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Cerebral vasculopathy

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https://www.readbyqxmd.com/read/28335992/varicella-zoster-virus-vasculopathy-the-expanding-clinical-spectrum-and-pathogenesis
#1
REVIEW
Maria A Nagel, Dallas Jones, Ann Wyborny
Varicella zoster virus (VZV) is a ubiquitous, human alphaherpesvirus that produces varicella on primary infection then becomes latent in ganglionic neurons along the entire neuraxis. In elderly and immunocompromised individuals, VZV reactivates and travels along nerve fibers peripherally resulting in zoster. However, VZV can also spread centrally and infect cerebral and extracranial arteries (VZV vasculopathy) to produce transient ischemic attacks, stroke, aneurysm, sinus thrombosis and giant cell arteritis, as well as granulomatous aortitis...
March 18, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28325644/dnase-active-trex1-frame-shift-mutants-induce-serologic-autoimmunity-in-mice
#2
Tomomi Sakai, Takuya Miyazaki, Dong-Mi Shin, Yong-Soo Kim, Chen-Feng Qi, Robert Fariss, Jeeva Munasinghe, Hongsheng Wang, Alexander L Kovalchuk, Parul H Kothari, Charles S Fermaintt, John P Atkinson, Fred W Perrino, Nan Yan, Herbert C Morse
TREX1/DNASE III, the most abundant 3'-5' DNA exonuclease in mammalian cells, is tail-anchored on the endoplasmic reticulum (ER). Mutations at the N-terminus affecting TREX1 DNase activity are associated with autoimmune and inflammatory conditions such as Aicardi-Goutières syndrome (AGS). Mutations in the C-terminus of TREX1 cause loss of localization to the ER and dysregulation of oligosacchryltransferase (OST) activity, and are associated with retinal vasculopathy with cerebral leukodystrophy (RVCL) and in some cases with systemic lupus erythematosus (SLE)...
March 18, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28314885/adverse-effects-of-levamisole-in-cocaine-users-a-review-and-risk-assessment
#3
REVIEW
Tibor Markus Brunt, Jorrit van den Berg, Ed Pennings, Bastiaan Venhuis
The immunomodulatory adjuvant and antihelminth levamisole is increasingly used as an adulterant in cocaine worldwide. An accumulating body of clinical and toxicological literature has appeared since 2010 describing neutropenia, agranulocytosis, leukoencephalopathy and vasculitis in cases associated with levamisole-adulterated cocaine. Mostly, neutropenia and agranulocytosis were reported, characterized by a decimation of neutrophils. A large proportion of cases also involved vasculopathy, characterized by pronounced black and purple skin purpura with cutaneous necrosis...
March 17, 2017: Archives of Toxicology
https://www.readbyqxmd.com/read/28293402/treosulfan-based-conditioning-regimen-in-sibling-and-alternative-donor-hematopoietic-stem-cell-transplantation-for-children-with-sickle-cell-disease
#4
Antonio Marzollo, Elisabetta Calore, Manuela Tumino, Marta Pillon, Maria Vittoria Gazzola, Roberta Destro, Raffaella Colombatti, Piero Marson, Tiziana Tison, Anna Colpo, Chiara Mainardi, Maria Gabelli, Maria Paola Boaro, Sara Rossin, Aurora Strano, Nadia Quaglia, Federica Menzato, Giuseppe Basso, Laura Sainati, Chiara Messina
BACKGROUND AND OBJECTIVES: Lack of suitable donors and regimen related toxicity are major barriers for hematopoietic stem cell transplantation (HSCT) in patients with sickle cell disease (SCD). The aim of the study is the assessment of efficacy and toxicity of Treosulfan-based conditioning regimen for SCD also when alternative donors such as mismatched unrelated donor and haploidentical donor are employed. METHODS: We report our single-center experience: 11 patients with SCD received HSCT with a Treosulfan/Thiotepa/Fludarabine/Anti-thymoglobulin conditioning regimen between 2010 and 2015...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28289923/tocilizumab-reverses-cerebral-vasculopathy-in-a-patient-with-homozygous-samhd1-mutation
#5
REVIEW
Michael Henrickson, Heng Wang
An auto-inflammatory syndrome consequent to SAMHD1 mutations involves cerebral vasculopathy characterized by multifocal stenosis and aneurysms within large arteries, moyamoya, chronic ischemia, and early-onset strokes (SAMS). While this condition involves the innate immune system, additional clinical features mimic systemic lupus erythematosus. Mutations in this gene can also cause a subset of the rare genetic condition Aicardi-Goutières syndrome. To date, no established therapy successfully prevents disease progression...
