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Cerebral vasculopathy

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https://www.readbyqxmd.com/read/27814292/hemorheological-alterations-in-sickle-cell-anemia-and-their-clinical-consequences-the-role-of-genetic-modulators
#1
Marisa Silva, Sofia Vargas, Andreia Coelho, Alexandra Dias, Teresa Ferreira, Anabela Morais, Raquel Maia, Paula Kjöllerström, João Lavinha, Paula Faustino
Sickle cell anemia (SCA) is an autosomal recessive disease caused by the HBB:c.20A>T mutation that leads to hemoglobin S synthesis. The disease presents with high clinical heterogeneity characterized by chronic hemolysis, recurrent episodes of vaso-oclusion and infection. This work aimed to characterize by in silico studies some genetic modulators of severe hemolysis and stroke risk in children with SCA, and understand their consequences at the hemorheological level.Association studies were performed between hemolysis biomarkers as well as the degree of cerebral vasculopathy and the inheritance of several polymorphic regions in genes related with vascular cell adhesion and vascular tonus in pediatric SCA patients...
November 4, 2016: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/27805576/protocol-for-isolating-the-mouse-circle-of-willis
#2
Justine Claire Hur, Régis Blaise, Isabelle Limon
The cerebral arterial circle (circulus arteriosus cerebri) or circle of Willis (CoW) is a circulatory anastomosis surrounding the optic chiasma and hypothalamus that supplies blood to the brain and surrounding structures. It has been implicated in several cerebrovascular disorders, including cerebral amyloid angiopathy (CAA)-associated vasculopathies, intracranial atherosclerosis and intracranial aneurysms. Studies of the molecular mechanisms underlying these diseases for the identification of novel drug targets for their prevention require animal models...
October 22, 2016: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/27773153/sporadic-case-of-retinal-vasculopathy-with-cerebral-leukodystrophy-hereditary-endotheliopathy-retinopathy-nephropathy-stroke-with-novel-trex1-mutation
#3
Bonnie Scurry, Thomas Wellings, Himanshu Goel, Anja Pluschke
No abstract text is available yet for this article.
February 2016: Pathology
https://www.readbyqxmd.com/read/27723828/role-of-transcranial-doppler-in-the-evaluation-of-vasculopathy-in-tuberculous-meningitis
#4
Mei-Ling Sharon Tai, Vijay K Sharma
BACKGROUND: Vascular complications are important causes of cerebral infarction in tuberculous meningitis (TBM).Transcranial Doppler ultrasonography (TCD) is a non-invasive tool that can provide real-time information about cerebral hemodynamics. However, the literature on the role of TCD in the diagnosis or monitoring of vasculopathy associated with TBM is scarce. We explored the role of TCD in the diagnosis and monitoring of TBM-related vasculopathy of the major intracranial arteries...
2016: PloS One
https://www.readbyqxmd.com/read/27693106/thrombotic-thrombocytopenic-purpura-with-concomitant-progressive-cerebral-microbleeds
#5
Nariman Noorbakhsh-Sabet, Ramin Zand
Thrombotic thrombocytopenic purpura (TTP) is a disease caused by excessive platelet thrombi and multisystem vasculopathy. We report a young patient with refractory TTP who has developed multiple multifocal cerebral microbleeds on his brain magnetic resonance imaging scans. TTP-associated thrombotic microangiopathy resulting in diffuse microthrombi formation in the microcirculation and hypoperfusion might be an underlying etiology.
September 28, 2016: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/27688895/fatal-cerebral-haemorrhage-in-a-hypertensive-seven-year-old-boy
#6
Herwig Brandtner, Fabio C Monticelli, Harald J Meyer, Ariane Biebl, Herbert Budka
A seven-year-old boy died from autopsy-proven brain haemorrhage due to hypertensive vasculopathy. This emphasizes the importance of early diagnosis and therapy of hypertension in children. Brain haemorrhage is a potentially fatal complication of paediatric hypertension.
