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https://www.readbyqxmd.com/read/28892037/induction-and-micro-ct-imaging-of-cerebral-cavernous-malformations-in-mouse-model
#1
Jaesung P Choi, Xi Yang, Matthew Foley, Xian Wang, Xiangjian Zheng
Mutations in the CCM1 (aka KRIT1), CCM2, or CCM3 (aka PDCD10) gene cause cerebral cavernous malformation (CCM) in humans. Mouse models of CCM disease have been established by tamoxifen induced deletion of Ccm genes in postnatal animals. These mouse models provide invaluable tools to investigate molecular mechanism and therapeutic approaches for CCM disease. An accurate and quantitative method to assess lesion burden and progression is essential to harness the full value of these animal models. Here, we demonstrate the induction of CCM disease in a mouse model and the use of the contrast enhanced X-ray micro computed tomography (micro-CT) method to measure CCM lesion burden in mouse brains...
September 4, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28870584/relevance-of-ccm-gene-polymorphisms-for-clinical-management-of-sporadic-cerebral-cavernous-malformations
#2
Carmela Rinaldi, Placido Bramanti, Concetta Scimone, Luigi Donato, Concetta Alafaci, Rosalia D'Angelo, Antonina Sidoti
Cerebral cavernous malformations (CCMs) are clusters of capillaries in the brain that may cause focal deficits or seizures in affected patients. They occur in both sporadic and inherited autosomal dominant form. Germline mutations in CCM1, CCM2 and CCM3 were identified in familial cases. Over the past 13years we performed sequencing and MLPA of the CCM genes in all sporadic and familial CCM cases coming from some hospital clinics of Neurology and Neurosurgery of Messina and other Italian cities. Our results showed that CCM sporadic patients, negative for previously reported CCM gene causative mutations, always carried known CCM polymorphisms...
September 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28866064/surgical-outcomes-of-posterolateral-sulcus-approach-for-spinal-intramedullary-tumors-tumor-resection-and-functional-preservation
#3
Toshihiro Takami, Kentaro Naito, Toru Yamagata, Shinichi Kawahara, Kenji Ohata
BACKGROUND: Selection of the access myelotomy is a key issue in surgery for spinal intramedullary tumors. This study focused on surgical outcomes with the posterolateral sulcus (PLS) approach, equivalent to dorsal root entry zone myelotomy. METHODS: This retrospective study of the 10-year period from 2007 to 2016 included 90 cases of spinal intramedullary lesions (99 operations). A PLS approach was indicated for intramedullary lesions situated laterally in the spinal cord showing no contact with the spinal cord surface...
August 30, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28851747/vascular-endothelial-ve-cadherin-endothelial-adherens-junctions-and-vascular-disease
#4
Maria Grazia Lampugnani, Elisabetta Dejana, Costanza Giampietro
Endothelial cell-cell adherens junctions (AJs) supervise fundamental vascular functions, such as the control of permeability and transmigration of circulating leukocytes, and the maintenance of existing vessels and formation of new ones. These processes are often dysregulated in pathologies. However, the evidence that links dysfunction of endothelial AJs to human pathologies is mostly correlative. In this review, we present an update of the molecular organization of AJ complexes in endothelial cells (ECs) that is mainly based on observations from experimental models...
August 29, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28844916/long-term-outcomes-of-surgical-treatment-in181-patients-with-supratentorial-cerebral-cavernous-malformation-associated-epilepsy
#5
Kangmin He, Shize Jiang, Zehan Wu, Liang Chen, Ying Mao
OBJECTIVE: To evaluate the efficacy of surgery as a treatment for supratentorial cerebral cavernous malformation-associated epilepsy (SCCMAE) and determine the factors that influence outcomes. METHODS: During the 5year period from2005 to 2009, this study included 181 consecutive patients who were diagnosed with SCCMAE and surgically treated in a single institute. Each patient was followed up for at least 5 years post-operatively. A time-to-event analysis was performed using Kaplan-Meier curves and Cox regression models to evaluate the associated risk factors...
