw381</ArticleId><ArticleId IdType="pubmed">28073886</ArticleId></ArticleIdList></Reference><Reference><Citation>Elencwajg B, L&#xf3;pez-Cabanillas N, Fischer A, et al. Multicenter prospective observational long-term follow-up study of endocardial cardiac resynchronization therapy using the Jurdham procedure. Heart Rhythm. 2019;16(10):1453&#x2013;1461. doi: 10.1016/j.hrthm.2019.07.017.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.hrthm.2019.07.017</ArticleId><ArticleId IdType="pubmed">31323347</ArticleId></ArticleIdList></Reference><Reference><Citation>Auricchio A, Delnoy PP, Butter C, et al. Feasibility, safety, and short-term outcome of leadless ultrasound-based endocardial left ventricular resynchronization in heart failure patients: results of the Wireless Stimulation Endocardially for CRT (WiSE-CRT) study. Europace. 2014;16:681&#x2013;688. doi: 10.1093/europace/eut435.</Citation><ArticleIdList><ArticleId IdType="doi">10.1093/europace/eut435</ArticleId><ArticleId IdType="pubmed">24497573</ArticleId></ArticleIdList></Reference><Reference><Citation>Reddy VY, Miller MA, Neuzil P, et al. Cardiac resynchronization therapy with wireless left ventricular endocardial pacing. J Am Coll Cardiol. 2017;69:2119&#x2013;2129. doi: 10.1016/j.jacc.2017.02.059.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.jacc.2017.02.059</ArticleId><ArticleId IdType="pubmed">28449772</ArticleId></ArticleIdList></Reference><Reference><Citation>Sieniewicz BJ, Betts TR, James S, et al. Real-world experience of leadless left ventricular endocardial cardiac resynchronization therapy: a multicenter international registry of the WiSE-CRT pacing system. Heart Rhythm. 2020 doi: 10.1016/j.hrthm.2020.03.002.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.hrthm.2020.03.002</ArticleId><ArticleId IdType="pmc">PMC7397503</ArticleId><ArticleId IdType="pubmed">32165181</ArticleId></ArticleIdList></Reference><Reference><Citation>Sidhu BS, Porter B, Gould J, et al. Leadless left ventricular endocardial pacing in nonresponders to conventional cardiac resynchronization therapy. Pacing Clin Electrophysiol. 2020;43(9):966&#x2013;973. doi: 10.1111/pace.13926.</Citation><ArticleIdList><ArticleId IdType="doi">10.1111/pace.13926</ArticleId><ArticleId IdType="pubmed">32330307</ArticleId></ArticleIdList></Reference><Reference><Citation>Singh JP, Abraham WT, Auricchio A, et al. Design and rationale for the stimulation of the left ventricular endocardium for cardiac resynchronization therapy in non-responders and previously untreatable patients (SOLVE-CRT) trial. Am Heart J. 2019;217:13&#x2013;22. doi: 10.1016/j.ahj.2019.04.002.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.ahj.2019.04.002</ArticleId><ArticleId IdType="pubmed">31472360</ArticleId></ArticleIdList></Reference><Reference><Citation>Singh JP, Walsh MN, Kubo SH, et al. Modified design of stimulation of the left ventricular endocardium for cardiac resynchronization therapy in nonresponders, previously untreatable and high-risk upgrade patients (SOLVE-CRT) trial. Am Heart J. 2021;235:158&#x2013;162. doi: 10.1016/j.ahj.2021.02.008.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.ahj.2021.02.008</ArticleId><ArticleId IdType="pubmed">33596412</ArticleId></ArticleIdList></Reference><Reference><Citation>Carabelli A, Jabeur M, Jacon P, et al. European experience with a&#xa0;first totally leadless cardiac resynchronization therapy pacemaker system. Europace. 2021;23:740&#x2013;747. doi: 10.1093/europace/euaa342.</Citation><ArticleIdList><ArticleId IdType="doi">10.1093/europace/euaa342</ArticleId><ArticleId IdType="pmc">PMC8139811</ArticleId><ArticleId IdType="pubmed">33313789</ArticleId></ArticleIdList></Reference><Reference><Citation>Sidhu BS, Gould J, Porter B, et al. Completely leadless cardiac resynchronization defibrillator system. JACC Clin Electrophysiol. 2020;6:5&#x2013;6. doi: 10.1016/j.jacep.2020.02.012.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.jacep.2020.02.012</ArticleId><ArticleId IdType="pubmed">32439047</ArticleId></ArticleIdList></Reference><Reference><Citation>Sharma PS, Vijayaraman P. Conduction system pacing for cardiac resynchronisation. Arrhythm Electrophysiol Rev. 2021;10:51&#x2013;58. doi: 10.15420/AER.2020.45.</Citation><ArticleIdList><ArticleId IdType="doi">10.15420/AER.2020.45</ArticleId><ArticleId IdType="pmc">PMC8076975</ArticleId><ArticleId IdType="pubmed">33936744</ArticleId></ArticleIdList></Reference><Reference><Citation>Salden FCWM, Luermans JGLM, Westra SW, et al. Short-term hemodynamic and electrophysiological effects of cardiac resynchronization by left ventricular septal pacing. J Am Coll Cardiol. 2020;75:347&#x2013;359. doi: 10.1016/j.jacc.2019.11.040.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.jacc.2019.11.040</ArticleId><ArticleId IdType="pubmed">32000945</ArticleId></ArticleIdList></Reference><Reference><Citation>Elliott MK, Jacon P, Sidhu BS, et al. Eur Heart J. 2021 doi: 10.1093/ehjcr/ytab379.</Citation><ArticleIdList><ArticleId IdType="doi">10.1093/ehjcr/ytab379</ArticleId><ArticleId IdType="pmc">PMC8633604</ArticleId><ArticleId IdType="pubmed">34859181</ArticleId></ArticleIdList></Reference></ReferenceList></PubmedData></PubmedArticle><PubmedArticle><MedlineCitation Status="MEDLINE" Owner="NLM" IndexingMethod="Automated"><PMID Version="1">34694286</PMID><DateCompleted><Year>2022</Year><Month>04</Month><Day>05</Day></DateCompleted><DateRevised><Year>2022</Year><Month>04</Month><Day>05</Day></DateRevised><Article PubModel="Electronic"><Journal><ISSN IssnType="Electronic">1940-087X</ISSN><JournalIssue CitedMedium="Internet"><Issue>176</Issue><PubDate><Year>2021</Year><Month>Oct</Month><Day>07</Day></PubDate></JournalIssue><Title>Journal of visualized experiments : JoVE</Title><ISOAbbreviation>J Vis Exp</ISOAbbreviation></Journal><ArticleTitle>Cryo-section Dissection of the Adult Subependymal Zone for Accurate and Deep Quantitative Proteome Analysis.</ArticleTitle><ELocationID EIdType="doi" ValidYN="Y">10.3791/63047</ELocationID><Abstract>The subependymal neurogenic niche consists of a paraventricular ribbon of the lateral ventricular wall of the lateral ventricle. The subependymal zone (SEZ) is a thin and distinct region exposed to the ventricles and cerebrospinal fluid. The isolation of this niche allows the analysis of a neurogenic stem cell microenvironment. However, extraction of small tissues for proteome analysis&#xa0;is challenging, especially for the maintenance of considerable measurement depth and the achievement of reliable robustness. A new method termed cryo-section-dissection (CSD), combining high precision with minimal tissue perturbation, was developed to address these challenges. The method is compatible with state-of-the-art mass spectrometry (MS) methods that allow the detection of low-abundant niche regulators. This study compared the CSD and its proteome data to the method and data obtained by laser-capture-microdissection (LCM) and a standard wholemount dissection. The CSD method resulted in twice the quantification depth in less than half the preparation time compared to the LCM and simultaneously clearly outperformed the dissection precision of the wholemount dissection. Hence, CSD is a superior method for collecting the SEZ for proteome analysis.
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Two novel truncating variants of the ASPM gene identified in a nonconsanguineous Chinese family associated with primary microcephaly.
Primary autosomal recessive microcephaly 5 (MCPH5) is a rare neurodevelopmental disorder with a relatively high incidence in regions where consanguineous marriage is widely practiced; So far, only a few MCPH5 cases have been reported from China. Here, we report clinical and molecular characteristics of two Chinese MCPH5 patients, a 24-year-old woman proband and her brother, a 19-year-old man, from a nonconsanguineous family. Main manifestations in the proband were small head circumference, premature closure of fontanelles, impaired concentration and moderate intellectual disability. The proband's brother had similar symptoms, but he was hyperactive and had a more severe sloping forehead. Brain imaging revealed global reduction in brain size, especially in the frontal lobes bilaterally and anterior horns of lateral ventricles. Sequencing results revealed that both patients carried a novel nonsense variant p.Tyr2004* (c.6012_6013delTA) and a novel frameshift variant p.Arg2005Serfs*48 (c.6015_6016delGG) in the ASPM gene. These variants were interpreted to be pathogenic in the in-silico analysis. Our findings help to expand the mutation spectrum of ASPM and provide new opportunities for assisting the traditional clinical diagnosis on the cases with atypical characteristics.
2,330,810
Eye Blink-Associated Saccades.
Saccades function to bring targets of interest into the field of view. They are one of the four types of basic eye movements in humans, all of which are generated and modulated by components of a complex eye movement network, involving cortical eye fields, thalami, basal ganglia, cerebellum, and brainstem structures. Similarly, blinks are presumed to be generated by a blink center involving complex cortical and subcortical pathways. An association between saccades and blinks is well established; when these circuits are disrupted, normal saccadic parameters change.&#xa0;We report a case of a 48-year-old female who presented with fatigue and weakness. She had a complicated medical history, including drug-resistant epilepsy with subsequent vagus nerve stimulator (VNS) placement, right anterior temporal lobectomy, and craniotomy for a residual right temporal lobectomy and amygdalohippocampectomy. The latter was complicated by ischemic right middle cerebral artery (MCA) territory stroke with residual&#xa0;left hemiplegia. Her examination was unremarkable with regards to the presenting complaints, but one unique finding was observed; she demonstrated abnormal conjugate eye movements to the left associated with each blink. These eye movements continued to be present even after the patient's ability to fixate on an object was removed.&#xa0;It was unclear how long this finding had been present. A review of her MRI of the brain from 10 months prior showed encephalomalacia and surrounding gliosis in the right MCA territory, right temporal laminar necrosis, right basal ganglia and parietal lobe microhemorrhages, ex vacuo dilatation of the right lateral ventricle, and a&#xa0;rightward midline shift.&#xa0;Saccadic abnormalities have been reported in a variety of conditions. The eye blink-associated saccades seen here are rare. To our knowledge, only one other patient has been reported with similar blink-associated eye movements after brain injury following a right MCA&#xa0;territory stroke. The exact mechanism underlying these eye movements is unclear, but may involve aberrant or disrupted neuronal signaling in cortical and/or basal ganglia components of the eye movement network, or related to an as yet unknown blink-saccadic regulatory mechanism.
2,330,811
The Typical Triad of Idiopathic Normal Pressure Hydrocephalus in a 62-Year-Old Male.
Normal pressure hydrocephalus (NPH) is a rare pathological condition of the brain in which the ventricles are enlarged due to cerebrospinal fluid accumulation and is associated with normal opening pressure on lumbar puncture with a large-volume cerebrospinal fluid (CSF) tap. This results in three classical symptoms: mental impairment, gait disturbance, and urinary or fecal incontinence. We present a case of idiopathic NPH in which a 64-year-old retired man with diabetes was brought to the emergency department after recurrent previous falls. The patient complained of an unsteady gait and presented with the typical triad of NPH which is mental impairment, gait disturbance, and incontinence. The patient was a known diabetic, and his gait was characterized by shuffling, bradykinesia, and mild drifting toward the right side. A head computed tomography scan revealed brain tissue volume loss, with disproportionate dilation of the lateral and third ventricles. A lumbar puncture with a large-volume CSF tapping produced normal opening pressure (18 mmHg); thus, the diagnosis of NPH was made. The patient underwent shunt surgery, and his balance and memory improved significantly after the procedure. Also, no event of fecal incontinence occurred. NPH resembles several neurodegenerative disorders. Due to this, it can be difficult to diagnose. Emergency physicians, as frontline healthcare providers, may encounter such cases.NPH should be considered in patients presenting with an unsteady gait, memory impairment, and urinary or fecal incontinence by taking a detailed history and conducting a physical examination to prevent future complications.
2,330,812
Retrospective evaluation of endoscopic treatment in colloid cyst of the third ventricle.
Endoscopic methods have gained a well-established position in surgical treatment of colloid cysts of third ventricle. However, the possibility of total tumor removal with this method and the long-term effectiveness of treatment are being questioned.</AbstractText>Personal twenty years' experience in treatment of third ventricle colloid cysts is presented on the basis of retrospective analysis.</AbstractText>The study group included 58 patients diagnosed by neuroimaging (head CT/MRI) with third ventricle colloid cyst. Post-hospital follow-up ranged from 18 to 42 months. Long-term follow-up head CT/MRI was performed in 39 patients.</AbstractText>The colloid cyst was removed totally in 47 (81%) patients. In 11 cases, the colloid cyst's wall was tightly adherent to the roof of the third ventricle, which limited the radicality of the procedure. Sixteen patients demonstrated memory impairments, 4 patients epilepsy and another 2 akinetic mutism in the direct postoperative course. One patient died as a result of complications unrelated to the procedure. The average hospitalization was 5 days. In the late period after surgery, remission of the most, previously, reported ailments and symptoms has been reported. Surgical treatment for hydrocephalus was needed in 7 patients. In 3 cases cyst recurrence was diagnosed which required reoperation.</AbstractText>The endoscopic methods allow the total removal of a third ventricle colloid cyst in most patients. Leaving a small coagulated fragment of the cyst rarely results in its recurrence. This method results in effective treatment with a low complications rate, shortens hospitalization time and brings the patient a high level of satisfaction with a quick recovery.</AbstractText>Copyright: &#xa9; 2021 Fundacja Videochirurgii.</CopyrightInformation>
2,330,813
The Epic of the Thalamus in Anatomical Language.
Understanding the origin of Greek and Latin words used as metaphors to label brain structures gives a unique window into how scientific and medical knowledge was produced, preserved, and transmitted through generations. The history of the term <i>thalamus</i> exemplifies the complex historical process that led to the current anatomical terminology. From its first mention by Galen of Pergamon in the 2nd century A.D. to its definitive and current use by Thomas Willis in 1664, the <i>thalamus</i> had an epical journey through 1500 years across Europe, the Middle East, and the North of Africa. The <i>thalamus</i> was confusingly described by Galen, in the Greek language, as a chamber to the brain ventricles. The term <i>thalamus</i> was transferred from Greek to Syriac through the translations of Galen's books done in Baghdad and also from Syriac to Arabic. Then, it was translated in Europe during the Middle Ages from the Arabic versions of Galen's books to Latin. Later, during the Early Renaissance, it was translated again to Latin directly from the Greek versions of Galen's books. Along this epical journey through languages, the term <i>thalamus</i> switched from referring to a hollow structure connected to brain ventricles to naming a solid structure at the rostral end of the brainstem. Finally, the <i>thalamus</i> was translated from Latin to modern languages, where it is used, until today, to name a nuclear complex of subcortical gray matter in the lateral walls of the third ventricle.
2,330,814
Mitigation of late cardiovascular effects of oxygen ion radiation by &#x3b3;-tocotrienol in a mouse model.
While there is concern about degenerative tissue effects of exposure to space radiation during deep-space missions, there are no pharmacological countermeasures against these adverse effects. &#x3b3;-Tocotrienol (GT3) is a natural form of vitamin E that has anti-oxidant properties, modifies cholesterol metabolism, and has anti-inflammatory and endothelial cell protective properties. The purpose of this study was to test whether GT3 could mitigate cardiovascular effects of oxygen ion (16</sup>O) irradiation in a mouse model.</AbstractText>Male C57BL/6&#xa0;J mice were exposed to whole-body 16</sup>O (600 MeV/n) irradiation (0.26-0.33&#xa0;Gy/min) at doses of 0 or 0.25&#xa0;Gy at 6 months of age and were followed up to 9 months after irradiation. Animals were administered GT3 (50&#xa0;mg/kg/day s.c.) or vehicle, on Monday - Friday starting on day 3 after irradiation for a total of 16 administrations. Ultrasonography was used to measure in vivo cardiac function and blood flow parameters. Cardiac tissue remodeling and inflammatory infiltration were assessed with histology and immunoblot analysis at 2 weeks, 3 and 9 months after radiation.</AbstractText>GT3 mitigated the effects of 16</sup>O radiation on cardiac function, the expression of a collagen type III peptide, and markers of mast cells, T-cells and monocytes/macrophages in the left ventricle.</AbstractText>GT3 may be a potential countermeasure against late degenerative tissue effects of high-linear energy transfer radiation in the heart.</AbstractText>Copyright &#xa9; 2021 The Committee on Space Research (COSPAR). Published by Elsevier B.V. All rights reserved.</CopyrightInformation>
2,330,815
A clinical study of high-dose urokinase for the treatment of the patients with hypertension induced ventricular hemorrhage.
This study discusses the therapeutic effect of high-dose urokinase treatment for hypertension ventricular hemorrhage.</AbstractText>A total of 60 patients with hypertension ventricular hemorrhage were randomly assigned to two groups: treatment group (n=30) and control group (n=30). Both groups received bilateral external ventricular drain. The treatment group was injected with 50,000IU urokinase to the lateral ventricle every day; the total injection volume per day was 100,000IU. The control group was injected with 20,000IU urokinase to the lateral ventricle every day with a total injection volume per day of 40,000IU. Lumbar puncture was performed in both groups after the later ventricular drain was removed to release cerebrospinal fluid (CSF). Head Computed tomography(CT) examination was performed regularly to observe changes in the ventricular hematoma as well as the occurrence of complications such as intracranial infection and hydrocephalus. Patient prognosis 6 weeks after surgery was compared between the two groups.</AbstractText>In the treatment group, the intraventricular hemorrhage clearance time and the number of instances of urokinase treatment were significantly less than those of the control group (P&lt;0.05). The total urokinase dosage of the treatment group was significantly higher than that of the control group (P&lt;0.05). With respect to post-surgery complications, in the treatment group, there were three cases of hydrocephalus and one case of intracranial infection. In the control group, there were four cases of hydrocephalus and three cases of intracranial infection. Intraventricular re-hemorrhage was not observed in either group. Intracranial infection was relieved after strengthened anti-infective therapy and continuous drainage. There was a statistically significant difference in the occurrence of complications between the treatment group and the control group (P&lt;0.05). The rate of good prognosis in the treatment group was higher than that of the control group (P&lt;0.05), and the inefficiency rate was lower (P&lt;0.05).</AbstractText>High-dose urokinase treatment produces a significant therapeutic effect in hypertension ventricular hemorrhage. This treatment can quickly eliminate intraventricular hemorrhage, shorten the ventricular drain tube indwelling time, decrease the occurrence of intracranial infection, and increase the likelihood of a good prognosis.</AbstractText>Copyright &#xa9; 2021 Elsevier B.V. All rights reserved.</CopyrightInformation>
2,330,816
Ultrasonic biomechanics method for vortex and wall motion of left ventricle: a phantom and in vivo study.
The non-invasive quantitative evaluation of left ventricle (LV) function plays a critical role in clinical cardiology. This study proposes a novel ultrasonic biomechanics method by integrating both LV vortex and wall motion to fully assess and understand the LV structure and function. The purpose of this study was to validate the ultrasonic biomechanics method as a quantifiable approach to evaluate LV function.</AbstractText>Firstly, B-mode ultrasound images were acquired and processed, which were utilized to implement parameters for quantifying the LV vortex and wall motion respectively. Next, the parameters were compared in polyvinyl alcohol cryogen (PVA) phantoms with different degree of stiffness corresponding to different freezing and thawing cycles in vitro. Finally, the parameters were computed in vivo during one cardiac cycle to assess the LV function in normal and abnormal subjects in vivo.</AbstractText>In vitro study, the velocity field of PVA phantom differed with stiffness (varied elasticity modulus). The peak of strain for wall motion decreases with the increase of elasticity modulus, and periodically changed values. Statistical analysis for parameters of vortex dynamics (energy dissipation index, DI; kinetic energy fluctuations, KEF; relative strength, RS; and vorticity, W) based on different elasticity (E) of phantom depicted the good viability of this algorithm. In vivo study, the results confirmed that subjects with LV dysfunction had lower vorticity and strain (S) compared to the normal group.</AbstractText>Ultrasonic biomechanics method can obtain the vortex and wall motion of left ventricle. The method may have potential clinical value in evaluation of LV dysfunction.</AbstractText>&#xa9; 2021. The Author(s).</CopyrightInformation>
2,330,817
Global hippocampal and selective thalamic nuclei atrophy differentiate chronic TBI from Non-TBI.