September 2016: JRSM Open
https://www.readbyqxmd.com/read/27625380/strictly-lobar-cerebral-microbleeds-are-associated-with-cognitive-impairment
#7
Chih-Ping Chung, Kun-Hsien Chou, Wei-Ta Chen, Li-Kuo Liu, Wei-Ju Lee, Liang-Kung Chen, Ching-Po Lin, Pei-Ning Wang
BACKGROUND AND PURPOSE: Different distributions of cerebral microbleeds (CMBs) are associated with distinct pathological mechanisms. Lobar CMBs are thought to be related to cerebral amyloid angiopathy, whereas deep or infratentorial CMBs are related to hypertensive vasculopathy. The present study aimed to evaluate the effects of CMBs and their locations on a variety of cognitive domains. METHODS: Study subjects were selected from the community-based I-Lan Longitudinal Aging Study...
October 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27619954/expanding-treatment-for-existing-neurological-disease-extend-an-open-label-phase-ii-clinical-trial-of-hydroxyurea-treatment-in-sickle-cell-anemia
#8
Angela E Rankine-Mullings, Courtney R Little, Marvin E Reid, Deanne P Soares, Carolyn Taylor-Bryan, Jennifer M Knight-Madden, Susan E Stuber, Asha V Badaloo, Karen Aldred, Margaret E Wisdom-Phipps, Teresa Latham, Russell E Ware
BACKGROUND: Cerebral vasculopathy in sickle cell anemia (SCA) begins in childhood and features intracranial arterial stenosis with high risk of ischemic stroke. Stroke risk can be reduced by transcranial doppler (TCD) screening and chronic transfusion therapy; however, this approach is impractical in many developing countries. Accumulating evidence supports the use of hydroxyurea for the prevention and treatment of cerebrovascular disease in children with SCA. Recently we reported that hydroxyurea significantly reduced the conversion from conditional TCD velocities to abnormal velocities; whether hydroxyurea can be used for children with newly diagnosed severe cerebrovascular disease in place of starting transfusion therapy remains unknown...
September 12, 2016: JMIR Research Protocols
https://www.readbyqxmd.com/read/27604306/retinal-vasculopathy-with-cerebral-leukoencephalopathy-and-systemic-manifestations
#9
Anine H Stam, Parul H Kothari, Aisha Shaikh, Andreas Gschwendter, Joanna C Jen, Suzanne Hodgkinson, Todd A Hardy, Michael Hayes, Peter A Kempster, Katya E Kotschet, Ingeborg M Bajema, Sjoerd G van Duinen, Marion L C Maat-Schieman, Paulus T V M de Jong, Marc D de Smet, Didi de Wolff-Rouendaal, Greet Dijkman, Nadine Pelzer, Grant R Kolar, Robert E Schmidt, JoAnne Lacey, Daniel Joseph, David R Fintak, M Gilbert Grand, Elizabeth M Brunt, Helen Liapis, Rula A Hajj-Ali, Mark C Kruit, Mark A van Buchem, Martin Dichgans, Rune R Frants, Arn M J M van den Maagdenberg, Joost Haan, Robert W Baloh, John P Atkinson, Gisela M Terwindt, Michel D Ferrari
Cerebroretinal vasculopathy, hereditary vascular retinopathy, and hereditary endotheliopathy, retinopathy, nephropathy and stroke are neurovascular syndromes initially described as distinct entities. Recently they were shown to be one disease caused by C-terminal frame-shift mutations in TREX1, which was termed 'retinal vasculopathy with cerebral leukodystrophy'. Here we defined the genetic and clinicopathologic spectrum of this clinically and pathophysiologically poorly characterized and frequently misdiagnosed fatal neurovascular disorder...
September 6, 2016: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/27585640/clinical-interpretation-of-high-resolution-vessel-wall-mri-of-intracranial-arterial-diseases
#10
Vance T Lehman, Waleed Brinjikji, David F Kallmes, John Huston, Giuseppe Lanzino, Alejandro A Rabinstein, Ashima Makol, Mahmud Mossa-Bosha
Intracranial arterial pathology has traditionally been evaluated with luminal imaging. Recently, high-resolution vessel wall imaging (HR-VWI) with MRI has facilitated submillimetre evaluation of the arterial walls. This technique can help differentiate various causes of intracranial steno-occlusive disease, identify culprit atherosclerotic plaques with a recent cerebral infarct, locate vessel wall pathology in areas with minimal or no narrowing on luminal imaging, predict aneurysm stability and identify a ruptured aneurysm when multiple aneurysms are present...