August 24, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28841124/the-epitrigeminal-approach-to-the-brainstem
#6
Georgios Andrea Zenonos, David Fernandes-Cabral, Maximiliano Nunez, Stefan Lieber, Juan Carlos Fernandez-Miranda, Robert Max Friedlander
OBJECTIVE Surgical approaches to the ventrolateral pons pose a significant challenge. In this report, the authors describe a safe entry zone to the brainstem located just above the trigeminal entry zone which they refer to as the "epitrigeminal entry zone." METHODS The approach is presented in the context of an illustrative case of a cavernous malformation and is compared with the other commonly described approaches to the ventrolateral pons. The anatomical nuances were analyzed in detail with the aid of surgical images and video, anatomical dissections, and high-definition fiber tractography (HDFT)...
August 25, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28819935/plasma-biomarkers-of-inflammation-reflect-seizures-and-hemorrhagic-activity-of-cerebral-cavernous-malformations
#7
Romuald Girard, Hussein A Zeineddine, Maged D Fam, Anoop Mayampurath, Ying Cao, Changbin Shi, Robert Shenkar, Sean P Polster, Michael Jesselson, Ryan Duggan, Abdul-Ghani Mikati, Gregory Christoforidis, Jorge Andrade, Kevin J Whitehead, Dean Y Li, Issam A Awad
The clinical course of cerebral cavernous malformations (CCMs) is highly variable. Based on recent discoveries implicating angiogenic and inflammatory mechanisms, we hypothesized that serum biomarkers might reflect chronic or acute disease activity. This single-site prospective observational cohort study included 85 CCM patients, in whom 24 a priori chosen plasma biomarkers were quantified and analyzed in relation to established clinical and imaging parameters of disease categorization and severity. We subsequently validated the positive correlations in longitudinal follow-up of 49 subjects...
August 17, 2017: Translational Stroke Research
https://www.readbyqxmd.com/read/28811547/up-regulation-of-nadph-oxidase-mediated-redox-signaling-contributes-to-the-loss-of-barrier-function-in-krit1-deficient-endothelium
#8
Luca Goitre, Peter V DiStefano, Andrea Moglia, Nicholas Nobiletti, Eva Baldini, Lorenza Trabalzini, Julie Keubel, Eliana Trapani, Vladimir V Shuvaev, Vladimir R Muzykantov, Ingrid H Sarelius, Saverio Francesco Retta, Angela J Glading
The intracellular scaffold KRIT1/CCM1 is an established regulator of vascular barrier function. Loss of KRIT1 leads to decreased microvessel barrier function and to the development of the vascular disorder Cerebral Cavernous Malformation (CCM). However, how loss of KRIT1 causes the subsequent deficit in barrier function remains undefined. Previous studies have shown that loss of KRIT1 increases the production of reactive oxygen species (ROS) and exacerbates vascular permeability triggered by several inflammatory stimuli, but not TNF-α...
August 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28791783/quantitative-susceptibility-mapping-as-a-monitoring-biomarker-in-cerebral-cavernous-malformations-with-recent-hemorrhage
#9
Hussein A Zeineddine, Romuald Girard, Ying Cao, Nicholas Hobson, Maged D Fam, Agnieszka Stadnik, Huan Tan, Jingjing Shen, Kiranj Chaudagar, Robert Shenkar, Richard E Thompson, Nichol McBee, Daniel Hanley, Timothy Carroll, Gregory A Christoforidis, Issam A Awad
BACKGROUND: Quantitative Susceptibility Mapping (QSM) MRI allows accurate assessment of iron content in cerebral cavernous malformations (CCM), and a threshold increase by 6% in QSM has been shown to reflect new symptomatic hemorrhage (SH) in previously stable lesions. PURPOSE/HYPOTHESIS: It is unclear how lesional QSM evolves in CCMs after recent SH, and whether this could serve as a monitoring biomarker in clinical trials aimed at preventing rebleeding in these lesions...
August 9, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28776215/-cavernomas-of-the-central-nervous-system-observational-study-of-111-patients
#10
C Mayer, U M Mauer, G Bluhm, R Mathieu, C Hackenbroch, S Mayer
PURPOSE: The prevalence of cavernomas (cavernous hemangioma) is 0.1-0.7%. Cavernomas are often found as an incidental finding within the framework of magnetic resonance imaging (MRI) examinations in mainly young and healthy patients. In the literature, the reported risk of hemorrhage varies greatly and is sometimes higher than that of aneurysms, which is surprising given that cavernomas are part of the low blood pressure system. After the diagnosis the medical practitioner and the patient have to decide on the further therapy, either surgical removal or the strategy of watchful waiting (conservative treatment)...