March 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28284448/growth-of-giant-intracranial-aneurysms-an-aneurysmal-wall-disorder
#6
F-X Ferracci, V Gilard, H Cebula, N Magne, J-P Lejeune, O Langlois, F Proust
The enlargement of giant intracranial aneurysms (IA) can be observed in 30 % of cases resulting in a neurological deficit and epilepsy due to its mass effect. This growth process could be due to a morphological disorder of the IA wall. The authors report on 2 cases of giant IA growth responsible for intracranial hypertension. The treatment of these giant IA required a microsurgical excision combined with a series of cerebral revascularization procedures. The role of vasa vasorum on the inflammatory granuloma outside the vessel, which induced the enlargement, is discussed...
March 8, 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28281108/recognizing-cadasil-a-secondary-cause-of-migraine-with-aura
#7
REVIEW
John Glenn Burkett, Carrie Dougherty
PURPOSE OF REVIEW: CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an important disease to consider in the differential diagnosis of migraine with aura. This review examines current literature regarding migraine in CADASIL, as well as diagnostic and treatment modalities. RECENT FINDINGS: Recent studies have shown that smoking is a modifiable risk factor for progression of CADASIL (Chabriat et al. in Stroke 47:4-11, 2015)...
April 2017: Current Pain and Headache Reports
https://www.readbyqxmd.com/read/28276529/the-contrast-enhancement-of-intracranial-arterial-wall-on-high-resolution-mri-and-its-clinical-relevance-in-patients-with-moyamoya-vasculopathy
#8
Maoxue Wang, Yongbo Yang, Fei Zhou, Ming Li, Renyuan Liu, Maobin Guan, Rui Li, Le He, Yun Xu, Bing Zhang, Bin Zhu, Xihai Zhao
The purpose of this study is to investigate the characteristics of intracranial vessel wall enhancement and its relationship with ischemic infarction in patients with Moyamoya vasculopathy (MMV). Forty-seven patients with MMV confirmed by angiography were enrolled in this study. The vessel wall enhancement of the distal internal carotid artery, anterior cerebral artery and middle cerebral artery was classified into eccentric and concentric patterns, as well as divided into three grades: grade 0, grade 1 and grade 2...
March 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28273147/a-quantitative-brain-map-of-experimental-cerebral-malaria-pathology
#9
Patrick Strangward, Michael J Haley, Tovah N Shaw, Jean-Marc Schwartz, Rachel Greig, Aleksandr Mironov, J Brian de Souza, Sheena M Cruickshank, Alister G Craig, Danny A Milner, Stuart M Allan, Kevin N Couper
The murine model of experimental cerebral malaria (ECM) has been utilised extensively in recent years to study the pathogenesis of human cerebral malaria (HCM). However, it has been proposed that the aetiologies of ECM and HCM are distinct, and, consequently, no useful mechanistic insights into the pathogenesis of HCM can be obtained from studying the ECM model. Therefore, in order to determine the similarities and differences in the pathology of ECM and HCM, we have performed the first spatial and quantitative histopathological assessment of the ECM syndrome...
March 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28270343/comprehensive-neuropsychological-evaluation-of-children-and-adolescents-with-sickle-cell-anemia-a-hospital-based-sample
#10
Samantha Nunes, Nayara Argollo, Marivania Mota, Camilo Vieira, Eduardo Pondé de Sena
BACKGROUND: Individuals with sickle cell anemia may suffer symptomatic or silent cerebral infarcts leading to neurocognitive complications. This study investigated the cognitive and intellectual performance of children and adolescents with sickle cell anemia. METHODS: The socioeconomic status, clinical aspects and behavioral profile of 15 young individuals with sickle cell anemia were evaluated. The Wechsler Intelligence Scale for Children, the Developmental Neuropsychological Assessment Test, and the Child Behavior Checklist were applied...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28254515/hereditary-cerebral-small-vessel-disease-and-stroke
#11
REVIEW
Christian Baastrup Søndergaard, Jørgen Erik Nielsen, Christine Krarup Hansen, Hanne Christensen
Cerebral small vessel disease is considered hereditary in about 5% of patients and is characterized by lacunar infarcts and white matter hyperintensities on MRI. Several monogenic hereditary diseases causing cerebral small vessel disease and stroke have been identified. The purpose of this systematic review is to provide a guide for determining when to consider molecular genetic testing in patients presenting with small vessel disease and stroke. CADASIL, CARASIL, collagen type IV mutations (including PADMAL), retinal vasculopathy with cerebral leukodystrophy, Fabry disease, hereditary cerebral hemorrhage with amyloidosis, and forkhead box C1 mutations are described in terms of genetics, pathology, clinical manifestation, imaging, and diagnosis...