Traumatic brain injury (TBI) may increase susceptibility to neurodegenerative diseases later in life. One neurobiological parallel between chronic TBI and neurodegeneration may be accelerated aging and the nature of atrophy across subcortical gray matter structures. The main aim of the present investigation is to evaluate and rank the degree that subcortical gray matter atrophy differentiates chronic moderate-severe TBI from non-TBI participants by evaluating morphometric differences between groups. Forty individuals with moderate-severe chronic TBI (9.23&#xa0;yrs from injury) and 33 healthy controls (HC) underwent high resolution 3D T1-weighted structural magnetic resonance imaging. Whole brain volume was classified into white matter, cortical and subcortical gray matter structures with hippocampi and thalami further segmented into subfields and nuclei, respectively. Extensive atrophy was observed across nearly all brain regions for chronic TBI participants. A series of multivariate logistic regression models identified subcortical gray matter structures of the hippocampus and thalamus as the most sensitive to differentiating chronic TBI from non-TBI participants (McFadden R<sup>2</sup>&#xa0;=&#xa0;.36, p&#xa0;&lt;&#xa0;.001). Further analyses revealed the pattern of hippocampal atrophy to be global, occurring across nearly all subfields. The pattern of thalamic atrophy appeared to be much more selective and non-uniform, with largest between-group differences evident for nuclei bordering the ventricles. Subcortical gray matter was negatively correlated with time since injury (r&#xa0;=&#xa0;-.31, p&#xa0;=&#xa0;.054), while white matter and cortical gray matter were not. Cognitive ability was lower in the chronic TBI group (Cohen's d&#xa0;=&#xa0;.97, p&#xa0;=&#xa0;.003) and correlated with subcortical structures including the pallidum (r<sup>2</sup>&#xa0;=&#xa0;.23, p&#xa0;=&#xa0;.038), thalamus (r<sup>2</sup>&#xa0;=&#xa0;.36, p&#xa0;=&#xa0;.007) and ventral diencephalon (r<sup>2</sup>&#xa0;=&#xa0;.23, p&#xa0;=&#xa0;.036). These data may support an accelerated aging hypothesis in chronic moderate-severe TBI that coincides with a similar neuropathological profile found in neurodegenerative diseases.
2,330,818
Multiple two-dimensional active shape model framework for right ventricular segmentation.
Segmentation of the right ventricle (RV) in MRI short axis images is very challenging due to its complex shape and various appearance among the different subjects and cross-sections. Active shape models (ASM) have shown potential for segmenting the complex structures, including the RV, through two formulations: two- and three-dimensional modeling with a reported trade-off between accuracy and complexity of each formulation. In this work, we propose a new framework for modeling the RV surface using multiple 2D contours, where information from multiple cross-sectional images are incorporated into the same model. The proposed method was tested using cardiac MRI images from 56 human subjects. Compared to a golden reference of manually delineated RV contours, the proposed method resulted in significantly lower error than (almost one half) that of the conventional 2D ASM especially at the apical slices. The mean absolute distance of the proposed method was 2.9&#xa0;&#xb1;&#xa0;2&#xa0;mm while the conventional 2D ASM resulted in an error of 6.6&#xa0;&#xb1;&#xa0;4.5&#xa0;mm. In addition, the computation time of the proposed method was 5&#xa0;s compared to 4&#xa0;&#xb1;&#xa0;1&#xa0;min previously reported for the 3D ASM formulation.
2,330,819
Quantitative analysis of size and regional distribution of corpora amylacea in the hippocampal formation of obstructive sleep apnoea patients.
Corpora amylacea (CoA) are spherical aggregates of glucose polymers and proteins within the periventricular, perivascular and subpial regions of the cerebral cortex and the hippocampal cornu ammonis (CA) subfields. The present study quantified the distribution of CoA in autopsied hippocampi of patients with obstructive sleep apnoea (OSA) using ethanolamine-induced fluorescence. CoA were observed in 29 of 30 patients (96.7%). They were most abundant in periventricular regions (wall of lateral ventricle, alveus, fimbria and CA4), rarely found in the CA3 and CA1, and undetectable in the CA2 or subiculum. A spatiotemporal sequence of CoA deposition was postulated, beginning in the fimbria and progressively spreading around the subpial layer until they extended medially to the wall of the lateral ventricle and laterally to the collateral sulcus. This ranked CoA sequence was positively correlated with CoA packing density (count and area fraction) and negatively correlated with CoA minimum diameters (p&#x2009;&lt;&#x2009;0.05). Although this sequence was not correlated with age or body mass index (BMI), age was positively correlated with the mean and maximum diameters of CoA. These findings support the view that the spatiotemporal sequence of CoA deposition is independent of age, and that CoA become larger due to the accretion of new material over time.
2,330,820
Modeling Hypoxic Stress In Vitro Using Human Embryonic Stem Cells Derived Cardiomyocytes Matured by FGF4 and Ascorbic Acid Treatment.
Mature cardiomyocytes (CMs) obtained from human pluripotent stem cells (hPSCs) have been required for more accurate in vitro modeling of adult-onset cardiac disease and drug discovery. Here, we found that FGF4 and ascorbic acid (AA) induce differentiation of BG01 human embryonic stem cell-cardiogenic mesoderm cells (hESC-CMCs) into mature and ventricular CMs. Co-treatment of BG01 hESC-CMCs with FGF4+AA synergistically induced differentiation into mature and ventricular CMs. FGF4+AA-treated BG01 hESC-CMs robustly released acute myocardial infarction (AMI) biomarkers (cTnI, CK-MB, and myoglobin) into culture medium in response to hypoxic injury. Hypoxia-responsive genes and potential cardiac biomarkers proved in the diagnosis and prognosis of coronary artery diseases were induced in FGF4+AA-treated BG01 hESC-CMs in response to hypoxia based on transcriptome analyses. This study demonstrates that it is feasible to model hypoxic stress in vitro using hESC-CMs matured by soluble factors.
2,330,821
Antioxidants Supplementation Reduces Ceramide Synthesis Improving the Cardiac Insulin Transduction Pathway in a Rodent Model of Obesity.
Obesity-related disruption in lipid metabolism contributes to cardiovascular dysfunction. Despite numerous studies on lipid metabolism in the left ventricle, there is no data describing the influence of n-acetylcysteine (NAC) and &#x3b1;-lipoic acid (ALA), as glutathione precursors, on sphingolipid metabolism, and insulin resistance (IR) occurrence. The aim of our experiment was to evaluate the influence of chronic antioxidants administration on myocardial sphingolipid state and intracellular insulin signaling as a potential therapeutic strategy for obesity-related cardiovascular IR. The experiment was conducted on male Wistar rats fed a standard rodent chow or a high-fat diet with intragastric administration of NAC or ALA for eight weeks. Cardiac and plasma sphingolipid species were assessed by high-performance liquid chromatography (HPLC). The proteins expressed from sphingolipid and insulin signaling pathways were determined by Western blot. Antioxidant supplementation markedly reduced ceramide accumulation by lowering the expression of selected proteins from the sphingolipid pathway and simultaneously increased the myocardial sphingosine-1-phosphate level. Moreover, NAC and ALA augmented the expression of GLUT4 and the phosphorylation state of Akt (Ser473) and GSK3&#x3b2; (Ser9), which improved the intracellular insulin transduction pathway. Based on our results, we may postulate that NAC and ALA have a beneficial influence on the cardiac ceramidose under IR conditions.
2,330,822
Transcortical approach surgery versus external ventricular drainage in treating intraventricular hemorrhage.
Intraventricular hemorrhage is a serious intracerebral hemorrhagic disease with high mortality and poor prognosis. This retrospective study designed to investigate the therapeutic effect of transcortical approach surgery versus extraventricular drainage (EVD) on patients with intraventricular hemorrhage.Patients with intraventricular hemorrhage in Zhongshan City People's Hospital from January 01, 2014 to June 01, 2019 were retrospectively examined. They were divided into transcortical approach surgery groups and EVD groups to analyze the clinical characteristics and prognosis.A total of 96 patients were enrolled in the study (24 in the transcortical approach surgery group and 72 in the EVD group). The efficiency of postoperative operation was 15/19 in the transcortical approach surgery group and 24/48 in the EVD group (P&#x200a;=&#x200a;.012). The Glasgow Outcome Scale was 3.63&#x200a;&#xb1;&#x200a;1.27 in the transcortical approach surgery group and 2.80&#x200a;&#xb1;&#x200a;1.87 in the EVD group (P&#x200a;=&#x200a;.049). The postoperative residual blood volume was 9.62&#x200a;&#xb1;&#x200a;3.64&#x200a;mL in the transcortical approach surgery group and 33.60&#x200a;&#xb1;&#x200a;3.53&#x200a;mL in the EVD group (P&#x200a;&lt;&#x200a;.001). The incidence of hydrocephalus after the operation was 1/23 in the transcortical approach surgery group and 19/53 in the EVD group. The 30-day postoperative mortality was 16/56 in the EVD group and 1/23 in the transcortical approach surgery group. The transcortical approach surgery group was significantly better compared with the EVD group.This study showed that the transcortical approach for ventricular hemorrhage compared with EVD improved the hematoma clearance rate, shortened catheterization time, reduced the incidence of postoperative hydrocephalus, decreased patient mortality, led to a better prognosis, and reduced complications of hydrocephalus.
2,330,823
Role of Transient Elastography to Stage Fontan-Associated Liver Disease (FALD) in Adults with Single Ventricle Congenital Heart Disease Correction.
Fontan-associated liver disease (FALD) is an arising clinical entity that can occur long after a successful Fontan operation for correction of single ventricle (SV) congenital heart disease (CHD). Occurrence of FALD is characterized by liver cirrhosis and other hepatic complications, and determinates an increased morbidity and mortality. Currently, there is no consensus on how to stage FALD. We report here our experience by an observational study in 52 patients with SV-CHD after Fontan operation that were recruited through a period of 36 &#xb1; 9.3 months. All cases underwent lab tests and liver and cardiac imaging evaluation, including liver stiffness (LS) measurement by transient elastography (TE) (FibroScan<sup>&#xae;</sup>). According to selective criteria for liver disease, we identified 23/43 (53.5%) cases with advanced FALD that showed: older age (<i>p</i> &lt; 0.05), larger hepatic and cava veins diameter (<i>p</i> &lt; 0.05)<i>,</i> worsened NYHA class (<i>p</i> &lt; 0.05), abnormal lymphocytes (<i>p</i> &lt; 0.01), platelet count (<i>p</i> &lt; 0.05), and GGT, prothrombin time (INR), albumin and cystatin C levels (<i>p</i> &lt; 0.05), with respect to cases without advanced FALD. LS values were significantly increased in cases with advanced FALD, at cut-off values higher than 22 kPa (<i>p</i> &lt; 0.001). LS, and its combined score with spleen diameter and platelet count (LSPS) successfully helped to detect 100% of cases with portal hypertension (<i>p</i> &lt; 0.001). In conclusion, LS can be effective to stage FALD and to uncover cases with severe risk of complications, avoiding higher morbidity and mortality related to advanced FALD.
2,330,824
Interthalamic adhesion in humans: a gray commissure?
Interthalamic adhesion is an inconstant part of the human diencephalic neuroanatomy, which some histological studies have indicated it is a gray commissure and others a white commissure. Its presence has been associated with alterations in health status, including schizophrenia, psychotic states, and hydrocephalus. Thirty-one fresh human brains were evaluated randomly, to determine the presence of interthalamic adhesion and its histological composition, by way of lamina terminalis puncture of the third ventricle. Photographic records were taken and histological processes was performed by hematoxylin-eosin staining, in the case of the existence of the adhesion. It was found that 51.71% did present interthalamic adhesion, and on histological examination, no neuron bodies were found in the median part, which implies that does not correspond to a gray commissure, but interthalamic adhesion in humans is variable, with a predominance of glial cells. There is no gray commissure in human interthalamic adhesions.
2,330,825
Parkinsonism and cerebrospinal fluid disorders.
Although various motor manifestations can be seen in patients with cerebrospinal fluid (CSF) disorders, such as hydrocephalus or intracranial hypotension, the clinical presentation with parkinsonism is not clearly elucidated.</AbstractText>We searched the literature for studies describing the occurrence of parkinsonism in subjects with normal pressure hydrocephalus (NPH), obstructive hydrocephalus, and intracranial hypotension. We analyzed the clinical presentation (particularly with respect to bradykinesia, rigidity, rest tremor, and gait disturbance/postural instability) as well as the response to treatment.</AbstractText>Parkinsonism was most commonly reported in NPH patients. Although gait disturbance/postural instability is a well-known motor symptom of NPH, other cardinal signs include upper limb involvement or asymmetric presentation. As for obstructive hydrocephalus, parkinsonism was mainly observed in subjects with aqueductal stenosis and more often after shunt surgery. Patients with NPH or obstructive hydrocephalus rarely improved with levodopa therapy, while most subjects only improved with shunt surgery. Although the mechanism is still controversial, a functional involvement of nigrostriatal pathway has been hypothesized based on imaging studies and case reports. Brain imaging is also helpful for atypical cases of intracranial hypotension presenting with parkinsonism. Parkinsonism improved after treatment in such cases as well.</AbstractText>Studies exploring the relationship between CSF disorders and parkinsonism are mainly descriptive and their quality is generally poor. However, considering that these disorders can be treated, clinicians' awareness of the differential diagnosis is important and future studies better exploring the underlying pathophysiological mechanisms are warranted. This article is part of the Special Issue "Parkinsonism across the spectrum of movement disorders and beyond" edited by Joseph Jankovic, Daniel D. Truong and Matteo Bologna.</AbstractText>Copyright &#xa9; 2021 Elsevier B.V. All rights reserved.</CopyrightInformation>
2,330,826
Identification of a choroid plexus vascular barrier closing during intestinal inflammation.
Up to 40% of patients with inflammatory bowel disease present with psychosocial disturbances. We previously identified a gut vascular barrier that controls the dissemination of bacteria from the intestine to the liver. Here, we describe a vascular barrier in the brain choroid plexus (PVB) that is modulated in response to intestinal inflammation through bacteria-derived lipopolysaccharide. The inflammatory response induces PVB closure after gut vascular barrier opening by the up-regulation of the wingless-type, catenin-beta 1 (Wnt/&#x3b2;-catenin) signaling pathway, rendering it inaccessible to large molecules. In a model of genetically driven closure of choroid plexus endothelial cells, we observed a deficit in short-term memory and anxiety-like behavior, suggesting that PVB closure may correlate with mental deficits. Inflammatory bowel disease&#x2013;related mental symptoms may thus be the consequence of a deregulated gut&#x2013;brain vascular axis.
2,330,827
Posterior fossa decompression for children with Chiari I malformation and hydrocephalus.
Chiari I malformation (CMI) and hydrocephalus often coexist, with no clear understanding of the cause-and-effect relationship. In the absence of other associated etiologies, the traditional teaching has been to treat the hydrocephalus first, partly to minimize the risk of cerebrospinal fluid (CSF) leak from CMI decompression in the setting of elevated ICP. We describe a series of consecutive pediatric patients with CMI and hydrocephalus, the majority of whom were managed with posterior fossa decompression.</AbstractText>A retrospective review was conducted on consecutive children who presented to the senior author with both hydrocephalus and CMI, with emphasis on rationale for and outcomes of surgical intervention, including the need for additional surgery.</AbstractText>There were 14 patients aged 2&#xa0;weeks to 16&#xa0;years (median 2&#xa0;years) who presented with Chiari I and hydrocephalus. Four of these underwent posterior fossa decompression without duraplasty (PFD) as first-line therapy (one of whom eventually required duraplasty), 7 had PFD with duraplasty (PFDD), 1 received a ventriculoperitoneal shunt (VPS), and two had endoscopic third ventriculostomy (ETV). Of the 11 who had PFD/D, 9 (90%) had significant symptom improvement/resolution, 7 (55%) showed decrease in ventricle size, and 1 (10%) required VPS placement for persistent hydrocephalus. Both ETV patients improved clinically, and 1 showed decrease in ventricle size. There were no pseudomeningoceles, infections, or neurological deficits. One CSF leak occurred after an ETV and was successfully treated with wound revision.</AbstractText>In patients with both CMI and hydrocephalus, treating the CMI first in an effort to avoid a shunt can be safe and effective. In this series, PFDD in the setting of hydrocephalus did not result in CSF leak or pseudomeningocele. While limited by a small sample size, these data support a causative relationship between CMI and hydrocephalus.</AbstractText>&#xa9; 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.</CopyrightInformation>
2,330,828
Premature ventricular complexes from a left ventricular diverticulum: a case report.<Pagination><StartPage>551</StartPage><MedlinePgn>551</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1093/europace/euab227</ELocationID><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Sultankhonov</LastName><ForeName>Sardorkhon</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>The Heart Team Clinic, Yunusabad-11, Ahmad Donish St. 59/64, Tashkent, Uzbekistan.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Nedios</LastName><ForeName>Sotirios</ForeName><Initials>S</Initials><Identifier Source="ORCID">0000-0003-4222-6633</Identifier><AffiliationInfo><Affiliation>Department of electrophysiology, Heart Center, University of Leipzig, Leipzig, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Sadiyev</LastName><ForeName>Akmal</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>The Heart Team Clinic, Yunusabad-11, Ahmad Donish St. 59/64, Tashkent, Uzbekistan.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Yakubov</LastName><ForeName>Akmal</ForeName><Initials>A</Initials><AffiliationInfo><Affiliation>The Heart Team Clinic, Yunusabad-11, Ahmad Donish St. 59/64, Tashkent, Uzbekistan.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Yarbekov</LastName><ForeName>Rustam</ForeName><Initials>R</Initials><AffiliationInfo><Affiliation>American Hospital, Iqbol street, 14, Choshtepa, Tashkent region, Uzbekistan.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Jumaniyazov</LastName><ForeName>Rasul</ForeName><Initials>R</Initials><AffiliationInfo><Affiliation>The Heart Team Clinic, Yunusabad-11, Ahmad Donish St. 59/64, Tashkent, Uzbekistan.</Affiliation></AffiliationInfo></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>England</Country><MedlineTA>Europace</MedlineTA><NlmUniqueID>100883649</NlmUniqueID><ISSNLinking>1099-5129</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D004240" MajorTopicYN="Y">Diverticulum</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName><QualifierName UI="Q000000981" MajorTopicYN="N">diagnostic imaging</QualifierName><QualifierName UI="Q000601" MajorTopicYN="N">surgery</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006330" MajorTopicYN="Y">Heart Defects, Congenital</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006352" MajorTopicYN="N">Heart Ventricles</DescriptorName><QualifierName UI="Q000000981" MajorTopicYN="N">diagnostic imaging</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D018879" MajorTopicYN="Y">Ventricular Premature Complexes</DescriptorName><QualifierName UI="Q000175" MajorTopicYN="N">diagnosis</QualifierName><QualifierName UI="Q000209" MajorTopicYN="N">etiology</QualifierName><QualifierName UI="Q000601" MajorTopicYN="N">surgery</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="received"><Year>2021</Year><Month>3</Month><Day>27</Day></PubMedPubDate><PubMedPubDate PubStatus="accepted"><Year>2021</Year><Month>8</Month><Day>10</Day></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2021</Year><Month>10</Month><Day>21</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2022</Year><Month>4</Month><Day>8</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2021</Year><Month>10</Month><Day>20</Day><Hour>17</Hour><Minute>29</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">34669938</ArticleId><ArticleId IdType="doi">10.1093/europace/euab227</ArticleId><ArticleId IdType="pii">6406437</ArticleId></ArticleIdList></PubmedData></PubmedArticle><PubmedArticle><MedlineCitation Status="Publisher" Owner="NLM"><PMID Version="1">34669499</PMID><DateRevised><Year>2021</Year><Month>10</Month><Day>20</Day></DateRevised><Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1555-9823</ISSN><JournalIssue CitedMedium="Internet"><PubDate><Year>2021</Year><Month>Oct</Month><Day>20</Day></PubDate></JournalIssue><Title>The American surgeon</Title><ISOAbbreviation>Am Surg</ISOAbbreviation></Journal>Gastrocardiac Fistula as a Complication of Prior Bariatric Surgery.
Gastrocardiac fistula is a rare, typically fatal, diagnosis which has been reported infrequently in post-esophagectomy patients and, to a much lesser extent, patients with history of Roux-en-Y gastric bypass. This case describes a patient with a history of an eroded adjustable gastric band and subsequent removal with conversion to Roux-en-Y gastric bypass that initially presented with an upper gastrointestinal bleed. She was found to have a transdiaphragmatic fistula between the gastric lumen and left ventricle.
2,330,829
Association Between Sleep Disturbances With Neurodevelopmental Problems and Decreased Health-Related Quality of Life in Children With Fontan Circulation.