August 3, 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/28768818/sofia-intermediate-catheter-and-the-snake-technique-safety-and-efficacy-of-the-sofia-catheter-without-guidewire-or-microcatheter-construct
#11
Jeremy J Heit, Johnny Hy Wong, Adrienne M Mofaff, Nicholas A Telischak, Robert L Dodd, Michael P Marks, Huy M Do
BACKGROUND: Neurointerventional surgeries (NIS) benefit from supportive endovascular constructs. Sofia is a soft-tipped, flexible, braided single lumen intermediate catheter designed for NIS. Sofia advancement from the cervical to the intracranial circulation without a luminal guidewire or microcatheter construct has not been described. OBJECTIVE: To evaluate the efficacy and safety of the new Sofia Non-wire Advancement techniKE (SNAKE) for advancement of the Sofia into the cerebral circulation...
August 2, 2017: Journal of Neurointerventional Surgery
https://www.readbyqxmd.com/read/28745674/-gene-mutations-in-patients-with-hereditary-cavernous-malformations
#12
O B Belousova, E S Bulygina, D N Okishev, E B Prohorchuk, S V Tsygankova, I N Pronin, L V Shishkina, M V Ryzhova, K G Skryabin, A N Konovalov
AIM: To identify mutations in cerebral cavernous malformation (CCM) genes in patients with hereditary and sporadic CCMs in the Russian population. MATERIAL AND METHODS: Blood samples from 73 randomly selected patients, including 29 MRI-confirmed familial cases, 8 clinically confirmed familial cases and 38 so-called sporadic cases, were examined. A search for large deletions/duplications was performed using multiplex ligation-dependent probe amplification (MPLA)...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28744689/pediatric-cerebellar-giant-cavernous-malformation-case-report-and-review-of-literature
#13
Javier Villaseñor-Ledezma, Marcelo Budke, Juan-Antonio Alvarez-Salgado, María-Angeles Cañizares, Luis Moreno, Francisco Villarejo
BACKGROUND AND IMPORTANCE: Giant cavernous malformations (GCM) are low flow, angiographically occult vascular lesions, with a diameter >4 cm. Cerebellar GCMs are extremely rare, with only seven cases reported based on English literature. These lesions are most commonly seen in the pediatric age group, which is known to have an increased risk of hemorrhage, being surgery clearly recommended. CLINICAL PRESENTATION: An 18-month-old girl presented with a 6-month history of cervical torticollis and upper extremities clumsiness...
July 25, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28735437/prospective-and-retrospective-study-of-videoconference-telemedicine-follow-up-after-elective-neurosurgery-results-of-a-pilot-program
#14
Melissa Reider-Demer, Pushpa Raja, Neil Martin, Mariel Schwinger, Diana Babayan
Existing literature suggests that use of telemedicine during postoperative appointments can increase access to care and is valued by patients and providers alike. While research examining the clinical equivalency of telemedicine visits for postoperative care has been growing, few studies have reported on telemedicine follow-up after neurosurgery. This study examined if a videoconferencing visit could substitute for an in-person clinic visit for elective neurosurgical cases in the USA. This was a single-center prospective study of patients who underwent elective neurosurgical procedures (aneurysm clipping, resection of cavernous angiomas, resection of arterial venous malformation, microvascular decompression for trigeminal neuralgia and hemifacial spasm, and certain benign brain tumors) and were offered telemedicine follow-up care by an allied health professional during the first 90 days after neurosurgery...