November 2016: British Journal of Radiology
https://www.readbyqxmd.com/read/27540084/presence-of-cerebral-microbleeds-is-associated-with-worse-executive-function-in-pediatric-brain-tumor-survivors
#11
Erika Roddy, Katherine Sear, Erin Felton, Benita Tamrazi, Karen Gauvain, Joseph Torkildson, Benedict Del Buono, David Samuel, Daphne A Haas-Kogan, Josephine Chen, Robert E Goldsby, Anuradha Banerjee, Janine M Lupo, Annette M Molinaro, Heather J Fullerton, Sabine Mueller
BACKGROUND: A specific form of small-vessel vasculopathy-cerebral microbleeds (CMBs)-has been linked to various types of dementia in adults. We assessed the incidence of CMBs and their association with neurocognitive function in pediatric brain tumor survivors. METHODS: In a multi-institutional cohort of 149 pediatric brain tumor patients who received cranial radiation therapy (CRT) between 1987 and 2014 at age <21 years and 16 patients who did not receive CRT, we determined the presence of CMBs on brain MRIs...
November 2016: Neuro-oncology
https://www.readbyqxmd.com/read/27507861/nonatheroscleotic-isolated-middle-cerebral-artery-disease-may-be-early-manifestation-of-moyamoya-disease
#12
Yeon-Jung Kim, Joo Kyung Lee, Sung-Ho Ahn, Bum Joon Kim, Dong-Wha Kang, Jong S Kim, Sun U Kwon
BACKGROUND AND PURPOSE: Middle cerebral artery steno-occlusive disease (MCAD) is not an uncommon cause of ischemic stroke in young Asians. Aside from atherosclerosis, the pathogenesis of MCAD include various nonatherosclerotic vasculopathies, most of which are yet to be defined. This study investigated the pathogenesis of symptomatic isolated MCAD in young Asian patients using high-resolution magnetic resonance imaging (HR-MRI) and mutation analysis of RNF213. METHODS: Patients aged <60 years with stroke or transient ischemic attack caused by MCAD were prospectively enrolled...
September 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27504091/a-young-woman-with-ischemic-stroke-should-we-pay-more-attention-to-varicella-zoster-infection
#13
Cláudia Borbinha, João Pedro Marto, Sofia Calado, Miguel Viana-Baptista
Ischemic and hemorrhagic stroke are recognized complications of Varicella zoster virus (VZV) infections, although uncommon and poorly documented. The authors report the case of a 31-year-old woman admitted with acute ischemic stroke of the right posterior cerebral artery and a history of a thoracic rash 1 month before. Aspirin and simvastatin were prescribed, but the patient suffered a stepwise deterioration the following days, with new areas of infarction on brain imaging. Despite no evidence of cardiac or large vessel embolic sources, anticoagulation was started empirically 6 days after stroke onset...
May 2016: Case Reports in Neurology
https://www.readbyqxmd.com/read/27478660/two-cases-of-legionella-pneumophila-pneumonia-with-prolonged-neurologic-symptoms-and-brain-hypoperfusion-on-single-photon-emission-computed-tomography
#14
Hiromitsu Ohta, Susumu Yamazaki, You Miura, Akira Seto, Minoru Kanazawa, Makoto Nagata
Cerebral and cerebellar symptoms are frequently associated with Legionnaires' disease. However, corresponding brain lesions are difficult to demonstrate using either computed tomography (CT) or magnetic resonance imaging (MRI). We report here two patients with Legionella pneumophila pneumonia accompanied by prolonged neurologic symptoms. In contrast to brain CT and MRI, which failed to detect any abnormalities, single-photon emission computed tomography (SPECT) showed multiple sites of hypoperfusion within the brains of both patients...