April 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28248918/stroke-associated-with-varicella-zoster-vasculopathy-a-clinicoradiological-profile-of-3-patients
#12
Pradeep K Maurya, Dinkar Kulshreshtha, Ajai K Singh, Anup K Thacker
INTRODUCTION: Varicella zoster (VZ) vasculopathy is a rare but well recognized cause of stroke. In the absence of zoster rash and infection in remote past, the disease can pose diagnostic challenge. We report 3 cases of anterior circulation stroke occurring in close temporal relation to VZ. Their clinical, radiologic, and angiographic features are discussed. CASE REPORT: Of the 3 patients, 2 had stroke within a span of 4 to 6 weeks of herpes zoster ophthalmicus while the third patient had zoster of cervical dermatome...
March 2017: Neurologist
https://www.readbyqxmd.com/read/28220365/binding-of-11-c-pittsburgh-compound-b-correlated-with-white-matter-injury-in-hypertensive-small-vessel-disease
#13
Tetsuya Hashimoto, Chiaki Yokota, Kazuhiro Koshino, Takashi Temma, Makoto Yamazaki, Satoshi Iguchi, Ryo Shimomura, Toshiyuki Uehara, Naoko Funatsu, Tenyu Hino, Kazuo Minematsu, Hidehiro Iida, Kazunori Toyoda
OBJECTIVE: (11)C-Pittsburgh compound-B ((11)C-PIB) positron emission tomography (PET) is used to visualize and quantify amyloid deposition in the brain cortex in pathological conditions such as Alzheimer's disease (AD). Intense (11)C-PIB retention is also observed in the white matter (WM) of both healthy individuals and AD patients. However, the clinical implications of this retention in brain WM have not been clarified. We investigated the relationship between the extent of white matter lesions (WMLs) and the binding potential of (11)C-PIB (BPND) in the WM in patients with hypertensive small vessel disease...
April 2017: Annals of Nuclear Medicine
https://www.readbyqxmd.com/read/28179050/cerebral-amyloid-angiopathy-in-stroke-medicine
#14
Frank Block, Manuel Dafotakis
BACKGROUND: Cerebral amyloid angiopathy (CAA) is a degenerative vasculopathy that is classically associated with lobar intracerebral or sulcal hemorrhage. Its prevalence is estimated at 30% in the seventh decade and 50% in the eighth and ninth decades. In this review, we summarize the risks linked to CAA with respect to the treatment and prevention of stroke. METHODS: This review is based on pertinent publications retrieved by a selective search employing the terms "amyloid cerebral angiopathy," "stroke," "intra - cerebral bleeding," and "acute stroke therapy...
January 20, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/28159432/large-vessel-vasculopathy-in-children-with-sickle-cell-disease-a-magnetic-resonance-imaging-study-of-infarct-topography-and-focal-atrophy
#15
Kristin P Guilliams, Melanie E Fields, Dustin K Ragan, Yasheng Chen, Cihat Eldeniz, Monica L Hulbert, Michael M Binkley, James N Rhodes, Joshua S Shimony, Robert C McKinstry, Katie D Vo, Hongyu An, Jin-Moo Lee, Andria L Ford
BACKGROUND: Large-vessel vasculopathy (LVV) increases stroke risk in pediatric sickle cell disease beyond the baseline elevated stroke risk in this vulnerable population. The mechanisms underlying this added risk and its unique impact on the developing brain are not established. METHODS: We analyzed magnetic resonance imaging and angiography scans of 66 children with sickle cell disease and infarcts by infarct density heatmaps and Jacobian determinants, a metric utilized to delineate focal volume change, to investigate if infarct location, volume, frequency, and cerebral atrophy differed among hemispheres with and without LVV...