July 22, 2017: Neurosurgical Review
https://www.readbyqxmd.com/read/28731396/contralateral-posterior-interhemispheric-approach-to-deep-medial-parietooccipital-vascular-malformations-surgical-technique-and-results
#15
Jan-Karl Burkhardt, Ethan A Winkler, Michael T Lawton
OBJECTIVE Deep medial parietooccipital arteriovenous malformations (AVMs) and cerebral cavernous malformations (CCMs) are traditionally resected through an ipsilateral posterior interhemispheric approach (IPIA), which creates a deep, perpendicular perspective with limited access to the lateral margins of the lesion. The contralateral posterior interhemispheric approach (CPIA) flips the positioning, with the midline positioned horizontally for retraction due to gravity, but with the AVM on the upper side and the approach from the contralateral, lower side...
July 21, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28728536/postnatal-delayed-exacerbation-of-dural-sinus-malformation-associated-with-brainstem-cavernous-malformations-a-case-report
#16
Katsuhiro Mizutani, Tomoru Miwa, Takenori Akiyama, Tokunori Kanazawa, Hideaki Nagashima, Kei Miyakoshi, Yasunari Niimi, Kazunari Yoshida
Dural sinus malformation (DSM) is a rare paediatric vascular malformation characterised by abnormal dilation of the posterior dural sinus. Owing to its rarity, the pathophysiology of DSM has not been fully elucidated. We report a case of prenatally diagnosed DSM with an unusual clinical course. We detected DSM in a male foetus in the 26th week of gestation by using foetal ultrasonography. Although the DSM regressed during the foetal stage and the arteriovenous shunt was insignificant in the neonate, the shunt rapidly developed four months after birth...
January 1, 2017: Interventional Neuroradiology
https://www.readbyqxmd.com/read/28721350/corrigendum-cerebral-cavernous-malformations-review-of-the-genetic-and-protein-protein-interactions-resulting-in-disease-pathogenesis
#17
Jacob F Baranoski, M Yashar S Kalani, Colin J Przybylowski, Joseph M Zabramski
[This corrects the article on p. 60 in vol. 3, PMID: 27896269.].
2017: Frontiers in Surgery
https://www.readbyqxmd.com/read/28695311/aberrant-paramagnetic-signals-outside-the-tumor-volume-on-routine-surveillance-mri-of-brain-tumor-patients
#18
Shlomit Yust-Katz, Edna Inbar, Natalia Michaeli, Dror Limon, Tali Siegal
Late complications of cerebral radiation therapy (RT) involve vascular injury with acquired cavernous malformation, telangiectasias and damage to vascular walls which are well recognized in children. Its incidence in adults is unknown. Blood products and iron deposition that accompany vascular injury create paramagnetic effects on MRI. This study retrospectively investigated the frequency of paramagnetic lesions on routine surveillance MRI of adult brain tumor patients. MRI studies of 115 brain tumor patients were reviewed...
July 10, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28686112/emergency-resection-of-brainstem-cavernous-malformations
#19
Abdulfettah Tumturk, Yiping Li, Yahya Turan, Ulas Cikla, Bermans J Iskandar, Mustafa K Baskaya
Brainstem cavernous malformations (CMs) pose significant challenges to neurosurgeons because of their deep locations and high surgical risks. Most patients with brainstem CMs present with sudden-onset cranial nerve deficits or ataxia, but uncommonly patients can present in extremis from an acute hemorrhage, requiring surgical intervention. However, the timing of surgery for brainstem CMs has been a controversial topic. Although many authors propose delaying surgery into the subacute phase, some patients may not tolerate waiting until surgery...
July 7, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28680728/intradural-extramedullary-cavernous-malformation-with-extensive-superficial-siderosis-of-the-neuraxis-case-report-and-review-of-literature
#20
Pedram Golnari, Sameer A Ansari, Ali Shaibani, Michael C Hurley, Matthew B Potts, Missia E Kohler, Patrick A Sugrue, Babak S Jahromi
BACKGROUND: Spinal cavernous malformations usually affect the vertebral bodies and are seldom intradural. Here, we report a rare spinal intradural-extramedullary cavernous malformation associated with extensive superficial siderosis along the neuraxis in a patient with radicular complaints. CASE DESCRIPTION: A 60-year-old male presented with subacute headaches, intermittent fever, and acute back and radicular leg pain for 1-2 weeks. Magnetic resonance imaging revealed an intradural-extramedullary lesion just below the conus medullaris (at the L2 level)...
2017: Surgical Neurology International
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