Background Children with Fontan circulation are known to be at increased risk for neurodevelopmental problems and decreased health-related quality of life (HRQOL), but many factors that may contribute to this risk are unknown. Sleep disturbances may be one previously unidentified factor that contributes to this risk. Methods and Results We analyzed data from the Pediatric Heart Network Fontan cross-sectional study to evaluate associations between a parent or child report of sleep disturbance with reported neurodevelopmental concerns and HRQOL in 558 children with Fontan circulation. Parent-reported sleep disturbance was present in 11% of participants and child-reported sleep disturbance was present in 15%. Parent-reported sleep disturbance was associated with a significantly higher risk of attention problems, anxiety, depression, behavioral problems, and developmental delay (<i>P</i>&lt;0.001 for all). Similarly, parent-reported disturbance was associated with decreased HRQOL on both parent and child-reported HRQOL (<i>P</i>&lt;0.001 for most domains). Child-reported sleep disturbances were associated with increased odds of anxiety, depression, and attention problems as well as worse HRQOL. These associations were present even after adjustment for cardiac, demographic, and socioeconomic factors that may affect HRQOL and neurodevelopmental status. Conclusions Sleep disturbances in children with Fontan circulation are associated with an increased risk of neurodevelopmental problems as well as reduced HRQOL compared with those without sleep disturbance. Better understanding of sleep disturbances is needed in children with Fontan circulation, as sleep disturbances may represent a reversible cause of neurodevelopmental problems and decreased HRQOL in this population.
2,330,830
Slit ventricle syndrome: Historical considerations, diagnosis, pathophysiology, and treatment review.
After the introduction of shunt treatment for the management of childhood hydrocephalus, a wide variety of complications related to this treatment modality have been recognized. The entity of slit ventricle syndrome (alternatively, symptomatic ventricular coaptation) is one of them, is frequently encountered in the pediatric population and its symptom complex resembles that of shunt failure. We conducted research on PubMed<sup>&#xae;</sup>, MEDLINE<sup>&#xae;</sup>, and Web of Science<sup>&#xae;</sup>, using the keywords: "slit ventricles," "slit ventricle syndrome," "SVS" and "ventricular coaptation." The aim of our review was to trace the advances made through the past decades, concerning our knowledge about the clinical characteristics, pathophysiology, and treatment options of this entity. The discrepancy among researchers about the offending etiology and the optimum treatment algorithm of this entity, as well as the necessity of an updated concept regarding shunt over drainage is analyzed. The multiple treatment modalities proposed and pathophysiologic mechanisms implicated for the treatment of slit ventricle syndrome illustrate the complexity of this entity. Consequently, the issue requires more detailed evaluation. In this review, we comment on all the main facets related to shunt over drainage and the resultant slit ventricle syndrome.
2,330,831
The entity of the trapped fourth ventricle: A review of its history, pathophysiology, and treatment options.
An isolated or trapped fourth ventricle is a relatively rare, although serious, adverse effect of hemorrhagic, infectious, or inflammatory processes that involve the central nervous system. This entity usually occurs after successful shunting of the lateral ventricles and may become clinically evident with the development of delayed clinical deterioration. This decline of the neurological status of the patient is evident after an initial period of improvement of the relevant symptoms. Surgical treatment options include cerebrospinal fluid shunting procedures, along with open surgical and endoscopic approaches. Complications related to its management are common and are related with obstruction of the fourth ventricular catheter, along with cranial nerve or brainstem dysfunction. We used the keywords: "isolated fourth ventricle," and "trapped fourth ventricle," in PubMed<sup>&#xae;</sup> and Web of Science<sup>&#xae;</sup>. Treatment of the trapped fourth ventricle remains a surgical challenge, although the neurosurgical treatment armamentarium has broadened. However, prompt recognition of the clinical and neurological findings that accompany any individual patient, in conjunction with the relevant imaging findings, is mandatory to organize our treatment plan on an individual basis. The current experience suggests that any individual intervention plan should be mainly based on the underlying pathological substrate of hydrocephalus. This could help us to preserve the patient's life, on an emergent basis, as well as to ensure an uneventful neurological outcome, maintaining at least the preexisting level of neurological function.
2,330,832
Suprasellar arachnoid cyst due to ectopic choroid plexus: case report.
Arachnoid cysts (ACs) are malformations that account for about 1% of all intracranial lesions. The aetiology and progression of these lesions have been debated, with one possible explanation being the production of cerebro-spinal fluid (CSF) by ectopic choroid plexus (CP). To our knowledge, only seven cases of ACs incorporating CP have been reported, and we believe this to be the first reported case of a suprasellar AC containing ectopic CP. A 1-year-old boy presented with developmental delay and macrocephaly. MRI scan revealed hydrocephalus due to a suprasellar AC. An endoscopic ventriculocisternostomy was undertaken. Intra-operatively, intra-cystic, pink frond-like tissue resembling choroid plexus was identified. Histologically, the cyst wall was composed of fibrous tissue, with layered arachnoid cells, while the frond-like tissue was composed of papillary structures in keeping with normal choroid plexus tissue. We postulate that the rest of the ectopic CP may have been trapped within the double layered arachnoid fold of the diencephalic leaf of Liliequist's membrane which may drive the formation and development of certain suprasellar ACs.
2,330,833
Acute right ventricular failure associated with pulmonary hypertension in pediatrics: understanding the hemodynamic profiles.
Pulmonary hypertension (PHTN) is a common pathology in pediatrics, arising from a diverse array of etiologies and manifesting in equally diverse patient populations. The inpatient management of these infants and children may be complicated by dynamic and at times severe increases in pulmonary vascular resistance (PVR) and right ventricular (RV) afterload. Yet absent are cognitively accessible heuristics in the field whereby providers can reconcile the various clinical manifestations they observe with an understanding of the cardiac physiology at play, and therefore, appropriate physiology-driven interventions. Described herein is a framework for understanding the pathophysiology of four clinical phenotypes which are driven by two echocardiographic patient characteristics: the presence or absence of an atrial communication and the capacity of the right ventricle to maintain ventricular-vascular coupling. Application of this paradigm may facilitate accurate interpretation of observed clinical data, and alignment of treatment strategies with the underlying pathophysiology.
2,330,834
Early Outcome of Endoscopic Third Ventriculostomy With Choroid Plexus Cauterization Versus Ventriculoperitoneal Shunt as Primary Treatment of Hydrocephalus in Children With Myelomeningocele: A Prospective Cohort Study.
Myelomeningocele is associated with hydrocephalus in 35% to 90% of cases. Hydrocephalus is usually treated with insertion of ventriculoperitoneal shunt; however, there is growing evidence that endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) is an alternative.</AbstractText>To compare the success rate and morbidity of ETV with CPC and ventriculoperitoneal shunt (VPS) as the primary treatment of hydrocephalus in patients with myelomeningocele.</AbstractText>A prospective study from January 2016 to February 2019, involving 46 patients with myelomeningocele who developed hydrocephalus after repair in a tertiary hospital in southwestern Nigeria. Biodata and preoperative features of hydrocephalus were documented. ETV&#xa0;+&#xa0;CPC or VPS was done using standard operative techniques. Patients were followed up monthly for 6 mo.</AbstractText>There were 23 patients in the ETV&#xa0;+&#xa0;CPC arm and 22 patients in the VPS arm. Morbidities were cerebrospinal fluid leak, 8.3% in the ETV&#xa0;+&#xa0;CPC arm and 4.5% in the VPS arm, wound dehiscence, 13.6% in the VPS arm, none in the ETV&#xa0;+&#xa0;CPC arm. At 6-mo follow-up, success rate for ETV&#xa0;+&#xa0;CPC was 60.9% and 59.1% for VPS, P&#xa0;=&#xa0;.9.</AbstractText>ETV&#xa0;+&#xa0;CPC had similar success rate with VPS at 6 mo with lower morbidity. ETV&#xa0;+&#xa0;CPC should be considered a viable alternative when treating patients with myelomeningocele and hydrocephalus.</AbstractText>&#xa9; Congress of Neurological Surgeons 2021.</CopyrightInformation>
2,330,835
Cannabidiol impairs the rewarding effects of methamphetamine: Involvement of dopaminergic receptors in the nucleus accumbens.
Cannabidiol, as component of cannabis, can potentially hinder the rewarding impact of drug abuse; however, its mechanism is ambiguous. Moreover, the nucleus accumbens (NAc), as a key area in the reward circuit, extensively receives dopaminergic projections from the ventral tegmentum area. To elucidate the role of accumbal D1 and D2 dopamine receptor families in Cannabidiol's inhibitory impact on the acquisition and expression phases of methamphetamine (MET), the conditioned place preference (CPP) procedure as a common method to assay reward characteristics of drugs was carried out. Six groups of rats were treated by various doses of SCH23390 or Sulpiride (0.25, 1, and 4&#xa0;&#x3bc;g/0.5&#xa0;&#x3bc;L) in the NAc as D1 or D2 dopamine receptor family antagonists, respectively, prior to infusion of Cannabidiol (10&#xa0;&#x3bc;g/5&#xa0;&#x3bc;L) in the lateral ventricle (LV) over conditioning phase in the acquisition experiments. In the second step of the study, animals received SCH23390 or Sulpiride in the NAc before Cannabidiol (50&#xa0;&#x3bc;g/5&#xa0;&#x3bc;L) infusion into the LV in the expression phase of MET to illuminate the influence of SCH23390 or Sulpiride on the inhibitory impact of Cannabidiol on the expression of MET-induced CPP. Intra-NAc administration of either SCH23390 or Sulpiride impaired Cannabidiol's suppressive impact on the expression phase, while just Sulpiride could suppress the Cannabidiol's impact on the acquisition phase of the MET-induced CPP. Also, the inhibitory impact of Sulpiride was stranger in both phases of MET reward. It seems that Cannabidiol prevents the expression and acquisition phases of MET-induced CPP partly through the dopaminergic system in the NAc.
2,330,836
A Neonatal ABO non-compatible heart transplant from a circulatory-determined death donor using NRP/Cold storage.
Donation after Circulatory death is gaining worldwide acceptance. Most protocols regard their first cases to be performed with donor and recipient in the same institution. Few records of children or distant procurement have been published.</AbstractText>Our institution was offered a heart from a 3-day-old, 3.4-kg child, blood group A, suffering irreversible encephalopathy. Parents accepted withdrawal of life-sustaining therapy and agreed to donation. The donor hospital was located 340&#xa0;km away. Concomitantly, a 2-month-old, 3.1&#xa0;kg, blood group type B and with non-compaction ventricles was awaiting for the heart transplant in our unit.</AbstractText>Thirty-seven minutes after withdrawal of life-sustaining therapy, the heart arrested. Five minutes afterwards, a sternotomy was performed. The supra-aortic vessels were clamped altogether. Aorta and right appendage were cannulated and connected to heart-lung machine. The innominate artery above the clamp was severed. The heart resumed spontaneous rhythm in less than 1&#xa0;min. Ventilation was restored and extracorporeal circulation was maintained for 32&#xa0;min. Upon cardiologic arrest, the graft was harvested as routinely. The heart was cold-stored and transported by plane to our Hospital. An orthotopic bicaval transplant was performed. Overall cold ischaemia was 245&#xa0;min. Ten weeks later, the child was discharged home in good condition.</AbstractText>Donation in circulatory death could increase the pool in neonates. Extracorporeal circulation proves successful for procurement in neonates. Distant procurement plus cold storage for donation in circulatory death is feasible. Donation in circulatory death and ABO non-compatible strategies are complementary to each other.</AbstractText>&#xa9; 2021 Wiley Periodicals LLC.</CopyrightInformation>
2,330,837
Fourth Ventricular Epidermoid Cyst - Case Series, Systematic Review and Analysis.
Epidermoid cysts are extra-axial, pearly white avascular lesions mostly found in the cerebellopontine region. They are slow-growing and mostly become symptomatic when they attain significant size. They do occur at other anatomical locations, but fourth ventricle is a rare location. Three representative cases with their outcomes are described here.</AbstractText>The systematic review was done with adherence to predefined criteria. The studied variables were age, gender, duration of symptoms (DOS), clinical features, hydrocephalus (HCP), extent of resection, postoperative complications, outcome, follow-up, and recurrence. Statistical analysis was done to identify predictive factors for outcome.</AbstractText>Final analysis included 58 studies containing 131 patients. The most common clinical feature was cerebellar dysfunction (93%). The most common cranial nerve involved was the abducens nerve (n</i> = 37, 28.46%). Preoperative HCP was present in nearly a third (35%) of patients. The outcomes were not different with age (P</i> = 0.23), gender (P</i> = 0.74), DOS (P</i> = 0.09), and HCP (P</i> = 0.50). Improved outcomes were associated with total resections (P</i> = 0.001), absence of preoperative cranial nerve dysfunctions (P</i> = 0.004), and presentation with features of raised intracranial pressure (P</i> = 0.005). Longer DOS (mean 76.74 months) was associated with significantly increased cranial nerve nuclei involvement (P</i> = 0.03). Aseptic meningitis was reported in 14.5% of cases. Recurrences were infrequently reported (n</i> = 9).</AbstractText>Although the fourth ventricular epidermoid lesions are difficult to detect in an innocuous stage, when found, they should be extirpated early and totally, as a longer DOS leads to cranial nerve dysfunctions and suboptimal outcomes.</AbstractText>Copyright: &#xa9; 2021 Asian Journal of Neurosurgery.</CopyrightInformation>
2,330,838
Transplantation after Mustard operation for transposition of the great arteries.
As long-term outcomes of congenital heart diseases improve, the probability of adult patients presenting for heart transplantation for late failure of congenitally corrected heart disease also increases. In patients with dextro-transposition of the great arteries (d-TGA) who were initially treated in the era of Mustard or Senning procedures and before Jatene procedure was introduced, progressive systemic right ventricular failure represents a problem in the very long-term follow-up. We report a rare case of heart transplantation as a third operation 36 years after Mustard procedure in a patient with d-TGA experiencing late failure of the systemic right ventricle.
2,330,839
Expression of CCDC85C, a causative protein for hydrocephalus, and intermediate filament proteins during lateral ventricle development in rats.
Coiled-coil domain containing 85c (Ccdc85c) is a causative gene for genetic hydrocephalus and subcortical heterotopia with frequent brain hemorrhage. In the present study, we examined the expression pattern of CCDC85C protein and intermediate filament proteins, such as nestin, vimentin, GFAP, and cytokeratin AE1/AE3, during lateral ventricle development in rats. CCDC85C was expressed in the neuroepithelial cells of the dorsal lateral ventricle wall, diminishing with development and almost disappearing at postnatal day 20. By immunoelectron microscopy, CCDC85C was localized in the cell-cell junction and apical membrane. The expression of nestin and vimentin was decreased in the wall of the lateral ventricle in manner similar to CCDC85C, but GFAP expression started immediately after birth and became stronger with age. Moreover, cytokeratin expression was found at postnatal day 13 and increased at postnatal day 20 in conjunction with the disappearance of CCDC85C expression. Taken together, CCDC85C is expressed in the cell-cell junctions lining the wall of the lateral ventricle and plays a role in neural development with other intermediate filaments in the embryonic and postnatal periods. Our chronological study will help to relate CCDC85C protein with intermediate filaments to elucidate the detailed role of CCDC85C protein during neurogenesis.
2,330,840
[Subacute cognitive impairment and urinary retention due to primary central nervous system post-transplant lymphoproliferative disorder: a case report].
We report a 66-year-old man with primary central nervous system post-transplant lymphoproliferative disorder (PCNS-PTLD). He had received a living-donor kidney transplantation at the age of 64 years. Although he had a good postoperative course by continuing to take oral immunosuppressive agents, he was admitted to our hospital for subacute cognitive impairment and urinary retention two years after the transplantation. Brain MRI revealed high-intensity lesions on FLAIR and T<sub>2</sub>-weighted images in the left parietal operculum, deep white matter around the anterior horn of the lateral ventricle, and the genu and splenium of the corpus callosum. A part of these lesions showed ring enhancement. The cerebrospinal fluid examination revealed lymphocytic pleocytosis, elevation of protein level, and mild hypoglycorrhachia. Blood tests showed no abnormalities except for positive serum VCA-IgG antibody of Epstein-Barr virus. A brain biopsy was performed and diagnosis of PCNS-PTLD was made. There was no evidence of systemic PTLD. We reduced the dose of immunosuppressive agents and started the initial treatment with methylprednisolone pulse therapy. The patient showed a partial response to the treatment and transferred to another hospital for subsequent chemotherapy. PTLD is an important post-transplant complication that can affect the patient's prognosis. The incidence of PTLD is increasing with the growing numbers of transplantations and older age of donors and recipients. Although CNS involvement is known to be rare, PCNS-PTLD is an important differential diagnosis when symptoms of CNS origin develop in post-transplant patients.
2,330,841
[A patient with recurrent multiloculated hydrocephalus after Cryptococcal ventriculitis].
We report here a rare case of adult-onset multiloculated hydrocephalus (MLH) after Cryptococcal meningitis. A 63-year-old man had Cryptococcal ventriculitis in 2011, and he recovered with treatment of antimycotic drugs. However, he was admitted again because of disorientation and amnesia, and brain MRI showed dilation of the inferior horn of the left lateral ventricle. He underwent a ventriculoperitoneal shunt (VPS) for noncommunicating hydrocephalus in 2019, and the disorientation and amnesia improved. One year after the VPS, he was admitted because of urinary dysfunction and gait disturbance. Brain MRI showed dilation of the bilateral anterior horns of the lateral ventricles. He underwent an additional VPS into the space in 2020, and urinary dysfunction and gait disturbance improved. This case was supposed that the symptom in agreement with the dilated ventricle by MLH was shown.
2,330,842
Relationship Between Brain Volumes and Objective Balance and Gait Measures in Parkinson's Disease.
Instrumented measures of balance and gait measure more specific balance and gait impairments than clinical rating scales. No prior studies have used objective balance/gait measures to examine associations with ventricular and brain volumes in people with Parkinson's disease (PD).</AbstractText>To test the hypothesis that larger ventricular and smaller cortical and subcortical volumes are associated with impaired balance and gait in people with PD.</AbstractText>Regional volumes from structural brain images were included from 96 PD and 50 control subjects. Wearable inertial sensors quantified gait, anticipatory postural adjustments prior to step initiation (APAs), postural responses to a manual push, and standing postural sway on a foam surface. Multiple linear regression models assessed the relationship between brain volumes and balance/gait and their interactions in PD and controls, controlling for sex, age and corrected for multiple comparisons.</AbstractText>Smaller brainstem and subcortical gray matter volumes were associated with larger sway area in people with PD, but not healthy controls. In contrast, larger ventricle volume was associated with smaller APAs in healthy controls, but not in people with PD. A sub-analysis in PD showed significant interactions between freezers and non-freezers, in several subcortical areas with stride time variability, gait speed and step initiation.</AbstractText>Our models indicate that smaller subcortical and brainstem volumes may be indicators of standing balance dysfunction in people with PD whereas enlarged ventricles may be related to step initiation difficulties in healthy aging. Also, multiple subcortical region atrophy may be associated with freezing of gait in PD.</AbstractText>
2,330,843
Rethinking modeling Alzheimer's disease progression from a multi-task learning perspective with deep recurrent neural network.
Alzheimer's disease (AD) is a severe neurodegenerative disorder that usually starts slowly and progressively worsens. Predicting the progression of Alzheimer's disease with longitudinal analysis on the time series data has recently received increasing attention. However, training an accurate progression model for brain network faces two major challenges: missing features, and the small sample size during the follow-up study. According to our analysis on the AD progression task, we thoroughly analyze the correlation among the multiple predictive tasks of AD progression at multiple time points. Thus, we propose a multi-task learning framework that can adaptively impute missing values and predict future progression over time from a subject's historical measurements. Progression is measured in terms of MRI volumetric measurements, trajectories of a cognitive score and clinical status. To this end, we propose a new perspective of predicting the AD progression with a multi-task learning paradigm. In our multi-task learning paradigm, we hypothesize that the inherent correlations exist among: (i). the prediction tasks of clinical diagnosis, cognition and ventricular volume at each time point; (ii). the tasks of imputation and prediction; and (iii). the prediction tasks at multiple future time points. According to our findings of the task correlation, we develop an end-to-end deep multi-task learning method to jointly improve the performance of assigning missing value and prediction. We design a balanced multi-task dynamic weight optimization. With in-depth analysis and empirical evidence on Alzheimer's Disease Neuroimaging Initiative (ADNI), we show the benefits and flexibility of the proposed multi-task learning model, especially for the prediction at the M60 time point. The proposed approach achieves 5.6%, 5.7%, 4.0% and 11.8% improvement with respect to mAUC, BCA and MAE (ADAS-Cog13 and Ventricles), respectively.
2,330,844
Utility of multi-material three-dimensional print model in preoperative simulation for glioma surgery.
Although the three-dimensional (3D) printing technology has spread in the field of neurosurgery, the use of 3D print models concerning glioma surgery has rarely reported. For glioma surgery, some preoperative and intraoperative assistive methods have been developed to avoid injury to the cortex and fiber that are related to the neurological function. Furthermore, in order to perform preoperative simulation of glioma surgery, we created a 3D print model using a multi-material 3D printer that provided the flexibility of adjusting the color, hardness, and translucency of each structure arbitrarily. The use of 3D print model was demonstrated in one case involving an intramedullary tumor in the right temporal lobe. The tumor, optic radiation, brain parenchyma, tentorium, ventricle, and sinus were constructed in a single model in one printing process. Design of the degree of resection, insertion of the fence-post, and tumor resection paying attention to the optic radiation were simulated preoperatively using this model. The surgery was performed generally as the simulation and gross total removal of the tumor was achieved. This model was useful for understanding the degree of resection, adequate insertion of the fence-post, and the relationship of the tumor with other important structures. A variety of printing materials contributed to make the model realistic and to understand anatomical relationship. In conclusion, the 3D print model can supplement an image of some portions that are not visible perioperatively and serve as a preoperative assistant modality.