2016: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/27375007/homozygosity-for-moyamoya-disease-risk-allele-leads-to-moyamoya-disease-with-extracranial-systemic-and-pulmonary-vasculopathy
#15
Hiroyuki Fukushima, Toshiki Takenouchi, Kenjiro Kosaki
Moyamoya disease is characterized by diffuse distal intracranial stenosis. Recently, RNF213 has been identified as a susceptibility gene in the development of this condition. Pulmonary hypertension is a rare progressive vasculopathy with an unknown etiology. The co-occurrence of pulmonary hypertension and Moyamoya disease has been described in four patients; however, whether this co-occurrence represents a chance association or a common vascular pathology has remained unknown. Here, we report two unrelated male patients who presented during their childhood with dyspnea on exertion...
September 2016: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/27357378/neuropsychological-effects-of-cerebral-amyloid-angiopathy
#16
REVIEW
Matthew Schrag, Howard Kirshner
Cerebral amyloid angiopathy is a condition of the cerebral arterioles and to a lesser extent capillaries and veins, wherein beta-amyloid is deposited. In arterioles, this preferentially targets vascular smooth muscle cells and in the later stages undermines the stability of the vessel. This condition is frequently comorbid with Alzheimer's disease and its role in cognitive impairment and dementia is a topic of considerable recent research. This article reviews recent literature which confirms that CAA independently contributes to cognitive impairment by potentiating the neurodegeneration of Alzheimer's disease, by predisposing to microhemorrhagic and microischemic injury to the brain parenchyma, and by interfering with the autoregulation of CNS blood flow...
August 2016: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/27216120/endovascular-treatment-using-woven-stents-for-ruptured-vertebral-artery-dissecting-aneurysm-induced-by-varicella-zoster-virus-case-report
#17
Kwangho Lee, Hyun Park, Insung Park, Jongwoo Han
Subarachnoid hemorrhage with ruptured cerebral artery dissection caused by varicella zoster (VZ) vasculopathy is rare. We report a rare case of vertebral artery (VA) dissection induced by VZ virus, successfully treated with stent-assisted coil embolization and systemic anti viral medication, on six month follow-up cerebral angiography with the review of literatures.
May 24, 2016: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/27163552/acute-thyrotoxicosis-of-graves-disease-associated-with-moyamoya-vasculopathy-and-stroke-in-latin-american-women-a-case-series-and-review-of-the-literature
#18
Nirav H Shah, Priyank Khandelwal, Gillian Gordon-Perue, Ashish H Shah, Eric Barbarite, Gustavo Ortiz, Alejandro M Forteza
OBJECTIVE: Moyamoya disease is a cerebral vasculopathy characterized by stenosis of the terminal internal carotid artery, proximal middle cerebral artery, and anterior cerebral artery. There is an association between moyamoya vasculopathy and Graves disease, primarily in Asian populations. Here, we present the largest series of non-Asian, predominantly Latino patients with moyamoya vasculopathy in the setting of Graves thyrotoxicosis, as well as the largest review of the literature to date...
August 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27106860/reduction-in-overt-and-silent-stroke-recurrence-rate-following-cerebral-revascularization-surgery-in-children-with-sickle-cell-disease-and-severe-cerebral-vasculopathy
#19
Erin M Hall, Jeffrey Leonard, Jodi L Smith, Kristin P Guilliams, Michael Binkley, Robert J Fallon, Monica L Hulbert
BACKGROUND: Children with sickle cell disease (SCD) and moyamoya may benefit from indirect cerebral revascularization surgery in addition to chronic blood transfusion therapy for infarct prevention. We sought to compare overt and silent infarct recurrence rates in children with SCD undergoing revascularization. METHODS: This was a retrospective cohort study of all children with SCD and moyamoya treated at two children's hospitals. Clinical events and imaging studies were reviewed...
August 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27082149/the-contemporary-management-of-intracranial-atherosclerotic-disease
#20
Xinyi Leng, Ka Sing Wong, Thomas W Leung
Intracranial atherosclerotic disease is the most common cause of cerebral vasculopathy and an important stroke etiology worldwide, with a higher prevalence in Asian, Hispanic and African ethnicities. Symptomatic intracranial atherosclerotic disease portends a recurrent stroke risk as high as 18% at one year. The key to secondary prevention is an understanding of the underlying stroke mechanism and aggressive control of conventional cardiovascular risks. Contemporary treatment includes antiplatelet therapy, optimal glycemic and blood pressure control, statin therapy and lifestyle modifications...
June 2016: Expert Review of Neurotherapeutics
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