December 7, 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/28149032/unseen-face-of-varicella-zoster-infection-in-adults
#16
Gunchan Paul, Birinder S Paul, Gagandeep Singh
Varicella infection is common in children caused by varicella-zoster virus (VZV). VZV is known to cause cerebral arterial vasculopathy and antibody-mediated hypercoagulable state leading to thrombotic complications in children. Such complications in adults are very rare. We report three cases that represent the unseen face of primary varicella infection in adults. Simultaneous involvement of cortical venous sinus thrombosis and deep vein thrombosis leading to clot in right atrium and pulmonary embolism in first case; cortical venous sinus thrombosis in second case; and deep vein thrombosis in third case...
December 2016: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28120049/update-on-mechanism-and-therapeutic-implications-of-spinal-cord-stimulation-and-cerebral-hemodynamics-a-narrative-review
#17
Elisabetta Peppucci, Rina Di Bonaventura, Vincenzo Esposito, Jun Zhong, Gerardo Iacopino, Giuseppe Barbagallo, Massimiliano Visocchi
Spinal cord stimulation (SCS) is well known for its early role in the management of chronic pain, mainly failed back surgery syndrome (FBSS), spasticity, and bowel and bladder dysfunction. In more recent years, SCS has been proposed for patients suffering from refractory angina or peripheral vasculopathies in order to gain symptom relief, thus indicating some hemodynamic effect on the peripheral circulation. Taking into account this scientific observation, since the late1980s, researchers have started to investigate the potential effect of SCS on cerebral blood flow (CBF) regulation and its possible application in certain pathological settings dealing with vascular pattern dysfunction, such as ischemia, subarachnoid hemorrhage, head trauma, and brain tumors...
2017: Acta Neurochirurgica. Supplement
https://www.readbyqxmd.com/read/28095086/retinal-findings-and-a-novel-tinf2-mutation-in-revesz-syndrome-clinical-and-molecular-correlations-with-pediatric-retinal-vasculopathies
#18
Mrinali P Gupta, Katherine E Talcott, David Y Kim, Suneet Agarwal, Shizuo Mukai
BACKGROUND: Revesz syndrome is a telomere disorder in the dyskeratosis congenita (DKC) spectrum characterized by exudative retinopathy, bone marrow failure, neuroradiographic abnormalities, and integumentary findings. MATERIALS/METHODS: We report the ophthalmologic findings, documented by examinations under anesthesia with clinical photography and fluorescein angiography, as well as the systemic manifestations and genetic and molecular testing, in identical twins with Revesz syndrome, and compare and contrast these features to those of other pediatric retinal vasculopathies...
January 2017: Ophthalmic Genetics
https://www.readbyqxmd.com/read/28087351/central-nervous-system-vasculitis-in-adults-an-update
#19
REVIEW
Lívia Almeida Dutra, Alexandre Wagner Silva de Souza, Gabriela Grinberg-Dias, Orlando Graziani Povoas Barsottini, Simone Appenzeller
Primary central nervous system vasculitis (PCNSV) is a challenging diagnosis due to broad clinical manifestations and variable specificity and sensitivity of laboratory and imaging diagnostic tools. Differential diagnosis includes reversible cerebral vasoconstriction syndrome (RCVS), secondary vasculitis of the CNS and other noninflammatory vasculopathies. Brain biopsy is essential for definitive diagnosis and to exclude mimickers. Recent data show that data large-vessel PCNSV present worse prognosis when compared to small-vessel PCNSV...
February 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28056747/micrornas-based-predictor-factor-in-patients-with-migraine-ischemic-stroke
#20
Luca Gallelli, Antonio Siniscalchi, Marco Carotenuto, Maria Cristina Caroleo, Erika Cione, Vincenzo Guidetti
Among the clinical spectrum of neurological diseases, migraine is often associated with cerebro-vasculopathy. Impairment of neuroimmune mediators in the central nervous system has been recognized in the pathophysiology of migraine-related stroke. Although genetic correlation was found in patients with migraine-related stroke, the epidemiology of this disease indicate a need in biomarker searching discovery and validation. In this view, small molecule, called microRNAs (miRNAs), able to regulate immune and neuronal processes has been reported in patients with migraine and ischemic stroke and unambiguous miRNAs related to these diseases could be established as new molecular indicator of precocity for clinical and/or pharmacological intervention...
January 4, 2017: MicroRNA
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