2,330,845
Non-invasive assessment of cerebrospinal fluid flow dynamics using phase-contrast magnetic resonance imaging in communicating hydrocephalus.
This work aims to evaluate the changes in cerebrospinal fluid (CSF) hydrodynamics in patients diagnosed with communicating hydrocephalus. Besides, we establish the relationship between CSF flow dynamic parameters on the midbrain aqueduct and intracranial pressure (ICP). CSF hydrodynamics analysis was performed using Phase-Contrast Magnetic Resonance Imaging (PC-MRI) techniques on the midbrain aqueduct of 41 patients diagnosed with communicating hydrocephalus and 22 healthy volunteers. The correlation between CSF average flow in the midbrain aqueduct and intracranial pressure measured by Lumbar Puncture (LP) was assessed in patients with hydrocephalus. Pearson correlation coefficient was used to establish the correction between the average CSF flow of midbrain aqueduct and ICP. CSF dynamic parameters of the midbrain aqueduct in hydrocephalus patients, including peak positive velocity (7.348&#x202f;cm/s), average velocity (0.623&#x202f;cm/s), average flow (50.799&#x202f;mm<sup>3</sup>/s), and regions of interest (ROI) area (9.978&#x202f;mm<sup>2</sup>) were significantly higher than in the healthy controls (p&#x202f;&lt;&#x202f;0.05). This was after adjusting the age, gender, heart rate, systolic blood pressure, diastolic blood pressure, and body mass index. However, only the peak negative velocity of the midbrain aqueduct did not significantly differ between the groups (p&#x202f;=&#x202f;0.209). A positive correlation was noted between the average flow (AF) of the midbrain aqueducts and ICP in hydrocephalus patients (y (AF)&#x202f;=&#x202f;0.386&#xd7; (ICP)-33.738, r&#x202f;=&#x202f;0.787, p&#x202f;&lt;&#x202f;0.05). Reference data of CSF flow dynamic parameters was obtained through the PC-MRI in middle-aged healthy volunteers and communicating hydrocephalus patients. Although the sample size was constrained, this study has significant contributions. For instance, a significant correlation was noted between the average CSF flow of the aqueduct and ICP. This therefore provides a reference for clinicians to monitor ICP in patients with hydrocephalus.
2,330,846
High-resistance proximal "scaled" ventricular catheters.
Prove the concept of high-resistance proximal catheters for valve-independent treatment of hydrocephalus.</AbstractText>A preliminary design process yielded optimal high-resistance proximal ventricular catheters with a "scaled" design and parallel-oriented, U-shaped inlets. Prototypes were manually constructed using carving tools to stamp through silicone tubings. A testing apparatus was developed to simulate cerebrospinal fluid flow through a catheter, and the prototypes were tested against a control catheter for exhibition of an "on/off" phenomenon whereby no flow occurs at low pressures, and flow begins beyond a pressure threshold. Flow distribution was visualized with India ink. Regression analysis was performed to determine linearity.</AbstractText>The new designs showed varying amounts of improved flow control with the "scaled" design showing the most practical flow rate control across various pressures, compared to the standard catheter; however, no true "on/off" phenomenon was observed. The "scaled" design showed various degrees of dynamism; its flow rate can be time dependent, and certain maneuvers such as flushing and bending increased flow rate temporarily. Variation in the number of inlets within each "scaled" prototype also affected flow rate. Contrastingly, the flow rate of standard catheters was found to be independent of the number of inlet holes. Ink flow showed even flow distribution in "scaled" prototypes.</AbstractText>This initial feasibility study showed that high-resistance ventricular catheters can be designed to mimic the current/valved system. The "scaled" design demonstrated the best flow control, and its unique features were characterized.</AbstractText>&#xa9; 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.</CopyrightInformation>
2,330,847
Does ventricle size contribute to cognitive outcomes in posthemorrhagic hydrocephalus? Role of early definitive intervention.
Posthemorrhagic hydrocephalus (PHH) is associated with significant morbidity, smaller hippocampal volumes, and impaired neurodevelopment in preterm infants. The timing of temporary CSF (tCSF) diversion has been studied; however, the optimal time for permanent CSF (pCSF) diversion is unknown. The objective of this study was to determine whether cumulative ventricle size or timing of pCSF diversion is associated with neurodevelopmental outcome and hippocampal size in preterm infants with PHH.</AbstractText>Twenty-five very preterm neonates (born at &#x2264; 32 weeks' gestational age) with high-grade intraventricular hemorrhage (IVH), subsequent PHH, and pCSF diversion with a ventriculoperitoneal shunt (n = 20) or endoscopic third ventriculostomy (n = 5) were followed until 2 years of age. Infants underwent serial cranial ultrasounds from birth until 1 year after pCSF diversion, brain MRI at term-equivalent age, and assessment based on the Bayley Scales of Infant and Toddler Development, Third Edition, at 2 years of age. Frontooccipital horn ratio (FOHR) measurements were derived from cranial ultrasounds and term-equivalent brain MRI. Hippocampal volumes were segmented and calculated from term-equivalent brain MRI. Cumulative ventricle size until the time of pCSF diversion was estimated using FOHR measurements from each cranial ultrasound performed prior to permanent intervention.</AbstractText>The average gestational ages at tCSF and pCSF diversion were 28.9 and 39.0 weeks, respectively. An earlier chronological age at the time of pCSF diversion was associated with larger right hippocampal volumes on term-equivalent MRI (Pearson's r = -0.403, p = 0.046) and improved cognitive (r = -0.554, p = 0.047), motor (r = -0.487, p = 0.048), and language (r = -0.414, p = 0.021) outcomes at 2 years of age. Additionally, a smaller cumulative ventricle size from birth to pCSF diversion was associated with larger right hippocampal volumes (r = -0.483, p = 0.014) and improved cognitive (r = -0.711, p = 0.001), motor (r = -0.675, p = 0.003), and language (r = -0.618, p = 0.011) outcomes. There was no relationship between time to tCSF diversion or cumulative ventricle size prior to tCSF diversion and neurodevelopmental outcome or hippocampal size. Finally, a smaller cumulative ventricular size prior to either tCSF diversion or pCSF diversion was associated with a smaller ventricular size 1 year after pCSF diversion (r = 0.422, p = 0.040, R2 = 0.178 and r = 0.519, p = 0.009, R2 = 0.269, respectively).</AbstractText>In infants with PHH, a smaller cumulative ventricular size and shorter time to pCSF diversion were associated with larger right hippocampal volumes, improved neurocognitive outcomes, and reduced long-term ventriculomegaly. Future prospective randomized studies are needed to confirm these findings.</AbstractText>
2,330,848
Radiologic and Clinical Outcomes of Stereotactic Radiosurgery for Intraventricular Metastases.
The optimal management of intraventricular metastases remains debatable. The aim of this study is to define the safety and efficacy of Gamma-Knife radiosurgery in the treatment of intraventricular metastases.</AbstractText>This retrospective, single-center study involved patients that were treated with stereotactic radiosurgery (SRS) for intraventricular metastases. The study end points included SRS-related toxicity, local and distal intracranial tumor control, as well as the incidence of post-treatment hydrocephalus and leptomeningeal dissemination. Factors associated with radiologic and clinical outcomes were also analyzed.</AbstractText>The cohort included 17 consecutive patients who underwent stereotactic radiosurgery for treatment of 41 intracranial metastases, of which 23 were primary intraventricular (intraventricular metastasis). Median overall survival from primary tumor diagnosis and from SRS treatment were 28 and 5 months, respectively. With a median radiological follow-up of 3 (interquartile range 3) months, 7 patients (41.18%) experienced overall intracranial disease progression, whereas 7 (27.27%) intraventricular metastases progressed radiologically. Four (23.53%) and 3 (17.65%) patients developed hydrocephalus and leptomeningeal dissemination post-SRS, respectively. Four patients (23.53%) died due to intracranial disease progression.</AbstractText>SRS offers a reasonable chance of local tumor control for patients with intraventricular brain metastasis. However, the risk of hydrocephalus and leptomeningeal spread of disease is not inconsequential and merits close follow-up for patients with brain metastasis involving the ventricular system.</AbstractText>Copyright &#xa9; 2021 Elsevier Inc. All rights reserved.</CopyrightInformation>
2,330,849
Inhibition of progesterone receptor membrane component-1 exacerbates neonatal hypoxic-ischemic cerebral damage in male mice.
This study investigated the expression of progesterone receptor membrane component 1 (pgrmc1) in the brains of male and female mice, and the effect of inhibiting pgrmc1 on neonatal hypoxic-ischemic (HI) cerebral injury in male mice. A mouse model of neonatal HI brain injury was established, and AG205, a specific antagonist of pgrmc1, was injected into the left lateral cerebral ventricle 1&#xa0;h before HI. Histological staining, behavior testing, Western blots, and quantitative PCR (qPCR) were employed to evaluate pgrmc1 expression, brain damage, neurological function, and molecular mechanisms. Results demonstrated that the mRNA and protein levels of pgrmc1 increased significantly in the cortex and hippocampus 72&#xa0;h after HI without sex differences. The inhibition of pgrmc1 exacerbated the neonatal brain damage in the acute stage of HI in male mice as seen in the increase in brain water content, infarction area, and neuronal death. Inhibition of pgrmc1 also aggravated the neurological dysfunction and anxiety induced by HI brain injury. In addition, inhibition of pgrmc1 activated the NF-kB signaling and NF-&#x3ba;B-mediated cytokines, and inhibited BDNF/PI3K/AKT pathway in the brains of the newborn HI mice. The results indicated that pgrmc1 inhibition exacerbated the brain damage in newborn male mice subjected to HI by activating I&#x3ba;B&#x3b1;/NF&#x3ba;B signaling and inhibiting BDNF/PI3K/Akt pathway.
2,330,850
Suicide attempt induced by drug-induced leukoencephalopathy: A case report.
Leukoencephalopathy is identified during the administration of anticancer drugs. Symptoms vary from neurological symptoms to psychiatric symptoms depending on the site of damage. There have been no previous reports of suicide attempts due to drug-induced leukoencephalopathy.</AbstractText>The patient was diagnosed with diffuse large B-cell lymphoma (DLBCL) infiltrating the pharyngeal lesion. Rituximab&#xa0;+&#xa0;methotrexate&#xa0;+&#xa0;oncovin&#xa0;+&#xa0;procarbazine (R-MPV) therapy, a methotrexate-containing chemotherapy, was initiated. At the end of the fifth course, the patient attempted suicide by hanging with an appliance cord, which was associated with delusion. A head MRI scan showed no evidence of lymphoma recurrence, but white matter lesions around the ventricles showed progression.</AbstractText>We report the case of a patient in whom drug-induced leukoencephalopathy related to methotrexate led to a suicide attempt. In addition to monitoring brain tumors, daily monitoring of psychiatric and neurological symptoms is important for patients with methotrexate-induced encephalopathy.</AbstractText>&#xa9; 2021 The Authors. Neuropsychopharmacology Reports published by John Wiley &amp; Sons Australia, Ltd on behalf of The Japanese Society of Neuropsychopharmacology.</CopyrightInformation>
2,330,851
Hemodynamic Response to Calcium Chloride Boluses in Single-Ventricle Patients with Parallel Circulation.
The purpose of this study is to assess the effect of calcium bolus in response to a hypotensive episode by assessing high-fidelity hemodynamic data obtained from children with single-ventricle physiology with parallel circulation. Single-center, retrospective analysis of hemodynamic data after calcium bolus administrations within the first 2&#xa0;weeks post-surgery. Time intervals were the baseline (-&#x2009;60 to&#x2009;-&#x2009;10&#xa0;min); the hypotensive episode (-&#x2009;10 to 0&#xa0;min); time point zero at the bolus administration; and the response (0 to 60&#xa0;min). The main responses assessed were the peak increase in mean blood pressure (mBP), duration of the response after the bolus, and markers of oximetric effects. These analyses included 128 boluses in 63 patients. Of the total boluses analyzed, 80% increased the mBP by 5&#xa0;mmHg or higher with the effect lasting at least 10&#xa0;min, whereas 10% of the boluses analyzed increased the mBP by 20&#xa0;mmHg or higher with the effect lasting at least 50&#xa0;min. The boluses induced a significant increase in arterial oxygen saturation and an upward trend in pulmonary-to-systemic flow ratio, without increasing renal or cerebral oxygen extraction ratios. Calcium chloride boluses are an effective rescue medication for hypotensive episodes in children with parallel circulation. They lead to an improvement in mBP, as well as an increase in pulmonary-to-systemic blood flow ratio. More importantly, these boluses do not compromise systemic oxygen delivery.
2,330,852
Changes in mitochondrial biogenesis and fatty liver indicators in rat following continuous and high intensity interval training.
Oxidative stress and mitochondrial dysfunction can be tracked down in most liver diseases like non-alcoholic fatty liver disease (NAFLD). The most recommended preventative method is lifestyle modification, especially exercise. The aim of this study was the investigation of changes in the indexes of mitochondrial biogenesis and fatty liver indicators in rat following continuous and high intensity interval training.</AbstractText>Thirty healthy male rats were divided into three control (C=10), Continuous swimming training (CT=10) and High intensity interval swimming training groups (HIIT=10). The training groups performed their specific exercises 5 days a week for 8 weeks. 24 h after the last training session in order to prepare the serum, a blood sample was taken from the left ventricle of the rats. In addition, liver tissue was extracted and the SIRT3, PGC-1&#x3b1;, GSH:GSSG, MDA, LDL, HDL, LDL:HDL, TG, TC, AST, ALT and FBS variables were measured by ELISA and analysis of blood biochemistry.</AbstractText>Continuous training (CT) increased the levels of PGC-1&#x3b1;, SIRT3 and significantly reduced LDL, LDL:HDL, TG and FBS (P&lt;0.05) levels. High-intensity interval training (HIIT) caused a significant increase in SIRT3 and a significant decrease in FBS (P&lt;0.05) levels.</AbstractText>Adaptations resulting from further aerobic exercise can increase mitochondrial biogenesis factors such as PGC-1&#x3b1; and SIRT3 in hepatocytes, improve this process in hepatocytes, and ultimately improve the fatty liver markers. Therefore, CT may be more effective than HIIT in preventing fatty liver disease.</AbstractText>
2,330,853
Preclinical assessment of thrombin-preconditioned human Wharton's jelly-derived mesenchymal stem cells for neonatal hypoxic-ischaemic brain injury.
Hypoxic-ischaemic encephalopathy (HIE) is a type of brain injury affecting approximately 1&#xa0;million newborn babies per year worldwide, the only treatment for which is therapeutic hypothermia. Thrombin-preconditioned mesenchymal stem cells (MSCs) exert neuroprotective effects by enriching cargo contents and boosting exosome biogenesis, thus showing promise as a new therapeutic strategy for HIE. This study was conducted to evaluate the tissue distribution and potential toxicity of thrombin-preconditioned human Wharton's jelly-derived mesenchymal stem cells (th-hWJMSCs) in animal models before the initiation of clinical trials. We investigated the biodistribution, tumorigenicity and general toxicity of th-hWJMSCs. MSCs were administered the maximum feasible dose (1&#xa0;&#xd7;&#xa0;10<sup>5</sup> cells/10&#xa0;&#xb5;L/head) once, or at lower doses into the cerebral ventricle. To support the clinical use of th-hWJMSCs for treating brain injury, preclinical safety studies were conducted in newborn Sprague-Dawley rats and BALB/c nude mice. In addition, growth parameters were evaluated to assess the impact of th-hWJMSCs on the growth of newborn babies. Our results suggest that th-hWJMSCs are non-toxic and non-tumorigenic in rodent models, survive for up to 7&#xa0;days in the brain and hold potential for HIE therapy.
2,330,854
Development and application of a diagnostic and severity scale to grade post-operative pediatric cerebellar mutism syndrome.
The post-operative pediatric cerebellar mutism syndrome (CMS) affects about one-third of children and adolescents following surgical removal of a posterior fossa tumor (PFT). According to the Posterior Fossa Society consensus working definition, CMS is characterized by delayed-onset mutism/reduced speech and emotional lability after cerebellar or 4th ventricle tumor surgery in children, and is frequently accompanied by additional features such as hypotonia and oropharyngeal dysfunction/dysphagia. The main objective of this work was to develop a diagnostic scale to grade CMS duration and severity. Thirty consecutively referred subjects, aged 1-17&#xa0;years (median 8&#xa0;years, IQR 3-10), were evaluated with the proposed Post-Operative Pediatric CMS Survey after surgical resection of a PFT and, in case of CMS, for 30&#xa0;days after the onset (T<sub>0</sub>) or until symptom remission. At day 30 (T<sub>1</sub>), CMS was classified into mild, moderate, or severe according to the proposed scale. CMS occurred in 13 patients (43%, 95% C.I.: 25.5-62.6%), with mild severity in 4 cases (31%), moderate in 4 (31%), and severe in 5 (38%). At T<sub>1</sub>, longer symptom persistence was associated with greater severity (p&#x2009;=&#x2009;0.01). Greater severity at T<sub>0</sub> predicted greater severity at T<sub>1</sub> (p&#x2009;=&#x2009;0.0001). Children with a midline tumor location and those aged under 5&#xa0;years at diagnosis were at higher risk of CMS (p&#x2009;=&#x2009;0.025 and p&#x2009;=&#x2009;0.008, respectively). In conclusion, the proposed scale is a simple and applicable tool for estimating the severity of CMS at its onset, monitoring its course over time, and providing an early prognostic stratification to guide treatment decisions.
2,330,855
Modified Protocol to Enable the Study of Hemorrhage and Hematoma in a Traumatic Brain Injury Mouse Model.
To date, many studies using the controlled cortical impact (CCI) mouse model of traumatic brain injury (TBI) have presented results without presenting the pathophysiology of the injury-core itself or the temporal features of hemorrhage (Hrr). This might be owing to the removal of the injury-core during the histological procedure. We therefore developed a modified protocol to preserve the injury-core. The heads of mice were obtained after perfusion and were post-fixed. The brains were then harvested, retaining the ipsilateral skull bone; these were post-fixed again and sliced using a cryocut. To validate the utility of the procedure, the temporal pattern of Hrr depending on the impacting depth was analyzed. CCI-TBI was induced at the following depths: 1.5 mm (mild Hrr), 2.5 mm (moderate Hrr), and 3.5 mm (severe Hrr). A pharmacological study was also conducted using hemodynamic agents such as warfarin (2 mg/kg) and coagulation factor VIIa (Coa-VIIa, 1 mg/kg). The current protocol enabled the visual observation of the Hrr until 7 days. Hrr peaked at 1-3 days and then decreased to the normal range on the seventh day. It expanded from the affected cortex (mild) to the periphery of the hippocampus (moderate) and the brain ventricle (severe). Pharmacological studies showed that warfarin pre-treatment produced a massively increased Hrr, concurrent with the highest mortality rate and brain injury. Coa-VIIa reduced the side effects of warfarin. Therefore, these results suggest that the current method might be suitable to conduct studies on hemorrhage, hematoma, and the injury-core in experiments using the CCI-TBI mouse model.
2,330,856
The wireless pacemaker is on again; from electro-stimulation to synchronization.
Leadless stimulation of the right ventricle is now a reality, especially in patients with very specific indications and clinical characteristics, even in the absence of randomized studies to support its use. The reduction of device costs and the refinement of atrioventricular synchronization algorithms will sanction its greater diffusion in the future. The possibility of using leadless technology also for resynchronization therapy, on the other hand, is currently a promising option but, pending randomized studies with robust case histories and adequate follow-ups, it should still be considered as a niche therapy, to be limited to centres highly specialized and in patients in whom conventional resynchronization has been impossible or ineffective.
2,330,857
Introducing dorsoventral patterning in adult regenerating lizard tails with gene-edited embryonic neural stem cells.
Lizards regenerate amputated tails but fail to recapitulate the dorsoventral patterning achieved during embryonic development. Regenerated lizard tails form ependymal tubes (ETs) that, like embryonic tail neural tubes (NTs), induce cartilage differentiation in surrounding cells via sonic hedgehog (Shh) signaling. However, adult ETs lack characteristically roof plate-associated structures and express Shh throughout their circumferences, resulting in the formation of unpatterned cartilage tubes. Both NTs and ETs contain neural stem cells (NSCs), but only embryonic NSC populations differentiate into roof plate identities when protected from endogenous Hedgehog signaling. NSCs were isolated from parthenogenetic lizard embryos, rendered unresponsive to Hedgehog signaling via CRISPR/Cas9 gene knockout of smoothened (Smo), and implanted back into clonally-identical adults to regulate tail regeneration. Here we report that Smo knockout embryonic NSCs oppose cartilage formation when engrafted to adult ETs, representing an important milestone in the creation of regenerated lizard tails with dorsoventrally patterned skeletal tissues.
2,330,858
Novel Human Insulin Isoforms and C&#x3b1;-Peptide Product in Islets of Langerhans and Choroid Plexus.
Human insulin (<i>INS</i>) gene diverged from the ancestral genes of invertebrate and mammalian species millions of years ago. We previously found that mouse insulin gene (<i>Ins2</i>) isoforms are expressed in brain choroid plexus (ChP) epithelium cells, where insulin secretion is regulated by serotonin and not by glucose. We further compared human <i>INS</i> isoform expression in postmortem ChP and islets of Langerhans. We uncovered novel <i>INS</i> upstream open reading frame isoforms and their protein products. In addition, we found a novel alternatively spliced isoform that translates to a 74-amino acid (AA) proinsulin containing a shorter 19-AA C-peptide sequence, herein designated C&#x3b1;-peptide. The middle portion of the conventional C-peptide contains &#x3b2;-sheet (GQVEL) and hairpin (GGGPG) motifs that are not present in C&#x3b1;-peptide. Islet amyloid polypeptide (<i>IAPP</i>) is not expressed in ChP, and its amyloid formation was inhibited in vitro more efficiently by C&#x3b1;-peptide than by C-peptide. Of clinical relevance, the ratio of the 74-AA proinsulin to proconvertase-processed C&#x3b1;-peptide was significantly increased in islets from type 2 diabetes mellitus autopsy donors. Intriguingly, 100 years after the discovery of insulin, we found that <i>INS</i> isoforms are present in ChP from insulin-deficient autopsy donors.
2,330,859
Longitudinal impact on rat cardiac tissue transcriptomic profiles due to acute intratracheal inhalation exposures to isoflurane.
Isoflurane (ISO) is a widely used inhalation anesthetic in experiments with rodents and humans during surgery. Though ISO has not been reported to impart long-lasting side effects, it is unknown if ISO can influence gene regulation in certain tissues, including the heart. Such changes could have important implications for use of this anesthetic in patients susceptible to heart failure/other cardiac abnormalities. To test if ISO could alter gene regulation/expression in heart tissues, and if such changes were reversible, prolonged, or late onset with time, SHR (spontaneously hypertensive) rats were exposed by intratracheal inhalation to a 97.5% air/2.5% ISO mixture on two consecutive days (2 hr/d). Control rats breathed filtered air only. On Days 1, 30, 240, and 360 post-exposure, rat hearts were collected and total RNA was extracted from the left ventricle for global gene expression analysis. The data revealed differentially-expressed genes (DEG) in response to ISO (compared to na&#xef;ve control) at all post-exposure timepoints. The data showed acute ISO exposures led to DEG associated with wounding, local immune function, inflammation, and circadian rhythm regulation at Days 1 and 30; these effects dissipated by Day 240. There were other significantly-increased DEG induced by ISO at Day 360; these included changes in expression of genes associated with cell signaling, differentiation, and migration, extracellular matrix organization, cell-substrate adhesion, heart development, and blood pressure regulation. Examination of consistent DEG at Days 240 and 360 indicated late onset DEG reflecting potential long-lasting effects from ISO; these included DEG associated with oxidative phosphorylation, ribosome, angiogenesis, mitochondrial translation elongation, and focal adhesion. Together, the data show acute repeated ISO exposures could impart variable effects on gene expression/regulation in the heart. While some alterations self-resolved, others appeared to be long-lasting or late onset. Whether such changes occur in all rat models or in humans remains to be investigated.
2,330,860
Cardiac involvement in patients recovered from COVID-19 identified using left ventricular longitudinal strain.<Pagination><StartPage>51</StartPage><EndPage>56</EndPage><MedlinePgn>51-56</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1007/s12574-021-00555-4</ELocationID><Abstract><AbstractText Label="INTRODUCTION">Coronavirus disease-19 (COVID-19) has been associated with subclinical myocardial dysfunction during its acute phase and a recurring pattern of reduced basal left ventricular longitudinal strain on speckle-tracking echocardiography (STE) in hospitalized patients. But a question still remains unanswered: speckle-tracking echocardiography might also be suitable to detect residual myocardial involvement after acute stage of COVID-19?</AbstractText><AbstractText Label="METHODS AND RESULTS">We studied 100 patients recovered from COVID-19 with STE to evaluate global (GLS) and segmentar longitudinal strain (LS) and compared with a control group of 100 healthy individuals. STE was performed at a median of 130.35&#x2009;&#xb1;&#x2009;76.06&#xa0;days after COVID-19 diagnostic. Demographic and echocardiographic parameters are similar in both groups. Left ventricular ejection faction (LVEF) and GLS were normal in COVID-19 patients (66.20&#x2009;&#xb1;&#x2009;1.98% and -&#xa0;19.51&#x2009;&#xb1;&#x2009;2.87%, respectively). A reduction in mean LS for the basal segments was found in COVID-19 (16.48&#x2009;&#xb1;&#x2009;5.41%) when compared to control group (19.09&#x2009;&#xb1;&#x2009;4.31%) (p&#x2009;&lt;&#x2009;0.001).</AbstractText><AbstractText Label="CONCLUSION">The present study suggests that COVID-19-induced cardiac involvement could persist after recovery of the disease and may be detected by deformation abnormalities using STE. COVID-19-induced myocardial involvement often shows specific LV deformation patterns due to pronounced edema and/or myocardial damage in basal LV segments.</AbstractText><CopyrightInformation>&#xa9; 2021. Japanese Society of Echocardiography.</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Caiado</LastName><ForeName>Luiz D C</ForeName><Initials>LDC</Initials><Identifier Source="ORCID">0000-0002-1327-3804</Identifier><AffiliationInfo><Affiliation>Division of Echocardiography, Cardioimagem Cl&#xed;nica Cardiol&#xf3;gica, SHIS QI7 conjunto 1 casa 11, Brasilia, DF, 71615-210, Brazil. ldcortez@uol.com.br.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Azevedo</LastName><ForeName>Nathalia C</ForeName><Initials>NC</Initials><AffiliationInfo><Affiliation>Division of Echocardiography, Cardioimagem Cl&#xed;nica Cardiol&#xf3;gica, SHIS QI7 conjunto 1 casa 11, Brasilia, DF, 71615-210, Brazil.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Azevedo</LastName><ForeName>Rafael R C</ForeName><Initials>RRC</Initials><AffiliationInfo><Affiliation>Division of Echocardiography, Cardioimagem Cl&#xed;nica Cardiol&#xf3;gica, SHIS QI7 conjunto 1 casa 11, Brasilia, DF, 71615-210, Brazil.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Caiado</LastName><ForeName>Brasil R</ForeName><Initials>BR</Initials><AffiliationInfo><Affiliation>Division of Echocardiography, Cardioimagem Cl&#xed;nica Cardiol&#xf3;gica, SHIS QI7 conjunto 1 casa 11, Brasilia, DF, 71615-210, Brazil.</Affiliation></AffiliationInfo></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList><ArticleDate DateType="Electronic"><Year>2021</Year><Month>10</Month><Day>14</Day></ArticleDate></Article><MedlineJournalInfo><Country>Japan</Country><MedlineTA>J Echocardiogr</MedlineTA><NlmUniqueID>101263153</NlmUniqueID><ISSNLinking>1349-0222</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000086382" MajorTopicYN="Y">COVID-19</DescriptorName><QualifierName UI="Q000150" MajorTopicYN="N">complications</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006352" MajorTopicYN="N">Heart Ventricles</DescriptorName><QualifierName UI="Q000000981" MajorTopicYN="N">diagnostic imaging</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000086402" MajorTopicYN="N">SARS-CoV-2</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D018487" MajorTopicYN="Y">Ventricular Dysfunction, Left</DescriptorName><QualifierName UI="Q000000981" MajorTopicYN="N">diagnostic imaging</QualifierName><QualifierName UI="Q000209" MajorTopicYN="N">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D016277" MajorTopicYN="N">Ventricular Function, Left</DescriptorName></MeshHeading></MeshHeadingList><KeywordList Owner="NOTNLM"><Keyword MajorTopicYN="N">COVID-19</Keyword><Keyword MajorTopicYN="N">Echocardiography</Keyword><Keyword MajorTopicYN="N">Myocardial dysfunction</Keyword><Keyword MajorTopicYN="N">Recovered</Keyword><Keyword MajorTopicYN="N">Strain</Keyword></KeywordList><CoiStatement>All authors declare that they have no conflict of interest.</CoiStatement></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="received"><Year>2021</Year><Month>7</Month><Day>6</Day></PubMedPubDate><PubMedPubDate PubStatus="accepted"><Year>2021</Year><Month>10</Month><Day>6</Day></PubMedPubDate><PubMedPubDate PubStatus="revised"><Year>2021</Year><Month>9</Month><Day>28</Day></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2021</Year><Month>10</Month><Day>15</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2022</Year><Month>3</Month><Day>5</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2021</Year><Month>10</Month><Day>14</Day><Hour>12</Hour><Minute>30</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">34648149</ArticleId><ArticleId IdType="pmc">PMC8514606</ArticleId><ArticleId IdType="doi">10.1007/s12574-021-00555-4</ArticleId><ArticleId IdType="pii">10.1007/s12574-021-00555-4</ArticleId></ArticleIdList><ReferenceList><Reference><Citation>Babapoor-Farrokan S, Gill D, Walker J, Rasekhi RT, Bozorgnia B, Amanullah A. 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JACC Cardiovasc Imaging. 2020;13(11):2340&#x2013;2342. doi: 10.1016/j.jcmg.2020.06.013.</Citation><ArticleIdList><ArticleId IdType="doi">10.1016/j.jcmg.2020.06.013</ArticleId><ArticleId IdType="pmc">PMC7332904</ArticleId><ArticleId IdType="pubmed">32771570</ArticleId></ArticleIdList></Reference><Reference><Citation>Patel VB, Mori J, McLean BA, et al. ACE2 deficiency worsens epicardial adipose tissue inflammation and cardiac dysfunction in response to diet-induced obesity. Diabetes. 2016;65(1):85&#x2013;95. doi: 10.2337/db15-0399.</Citation><ArticleIdList><ArticleId IdType="doi">10.2337/db15-0399</ArticleId><ArticleId IdType="pmc">PMC4686955</ArticleId><ArticleId IdType="pubmed">26224885</ArticleId></ArticleIdList></Reference><Reference><Citation>Abbara S, Desai JC, Cury RC, Butler J, Nieman K, Reddy V. Mapping epicardial fat with multi-detector computed tomography to facilitate percutaneous transepicardial arrhythmia ablation. 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Echocardiography. 2015;32(12):1880&#x2013;1884. doi: 10.1111/echo.13024.</Citation><ArticleIdList><ArticleId IdType="doi">10.1111/echo.13024</ArticleId><ArticleId IdType="pubmed">26234484</ArticleId></ArticleIdList></Reference><Reference><Citation>Esposito R, Galderisi M, Santoro C, et al. Prominent longitudinal strain reduction of left ventricular basal segments in treatment-na&#xef;ve Anderson&#x2013;Fabry disease patients. Eur Heart J Cardiovasc Imaging. 2019;204:438&#x2013;445. doi: 10.1093/ehjci/jey108.</Citation><ArticleIdList><ArticleId IdType="doi">10.1093/ehjci/jey108</ArticleId><ArticleId IdType="pubmed">30085001</ArticleId></ArticleIdList></Reference></ReferenceList></PubmedData></PubmedArticle><PubmedArticle><MedlineCitation Status="Publisher" Owner="NLM"><PMID Version="1">34648132</PMID><DateRevised><Year>2021</Year><Month>10</Month><Day>14</Day></DateRevised><Article PubModel="Print-Electronic"><Journal><ISSN IssnType="Electronic">1573-6822</ISSN><JournalIssue CitedMedium="Internet"><PubDate><Year>2021</Year><Month>Oct</Month><Day>14</Day></PubDate></JournalIssue><Title>Cell biology and toxicology</Title><ISOAbbreviation>Cell Biol Toxicol</ISOAbbreviation></Journal>METTL14 promotes doxorubicin-induced cardiomyocyte ferroptosis by regulating the KCNQ1OT1-miR-7-5p-TFRC axis.
Doxorubicin (DOX) has toxic effects on the heart, causing cardiomyopathy and heart injury, but the underlying mechanisms of these effects require further investigation. This study investigated the role of DOX in promoting ferroptosis to induce myocardial injury. AC16 cardiomyocyte and neonatal rat ventricle cardiomyocytes were used as an in vitro model to study the molecules involved in myocardial injury using gene silencing, ectopic expression, and RNA immunoprecipitation. Messenger RNA and protein level analyses showed that DOX treatment resulted in the upregulation of methyltransferase-like 14 (METTL14), which catalyzes the m6A modification of the long non-coding RNA KCNQ1OT1, a miR-7-5p sponge. The RNA-binding protein IGF2BP1 is associated with KCNQ1OT1 to increase its stability and robustly inhibit miR-7-5p activity. Furthermore, a lack of miR-7-5p expression led to increased levels of transferrin receptor, promoting the uptake of iron and production of lipid reactive oxygen species and demonstrating that DOX-induced ferroptosis occurs in AC16 cells. Additionally, we found that miR-7-5p targets METTL14 in AC16 cells. Meanwhile, the role of METTL14/KCNQ1OT1/miR-7-5p axis in regulating ferroptosis in neonatal rat ventricle cardiomyocytes was also confirmed. Our results indicate that selectively inhibiting ferroptosis mediated by a METTL14/KCNQ1OT1/miR-7-5p positive feedback loop in cardiomyocytes could provide a new therapeutic approach to control DOX-induced cardiac injury.
2,330,861
Computer simulation of tumour resection-induced brain deformation by a meshless approach.
Tumour resection requires precise planning and navigation to maximise tumour removal while simultaneously protecting nearby healthy tissues. Neurosurgeons need to know the location of the remaining tumour after partial tumour removal before continuing with the resection. Our approach to the problem uses biomechanical modelling and computer simulation to compute the brain deformations after the tumour is resected. In this study, we use meshless Total Lagrangian explicit dynamics as the solver. The problem geometry is extracted from the patient-specific magnetic resonance imaging (MRI) data and includes the parenchyma, tumour, cerebrospinal fluid and skull. The appropriate non-linear material formulation is used. Loading is performed by imposing intra-operative conditions of gravity and reaction forces between the tumour and surrounding healthy parenchyma tissues. A finite frictionless sliding contact is enforced between the skull (rigid) and parenchyma. The meshless simulation results are compared to intra-operative MRI sections. We also calculate Hausdorff distances between the computed deformed surfaces (ventricles and tumour cavities) and surfaces observed intra-operatively. Over 80% of points on the ventricle surface and 95% of points on the tumour cavity surface were successfully registered (results within the limits of two times the original in-plane resolution of the intra-operative image). Computed results demonstrate the potential for our method in estimating the tissue deformation and tumour boundary during the resection.
2,330,862
Myelin Basic Protein and Cardiac Sympathetic Neurodegeneration in Nonhuman Primates.
Minimal myelination is proposed to be a contributing factor to the preferential nigral neuronal loss in Parkinson's disease (PD). Similar to nigral dopaminergic neurons, sympathetic neurons innervating the heart have long, thin axons which are unmyelinated or minimally myelinated. Interestingly, cardiac sympathetic loss in PD is heterogeneous across the heart, yet the spatial relationship between myelination and neurodegeneration is unknown. Here, we report the mapping of myelin basic protein (MBP) expression across the left ventricle of normal rhesus macaques (<i>n</i>&#x2009;=&#x2009;5) and animals intoxicated with systemic 6-OHDA (50&#x2009;mg/kg iv) to model parkinsonian cardiac neurodegeneration (<i>n</i>&#x2009;=&#x2009;10). A subset of 6-OHDA-treated rhesus received daily dosing of pioglitazone (5&#x2009;mg/kg po; <i>n</i>&#x2009;=&#x2009;5), a PPAR<i>&#x3b3;</i> agonist with neuroprotective properties. In normal animals, MBP-immunoreactivity (-ir) was identified surrounding approximately 14% of axonal fibers within nerve bundles of the left ventricle, with more myelinated nerve fibers at the base level of the left ventricle than the apex (<i>p</i> &lt; 0.014). Greater MBP-ir at the base was related to a greater number of nerve bundles at that level relative to the apex (<i>p</i> &lt; 0.05), as the percent of myelinated nerve fibers in bundles was not significantly different between levels of the heart. Cardiac sympathetic loss following 6-OHDA was associated with decreased MBP-ir in cardiac nerve bundles, with the percent decrease of MBP-ir greater in the apex (84.5%) than the base (52.0%). Interestingly, cardiac regions and levels with more MBP-ir in normal animals showed attenuated sympathetic loss relative to areas with less MBP-ir in 6-OHDA&#x2009;+&#x2009;placebo (<i>r</i>&#x2009;=&#x2009;-0.7, <i>p</i> &lt; 0.014), but not in 6-OHDA&#x2009;+&#x2009;pioglitazone (<i>r</i>&#x2009;=&#x2009;-0.1) subjects. Our results demonstrate that myelination is present around a minority of left ventricle nerve bundle fibers, is heterogeneously distributed in the heart of rhesus macaques, and has a complex relationship with cardiac sympathetic neurodegeneration and neuroprotection.
2,330,863
Ventricular longitudinal shortening is an independent predictor of death in heart failure patients with reduced ejection fraction.
Reduced ventricular longitudinal shortening measured by atrioventricular plane displacement (AVPD) and global longitudinal strain (GLS) are prognostic markers in heart disease. This study aims to determine if AVPD and GLS with cardiovascular magnetic resonance (CMR) are independent predictors of cardiovascular (CV) and all-cause death also in heart failure with reduced ejection fraction (HFrEF). Patients (n&#x2009;=&#x2009;287) were examined with CMR and AVPD, GLS, ventricular volumes, myocardial fibrosis/scar were measured. Follow-up was 5&#xa0;years with cause of death retrieved from a national registry. Forty CV and 60 all-cause deaths occurred and CV non-survivors had a lower AVPD (6.4&#x2009;&#xb1;&#x2009;2.0 vs 8.0&#x2009;&#xb1;&#x2009;2.4&#xa0;mm, p&#x2009;&lt;&#x2009;0.001) and worse GLS (-&#x2009;6.1&#x2009;&#xb1;&#x2009;2.2 vs -&#x2009;7.7&#x2009;&#xb1;&#x2009;3.1%, p&#x2009;=&#x2009;0.001). Kaplan-Meier analyses displayed increased survival for patients in the highest AVPD- and GLS-tertiles vs. the lowest tertiles (AVPD: p&#x2009;=&#x2009;0.001, GLS: p&#x2009;=&#x2009;0.013). AVPD and GLS showed in univariate analysis a hazard ratio (HR) of 1.30 (per-mm-decrease) and 1.19 (per-%-decrease) for CV death. Mean AVPD and GLS were independent predictors of all-cause death (HR&#x2009;=&#x2009;1.24 per-mm-decrease and 1.15 per-%-decrease), but only AVPD showed incremental value over age, sex, body-mass-index, EF, etiology and fibrosis/scar for CV death (HR&#x2009;=&#x2009;1.33 per-mm-decrease, p&#x2009;&lt;&#x2009;0.001). Ventricular longitudinal shortening remains independently prognostic for death in HFrEF even after adjusting for well-known clinical risk factors.
2,330,864
Upward gaze palsy and convergence insufficiency as a rare presentation of primary intraventricular haemorrhage.
A primary intraventricular haemorrhage (PIVH) usually presents with non-localised neurological symptoms since the haematoma is limited to the ventricles. However, it is sometimes associated with focal neurological signs, whose pathophysiologies are not confirmed. Here, we report on a case of PIVH who showed rare manifestations in the acute stage: upward gaze palsy and convergence insufficiency. The CT and MRI showed intraventricular haematoma without evidence of parenchymal haemorrhage, local mass effect around midbrain or hydrocephalus. There had been bilateral papilloedema, and it resolved along with improvement of the ophthalmic symptoms, suggesting a possible causal relation to increased intracranial pressure. The ophthalmic abnormalities suggested injury of the rostral part of the midbrain, especially the region around the dorsal midbrain tectum. It should be known that PIVH is one of the causes of acutely developing upward gaze palsy and convergence insufficiency.
2,330,865
Speckle-Tracking Global Longitudinal and Regional Strain Analysis in Neonates with Coarctation of Aorta: A Case-Control Study.
Our objectives are to compare speckle-tracking peak global longitudinal (pGLS) and regional strain values in neonates with coarctation of aorta (CoA) and control groups. Echocardiographic parameters measured by speckle-tracking were studied in a retrospective single-center study. A comparison of pGLS and segmental deformation between neonates with CoA and control group was performed using a three-way mixed ANOVA model. There was a significant difference in the means of segmental strain values between CoA and control group at the apical (<i>p</i> = 0.018) and basal segments (<i>p</i> = 0.031) of the interventricular septum and at the apical segment (<i>p</i> = 0.026) of the left ventricle (LV). After correcting for multiple comparisons, the results had a tendency toward statistical significance (adjusted-<i>p</i> &lt; 0.10). There was significant difference in the mean values of pGLS [F(1, 39) = 7.61, <i>p</i> = 0.009, adjusted <i>p</i> = 0.018] between the studied groups. The results of ROC analysis showed that a cut-off value of -16.60% for pGLS provided an estimated sensitivity of 92.31% (95% CI: [63.97, 99.81]) and 71.43% specificity (95% CI: [51.33, 86.78]) for the diagnosis of CoA in neonates (AUC = 0.794, 95% CI: [0.66, 0.93]). pGLS can be regarded as a feasible and reproducible parameter reflecting LV dysfunction in newborns with CoA when compared to newborns with a false-positive diagnosis.
2,330,866
Sex- and age- specific normal values of left ventricular functional and myocardial mass parameters using threshold-based trabeculae quantification.
The threshold-based (TB) trabeculated and papillary muscle mass (TPM) quantification method for cardiac MRI (CMR) calculates different values than conventional contouring techniques. We aimed to identify the sex- and age-related normal reference ranges for left ventricular (LV) myocardial mass values, volumetric and functional parameters and the correspondence of these parameters using the TB method.</AbstractText>Healthy European adults (n = 200, age: 39.4 &#xb1; 12 years, males: 100) were examined with CMR and evaluated with a TB postprocessing method. They were stratified by sex and age (Group A: 18-29, Group B: 30-39, Group C: 40-49, Group D: &gt;50 years). The calculated parameters were indexed to body surface area (i).</AbstractText>The normal reference ranges for the studied parameters were assessed in each age group. Significant biometric differences in LV parameters and mass-to-volume ratios were found between males and females, and the left ventricular compacted myocardial mass (LVCMi) and TPMi differences remained significant after stratification by age. Unlike other LV volumetric and functional parameters and mass-to-volume ratios, the TPMi, the LVCMi and the TPMi-to-LVCMi ratio did not differ among age groups in males or females. This finding was strengthened by the lack of correlation between TPMi and age.</AbstractText>Age- and sex-related normal reference ranges for LV volumetric and functional parameters and LVCMi and TPMi values were established using a TB postprocessing method. TPMi, LVCMi and their ratio did not change over time. The TPMi-to-LVCMi and the mass-to-volume ratios might have clinical utility in the differential diagnosis of conditions with LV hypertrabeculation.</AbstractText>
2,330,867
Intracranial Transthecal Fat Migration After a Sacral Fracture: 2 Case Reports.
BACKGROUND The presence of fat droplets within the subarachnoid space is an uncommon finding, which is almost exclusively associated with a ruptured dermoid cyst. In a trauma setting, transthecal migration of fat droplets is an extremely rare occurrence. We present 2 case reports of intracranial transthecal migration of fatty bone marrow after sacral fractures. CASE REPORT Both patients presented to the Emergency Department (ED) after falls from a standing height. The first patient, an 84-year-old woman, suffered a stable sacral fracture extending into a large meningeal cyst within the right S2 foramen. Her initial neurological assessment and computed tomography (CT) of the head were unremarkable. As the fracture did not require surgical treatment, she was discharged home and prescribed bed rest, analgesics, and venous thromboembolism prophylaxis. Three days after the injury, she was readmitted to the ED with a mild headache, dizziness, and an episode of nausea and vomiting. A follow-up head CT revealed fat droplets in the subarachnoid space and lateral ventricles. After successful symptomatic treatment, she was discharged home in good general condition. The second patient, a 60-year-old man, underwent a head CT for a scalp hematoma, which revealed fat droplets in the 3rd ventricle and right lateral ventricle. The pelvic CT revealed a large sacral meningeal cyst with microfractures in its wall. He was discharged home on the same day and prescribed bed rest and analgesics. CONCLUSIONS The detection of intracranial intrathecal fat droplets in association with a specific trauma mechanism should initiate the search for a sacral fracture.
2,330,868
Brain Injury Patterns in Neonates With Hypernatremic Dehydration: Single Center Experience.
To find out the incidence, spectrum, and topographical distribution of brain lesions in neonatal hypernatremic dehydration.</AbstractText>We prospectively enrolled 100 consecutive neonates admitted with hypernatremic dehydration. 93 neonates underwent magnetic resonance imaging brain to identify the nature and site of neurological injury.</AbstractText>Neuroradiological lesions were found in 42 (45.2%) babies. Edema was the most common finding in 37 (39.8%), followed by hemorrhage in 13 (13.9%) and thrombosis in 6 (6.4%). Edema predominantly affected juxtacortical/subcortical white matter followed by periventricular white matter and centrum semiovale, posterior part of internal capsule, and basal ganglia/thalamus. Occipital horns of lateral ventricle were the main sites of hemorrhage. Thrombotic lesions predominantly involved sagittal, straight and transverse sinuses. Brain lesions were observed only in severe hypernatremia group.</AbstractText>In neonatal hypernatremic dehydration, edema was the most common neurological lesion, followed by hemorrhage and thrombosis. Subcortical/juxtacortical white matter was the most commonly affected site.</AbstractText>
2,330,869
Nucleated red blood cells as a biomarker for mortality in infants and neonates requiring veno-arterial extracorporeal membrane oxygenation for cardiac disease.
Nucleated red blood cells (NRBC) are rare in the peripheral circulation of healthy individuals and their presence have been associated with mortality in adults and very low birth weight newborns, however, its value as a biomarker for mortality in infants requiring veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) has yet to be studied. We sought to determine if NRBC can serve as a biomarker for ECMO mortality and inpatient mortality in infants requiring V-A ECMO.</AbstractText>A single-center retrospective chart review analyzing infants &lt;1&#x2009;year of age requiring VA ECMO due to myocardial dysfunction or post-cardiotomy between January 1, 2011 to June 30, 2020.</AbstractText>One hundred two patients required VA ECMO. Sixty-five patients required ECMO post-cardiotomy, 19 for perioperative deterioration, and 18 for myocardial dysfunction. Fifty-one patients (50%) died (21 died on ECMO, 30 died post-ECMO decannulation). Multivariable analysis found Age</i> &lt;60&#x2009;days</i> (OR 13.0, 95% CI 1.9-89.6, p&#x2009;=&#x2009;0.009), NRBC increase by &gt;50% post-ECMO decannulation</i> (OR 17.1, 95% CI 3.1-95.1, p&#x2009;=&#x2009;0.001), Single Ventricle</i> (OR 9.0, 95% CI 1.7-47.7, p&#x2009;=&#x2009;0.01), and lactate at ECMO decannulation</i> (OR 3.0, 95% CI 1.3-7.1, p&#x2009;=&#x2009;0.011) to be independently associated with inpatient mortality. ROC curves evaluating NRBC pre-ECMO decannulation as a biomarker for mortality on ECMO (AUC 0.80, 95% CI 0.68-0.92, p&#x2009;&#x2a7d;</i>&#x2009;0.001) and post-ECMO decannulation (AUC 0.75, 95% CI 0.65-0.84, p&#x2009;&#x2a7d;</i>&#x2009;0.001) show NRBC to be an accurate biomarker for mortality.</AbstractText>Greater than 50% increase in NRBC post-ECMO decannulation is associated with inpatient mortality. NRBC value pre-ECMO decannulation may be a useful biomarker for mortality while on ECMO and post-decannulation.</AbstractText>
2,330,870
Associations of the Dietary Approaches to Stop Hypertension dietary pattern with cardiac structure and function.
Various food groups have been associated with measures of left ventricular geometry and function. Whether the Dietary Approaches to Stop Hypertension (DASH) dietary pattern in mid-life is associated with a favorable cardiac structure and function later in life is unknown.</AbstractText>The study population consisted of the Atherosclerosis Risk in Communities study participants free of cardiovascular disease at study visit 3 in 1993-1995. Dietary intake was assessed by food frequency questionnaire at study visits 1 (1987-1989) and 3 (1993-1995). Participants who underwent transthoracic echocardiograms at the Jackson field center at visit 3 (n&#xa0;=&#xa0;1974) and at all field centers at study visit 5 (2011-2013; n&#xa0;=&#xa0;4651) were included in this study. General linear regression was used to evaluate associations between dietary intake and markers of cardiac structure and function adjusting for potential confounders. Higher DASH score was associated with lower left ventricle mean wall thickness and higher absolute value of longitudinal strain at visit 5 (ptrend</sub>&#xa0;=&#xa0;0.004 and&#xa0;&lt;&#xa0;0.001, respectively).</AbstractText>The DASH dietary pattern in midlife was favorably associated with left ventricle structure and systolic function later in life. These results emphasize the importance of adhering to a healthy eating plan as one lifestyle measure to preserve cardiac structure and function.</AbstractText>Copyright &#xa9; 2021 The Italian Diabetes Society, the Italian Society for the Study of Atherosclerosis, the Italian Society of Human Nutrition and the Department of Clinical Medicine and Surgery, Federico II University. Published by Elsevier B.V. All rights reserved.</CopyrightInformation>
2,330,871
How growing tumour impacts intracranial pressure and deformation mechanics of brain.
Brain is an actuator for control and coordination. When a pathology arises in cranium, it may leave a degenerative, disfiguring and destabilizing impact on brain physiology. However, the leading consequences of the same may vary from case to case. Tumour, in this context, is a special type of pathology which deforms brain parenchyma permanently. From translational perspective, deformation mechanics and pressures, specifically the intracranial cerebral pressure (ICP) in a tumour-housed brain, have not been addressed holistically in literature. This is an important area to investigate in neuropathy prognosis. To address this, we aim to solve the pressure mystery in a tumour-based brain in this study and present a fairly workable methodology. Using image-based finite-element modelling, we reconstruct a tumour-based brain and probe resulting deformations and pressures (ICP). Tumour is grown by dilating the voxel region by 16 and 30 mm uniformly. Cumulatively three cases are studied including an existing stage of the tumour. Pressures of cerebrospinal fluid due to its flow inside the ventricle region are also provided to make the model anatomically realistic. Comparison of obtained results unequivocally shows that as the tumour region increases its area and size, deformation pattern changes extensively and spreads throughout the brain volume with a greater concentration in tumour vicinity. Second, we conclude that ICP pressures inside the cranium do increase substantially; however, they still remain under the normal values (15 mmHg). In the end, a correlation relationship of ICP mechanics and tumour is addressed. From a diagnostic purpose, this result also explains why generally a tumour in its initial stage does not show symptoms because the required ICP threshold has not been crossed. We finally conclude that even at low ICP values, substantial deformation progression inside the cranium is possible. This may result in plastic deformation, midline shift etc. in the brain.
2,330,872
[Cardiac glomus tumor: An unusual localization of pericytic (perivascular) tumor].
Glomus tumor are rare mesenchymal neoplasm, belonging to the pericytic (perivascular) tumor family, witch recent molecular characterization has allowed highlight recurrent molecular abnormalities. In fact, glomus tumor involves frequent MIR143-NOTCH gene fusion whereas others pericytic tumor (myopericytoma and myofibroma) involve mutations of PDGFRB gene. Glomus tumor are usually developed in superficial localization. However visceral locations have been described. Cardiac location is exceptional with only one case reported in literature. Here, we report the case of cardiac glomus tumor (glomangiomyoma) developed in the left ventricle in a 34 year-old patient, diagnosed after chest pain. The length of tumor was 4cm in greatest dimension. Histologically, the tumor concerned both round glomus cells and smooth muscle cells with prominent branching thin-walled vessels. By immunohistochemistry, these two contingents exhibited diffuse expression of smooth muscle actin and heterogeneous expression of H-caldesmone whereas cytokeratins, melanocytic markers and chomogranine were negative. Next Generation molecular analysis using RNA sequencing highlighted the characteristic MIR143-NOTCH gene fusion witch supports the diagnosis of glomus tumor. In this observation, we recall histological and immunohistochemistry features of glomus tumor and we make a synthesis concerning the molecular data recently described in sporadic glomus tumor.
2,330,873
Bipolar disorder with Melnick-Needles syndrome and periventricular nodular heterotopia: two case reports&#xa0;and&#xa0;a review of the&#xa0;literature.
Melnick-Needles syndrome and periventricular nodular heterotopia are two usually mutually exclusive phenotypes of F-actin-binding cytoskeletal phosphoprotein Filamin-A mutations. Melnick-Needles syndrome is a rare X-linked condition that is lethal in males and shows great phenotypic variability in affected females. It is caused by mutations in Filamin-A gene, which encodes the protein Filamin A. Defects of the human Filamin-A gene also cause X-linked periventricular nodular heterotopia, a malformation of neuronal migration characterized by nodules of neurons in inappropriate location adjacent to the walls of the lateral ventricles.</AbstractText>We report on two Caucasian adolescent females, sisters, diagnosed with Melnick-Needles syndrome and bilateral periventricular nodular heterotopia, who developed bipolar disorder and somatic symptoms disorder at a young age. We also present a review of the literature about mental disorders associated with periventricular nodular heterotopia. Our report shows that patients presenting with atypical and heterogeneous psychiatric disease may have an underrecognized anatomical brain abnormality on genetic basis.</AbstractText>We found records of psychiatric disorders associated with periventricular nodular heterotopia; nevertheless, this is the first report of bipolar disorder occurring in individuals with periventricular nodular heterotopia, and the first report of any psychiatric disorder in individuals affected by Melnick-Needles syndrome. In conclusion, this case report may contribute to characterizing the phenotype of this very rare syndrome.</AbstractText>&#xa9; 2021. The Author(s).</CopyrightInformation>
2,330,874
Function of Oncogene Mycn in Adult Neurogenesis and Oligodendrogenesis.
Human MYCN is an oncogene amplified in neuroblastoma and many other tumors. Both human MYCN and mouse Mycn genes are important in embryonic brain development, but their functions in adult healthy nerve system are completely unknown. Here, with Mycn-eGFP mice and quantitative RT-PCR, we found that Mycn was expressed in specific brain regions of young adult mice, including subventricular zone (SVZ), subgranular zone (SGZ), olfactory bulb (OB), subcallosal zone (SCZ), and corpus callosum (CC). With immunohistochemistry (IHC), we found that many Mycn-expressing cells expressed neuroblast marker doublecortin (DCX) and proliferation marker Ki67. With Dcx-creER and Mki67-creER mouse lines, we fate mapped Dcx-expressing neuroblasts and Mki67-expressing proliferation cells, along with deleting Mycn from these cells in adult mice. We found that knocking out Mycn from adult neuroblasts or proliferating cells significantly reduced cells in proliferation in SVZ, SGZ, OB, SCZ, and CC. We also demonstrated that the Mycn-deficient neuroblasts in SGZ matured quicker than wild-type neuroblasts, and that Mycn-deficient proliferating cells were more likely to survive in SVZ, SGZ, OB, SCZ, and CC compared to wild type. Thus, our results demonstrate that, in addition to causing tumors in the nervous system, oncogene Mycn has a crucial function in neurogenesis and oligodendrogenesis in adult healthy brain.
2,330,875
The eagle sign: a new preoperative MRI-based tool for predicting topographic correlation between craniopharyngioma and hypothalamus.
Accurate prediction of topographical correlation between craniopharyngiomas (CPs) and hypothalamus is important for treatment. This study sought to develop a predicting tool based on preoperative-MRI through radiological-surgical-pathological-outcome analysis.</AbstractText>Third ventricle floor (TVF), mammillary bodies and cerebral peduncle were evaluated through preoperative-MRI. An eagle-head-like sign named "eagle sign" was observed. Normal TVF on sagittal-MRI was defined as the baseline. Variants of the sign were analyzed by comparing with the baseline and corresponding correlations of CPs with hypothalamus were verified using intraoperative records, histopathology and outcome evaluation.</AbstractText>A total of 146 CPs patients, who undergone endoscopic endonasal procedure were divided into four groups based on the variants of "eagle sign". Group A: 24 patients with the upward sign; group B: 81 with the downward sign; group C: 21 with the anterior TVF upward sign and group D: 20 with the unidentifiable sign. Surgical-pathological analysis showed significant correlations between 95.8% CPs in group A and 95.2% in group C with tumor topography and tumor adherence to the hypothalamus. These CPs had their origins beneath the hypothalamus. In contrast, groups B and D, with hypothalamic origin, showed hypothalamic infiltration by tumor in 97.5% and 95% of cases in groups B and D, respectively. Outcomes of groups A and C were relatively better than groups B and D. Predictive sensitivity and specificity of "eagle sign" were more than 90%.</AbstractText>"Eagle sign" is an accurate tool for predicting topographic correlations between CPs and hypothalamus with high sensitivity and specificity.</AbstractText>&#xa9; 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.</CopyrightInformation>
2,330,876
Subventricular zone involvement is associated with worse outcome in glioma WHO grade 2 depending on molecular markers.
Neural stem cells within the subventricular zone were identified as cells of origin driving growth of high-grade gliomas, and anatomical involvement of the subventricular zone has been associated with an inferior clinical outcome. Whether the association between poor outcome and subventricular zone involvement also applies to glioma of lower grades is unclear. We therefore analysed a retrospective cohort of 182 patients with glioma grade 2 (according to the WHO 2016 classification) including 78 individuals (43%) with subventricular zone involvement. Patients with and without subventricular zone involvement did not differ in regard to demographics, histopathology, and molecular markers. Notably, subventricular zone involvement was a negative prognostic marker for malignant progression and overall survival on uni- and multivariate analysis. When patients were stratified according to the cIMPACT-NOW update 6, subventricular zone involvement was negatively associated with outcome in IDH-wildtype astrocytomas and 1p19q-codeleted oligodendrogliomas but not in IDH-mutant astrocytomas. Collectively, subventricular zone involvement may represent a risk factor for worse outcome in glioma WHO grade 2 depending on the molecular tumor signature. The present data confirm the relevance of molecular glioma classifications as proposed by the cIMPACT-NOW update 6. These findings warrant evaluation in prospective cohorts.
2,330,877
BRAF mutations may identify a clinically distinct subset of glioblastoma.
Glioblastoma (GBM) is the most common primary malignant brain tumor in adults. Prior studies examining the mutational landscape of GBM revealed recurrent alterations in genes that regulate the same growth control pathways. To this regard,&#x2009;~&#x2009;40% of GBM harbor EGFR alterations, whereas BRAF variants are rare. Existing data suggests that gain-of-function mutations in these genes are mutually exclusive. This study was designed to explore the clinical, pathological, and molecular differences between EGFR- and BRAF-mutated GBM. We reviewed retrospective clinical data from 89 GBM patients referred for molecular testing between November 2012 and December 2015. Differences in tumor mutational profile, location, histology, and survival outcomes were compared in patients with EGFR- versus BRAF-mutated tumors, and microarray data from The Cancer Genome Atlas was used to assess differential gene expression between the groups. Individuals with BRAF-mutant tumors were typically younger and survived longer relative to those with EGFR-mutant tumors, even in the absence of targeted treatments. BRAF-mutant tumors lacked distinct histomorphology but exhibited unique localization in the brain, typically arising adjacent to the lateral ventricles. Compared to EGFR- and IDH1-mutant tumors, BRAF-mutant tumors showed increased expression of genes related to a trophoblast-like phenotype, specifically HLA-G and pregnancy specific glycoproteins, that have been implicated in invasion and immune evasion. Taken together, these observations suggest a distinct clinical presentation, brain location, and gene expression profile for BRAF-mutant tumors. Pending further study, this may prove useful in the stratification and management of GBM.
2,330,878
Suboccipital Transhorizontal Fissure Approach for Posterior Cranial Fossa Lesions: A Cadaveric Study and First Clinical Experience.
Surgical treatment of pathological lesions in the deep cerebellar hemisphere, cerebellopontine angle (CPA), and fourth ventricle of the posterior cranial fossa (PCF) is challenging. Conventional neurosurgical approaches to these lesions are associated with risk of various complications. Mastery of efficient fissure dissection is imperative when approaching deep-seated lesions. The horizontal fissure (HF) is the largest and deepest fissure of the cerebellum.</AbstractText>To conduct an anatomical study and introduce a novel suboccipital trans-HF (SOTHF) approach to access lesions of the deep cerebellar hemispheres, CPA, and upper fourth ventricle of the PCF.</AbstractText>We performed a cadaveric dissection study focusing on anatomical landmarks and surgical feasibility of the SOTHF approach then implemented it in 2 patients with a deep cerebellar hemispheric tumor.</AbstractText>Anatomical feasibility of the SOTHF approach was demonstrated and compared with conventional approaches in the cadaveric study. Opening the suboccipital surface of the HF to create medial, intermediate, and lateral surgical corridors provided optimal viewing angles and wide access to the deep cerebellar hemispheres, CPA, and upper fourth ventricle without heavy cerebellar retraction. Sacrificing cerebellar neural structures and complex skull base techniques were not required to obtain adequate exposure. The SOTHF approach was successfully applied without complication in 2 patients with a deep cerebellar hemispheric tumor.</AbstractText>The HF is an important cerebellar fissure that provides a gateway to deep areas of the PCF. Further studies are needed to define and expand applications of the SOTHF approach.</AbstractText>&#xa9; Congress of Neurological Surgeons 2021.</CopyrightInformation>
2,330,879
Cerebrospinal fluid shunting protocol for idiopathic intracranial hypertension for an improved revision rate.
Cerebrospinal fluid (CSF) shunting in idiopathic intracranial hypertension (IIH) is associated with high complication rates, primarily because of the technical challenges that are related to small ventricles and a large body habitus. In this study, the authors report the benefits of a standardized protocol for CSF shunting in patients with IIH as relates to shunt revisions.</AbstractText>This was a retrospective study of consecutive patients with IIH who had undergone primary insertion of a CSF shunt between January 2014 and December 2020 at the authors' hospital. In July 2019, they implemented a surgical protocol for shunting in IIH. This protocol recommended IIH shunt insertion by neurosurgeons with expertise in CSF disorders, a frontal ventriculoperitoneal (VP) shunt with an adjustable gravitational valve and integrated intracranial pressure monitoring device, frameless stereotactic insertion of the ventricular catheter, and laparoscopic insertion of the peritoneal catheter. Thirty-day revision rates before and after implementation of the protocol were compared in order to assess the impact of standardizing shunting for IIH on shunt complications.</AbstractText>The 81 patients included in the study were predominantly female (93%), with a mean age of 31 years at primary surgery and mean body mass index (BMI) of 37 kg/m2. Forty-five patients underwent primary surgery prior to implementation of the protocol and 36 patients after. Overall, 12 (15%) of 81 patients needed CSF shunt revision in the first 30 days, 10 before and 2 after introduction of the protocol. This represented a significant reduction in the early revision rate from 22% to 6% after the protocol (p = 0.036). The most common cause of shunt revision for the whole cohort was migration or misplacement of the peritoneal catheter, occurring in 6 of the 12 patients. Patients with a higher BMI were significantly more likely to have a shunt revision within 30 days (p = 0.022).</AbstractText>The Birmingham standardized IIH shunt protocol resulted in a significant reduction in revisions within 30 days of primary shunt surgery in patients with IIH. The authors recommend standardization for shunting in IIH as a method for improving surgical outcomes. They support the notion of subspecialization for IIH shunts, the use of a frontal VP shunt with sophisticated technology, and laparoscopic insertion of the peritoneal end.</AbstractText>
2,330,880
Postoperative speech impairment and surgical approach to posterior fossa tumours in children: a prospective European multicentre cohort study.
Brain tumours are the most common solid tumours in childhood. Half of these tumours occur in the posterior fossa, where surgical removal is complicated by the risk of cerebellar mutism syndrome, of which postoperative speech impairment (POSI) is a cardinal symptom, in up to 25% of patients. The surgical approach to midline tumours, mostly undertaken by transvermian or telovelar routes, has been proposed to influence the risk of POSI. We aimed to investigate the risk of developing POSI, the time course of its resolution, and its association with surgical approach and other clinical factors.</AbstractText>In this observational prospective multicentre cohort study, we included children (aged &lt;18 years) undergoing primary surgery for a posterior fossa tumour at 26 centres in nine European countries. Within 72 h of surgery, the operating neurosurgeon reported details on the tumour location, surgical approach used, duration of surgery, use of traction, and other predetermined factors, using a standardised surgical report form. At 2 weeks, 2 months, and 1 year after surgery, a follow-up questionnaire was filled out by a paediatrician or neurosurgeon, including neurological examination and assessment of speech. Speech was classified as mutism, reduced speech, or habitual speech. POSI was defined as either mutism or severely reduced speech. Ordinal logistic regression was used to analyse the risk of POSI.</AbstractText>Between Aug 11, 2014, and Aug 24, 2020, we recruited 500 children. 426 (85%) patients underwent primary tumour surgery and had data available for further analysis. 192 (45%) patients were female, 234 (55%) patients were male, 81 (19%) patients were aged 0-2 years, 129 (30%) were aged 3-6 years, and 216 (51%) were aged 7-17 years. 0f 376 with known postoperative speech status, 112 (30%) developed POSI, 53 (14%) developed mutism (median 1 day [IQR 0-2]; range 0-10 days), and 59 (16%) developed reduced speech after surgery (0 days [0-1]; 0-4 days). Mutually adjusted analyses indicated that the independent risk factors for development of POSI were younger age (linear spline, p=0&#xb7;0087), tumour location (four levels, p=0&#xb7;0010), and tumour histology (five levels, p=0&#xb7;0030); surgical approach (six levels) was not a significant risk factor (p=0&#xb7;091). Tumour location outside the fourth ventricle and brainstem had a lower risk of POSI (with fourth ventricle as reference, odds ratio (OR) for cerebellar vermis 0&#xb7;34 [95% CI 0&#xb7;14-0&#xb7;77] and OR for cerebellar hemispheres 0&#xb7;23 [0&#xb7;07-0&#xb7;70]). Compared with pilocytic or pilomyxoid astrocytoma, a higher risk of POSI was seen for medulloblastoma (OR 2&#xb7;85 [1&#xb7;47-5&#xb7;60]) and atypical teratoid rhabdoid tumour (10&#xb7;30 [2&#xb7;10-54&#xb7;45]). We did not find an increased risk of POSI for transvermian surgical approach compared with telovelar (0&#xb7;89 [0&#xb7;46-1&#xb7;73]). Probability of speech improvement from mutism reached 50% around 16 days after mutism onset.</AbstractText>Our data suggest that a midline tumour location, younger age, and high-grade tumour histology all increase the risk of speech impairment after posterior fossa tumour surgery. We found no evidence to recommend a preference for telovelar over transvermian surgical approach in the management of posterior fossa tumours in children in relation to the risk of developing POSI.</AbstractText>The Danish Childhood Cancer Foundation, the Swedish Childhood Cancer Foundation, the UK Brain Tumour Charity, the Danish Cancer Society, Det Kgl Kj&#xf8;benhavnske Skydeselskab og Danske Broderskab, the Danish Capitol Regions Research Fund, Dagmar Marshall Foundation, Rigshospitalet's Research Fund, and Brainstrust.</AbstractText>Copyright &#xa9; 2021 Elsevier Ltd. All rights reserved.</CopyrightInformation>
2,330,881
Versatile Use of Intraoperative Ultrasound Guidance for Brain Puncture.
Intraoperative ultrasound (iUS) is an effective guidance and imaging system commonly used in neuro-oncological surgery. Despite the versatility of iUS, its utility for single burr hole puncture guidance remains fairly underappreciated.</AbstractText>To highlight the simplicity, versatility, and effectiveness of iUS guidance in brain puncture by presenting the current case series and technical note collection.</AbstractText>We present 4 novel uses of iUS guidance for single burr hole brain puncture: cannulation of normal-sized ventricles, endoscopic third ventriculostomy (ETV) guidance, evacuation of interhemispheric empyema, and stereotactic biopsy assistance.</AbstractText>All techniques were performed successfully in a total of 16 patients. Normal-sized ventricles were cannulated in 7 patients, among whom 5 underwent Ommaya reservoir placement and 2 underwent ventriculoperitoneal shunt placement for idiopathic intracranial hypertension. No more than 1 attempt was needed for cannulation. All ventricular tip positions were optimal as shown by postoperative imaging. iUS guidance was used in 5 ETV procedures. The working cannula was successfully introduced to the lateral ventricle, providing the optimal trajectory to the third ventricular floor in these cases. Interhemispheric subdural empyema was aspirated with iUS guidance in 1 patient. Volume reduction was clearly visible, allowing near-total evacuation of the empyema. iUS guidance was used for assistive purposes during stereotactic biopsy in 3 patients. No major perioperative complications were observed throughout this series.</AbstractText>iUS is an effective and versatile guidance system that allows for real-time imaging and can be easily and safely employed for various brain puncture procedures.</AbstractText>&#xa9; Congress of Neurological Surgeons 2021.</CopyrightInformation>
2,330,882
How I do it: surgical resection of a subcallosal gyrus cavernous malformation by a contralateral anterior interhemispheric approach.
Cavernous malformation (CM) of the subcallosal gyrus is extremely rare, and due to its close relationship with the lateral ventricle's frontal horn, it may be misdiagnosed as caudate head or ventricle CM.</AbstractText>This video shows a rare case of subcallosal gyrus CM successfully cured by a contralateral anterior interhemispheric approach.</AbstractText>We hope this case provides new insights into anatomical knowledge and surgical approach techniques for subcallosal lesions.</AbstractText>&#xa9; 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.</CopyrightInformation>
2,330,883
Endoscopic third ventriculostomy in children with chronic communicating congenital hydrocephalus: a single-center cohort retrospective analysis.
The aim of this study was to analyze the role of endoscopic third ventriculostomy (ETV) in the treatment of pediatric chronic communicating congenital hydrocephalus (CCCH).</AbstractText>This retrospective study comprised a series of 11 children with CCCH treated with ETV. Data were recorded on gender, history, presenting symptoms, age at surgery, complications during surgery, clinical evolution, ETV survival, and follow-up period. Radiological variables including ventricular and cephalic diameters were also recorded to determine a series of ventricular indexes in magnetic resonance imaging (MRI) before and after the ETV procedure. The procedure was considered to be successful when there was clinical stability or improvement accompanied by a reduction in the radiological indexes in the postoperative control images, such that there was no need to place an extrathecal cerebrospinal fluid shunt.</AbstractText>Over a mean follow-up period of 35.8&#xa0;months (range: 6-108&#xa0;months) from the ETV procedure, three patients required shunt placement; one of these was due to early failure in an 8-month old girl, the only patient younger than 12&#xa0;months in our series. The radiological indexes were reduced in all patients except for one of the cases of ETV failure. The mean ETV survival among the successful cases was 32.1&#xa0;months (range: 6-108&#xa0;months), whilst that of the failed cases was 16&#xa0;months (range: 6-108&#xa0;months).</AbstractText>Although studies with larger sample sizes are needed, ETV appears to be a promising option for the treatment of this type of patient with CCCH.</AbstractText>&#xa9; 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.</CopyrightInformation>
2,330,884
[Effect and Mechanism of Treating Experimental Autoimmune Encephalomyelitis in Mice with Butylphthalide Combined with Bone Marrow Mesenchymal Stem Cells].
To explore the efficacy and mechanism of using 3-n-butylphthalide (NBP) in combination with bone marrow mesenchymal stem cells (BMSCs) in the treatment of experimental autoimmune encephalomyelitis (EAE) in mice.</AbstractText>Myelin oligodendrocyte glycoprotein (MOG35-55) was used for the induction and establishment of the EAE model in C57BL/6 mice. The mice were randomly assigned to the EAE group, which received intraperitoneal injection of phosphate-buffered saline (PBS), the NBP-treated EAE group, or the NBP group, which received intraperitoneal injection of NBP, the BMSCs transplantion EAE group, or the BMSCs group, which received BMSCs injected into the lateral ventricle and intraperitoneal injection of PBS, and the BMSCs and NBP combination treatment EAE group, or the BMSCs+NBP group, which received BMSCs injected into the lateral ventricle and intraperitoneal injection of NBP. Each group had 10 mice, while ten normal mice were used as the blank control group receiving intraperitoneal injection of PBS. The neurological function scores were documented daily. The mice were sacrificed 22 days after EAE induction, and the demyelination state of of the spinal cords was observed through Luxol fast blue (LFB) staining. In addition, the levels of serum interleukin-6 (IL-6), IL-10, IL-17, IL-22 and transforming growth factor-&#x3b2; (TGF-&#x3b2;) were examined with ELISA. The levels of glial fibrillary acidic protein (GFAP), microtubule associated protein-2 (MAP-2) and myelin basic protein (MBP) in the brain were examined with immunofluorescence staining. Western blot was used to check the expressions of nuclear factor (NF)-&#x3ba;B pathway, phosphoinositide-3 kinase (PI3K)/protein kinase B (PKB or Akt) pathway, IL-17 and forkhead box P3 (Foxp3) in the spinal cords.</AbstractText>The neurological function scores and average scores of each treatment group were significantly lower than those of the EAE group ( P</i>&lt;0.05). The scores of the BMSCs+NBP group decreased more significantly than those of the single treatment groups (the NBP group and the BMSCs group) ( P</i>&lt;0.05). LFB staining results of the spinal cords were consistent with the neurological function scores and the average scores. Compared with the EAE group, the levels of pro-inflammatory cytokines, including IL-6, IL-17 and IL-22, significantly decreased ( P</i>&lt;0.05), and the levels of anti-inflammatory cytokines IL-10 and TGF-&#x3b2; significantly increased ( P</i>&lt;0.05). The change in cytokine expression was more significant in the BMSCs+NBP group ( P</i>&lt;0.05). The expressions of GFAP, MAP-2 and MBP in the BMSCs+NBP group were significantly higher than those of the BMSCs group ( P</i>&lt;0.05). Compared with the EAE group, the p-NF-&#x3ba;B/NF-&#x3ba;B ratio and the IL-17/Foxp3 ratio in NBP group, BMSCs group and BMSCs+NBP group decreased, while P-I&#x3ba;B&#x3b1;/I&#x3ba;B&#x3b1;, p-pI3k/PI3K and P-Akt/Akt ratios increased, especially in the BMSCs+NBP group( P</i>&lt;0.05).</AbstractText>The combined treatment of NBP and BMSCs can help alleviate the symptoms of EAE model mice, showing better efficacy than treatment with NBP or BMSCs alone. The mechanism is related to the inhibition of the NF-&#x3ba;B pathway to regulate Th17/Foxp3 ratio and the activation of the PI3K/Akt pathway to promote the neurogenic differentiation of BMSCs.</AbstractText>Copyright&#xa9; by Editorial Board of Journal of Sichuan University (Medical Sciences).</CopyrightInformation>
2,330,885
Efficacy of Whole-Ventricular Radiotherapy in Patients Undergoing Maximal Tumor Resection for Glioblastomas Involving the Ventricle.
Patients with glioblastoma (GBM) involving the ventricles are at high risk of ventricle opening during surgery and potential ventricular tumor spread. We evaluated the effectiveness of whole-ventricular radiotherapy (WVRT) in reducing intraventricular seeding in patients with GBM and identified patients who could benefit from this approach.</AbstractText>We retrospectively reviewed the data of 382 patients with GBM who underwent surgical resection and temozolomide-based chemoradiotherapy. Propensity score matching was performed to compensate for imbalances in characteristics between patients who did [WVRT (+); n=59] and did not [WVRT (-); n=323] receive WVRT. Local, outfield, intraventricular, and leptomeningeal failure rates were compared.</AbstractText>All patients in the WVRT (+) group had tumor ventricular involvement and ventricle opening during surgery. In the matched cohort, the WVRT (+) group exhibited a significantly lower 2-year intraventricular failure rate than the WVRT (-) group (2.1% vs</i>. 11.8%; P=0.045), with no difference in other outcomes. Recursive partitioning analysis stratified the patients in the WVRT (-) group at higher intraventricular failure risk (2-year survival, 14.2%) due to tumor ventricular involvement, MGMT unmethylation, and ventricle opening. WVRT reduced the intraventricular failure rate only in high-risk patients (0% vs</i>. 14.2%; P=0.054) or those with MGMT-unmethylated GBM in the matched cohort (0% vs</i>. 17.3%; P=0.036).</AbstractText>WVRT reduced the intraventricular failure rate in patients with tumor ventricular involvement and ventricle opening during surgery. The MGMT-methylation status may further stratify patients who could benefit from WVRT. Further prospective evaluation of WVRT in GBM is warranted.</AbstractText>Copyright &#xa9; 2021 Kim, Yoo, Kim, Moon, Byun, Kang, Chang, Yoon and Suh.</CopyrightInformation>
2,330,886
Tuberculoma mimicking postoperative VP shunt seeding of craniopharyngioma: A rare case report.
Tuberculosis (TB) is still a big problem in developing and TB endemic countries such as Indonesia. The most common manifestations of TB in the central nervous system are tuberculous meningitis and tuberculoma. In developing and TB endemic countries, tuberculomas account for 33% of intracranial space-occupying lesions. Isolated tuberculoma without systemic TB is rarely seen. On physical and radiological examination, tuberculoma often gives an atypical appearance. From imaging, tuberculoma often mimics another intracranial tumor. Oftentimes the accurate diagnosis can only be made after postoperative histopathological and microbiology examination.</AbstractText>An 11-year-old, Indonesian girl has been complaining persistent headache in the past 3 years. The patient had a history of surgical excision of craniopharyngioma 8 years ago, and placement of ventriculoperitoneal shunt due to postoperative hydrocephalus. Patient was immunocompetent with no sign of systemic TB nor tuberculous meningitis. Brain magnetic resonance imaging (MRI) revealed a 4 &#xd7; 2.3 &#xd7; 2.1 cm mass surrounding the ventricular drain which was attached in the anterior horn of the right lateral ventricle to the right frontal cortex. From dynamic susceptibility contrast MRI perfusion and MR Spectroscopy suggested a process of seeding metastases surrounding the ventricular drain. Postoperative histopathological examination results were consistent with tuberculoma.</AbstractText>Tuberculoma should always be considered as one of the differential diagnoses along with primary and secondary intracranial neoplasm, particularly in developing and TB endemic countries, and inpatient with immunocompromised state.</AbstractText>Copyright: &#xa9; 2021 Surgical Neurology International.</CopyrightInformation>
2,330,887
TRIP6 functions in brain ciliogenesis.
TRIP6, a member of the ZYXIN-family of LIM domain proteins, is a focal adhesion component. Trip6 deletion in the mouse, reported here, reveals a function in the brain: ependymal and choroid plexus epithelial cells are carrying, unexpectedly, fewer and shorter cilia, are poorly differentiated, and the mice develop hydrocephalus. TRIP6 carries numerous protein interaction domains and its functions require homodimerization. Indeed, TRIP6 disruption in vitro (in a choroid plexus epithelial cell line), via RNAi or inhibition of its homodimerization, confirms its function in ciliogenesis. Using super-resolution microscopy, we demonstrate TRIP6 localization at the pericentriolar material and along the ciliary axoneme. The requirement for homodimerization which doubles its interaction sites, its punctate localization along the axoneme, and its co-localization with other cilia components suggest a scaffold/co-transporter function for TRIP6 in cilia. Thus, this work uncovers an essential role of a LIM-domain protein assembly factor in mammalian ciliogenesis.
2,330,888
Evolution of MRI Findings in Patients with Idiopathic Intracranial Hypertension after Venous Sinus Stenting.
The correlation between imaging findings and clinical status in patients with idiopathic intracranial hypertension is unclear. We aimed to examine the evolution of idiopathic intracranial hypertension-related MR imaging findings in patients treated with venous sinus stent placement.</AbstractText>Thirteen patients with idiopathic intracranial hypertension (median age, 26.9&#x2009;years) were assessed for changes in the CSF opening pressure, transstenotic pressure gradient, and symptoms after venous sinus stent placement. Optic nerve sheath diameter, posterior globe flattening and/or optic nerve protrusion, empty sella, the Meckel cave, tonsillar ectopia, the ventricles, the occipital emissary vein, and subcutaneous fat were evaluated on MR imaging before and 6&#x2009;months after venous sinus stent placement. Data are expressed as percentages, medians, or correlation coefficients (r</i>) with P</i> values.</AbstractText>Although all patients showed significant reductions of the CSF opening pressure (31 versus 21 cm H2</sub>O; P&#x2009;</i>=&#x2009;.005) and transstenotic pressure gradient (22.5&#x2009;versus 1.5&#x2009;mm Hg; P</i> = .002) and substantial improvement of clinical symptoms 6&#x2009;months after venous sinus stent placement, a concomitant reduction was observed only for posterior globe involvement (61.5% versus 15.4%; P&#x2009;</i>=&#x2009;.001), optic nerve sheath diameter (6.8&#x2009; versus 6.1&#x2009;mm; P&#x2009;</i>&lt;&#x2009;.001), and subcutaneous neck fat (8.9&#x2009; versus 7.4&#x2009;mm; P&#x2009;</i>=&#x2009;.001). Strong correlations were observed between decreasing optic nerve sheath diameters and improving nausea/emesis (right optic nerve sheath diameter, r</i>&#x2009;= &#x2009;0.592, P&#x2009;</i>=&#x2009;.033; left optic nerve sheath diameter, r</i>&#x2009;=&#x2009;0.718, P&#x2009;</i>=&#x2009;.006), improvement of posterior globe involvement and decreasing papilledema (r</i>&#x2009;=&#x2009;0.775, P&#x2009;</i>=&#x2009;.003), and decreasing occipital emissary vein diameter and decreasing headache frequency (r</i>&#x2009;=&#x2009;0.74, P&#x2009;</i>=&#x2009;.035). Decreasing transstenotic pressure gradient at 6&#x2009;months strongly correlated with decreasing empty sella (r</i>&#x2009;=&#x2009;0.625, P&#x2009;</i>=&#x2009;.022) and regressing cerebellar ectopia (r&#x2009;</i>=&#x2009;0.662, P&#x2009;</i>=&#x2009;.019).</AbstractText>Most imaging findings persist long after normalization of intracranial pressure and clinical improvement. However, MR imaging findings related to the optic nerve may reflect treatment success.</AbstractText>&#xa9; 2021 by American Journal of Neuroradiology.</CopyrightInformation>
2,330,889
Deletion of Sphingosine 1-Phosphate receptor 1 in cardiomyocytes during development leads to abnormal ventricular conduction and fibrosis.
Sphingosine 1-Phosphate receptor 1 (S1P<sub>1</sub> , encoded by S1pr1) is a G protein-coupled receptor that signals in multiple cell types including endothelial cells and cardiomyocytes. Cardiomyocyte-specific deletion of S1pr1 during mouse development leads to ventricular noncompaction, with 44% of mutant mice surviving to adulthood. Adult survivors of embryonic cardiomyocyte S1pr1 deletion showed cardiac hypertrabeculation consistent with ventricular noncompaction. Surprisingly, systolic function in mutant mice was preserved through at least 1&#xa0;year of age. Cardiac conduction was abnormal in cardiomyocyte S1pr1 mutant mice, with prolonged QRS intervals in mutants as compared with littermate control mice. Immunostaining of hearts from S1pr1 mutant embryos displayed a zone of intermediate Connexin 40 (Cx40) expression in the trabecular myocardium. However, we observed no significant differences in Cx40 and Connexin 43 immunostaining in hearts from adult survivors of embryonic cardiomyocyte S1pr1 deletion, which suggests normalized development of the ventricular conduction system in mutant mice. By contrast, the adult survivors of embryonic cardiomyocyte S1pr1 deletion showed increased cardiac fibrosis as compared with littermate controls. These results demonstrate that ventricular hypertrabeculation caused by embryonic deletion of cardiomyocyte S1pr1 correlates with cardiac fibrosis, which contributes to abnormal ventricular conduction. These results also reveal conduction abnormalities in the setting of hypertrabeculation with normal systolic function, which may be of clinical relevance in humans with ventricular hypertrabeculation.
2,330,890
Cognitive impairment caused by hypoxia: from clinical evidences to molecular mechanisms.
Hypoxia is a state of reduced oxygen supply and excessive oxygen consumption. According to the duration of hypoxic period, it can be classified as acute and chronic hypoxia. Both acute and chronic hypoxia could induce abundant neurological deficits. Although there have been significant advances in the pathophysiological injuries, few studies have focused on the cognitive dysfunction. In this review, we focused on the clinical evidences and molecular mechanisms of cognitive impairment under acute and chronic hypoxia. Hypoxia can impair several cognitive domains such as attention, learning and memory, procession speed and executive function, which are similar in acute and chronic hypoxia. The severity of cognitive deficit correlates with the duration and degree of hypoxia. Recovery can be achieved after acute hypoxia, while sequelae or even dementia can be observed after chronic hypoxia, perhaps due to the different molecular mechanisms. Cardiopulmonary compensatory response, glycolysis, oxidative stress, calcium overload, adenosine, mitochondrial disruption, inflammation and excitotoxicity contribute to the molecular mechanisms of cognitive deficit after acute hypoxia. During the chronic stage of hypoxia, different adaptive responses, impaired neurovascular coupling, apoptosis, transcription factors-mediated inflammation, as well as A&#x3b2; accumulation and tau phosphorylation account for the neurocognitive deficit. Moreover, brain structural changes with hippocampus and cortex atrophy, ventricle enlargement, senile plaque and neurofibrillary tangle deposition can be observed under chronic hypoxia rather than acute hypoxia.
2,330,891
Wernicke's encephalopathy in a rectal cancer patient with atypical radiological features: A case report.
Wernicke's encephalopathy is a disease caused by thiamine deficiency. The lesions usually involve the periphery of the aqueduct, midbrain, tectum, third ventricle, papillary body, and thalamus. It is very rare to affect the vermis and cerebellar hemispheres.</AbstractText>We report a 77-year-old female patient admitted to the emergency department of our hospital for 2 d of unconsciousness. Brain magnetic resonance imaging showed increased diffusion weighted imaging signals in the bilateral thalamus, periventricular regions of the third ventricle, corpora quadrigemina, vermis, and cerebellar hemispheres. Wernicke's encephalopathy was considered. She was given thiamine therapy and became conscious after the treatment.</AbstractText>Wernicke's encephalopathy may have various imaging manifestations. Clinicians should keep in mind that Wernicke's encephalopathy may occur in patients who experience prolonged periods of malnutrition.</AbstractText>&#xa9;The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.</CopyrightInformation>
2,330,892
Pulmonary hypertension associated with busulfan.
Busulfan is widely used to treat malignant diseases, particularly for therapeutic intensification prior to an autologous stem cell graft. Numerous side effects consecutive to busulfan are described, but few descriptions of pulmonary hypertension exist, while bronchiolitis obliterans remains a rare complication. We report the clinical observations of four patients from the French Pulmonary Hypertension Registry who experienced subacute pulmonary hypertension after receiving busulfan as preparation regimen before an autologous stem cell graft for malignancies (Hodgkin's disease, Ewing's sarcoma and primary large B cell lymphoma of the brain). Patients experienced severe pulmonary arterial hypertension 2 to 4.5 months after busulfan administration. Pulmonary hypertension improved after treatment with approved drugs for pulmonary arterial hypertension and/or corticosteroids. During the follow-up period, two patients developed chronic respiratory insufficiency due to interstitial lung disease, leading to double lung transplantation. The pathological assessment of explanted lungs revealed interstitial lung fibrosis with advanced bronchiolar lesions and severe pulmonary vascular damage. Three of the four patients were still alive after 36 to 80 months and the fourth died unexpectedly and suddenly after 5 months. In conclusion, PAH is a rare but severe complication associated with busulfan chemotherapy in adults. Histological examinations provide evidence for diffuse pulmonary vascular damage combined with interstitial lung injury in most cases.
2,330,893
Features of myocardial injury detected by cardiac magnetic resonance in a patient with desmin-related restrictive cardiomyopathy.
Myocardial fibrosis detected by cardiac magnetic resonance (CMR) has been reported in patients with desmin-related myopathy, although its characteristics remain unclear. Here, we describe a case of desmin-related restrictive cardiomyopathy wherein CMR imaging revealed myocardial oedema, ischaemia, and fibrosis in the left ventricle; the different types and processes of myocardial injury were detected by CMR. Middle wall left ventricular enhancement may be a feature of late gadolinium enhancement, and the lateral wall is often involved in cases of myocardial injury. CMR is useful for the early detection of cardiac involvement and the prediction of prognosis in patients diagnosed with desmin-related myopathy.
2,330,894
Risk factors for left ventricular dysfunction in adulthood: role of low birth weight.<Pagination><StartPage>5403</StartPage><EndPage>5414</EndPage><MedlinePgn>5403-5414</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1002/ehf2.13632</ELocationID><Abstract><AbstractText Label="AIMS">This study aimed to determine the relationship of low birth weight (LBW) with adult cardiac structure and function and investigate potential causal pathways.</AbstractText><AbstractText Label="METHODS AND RESULTS">A population-based sample of 925 Australians (41.3% male) were followed from childhood (aged 7-15&#xa0;years) to young adulthood (aged 26-36&#xa0;years) and mid-adulthood (aged 36-50&#xa0;years). Left ventricular (LV) global longitudinal strain (GLS, %), LV mass index (LVMi, g/m<sup>2.7</sup> ), LV filling pressure (E/e'), and left atrial volume index (g/m<sup>2</sup> ) were measured by transthoracic echocardiography in mid-adulthood. Birth weight category was self-reported in young adulthood and classified as low (&#x2264;5&#xa0;lb or &#x2264;2270&#xa0;g), normal (5-8&#xa0;lb or 2271-3630&#xa0;g), and high (&gt;8&#xa0;lb or &gt;3630&#xa0;g). Of the 925 participants, 7.5% (n&#xa0;=&#xa0;69) were classified as LBW. Compared with participants with normal birth weight, those with LBW had 2.01-fold (95% confidence interval: 1.19, 3.41, P&#xa0;=&#xa0;0.009) higher risks of impaired GLS (GLS&#xa0;&gt;&#xa0;-18%) and 2.63-fold (95% confidence interval: 0.89, 7.81, P&#xa0;=&#xa0;0.08) higher risks of LV hypertrophy (LVMi&#xa0;&gt;&#xa0;48&#xa0;g/m<sup>2.7</sup> in men or &gt;44&#xa0;g/m<sup>2.7</sup> in women) in adulthood, independent of age, sex, and any socio-economic factors. Participants with LBW significantly increased body fat from childhood to adulthood relative to their peers and had greater levels of triglycerides, fasting glucose, and arterial stiffness in adulthood. These risk factors were the strongest mediators and explained 54% of the LBW effect size on adult GLS and 33% of the LBW effect size on LVMi. The remaining of these associations was independent of any of the measured risk factors.</AbstractText><AbstractText Label="CONCLUSIONS">Low birth weight was associated with impaired cardiac structure and function in mid-adulthood. This association was only partially explained by known risk factors.</AbstractText><CopyrightInformation>&#xa9; 2021 The Authors. ESC Heart Failure published by John Wiley &amp; Sons Ltd on behalf of European Society of Cardiology.</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Huynh</LastName><ForeName>Quan L</ForeName><Initials>QL</Initials><Identifier Source="ORCID">0000-0003-1368-5160</Identifier><AffiliationInfo><Affiliation>Baker Heart and Diabetes Institute, 75 Commercial Road, Melbourne, Vic, 3004, Australia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Menzies Institute for Medical Research, University of Tasmania, Hobart, Australia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Venn</LastName><ForeName>Alison J</ForeName><Initials>AJ</Initials><AffiliationInfo><Affiliation>Menzies Institute for Medical Research, University of Tasmania, Hobart, Australia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Magnussen</LastName><ForeName>Costan G</ForeName><Initials>CG</Initials><AffiliationInfo><Affiliation>Menzies Institute for Medical Research, University of Tasmania, Hobart, Australia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Research Centre of Applied and Preventive Cardiovascular Medicine, University of Turku, Turku, Finland.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Centre for Population Health Research, University of Turku and Turku University Hospital, Turku, Finland.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Yang</LastName><ForeName>Hong</ForeName><Initials>H</Initials><AffiliationInfo><Affiliation>Menzies Institute for Medical Research, University of Tasmania, Hobart, Australia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Dwyer</LastName><ForeName>Terence</ForeName><Initials>T</Initials><AffiliationInfo><Affiliation>Menzies Institute for Medical Research, University of Tasmania, Hobart, Australia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Nuffield Department of Women's &amp; Reproductive Health, University of Oxford, Oxford, UK.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Marwick</LastName><ForeName>Thomas H</ForeName><Initials>TH</Initials><AffiliationInfo><Affiliation>Baker Heart and Diabetes Institute, 75 Commercial Road, Melbourne, Vic, 3004, Australia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Menzies Institute for Medical Research, University of Tasmania, Hobart, Australia.</Affiliation></AffiliationInfo></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType></PublicationTypeList><ArticleDate DateType="Electronic"><Year>2021</Year><Month>10</Month><Day>05</Day></ArticleDate></Article><MedlineJournalInfo><Country>England</Country><MedlineTA>ESC Heart Fail</MedlineTA><NlmUniqueID>101669191</NlmUniqueID><ISSNLinking>2055-5822</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D000293" MajorTopicYN="N">Adolescent</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D001315" MajorTopicYN="N" Type="Geographic">Australia</DescriptorName><QualifierName UI="Q000453" MajorTopicYN="N">epidemiology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D002648" MajorTopicYN="N">Child</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D005260" MajorTopicYN="N">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D007230" MajorTopicYN="N">Infant, Low Birth Weight</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D007231" MajorTopicYN="N">Infant, Newborn</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008875" MajorTopicYN="N">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D012307" MajorTopicYN="N">Risk Factors</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D018487" MajorTopicYN="Y">Ventricular Dysfunction, Left</DescriptorName><QualifierName UI="Q000453" MajorTopicYN="N">epidemiology</QualifierName><QualifierName UI="Q000209" MajorTopicYN="N">etiology</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D016277" MajorTopicYN="Y">Ventricular Function, Left</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D055815" MajorTopicYN="N">Young Adult</DescriptorName></MeshHeading></MeshHeadingList><KeywordList Owner="NOTNLM"><Keyword MajorTopicYN="N">Global longitudinal strain</Keyword><Keyword MajorTopicYN="N">LV hypertrophy</Keyword><Keyword MajorTopicYN="N">Left ventricle</Keyword><Keyword MajorTopicYN="N">Low birth weight</Keyword></KeywordList><CoiStatement>None declared.</CoiStatement></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="revised"><Year>2021</Year><Month>9</Month><Day>4</Day></PubMedPubDate><PubMedPubDate PubStatus="received"><Year>2021</Year><Month>5</Month><Day>21</Day></PubMedPubDate><PubMedPubDate PubStatus="accepted"><Year>2021</Year><Month>9</Month><Day>11</Day></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2021</Year><Month>10</Month><Day>7</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2022</Year><Month>3</Month><Day>22</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2021</Year><Month>10</Month><Day>6</Day><Hour>7</Hour><Minute>15</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">34612017</ArticleId><ArticleId IdType="pmc">PMC8712853</ArticleId><ArticleId IdType="doi">10.1002/ehf2.13632</ArticleId></ArticleIdList><ReferenceList><Reference><Citation>Barker DJ, Osmond C, Golding J, Kuh D, Wadsworth ME. 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We investigated the organ-sparing effect of the deep inspiration breath hold (DIBH) technique among different levels of lung expansion for left-side breast radiotherapy. This retrospective study enrolled 30 patients who received adjuvant left breast radiotherapy after breast-conserving surgery (BCS). Simulation scans of both DIBH and deep breathing four-dimensional computed tomography (4DCT) were acquired, and three treatment plans were generated for each patient. One plan was based on the DIBH images, and the other two plans were based on the mid-lung expansion (ME) and initial lung expansion (IE) phases retrieved from 4DCT data sets. Dosimetric comparisons and normal tissue complication probability (NTCP) models were conducted. We used image registration for displacement analysis and sought potential factors related to the dose benefit of DIBH. The DIBH plans resulted significantly lower doses to the heart, left ventricle (LV) and left anterior descending coronary artery (LAD), including the high- to low-dose areas, followed by the ME plans and IE plans (p &lt; 0.05). DIBH reduced the risk of long-term cardiac mortality by 40% and radiation pneumonitis of the left lung by 37.96% compared with the IE plans (p &lt; 0.001). The reduction in the mean dose to the heart and LV significantly correlated with anterior displacement of the left lung. The DIBH technique is a feasible tool to provide dosimetric and clinical advantages for adjuvant left-sided breast radiotherapy. Breathing pattern and the level of lung expansion seem to play an important role.
2,330,895
Diversity and function of motile ciliated cell types within ependymal lineages of the zebrafish brain.
Motile cilia defects impair cerebrospinal fluid (CSF) flow and can cause brain and spine disorders. The development of ciliated cells, their impact on CSF flow, and their function in brain and axial morphogenesis are not fully understood. We have characterized motile ciliated cells within the zebrafish brain ventricles. We show that the ventricles undergo restructuring through development, involving a transition from mono- to multiciliated cells (MCCs) driven by gmnc. MCCs co-exist with monociliated cells and generate directional flow patterns. These ciliated cells have different developmental origins and are genetically heterogenous with respect to expression of the Foxj1 family of ciliary master regulators. Finally, we show that cilia loss from the tela choroida and choroid plexus or global perturbation of multiciliation does not affect overall brain or spine morphogenesis but results in enlarged ventricles. Our findings establish that motile ciliated cells are generated by complementary and sequential transcriptional programs to support ventricular development.
2,330,896
Intra-operative neurophysiological mapping to identify distorted functional anatomy of the 4th ventricle in a 5-month-old infant.
Neurophysiological brainstem mapping techniques facilitate the intra-operative localisation of cranial nerve nuclei amidst distorted anatomy. Neurophysiological recording in young infants can be limited due to immature myelination and synaptogenesis, as well as an increased sensitivity to anaesthetic agents.</AbstractText>A 5-month-old boy was diagnosed with a cystic brainstem lesion located dorsally within the pons and upper medulla. An open surgical biopsy was undertaken via a posterior fossa craniotomy, revealing a grossly distorted fourth ventricular floor. Intra-operative neurophysiological mapping produced oculomotor, facial, glossopharyngeal and vagal muscle responses allowing a deviated functional midline to be identified. Direct stimulation was used to identify an area in the floor of the fourth ventricle eliciting no cranial nerve responses and allow safe entry into the tumour cavity and biopsy. Transcranial motor evoked responses (TcMEPs), short-latency somatosensory evoked potentials (SSEPs) and brainstem auditory evoked potentials (BAEPs) were all successfully recorded throughout the procedure, despite the use of halogenated gaseous anaesthesia.</AbstractText>We describe the use of brainstem mapping techniques for identification of a distorted midline on the floor of the 4th ventricle in an infant, with reproducible recordings of intra-operative TcMEPs, SSEPs and BAEPs.</AbstractText>&#xa9; 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.</CopyrightInformation>
2,330,897
Surgical Outcome of CSF Drainage in Paediatric Obstructive Hydrocephalus.
Hydrocephalus (HCP) is occurred when there is inequality in the formation and absorption of CSF to such a level which causes accumulation of fluid and causing raised intracranial pressure. Hydrocephalus is the most frequent neurosurgical problem encountered in the paediatric age group. CSF diversion surgically is needed as treatment for certain condition. Endoscopic third ventriculostomy (ETV) and Ventriculo-peritoneal shunt (VPS) are most widely used CSF diversion procedure. Therefore, this study was conducted for comparing the surgical outcome of ETV and VP shunt in obstructive hydrocephalus. This is a prospective experimental study conducted in the Department of Neurosurgery, Dhaka Medical College and Hospital (DMCH) and Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from January 2017 to December 2019. Paediatric patients of both sex and 6 months to 18 years of age with obstructive hydrocephalus undergone endoscopic third ventriculostomy (ETV) or ventriculo-peritoneal shunt (VP shunt) were included after fulfilling inclusion and exclusion criteria. The patients divided into two groups (Group A and Group B). The Group A included 30 patients who treated by Endoscopic third ventriculostomy and the Group B include 30 patients, who was treated by Ventriculo-peritoneal shunt. Surgical outcome was analyzed in two procedures separately. In Group A (ETV group) symptomatic improvement, GCS score improves earlier and post-operative complications relatively less than Group B (VP shunt group). But in VP shunt group 3 months follow up reveals- improvement of papilledema, Occipital frontal circumference (OFC) regression and fontanelle size improvement was more than that of ETV group. This study concluded with suggesting that Endoscopic third ventriculostomy (ETV) is technologically superior surgical technique than VP shunt. Though ETV showed relatively more immediate procedural failure and intraventricular hemorrhage but long-term outcome is better and also associated with lower incidence of infection and re-operation in comparison to VP shunt.
2,330,898
Developing a biomechanical model-based elasticity imaging method for assessing hormone receptor positive breast cancer treatment-related myocardial stiffness changes.
<b>Purpose:</b> Assessing cardiotoxicity as a result of breast cancer therapeutics is increasingly important as breast cancer diagnoses are trending younger and overall survival is increasing. With evidence showing that prevention of cardiotoxicity plays a significant role in increasing overall survival, there is an unmet need for accurate non-invasive methods to assess cardiac injury due to cancer therapies. Current clinical methods are too coarse and emerging research methods have not yet achieved clinical implementation. <b>Approach</b>: As a proof of concept, we examine myocardial elasticity imaging in the setting of premenopausal women diagnosed with hormone receptor positive (HR-positive) breast cancer undergoing severe estrogen depletion, as cardiovascular injury from early estrogen depletion is well-established. We evaluate the ability of our model-based cardiac elasticity imaging analysis method to indicate subclinical cancer therapy-related cardiac decline by examining differences in the change in cardiac elasticity over time in two cohorts of premenopausal women either undergoing severe estrogen depletion for HR-positive breast cancer or triple negative breast cancer patients as comparators. <b>Results</b>: Our method was capable of producing functional mechanical elasticity maps of the left ventricle (LV). Using these elasticity maps, we show significant differences in cardiac mechanical elasticity in the HR-positive breast cancer cohort compared to the comparator cohort. <b>Conclusions</b>: We present our methodology to assess the mechanical stiffness of the LV by interrogating cardiac magnetic resonance images within a computational biomechanical model. Our preliminary study suggests the potential of this method for examining cardiac tissue mechanical stiffness properties as an early indicator of cardiac decline.
2,330,899
Asymmetry and Heterogeneity: Part and Parcel in Cardiac Autonomic Innervation and Function.
The cardiac autonomic nervous system (cANS) regulates cardiac adaptation to different demands. The heart is an asymmetrical organ, and in the selection of adequate treatment of cardiac diseases it may be relevant to take into account that the cANS also has sidedness as well as regional differences in anatomical, functional, and molecular characteristics. The left and right ventricles respond differently to adrenergic stimulation. Isoforms of nitric oxide synthase, which plays an important role in parasympathetic function, are also distributed asymmetrically across the heart. Treatment of cardiac disease heavily relies on affecting left-sided heart targets which are thought to apply to the right ventricle as well. Functional studies of the right ventricle have often been neglected. In addition, many principles have only been investigated in animals and not in humans. Anatomical and functional heterogeneity of the cANS in human tissue or subjects is highly valuable for understanding left- and right-sided cardiac pathology and for identifying novel treatment targets and modalities. Within this perspective, we aim to provide an overview and synthesis of anatomical and functional heterogeneity of the cANS in tissue or subjects, focusing on the human heart.